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Sample records for adult malignant fibrous

  1. Malignant transformation of orbital solitary fibrous tumor.

    PubMed

    Wang, Xiangning; Qian, Jiang; Bi, Yingwen; Ping, Bo; Zhang, Rui

    2013-06-01

    Orbital solitary fibrous tumor (SFT) is a rare tumor and may recur or undergo malignant transformation without complete excision. We present a case of orbital SFT which recurred twice and underwent malignant transformation. The patient was treated with en bloc excision via a lateral orbitotomy. The postoperative histopathologic diagnosis of this case was an adult fibrosarcoma. Postoperative adjuvant radiation therapy was given. In 18 months of further follow-up, there has been no evidence of recurrence, both clinically and in regular imaging studies.

  2. Malignant Solitary Fibrous Tumor of the Scalp.

    PubMed

    Shirley, Brett M; Kang, David R; Sakamoto, Aya Hamao

    2016-07-01

    Solitary fibrous tumors are an uncommon slow growing benign neoplasm originally described as a pleural neoplasm but can also be found in the lung, mediastinum, peritoneum, or any other sites including the head and neck. Malignant solitary fibrous tumors (MSFT) are extremely rare and only few cases have been published in the literature. There have been 19 cases reported of MSFT in the head and neck, but there are no reports of MSFT located within the scalp in the English language literature. We present a case of MSFT arising in the scalp and describe our experience with the clinical presentation, surgical management, and outcome in this pathological condition. PMID:27408445

  3. Malignant solitary fibrous tumor of the esophagus.

    PubMed

    Lococo, Filippo; Cesario, Alfredo; Mulè, Antonino; Margaritora, Stefano

    2011-04-01

    Solitary fibrous tumor (SFT) of the esophagus has been very rarely reported in literature. Herein, we report a case of a successful surgically treated malignant solitary fibrous tumor of the esophagus. A 36-year-old woman was admitted at our hospital with an erroneous ultrasound-based diagnosis of cervico-mediastinal goiter. Surprisingly, the preoperative diagnostic work-up, including a computed tomographic chest scan, endoscopy, and endoscopic ultrasonography, revealed a pedunculated intraluminal mass in the esophagus. The tumor was radically removed through left antero-lateral cervicotomy. Pathologic and immunohistochemical examination was concluded for a malignant SFT, a rare variant not previously described in relation to the esophagus. The patient underwent adjuvant radiotherapy and is alive with no signs of tumor recurrence 32 months after surgery.

  4. Myxoid Malignant Fibrous Histiocytoma with Multiple Primary Sites

    PubMed Central

    Muler, Jeffrey H.; Paulino, Augusto F.; Roulston, Diane

    2002-01-01

    Malignant fibrous histiocytoma (MFH) is one of the most common types of soft tissue sarcomas in adults. The most common location of MFH are the extremities and the trunk, with the most common site for distant metastases being the lung. We describe a case with multiple synchronous sites of myxoid MFH but no lung metastases and presence of abnormalities of 19p13. PMID:18521346

  5. Retroperitoneal malignant fibrous histiocytoma can mimic a hydatid cyst.

    PubMed

    Erbay, Gurcan; Ulusan, Serife; Koc, Zafer; Canpolat, Emine Tuba; Calıskan, Kenan

    2011-01-01

    Malignant fibrous histiocytoma is the second most common soft-tissue sarcoma in adults. After the extremities, the retroperitoneal space is the second most common site of this tumor. A 50-year-old man presented with a right retroperitoneal, thick-walled, cystic multilocular mass measuring 10 × 10 cm that was thought to be a type CE 5 hydatid cyst preoperatively. However, the postoperative histopathology did not agree with the radiological findings and instead showed a malignant fibrous histiocytoma. The computed tomography and ultrasound/Doppler ultrasound findings of this retroperitoneal mass mimicked a type CE 5 hydatid cyst. We present this case because the surgical management of these two lesions differs and misdiagnosis can be problematic.

  6. Malignant fibrous histiocytomas of salivary glands.

    PubMed

    Benjamin, E; Wells, S; Fox, H; Reeve, N L; Knox, F

    1982-09-01

    The light microscopic, immunohistological and ultrastructural findings in two cases of malignant fibrous histiocytoma arising in salivary glands are presented and the features of seven previously reported cases are reviewed. This neoplasm is extremely rare in this site and may pose problems in diagnosis. It has to be distinguished from other spindled cell tumours, in particular from epithelial tumours of predominantly spindled cell pattern; immunohistological markers for histiocytic cells may be of value. The histogenesis of this neoplasm is controversial but our electron microscopic findings support an origin from mesenchymal cells which differentiate along a broad fibrohistiocytic spectrum.

  7. Malignant Solitary Fibrous Tumor of the Pancreas.

    PubMed

    Estrella, Jeannelyn S; Wang, Huamin; Bhosale, Priya R; Evans, Harry L; Abraham, Susan C

    2015-08-01

    Solitary fibrous tumor (SFT) arising in the pancreas is exceedingly rare, with only 11 cases reported in the English literature. All cases described thus far have exhibited benign histology. We report the first case of malignant SFT of the pancreas. The patient was a 52-year-old woman who presented with obstructive jaundice and a 15-cm pancreatic head mass. The mass showed areas with typical histologic features for SFT including small fibroblastlike cells arranged in the well-characterized "patternless pattern" of architecture, hemangiopericytomalike vessels, areas with dense collagen and infrequent mitoses (0-2 per 10 high-power fields [HPFs]). In addition, multiple areas with an overtly sarcomatous morphology were present, containing large spindle and epithelioid cells with nuclear pleomorphism, marked cellularity, up to17 mitoses per 10 HPFs, and necrosis. Immunohistochemical stains were positive for CD34 and B-cell CLL/lymphoma 2 (Bcl-2) in both benign and malignant components and showed strong, diffuse p53 and p16 staining in the malignant component. At last follow-up (40 months), the patient was alive and well without evidence of disease. However, given that the presence of a malignant component in extrapancreatic SFT has been associated with recurrence/metastasis and death, complete surgical resection and close long-term follow-up is required.

  8. Malignant Solitary Fibrous Tumor of the Pancreas.

    PubMed

    Estrella, Jeannelyn S; Wang, Huamin; Bhosale, Priya R; Evans, Harry L; Abraham, Susan C

    2015-08-01

    Solitary fibrous tumor (SFT) arising in the pancreas is exceedingly rare, with only 11 cases reported in the English literature. All cases described thus far have exhibited benign histology. We report the first case of malignant SFT of the pancreas. The patient was a 52-year-old woman who presented with obstructive jaundice and a 15-cm pancreatic head mass. The mass showed areas with typical histologic features for SFT including small fibroblastlike cells arranged in the well-characterized "patternless pattern" of architecture, hemangiopericytomalike vessels, areas with dense collagen and infrequent mitoses (0-2 per 10 high-power fields [HPFs]). In addition, multiple areas with an overtly sarcomatous morphology were present, containing large spindle and epithelioid cells with nuclear pleomorphism, marked cellularity, up to17 mitoses per 10 HPFs, and necrosis. Immunohistochemical stains were positive for CD34 and B-cell CLL/lymphoma 2 (Bcl-2) in both benign and malignant components and showed strong, diffuse p53 and p16 staining in the malignant component. At last follow-up (40 months), the patient was alive and well without evidence of disease. However, given that the presence of a malignant component in extrapancreatic SFT has been associated with recurrence/metastasis and death, complete surgical resection and close long-term follow-up is required. PMID:26166470

  9. Malignant solitary fibrous tumor of the meninges.

    PubMed

    Ogawa, Kumiko; Tada, Toyohiro; Takahashi, Satoru; Sugiyama, Naotake; Inaguma, Shingo; Takahashi, Seishiro S; Shirai, Tomoyuki

    2004-05-01

    Increasing numbers of solitary fibrous tumors (SFTs) in the meninges have been reported since this entity was first recognized. While most cases previously reported were considered to be benign, the malignant potential of extrathoracic SFTs has not been excluded. The authors report a rare case of a meningeal SFT with malignant behavior occurring in a Japanese female patient, initially resected when she was 44 years old and recurring in the same place four times during a 26-year follow-up period. A metastatic tumor to the right lung arose 25 years after the resection of the first meningeal tumor and focal invasion into the cerebellum was also observed with her last (5th) meningeal tumor. Immunohistochemical analysis showed all tumors to be diffusely positive for CD34 and negative for EMA, with a so-called "patternless" histological pattern, featuring thin collagen fibers between tumor cells. A focal "staghorn" vascular pattern was also observed. Ki67 (MIB-1) labeling indices and mitosis rates were 3.1+/-1.2% and less than 1/10 high power fields (HPF) in the first meningeal tumor and 16.1+/-6.4% and 6/10HPF in the last (5th) one, respectively. Thus, the present case suggests that meningeal SFTs possess malignant potential so that careful long-term follow up is required.

  10. An intracranial malignant solitary fibrous tumor.

    PubMed

    Choi, Chan-Young; Han, Seong-Rok; Yee, Gi-Taek; Joo, Mee

    2011-04-01

    Intracranial malignant solitary fibrous tumor (SFT) is very rare. It was identified in a 39-year-old female patient who underwent malignant transformation over 6 months. MRI revealed an 8 × 5 × 6 cm mass with heterogenous strong enhancement in the left occipital lobe. Histologic findings and immunophenotype (positive for CD34, bcl-2 and vimentin, and negative for epithelial membrane antigen or S100 protein) of the primary tumor were typical of SFT. However, there was a focal area (<10% of tumor volume) showing hypercellularity, nuclear pleomorphism and increased Ki-67 labeling index (LI) of 10% (average, 1%). At the second operation, the recurrent tumor revealed full-blown histologic features of malignant SFT, such as infiltrative brain invasion, marked nuclear pleomorphism, frequent mitotic figures (15/10 high power fields), and high Ki-67 LI (25%). The presence of atypical histologic finding or increased Ki-67 LI in the typical SFT, although it is focal, needs to be mentioned in the diagnosis and also may require more aggressive surgical management.

  11. Inflammatory malignant fibrous histiocytoma of the retroperitoneum.

    PubMed

    Cvjetko, Ivan; Kocman, Branko; Sitić, Sanda

    2011-03-01

    The authors present a case of inflammatory malignant fibrous histiocytoma located in the left retroperitoneum. The tumor was resected enblock with kidney and suprarenal gland. During the resection the system of retractors called the pillars of Kocman was used which allowed wide exposure of the abdominal cavity. The tumor measured 23 x 17 x 10 cm with the left kidney and suprarenal incorporated. The tumor was centrally pseudocystic made of xanthomatous cells, foamy cells and rare giant cells with storiform formations and infiltrated with neutrophils. Imunohistochemically, the tumor cells were vimentin and CD 68 positive and CD 20, CD3, EMA, S-100, HMB 45, CD 34 and CD 1a negative. Neutrophils were CD 15 positive.

  12. A large retroperitoneal malignant solitary fibrous tumor.

    PubMed

    Yoh, Tomoaki; Sata, Ritsuko; Kobayashi, Atsushi; Wada, Seidai; Nakamura, Yuya; Kato, Tatsushi; Nakayama, Hiroyuki; Okamura, Ryuji

    2014-01-01

    We report on a large, retroperitoneal, malignant, solitary fibrous tumor (SFT) with high proliferation activity. A 43-year-old man was admitted to our department complaining of a palpable mass. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a large retroperitoneal tumor occupying the entire abdominal cavity. A laparotomy was performed for diagnosis and treatment, which revealed a tumor in the retroperitoneum but with no invasion to the surrounding organs, thereby allowing safe macroscopic excision. Histologically, the tumor was composed of spindle-shaped cells with patternless pattern and a hemangiopericytomatous appearance. Moreover, immunohistochemical staining was positive for CD34, vimentin, Bcl-2, and CD99 and negative for desmin, S-100p, and smooth muscle actin (AMA). The tumor exhibited high cellularity, moderate mitotic activity, pleomorphism, necrosis, and hemorrhagic changes. In addition, the Ki-67 labeling index was 37%. These findings confirmed the diagnosis of malignant SFT with high proliferation activity. Subsequently, adjuvant doxorubicin plus ifosfamide chemotherapy was performed. No signs of recurrence were observed 12 months after the surgery.

  13. [Malignant fibrous histiocytoma of the penis].

    PubMed

    Crisman, G; Margiotta, G; Calabresi, M; Discepoli, S; Leocata, P

    2015-07-01

    According to the most recent World Health Organization (WHO) guidelines a diagnosis of malignant fibrous histiocytoma (MFH) should be made only for lesions composed of tumor cells without evidence of a specific line of differentiation. This is therefore a diagnosis by exclusion which is why the name of undifferentiated high-grade pleomorphic sarcoma (UPS) should be preferred. Soft tissue sarcomas currently have an incidence in all body regions of approximately 20 cases per 1 million inhabitants per year. Soft tissue tumors of the penis represent approximately 5 % of all penile tumors and the incidence of penile sarcomas is estimated to be approximately 0.6-1 case per 100,000 patients. Only seven cases have so far been reported in the literature. This article describes the case of a 61-year-old Caucasian male who presented with a painless mass sited in the upper part of the corpus cavernosa. An incisional biopsy with a subsequent investigation using an extensive immunohistochemical panel were performed and a high-grade undifferentiated pleomorphic sarcoma or pleomorphic storiform MFH was diagnosed. In addition to the case report a literature review is presented to elaborate the discussion on the differential diagnoses of these kinds of lesions.

  14. General Information about Osteosarcoma and Malignant Fibrous Histiocytoma of Bone

    MedlinePlus

    ... Histiocytoma of Bone Treatment (PDQ®)–Patient Version General Information About Osteosarcoma and Malignant Fibrous Histiocytoma of Bone ... the PDQ Pediatric Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  15. Retroperitoneal malignant fibrous histiocytoma without histological atypias.

    PubMed

    Laky, D; Penciu, M; Rădulescu, D

    1993-01-01

    A case of a 36-year-old woman suspected of hepatic hydatid cyst with dyseptic symptoms, hepatomegalia, profound pains in the right hypochondrium is presented. During the operation a 15/12/17 cm well-incapsulated tumour is extirpated. The tumour stretched from the upper right renal pole behind the right hepatic lobe as far as the diaphragm and while sectioning it was grey and orange. Histopathologically we found a fibrous histiocytoma with zones of storiform fascicular pattern alternating with areas of histocytic cells with vacuolar-foamy cytoplasm, rich in lipids and without atypias or mitoses. We report the frequency, histological aspects of the fibrous histiocytomas as well as some particularities of their retroperitoneal location.

  16. Suboccipital malignant solitary fibrous tumor: report of a case.

    PubMed

    Zuniga, M Geraldine; Orr, Brent A; Chang, Steven S; Tufano, Ralph P

    2012-02-01

    Solitary fibrous tumors (SFTs) are rare soft-tissue sarcomas usually arising in the pleura of the thoracic cavity. Thirty percent of SFTs present in extrapleural locations, and infrequently in the head and neck region. While the majority of SFTs are benign, aggressive variants have been described. We report a case of a patient with a malignant SFT arising in the suboccipital soft tissues of the neck. We elucidate the diagnostic and therapeutic challenges in managing this tumor in this unusual location.

  17. Malignant fibrous histiocytoma of the lateral conjunctiva and anterior orbit.

    PubMed

    Boehlke, Christopher S; Frueh, Bartley R; Flint, Andrew; Elner, Victor M

    2007-01-01

    A 25-year-old woman presented for evaluation of a rapidly enlarging mass of the left bulbar conjunctiva. Debulking biopsy was performed, which led to the diagnosis of myxoid malignant fibrous histiocytoma. The patient underwent left orbital exenteration with clear margins. Histopathology showed involvement of the conjunctiva substantia propria, superior conjunctival fornix, and episclera, as well as extension from the lateral rectus muscle sheath. Immunohistochemical staining was strongly positive for CD68, alpha1-antitrypsin, CD34, and vimentin.

  18. The ultrastructure of malignant fibrous histiocytoma (case report).

    PubMed

    Lombardi, L; Pilotti, S; Carbone, A; Rilke, F

    1977-01-01

    A soft tissue malignant fibrous histiocytoma was studied by light and electron microscopy. The presence of fibroblast-like cells, histiocyte-like cells, undifferentiated stem cells, and xanthomatous cells was confirmed. Fibroblast- and histiocyte-like multinucleated giant cells were also observed. The observation of lysosomes in some fibroblast-like cells, the features of undifferentiated stem cells, and the presence of cells and intermediate characteristics common to xanthomatous, histiocytic, and stem cells suggest a strict relationship among these cellular types.

  19. Malignant solitary fibrous tumor of the urinary bladder.

    PubMed

    Cheng, Shih-Hao; Wang, Shian-Shiang; Lee, Chen-Hui; Ou, Yen-Chung; Cheng, Chen-Li

    2012-09-01

    A 67-year-old man was examined for persistent pain over his lower abdomen and was found to have a large pelvic tumor. During surgery, we detected a 16 × 9 × 9 cm(3) urinary bladder tumor with small intestinal adhesions, and performed partial cystectomy and segmental resection. Histological and immunohistochemical examinations established the diagnosis of malignant solitary fibrous tumor (SFT). Only 10 cases of urinary bladder SFT have been reported in the English literature; our patient is the second one with malignancy and had a longer follow-up period than the other case.

  20. Malignant solitary fibrous tumor of breast: a rare case report.

    PubMed

    Yang, Lian-He; Dai, Shun-Dong; Li, Qing-Chang; Xu, Hong-Tao; Jiang, Gui-Yang; Zhang, Yong; Wang, Liang; Fan, Chui-Feng; Wang, En-Hua

    2014-01-01

    Solitary fibrous tumor (SFT) is rare mesenchymal neoplasm that has been originally and most often documented in the pleura. Recently, the ubiquitous nature of the SFT has been recognized with reports of involvement of numerous sites all over the body such as: upper respiratory tract, somatic tissue, mediastinum, head, and neck. Less than 10 cases SFT of breast have been reported. Herein, we presented a 52-year-old Asian female with SFT of breast, this tumor showed predominant malignant features. To our knowledge, SFT of breast with such malignant evidence is extremely rare.

  1. Disseminated malignant solitary fibrous tumor of the pleura.

    PubMed

    Zhang, Hongquan; Lucas, David R; Pass, Harvey I; Che, Mingxin

    2004-02-01

    Solitary fibrous tumor (SFT) of the pleura typically forms a localized pleura-based mass, and most are benign. A rare case of disseminated malignant SFT of the pleura is reported. The patient was a 71-year-old man who presented with complaints of shortness of breath to his primary care physician. A diagnosis of malignant mesothelioma was suspected, based on clinical, radiological and needle biopsy findings. He was referred to our institution for surgery. An extrapleural pneumonectomy, encompassing all pleural masses, was performed. Gross examination of the resected specimen was remarkable for numerous masses, ranging in size from 0.2 to 13.5 cm, covering the majority of the visceral pleura. Histologically, the tumor was composed of short spindle cells admixed with variable proportions of collagenous stroma. There were great intra- and intertumoral heterogeneity in tumor growth pattern, cellularity, pleomorphism and mitoses. Histologically malignant areas were present in all of the masses examined. The neoplastic cells were diffusely and intensely positive for bcl-2. Most tumor cells were also strongly stained for CD34 and CD99. Staining for cytokeratin was negative. The tumor also revealed p53 over-expression. Thus, the histological and immunohistochemical features of the tumor were consistent with a disseminated malignant SFT. This report shows that SFT rarely presents with disseminated pleural involvement, and a panel with CD34, bcl-2 and cytokeratin are valuable for differentiating SFT from malignant mesothelioma and other malignant spindle cell neoplasms of the pleura.

  2. Malignant giant solitary fibrous tumor of the mediastinum.

    PubMed

    De Raet, Jan; Sacré, Robert; Hoorens, Anne; Fletcher, Christopher; Lamote, Jan

    2008-09-01

    Malignant giant solitary fibrous tumor (SFT) of the mediastinum is a rare neoplasm derived from mesenchymal tissue. Owing to its large size, a complete resection of the tumor can present many challenges, particularly given its proximity to vital neighboring structures. We report a successful en-bloc resection of a massive mediastinal SFT, which was compressing the inferior trachea and heart, by means of a median sternotomy and an anterior left thoracotomy. We emphasize the rarity of this uncommon mediastinal mass. Key points of mediastinal SFT are discussed.

  3. Recurrent Intrapulmonary Solitary Fibrous Tumor With Malignant Transformation.

    PubMed

    Inoue, Takuya; Owada, Yuki; Watanabe, Yuzuru; Muto, Satoshi; Okabe, Naoyuki; Yonechi, Atsushi; Kanno, Ryuzo; Suzuki, Hiroyuki

    2016-07-01

    Intrapulmonary solitary fibrous tumor (SFT) of the pleura; the so-called inverted pattern, which appears to grow into the lung parenchyma, is extremely rare. We experienced a 66-year-old woman with an intrapulmonary SFT that recurred locally with malignant transformation 2 years after wedge resection of the left upper lobe for the primary tumor. Subsequently, she underwent a lobectomy of the residual left upper lobe. Six years after the second operation she was well, without rerecurrence. Complete excision and long-term follow-up of intrapulmonary SFTs of the pleura are important, even when the primary tumor displays benign histopathologic features.

  4. Recurrent Intrapulmonary Solitary Fibrous Tumor With Malignant Transformation.

    PubMed

    Inoue, Takuya; Owada, Yuki; Watanabe, Yuzuru; Muto, Satoshi; Okabe, Naoyuki; Yonechi, Atsushi; Kanno, Ryuzo; Suzuki, Hiroyuki

    2016-07-01

    Intrapulmonary solitary fibrous tumor (SFT) of the pleura; the so-called inverted pattern, which appears to grow into the lung parenchyma, is extremely rare. We experienced a 66-year-old woman with an intrapulmonary SFT that recurred locally with malignant transformation 2 years after wedge resection of the left upper lobe for the primary tumor. Subsequently, she underwent a lobectomy of the residual left upper lobe. Six years after the second operation she was well, without rerecurrence. Complete excision and long-term follow-up of intrapulmonary SFTs of the pleura are important, even when the primary tumor displays benign histopathologic features. PMID:27343529

  5. Malignant solitary fibrous tumor in the extremity: Cytopathologic findings.

    PubMed

    Khanchel, Fatma; Driss, Maha; Mrad, Karima; Romdhane, Khaled Ben

    2012-04-01

    Malignant solitary fibrous tumor (SFT) is an extremely rare neoplasm. There are only rare published accounts of the cytopathologic features of this tumor. We report a case of a 59-year-old woman presented with a 10-year history of a right thigh mass. A preoperative fine needle aspiration (FNA) was performed. Smears were hypercellular, with cohesive and crowded tissue fragments, haphazard cell arrangements and many single cells. The tumor cells were polymorphous, plump spindled or round with often indented or bare nuclei. A differential diagnosis of low grade sarcoma was favored. The diagnosis of malignant SFT is extremely difficult on FNA and must be included in the differential diagnosis of spindle cell neoplasms.

  6. Malignant solitary fibrous tumor of the right atrium.

    PubMed

    Zhao, Xiao-Gang; Wang, Hao; Wang, Yan-Li; Chen, Gang; Jiang, Ge-Ning

    2012-11-01

    Cardiac solitary fibrous tumors (SFTs) are extremely rare, with only 4 cases described to date. Here, we describe the case of a 55-year-old man with a primary, malignant SFT that arose in the right atrium of his heart. His presenting complaint was of chest distress and shortness of breath. Physical examination revealed jugular venous engorgement. Computed tomography showed a tumor of 15 × 10 × 8 cm in the anterior mediastinum. The tumor was surgically resected under cardiopulmonary bypass. A grayish white neoplasm was discovered lying within the right atrium. The histological appearance of the tumor was of a hypercellular distribution of spindle cells with nuclear atypia, arranged in a storiform pattern and intermixed with collagenous stroma. Immunohistochemistry showed that the tumor was positive for CD34. Based on the aforementioned features, the diagnosis of malignant SFT was made. The patient subsequently died as a result of respiratory and circulatory failure.

  7. Malignant solitary fibrous tumor of the nasal cavity.

    PubMed

    Zeitler, Daniel M; Kanowitz, Seth J; Har-El, Gady

    2007-07-01

    Solitary fibrous tumors (SFTs) are unusual mesenchymal tumors that were first described as primary spindle-cell neoplasms of the pleura. These tumors have been described in many other locations, including the urogenital system, orbit, mediastinum, and upper respiratory tract. Twenty-two cases of an SFT of the paranasal sinuses and nasal cavity have been reported, but none described a malignant SFT extending through the anterior skull base. A 70-year-old man had a 6-month history of unilateral left-sided epiphora and nasal obstruction. Computed tomography and magnetic resonance imaging showed a large left-sided nasal cavity mass with extension into the left extraconal orbit and intracranial extension through the left cribriform plate and ethmoid roof. The patient underwent preoperative embolization of the internal maxillary artery and a subsequent anterior craniofacial resection via a midfacial degloving approach and a left anterior craniotomy. Histopathological analysis of the specimen was consistent with a malignant SFT.

  8. Solitary fibrous tumor with malignant potential arising in sublingual gland.

    PubMed

    Ogawa, Ikuko; Sato, Sunao; Kudo, Yasusei; Miyauchi, Mutsumi; Sugiyama, Masaru; Suei, Yoshikazu; Takata, Takashi

    2003-01-01

    A rare case is described of a solitary fibrous tumor (SFT) with malignant potential arising in the sublingual gland. A 59-year-old man presented with a 4-month history of a slowly enlarging painless mass in the center of the floor of the mouth. The tumor was a well-demarcated, firm mass with a multicystic lesion. The tumor exhibited highly cellular areas of spindle cells with patternless architecture alternating with hypocellular areas. The tumor cells were positive for CD34 and bcl-2 as well as vimentin, and negative for epithelial, myogenic, neurogenic and histiocytic markers. The tumor cells formed multiple satellite nodules around dilated ducts in the multicystic lesion, indicating infiltrative growth. In addition, areas exhibiting higher cellularity with increased mitoses were noticed in the satellite nodules, although cellular atypia was not obvious. These findings led to a final diagnosis of SFT with malignant potential. There has been no recurrence or metastasis for 27 months after the surgery. Solitary fibrous tumor of the salivary gland must be differentiated from various spindle cell neoplasms including myogenic, peripheral nerve sheath, fibroblastic and fibro-histiocytic spindle cell neoplasms, hemangiopericytoma and myoepithelioma. In addition to characteristic morphological features, an immunohistochemical positivity for CD34 and bcl-2 may aid in the diagnosis of SFT.

  9. De novo malignant solitary fibrous tumor of the kidney.

    PubMed

    Hsieh, Tsan-Yu; ChangChien, Yi-Che; Chen, Wen-Hsiang; Chen, Siu-Chung; Chang, Liang-Che; Hwang, Cheng-Cheng; Chein, Hui-Ping; Chen, Jim-Ray

    2011-01-01

    The kidney is a relatively infrequent site for solitary fibrous tumor (SFT). Among the previously reported cases, only two cases of malignant renal SFT developing via dedifferentiation from a pre-existing benign SFT have been reported. Here we reported a case of de novo malignant renal SFT clinically diagnosed as renal cell carcinoma in a 50-year-old woman. The tumor was circumscribed but unencapsulated and showed obvious hemorrhagic necrosis. Microscopically, the tumor was composed of patternless sheets of alternating hypercellular and hypocellular areas of spindle cells displaying mild to moderate nuclear atypia, frequent mitoses up to 8 per 10 high power fields, and a 20% Ki-67 proliferative index. Immunohistochemical studies revealed reactivity for CD34, CD99 and vimentin, with no staining for all other markers, confirming the diagnosis of SFT. No areas of dedifferentiation were seen after extensive sampling. Based on the pathologic and immunohistochemical features, a diagnosis of de novo malignant renal SFT was warranted. Our report expands the spectrum of malignant progression in renal SFTs. Even though this patient has been disease-free for 30 months, long-term follow-up is still mandatory.

  10. Malignant solitary fibrous tumor of the pleura with liposarcomatous differentiation.

    PubMed

    Bai, H; Aswad, B I; Gaissert, H; Gnepp, D R

    2001-03-01

    Pleural solitary fibrous tumors (SFTs) are uncommon tumors. Although these tumors have been well characterized, malignant pleural SFTs with liposarcomatous differentiation have not been reported. We report an unusual malignant pleural SFT intermixed with foci of well-differentiated liposarcoma. The patient was a 66-year-old, white man with a large, solid right pleural mass that measured 13.5 x 10.3 x 8.5 cm. The tumor was composed of spindle-shaped and plump cells embedded in dense collagenous stroma. The tumor cells were arranged in interlacing fascicles or in a patternless pattern. Marked nuclear atypia, a high mitotic rate (21 mitoses per 10 high-power fields), and areas of prominent necrosis were evident. In addition, numerous adipocytes mixed with typical lipoblasts were seen scattered throughout portions of the tumor. Immunohistochemistry revealed the tumor cells were strongly positive for CD34 and vimentin and negative for cytokeratin, desmin, smooth muscle actin (IA4), and S100. To the best of our knowledge, this case represents the first example of a malignant SFT with liposarcomatous differentiation.

  11. Metachronous Malignant Solitary Fibrous Tumor of Kidney: Case Report and Review of Literature.

    PubMed

    Cheung, Felix; Talanki, Varun R; Liu, Jingxuan; Davis, James E; Waltzer, Wayne C; Corcoran, Anthony T

    2016-01-01

    Solitary fibrous tumors are well described in the pleura, but rare extra-pleural neoplasms have been reported. We describe a patient with a solitary left renal fibrous tumor who after undergoing a nephrectomy, presented 8 years later with a contralateral metachronous solitary fibrous tumor. Malignant metastatic extra-pleural solitary fibrous tumors are extremely rare, and to our knowledge, this is the first case of contralateral recurrence of solitary renal fibrous tumor. The patient underwent a robotic assisted partial nephrectomy of the right renal mass. Both tumors showed overlapping histopathology.

  12. Metachronous Malignant Solitary Fibrous Tumor of Kidney: Case Report and Review of Literature.

    PubMed

    Cheung, Felix; Talanki, Varun R; Liu, Jingxuan; Davis, James E; Waltzer, Wayne C; Corcoran, Anthony T

    2016-01-01

    Solitary fibrous tumors are well described in the pleura, but rare extra-pleural neoplasms have been reported. We describe a patient with a solitary left renal fibrous tumor who after undergoing a nephrectomy, presented 8 years later with a contralateral metachronous solitary fibrous tumor. Malignant metastatic extra-pleural solitary fibrous tumors are extremely rare, and to our knowledge, this is the first case of contralateral recurrence of solitary renal fibrous tumor. The patient underwent a robotic assisted partial nephrectomy of the right renal mass. Both tumors showed overlapping histopathology. PMID:26793578

  13. An unresectable retroperitoneal malignant fibrous histiocytoma: A case report

    PubMed Central

    HSIAO, PO-JEN; CHEN, GUANG-HENG; CHANG, YI-HUEI; CHANG, CHAO-HSIANG; CHANG, HAN; BAI, LI-YUAN

    2016-01-01

    Malignant fibrous histiocytoma (MFH) is most commonly observed in the extremities and the trunk but rarely in retroperitoneum. The present case report documents a 64-year-old man who was admitted with an abdominal palpable mass for 6 months. After a thorough investigation, a tumor of the retroperitoneum was identified adhered to adjacent organs and vessels. The patient experienced mild hydronephrosis and hydroureter as a result of the tumor compression. A number of previous surgeons considered the tumor unresectable and suggested palliative treatment. En bloc resection of the tumor was attempted but incomplete surgery was performed initially as the tumor was friable and prone to bleeding. Therefore, a biopsy of the tumor was performed and a double J ureteral stent was set for hydronephrosis. Histopathological examination confirmed the tumor was an MFH. The patient received neo-adjuvant chemotherapy with 4 cycles of mesna, doxorubicin, ifosfamide, and dacarbazine (MAID). A computed tomography scan demonstrated that the tumor had reduced in size following chemotherapy. En bloc resection of the tumor was arranged again 6 months later. The tumor exhibited a complete response to neo-adjuvant chemotherapy after the formal pathological evaluation. The patient survives without tumor recurrence >5 years without recurrence. PMID:27073487

  14. Extrathoracic solitary fibrous tumor of the pelvic peritoneum with central malignant degeneration on CT and MRI.

    PubMed

    Vossough, Arastoo; Torigian, Drew A; Zhang, Paul J; Siegelman, Evan S; Banner, Marc P

    2005-11-01

    We describe a 61-year-old man who presented with an extrathoracic solitary fibrous tumor (SFT) of the pelvic peritoneum with central malignant degeneration as seen on computed tomography (CT), magnetic resonance imaging (MRI), and histopathology. When a central focus of heterogeneity and variable contrast enhancement are identified within a fibrous tumor of the pelvis on CT or MRI, malignant degeneration of an extrathoracic SFT, although rare, should be considered as a diagnostic possibility.

  15. Retroperitoneal malignant fibrous histiocytoma surgically removed six times using a harmonic scalpel.

    PubMed

    Maruta, F; Sugiyama, A; Murakami, T; Murakami, M; Ikeno, T; Shimizu, F; Ishida, K; Matsushita, K; Ota, H; Kawasaki, S

    2000-01-01

    This paper describes a patient with retroperitoneal malignant fibrous histiocytoma who underwent surgical removal of the tumor 6 times. It is difficult to remove locally recurrent retroperitoneal malignant fibrous histiocytoma many times by repeat surgery, since the tumor tends to adhere tightly to the surrounding scar tissue. In the 5th and 6th operations we conducted on the patient, a harmonic scalpel was useful for separating the tumor from the scar tissue at the previous surgical site.

  16. Pleural malignant solitary fibrous tumor with sarcomatous overgrowth showing PDGFRbeta mutation.

    PubMed

    Rossi, Giulio; Schirosi, Laura; Giovanardi, Filippo; Sartori, Giuliana; Paci, Massimiliano; Cavazza, Alberto

    2006-08-01

    Pleural malignant solitary fibrous tumors (SFTs) are uncommon, and little is known about their histogenesis and molecular features. We report a case of pleural SFT with sarcomatous overgrowth that showed expression for PDGFRbeta and a missense mutation on exon 18 of the PDGFRbeta gene. The involvement of the PDGFRbeta gene in SFT is compatible with a pericytic derivation, also supporting a possible role of this tyrosine kinase in malignant transformation and in the adoption of novel molecular therapies.

  17. Radical resection of a giant, invasive and symptomatic malignant Solitary Fibrous Tumour (SFT) of the pleura.

    PubMed

    Filosso, Pier Luigi; Asioli, Sofia; Ruffini, Enrico; Rovea, Paolo; Macri', Luigia; Sapino, Anna; Bretti, Sergio; Lyberis, Paraskevas; Oliaro, Alberto

    2009-04-01

    Solitary Fibrous Tumours (SFTs) of the pleura are rare neoplasms, with unpredictable biological behaviour. Although usually benign, malignant SFTs are described, and they are often associated with large, necrotic and locally invasive tumours. Radical resection represents the treatment of choice in all cases; recurrences are uncommon, and redo-surgery should be considered. The case of a giant, invasive, radically resected malignant SFT, is described. The role of postoperative radiotherapy, to reduce the risk of recurrence, is also discussed.

  18. The use of clustering software for the classification of comparative genomic hybridization data. an analysis of 109 malignant fibrous histiocytomas.

    PubMed

    Chibon, Frédéric; Mariani, Odette; Mairal, Aline; Derré, Josette; Coindre, Jean-Michel; Terrier, Philippe; Lagacé, Réal; Sastre, Xavier; Aurias, Alain

    2003-02-01

    Malignant fibrous histiocytoma (MFH) is considered the most frequent soft-tissue sarcoma of late adult life. Nevertheless, the validity of this entity has been recurrently questioned by pathologists. Preliminary analyses by comparative genomic hybridization (CGH) of series of MFH have suggested that this tumor group is heterogeneous at the genomic level, and that at least two main genetic subgroups exist. We report an analysis by CGH of a large series of 109 MFH and on the use of clustering software for an objective classification of these tumors. We confirm our preliminary CGH results and demonstrate that two main clusters of tumors are present in the series analyzed. PMID:12581902

  19. Solitary fibrous tumor: histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites.

    PubMed

    Hanau, C A; Miettinen, M

    1995-04-01

    Twenty-nine tumors (from 26 patients, including two with recurrent disease) diagnosed as solitary fibrous tumor (SFT) of the pleura (n = 23), mediastinum (n = 4), abdominal cavity (n = 1), and parotid gland (n = 1) were studied immunohistochemically. Three histologically malignant tumors showed areas of high cellularity and mitotic activity (more than 4 mitoses/10 high-power fields) with features resembling malignant fibrous histiocytoma, malignant hemangiopericytoma, or fibrosarcoma, together with areas typical of benign solitary fibrous tumor. Formaldehyde-fixed, paraffin-embedded tissues and avidin-biotin-complex immunostaining were used. All of the tumors showed vimentin positivity and did not stain for cytokeratin, glial fibrillary acidic protein, or muscle cell markers, except for focal desmin reactivity in seven tumors, mostly seen in frozen sections, and focal keratin reactivity in one histologically malignant tumor. The neoplastic cells were positive for CD34 and negative for CD31; these patterns also were seen in the three histologically malignant cases. In nine of the cases acetone-fixed frozen sections showed variable focal positivity for neurofilament proteins of 68 kd. We conclude that SFT is a neoplasm of fibroblasts/primitive mesenchymal cells with features of multidirectional differentiation. We also report the finding of a novel site for SFT, the parotid gland.

  20. Postoperative Radiotherapy for the Treatment of Solitary Fibrous Tumor With Malignant Transformation of the Pelvic

    PubMed Central

    Gao, Chao; Zhang, Yong; Jing, Ming; Qu, Wei; Li, Jia; Zhao, Xiang-Rong; Yu, Yong-Hua

    2016-01-01

    Abstract Solitary fibrous tumor of the pelvic is an uncommon neoplasm with nonspecific symptoms. Reports of malignant transformation are especially rare. We report a case of solitary fibrous tumor in pelvic. A unique feature of our case compared with previously reported is that this patient relapsed with malignant transformation and had significant response to radiotherapy. The patient was initially treated with surgery, followed by postoperative dimensional conformal intensity modulated radiation therapy (dynamic MLC VRIAN 23EX Linac, inversely optimized by the Eclipse system) to provide a radical cure for residual tumor. In this case, there were no signs of recurrence after six and a half years of further follow-up, indicating that postoperation radiotherapy may be an effective treatment for SFT with malignant transformation in pelvic. PMID:26765426

  1. Cytological patterns of primary malignant uterine fibrous histiocytoma. A case report.

    PubMed

    Torrisi, A; Giorgino, F; Onnis, G L; Minucci, D

    1990-01-01

    The Authors have reported the cytological patterns of malignant primitive uterine fibrous histiocytoma of a 57 year old woman. The endometrial cytological sampling was performed by an aspiration technique using a feeding-tube; a necrotic-haemorrhagic background filled with hymphocytes and polymorphonuclear leukocytes, and a mixture of highly anaplastic histiocytic and fibroblastic type cells, associated with undifferentiated small round cells, has been described. The result of the histological examination performed on the surgical specimen was the following: polymorphic malignant neoplasia characterized by spindle-like and giant plurinucleated cells, phagocytosis features, endoluminal polypoid growth, wide infiltration of the miometrium two thirds deep, vascular space invasion of the uterine wall and the hilus of the ovaries. The histological features and immunohistochemical analysis were consistent with malignant fibrous histiocytoma in the pleomorphic variety. PMID:1965802

  2. Multiple recurrent malignant solitary fibrous tumors: long-term follow-up of 24 years.

    PubMed

    Park, Chang Kwon; Lee, Deok Heon; Park, Ji Young; Park, Sun Hwa; Kwon, Kun Young

    2011-04-01

    Solitary fibrous tumor (SFT) of the pleura is a rare, generally benign, neoplasm that accounts for less than 5% of all pleural tumors. However, 10% to 30% of SFTs display aggressive behavior with local recurrence, malignant transformation, and distant metastasis. We report a case of multiple recurrent SFTs of the thorax in a 77-year-old Korean woman. During a 24-year period, she underwent six surgical resections for six recurrent SFTs. The tumor eventually underwent morphologically malignant transformation. This unique case highlights the necessity of long-term follow-up in cases of SFT because of the potentially malignant biological behavior of this type of tumor.

  3. [Recurrent malignant solitary fibrous tumor of the orbit].

    PubMed

    Manousaridis, K; Stropahl, G; Guthoff, R F

    2011-03-01

    A 53-year-old female patient presented with a palpable mass in the right medial orbit which had been present for 18 months. The left eye had been enucleated in childhood due to a perforating injury. An orbital biopsy performed ex domo showed unspecific inflammatory tissue but no definite diagnosis was made and systemic therapy with corticosteroids was unsuccessful. A second orbital biopsy was performed in our clinic and revealed a solitary fibrous tumor (SFT) of the orbit. Medial orbitotomy with tumor excision followed and the histological examination of the excised tumor established the diagnosis of SFT of the orbit with focal sarcomatous transformation. The patient presented 3 years later with a new orbital mass in a control MRI examination. Relapse was suspected and orbitotomy with excision of the mass was performed. The histological examination revealed a relapse of the SFT with sarcomatous transformation in the whole extent of the tumor. Radiotherapy with a dosis of 59.4 Gy was subsequently performed. The patient remained tumor-free in the most recent control examination 3 years later.

  4. Most malignant fibrous histiocytomas developed in the retroperitoneum are dedifferentiated liposarcomas: a review of 25 cases initially diagnosed as malignant fibrous histiocytoma.

    PubMed

    Coindre, Jean-Michel; Mariani, Odette; Chibon, Frédéric; Mairal, Aline; De Saint Aubain Somerhausen, Nicolas; Favre-Guillevin, Elizabeth; Bui, Nguyen Binh; Stoeckle, Eberhard; Hostein, Isabelle; Aurias, Alain

    2003-03-01

    Forty-four samples from 25 cases of retroperitoneal sarcoma initially diagnosed as malignant fibrous histiocytoma were histologically reviewed. Immunohistochemistry for mdm2 and cdk4 was performed on 20 cases. Comparative genomic hybridization was performed on 18 samples from 13 patients. Seventeen cases were reclassified as dedifferentiated liposarcoma. Twenty-one of 32 samples from these patients showed areas of well-differentiated liposarcoma, allowing the diagnosis of dedifferentiated liposarcoma. Immunohistochemistry performed in 15 of these cases showed positivity for mdm2 and cdk4. Comparative genomic hybridization analysis performed on 15 samples from 11 of these patients showed an amplification of the 12q13-15 region. Eight cases were reclassified as poorly differentiated sarcoma. Twelve samples from these patients showed no area of well-differentiated liposarcoma. Immunohistochemistry showed positivity for mdm2 and cdk4 in one of six of these patients and showed positivity for CD34 in another one. Comparative genomic hybridization analysis performed on three samples from two of these patients showed no amplification of the 12q13-15 region but showed complex profiles. This study shows that most so-called malignant fibrous histiocytomas developed in the retroperitoneum are dedifferentiated liposarcoma and that a poorly differentiated sarcoma in this area should prompt extensive sampling to demonstrate a well-differentiated liposarcoma component, immunohistochemistry for mdm2 and cdk4, and if possible, a cytogenetic or a molecular biology analysis. PMID:12640106

  5. An aggressive solitary fibrous tumor with evidence of malignancy: a rare case report.

    PubMed

    Vimi, S; Punnya, V A; Kaveri, H; Rekha, K

    2008-09-01

    Solitary fibrous tumor (SFT) is rare mesenchymal neoplasm that has been originally and most often documented in the pleura. Recently, the ubiquitous nature of the SFT has been recognized with reports of involvement of numerous sites all over the body, i.e, upper respiratory tract, breast, somatic tissue, mediastinum, head, and neck, etc. The diagnosis of SFT still remains an enigma in our field. Furthermore, malignant SFT is extremely rare and only two cases have been reported in the oral cavity till date. Here, we present a rare case report of an aggressive solitary fibrous tumor which presented as a palatal mass and extended throughout the middle cranial fossa and exhibited features of malignancy.

  6. [Pulmonary malignant fibrous histiocytoma (inflammatory type) showing intracavitary fungus ball-like shadow].

    PubMed

    Kobayashi, T; Takenaka, M; Kido, T; Tanio, Y

    1999-02-01

    We report a rare case of primary malignant fibrous histiocytoma of the lung showing intracavitary fungus ball-like shadows. Fiberoptic bronchoscopy revealed no visible tumor, but adenocarcinoma cells were detected in samples of lavage fluid from the cavitary lesion. Staging procedures (T 2 N 0 M 0) confirmed that there were no metastatic lesions. A complete resection of the left lower lobe was performed. The tumor showed polypoid growth that obstructed a small peripheral bronchus, and formed a cavitary lesion. It was histologically diagnosed as an inflammatory type of malignant fibrous histiocytoma, and consisted of atypical histiocyte-like cells, neutrophils, lymphocytes, foamy cells, and fibroblast-like cells in a storiform pattern. The patient has been in complete remission for 3 years after surgery. PMID:10214047

  7. Malignant fibrous histiocytoma (giant cell type) associated with a malignant mixed tumor in the salivary gland of a dog.

    PubMed

    Pérez-Martínez, C; García Fernández, R A; Reyes Avila, L E; Pérez-Pérez, V; González, N; García-Iglesias, M J

    2000-07-01

    A 12-year-old male Boxer dog presented with a 5 x 5 x 7-cm partially encapsulated mass in the right mandibular salivary gland. Histologically, the mass was composed of neoplastic epithelial and mesenchymal cells. The mesenchymal component consisted of two cell populations arranged in different patterns: coalescing nodules of neoplastic mononuclear cells with rare osteoid and numerous osteoclastlike giant cells; and sheets of neoplastic spindle cells intermingled with neoplastic epithelial cells and containing osteoid and well-formed bone trabeculae lined by osteoblasts and few osteoclastlike giant cells. On the basis of these histological features, two malignant salivary tumors were diagnosed: a malignant fibrous histiocytoma (giant cell type) and a malignant mixed tumor. Immunohistochemical studies demonstrated keratin 5 and 8 expression by the neoplastic epithelial cells, indicating a probable salivary ductal origin, and vimentin expression by all mesenchymal elements, suggesting a fibroblastic line of differentiation.

  8. Basic FGF and Ki-67 proteins useful for immunohistological diagnostic evaluations in malignant solitary fibrous tumor.

    PubMed

    Sun, Yuliang; Naito, Zenya; Ishiwata, Toshiyuki; Maeda, Shotaro; Sugisaki, Yuichi; Asano, Goro

    2003-05-01

    Solitary fibrous tumor (SFT) is an uncommon soft tissue tumor initially reported in the pleura but recently described in other sites in the body. Morphological distinction between benign and malignant SFT is often difficult. An immunohistochemical study was performed in pleural and extrapleural sites. The aim of this study was to determine if an immunohistochemical method is helpful in distinguishing benign SFT from malignant SFT, and providing valid information to predict the prognosis associated with malignant SFT. Twenty-four cases of benign (14 patients) and malignant (10 patients) SFT in the pleura, pelvic space, prostate and other sites of soft tissue were analyzed. Tumors from 10 patients were diagnosed as malignant on the basis of markedly increased cellularity, mitotic activity (>4/10 high-power fields), nuclear pleomorphism and areas of necrosis. Immunohistochemically, we found a mean basic fibroblast growth factor (bFGF) labeling index of 48.67% (48.67 +/- 8.52%) for benign SFT and 74.5% (74.5 +/- 6.92%) for malignant SFT (P < 0.05). We also found a mean Ki-67 labeling index of 1.9% (1.9 +/- 0.43%) for benign SFT and 6.11% (6.11 +/- 1.05%) for malignant SFT (P < 0.05). Our results suggest that bFGF and Ki-67 are diagnostically relevant to the evaluation of malignant SFT and these proteins are thought to be potentially useful markers for prognosis of SFT.

  9. A clinicopathologic comparison of malignant fibrous histiocytoma and liposarcoma.

    PubMed

    Spanier, S S; Floyd, J

    1989-01-01

    The diagnosis of MFH depends on the demonstration of histiocytic and fibroblastic functions. MFHs may phagocytose fat; therefore, lipid stains are useless. By electron microscopy, cytoplasmic lipid is membrane-bound. Immunohistochemical staining for vimentin and histiocytic markers may be helpful. Liposarcoma is diagnosed only when there is convincing evidence of synthesis and storage of fat by the tumor cells. By electron microscopy, cytoplasmic lipid is nonmembrane-bound. Both MFH and liposarcoma have subtypes. In MFH, the pleomorphic forms are the most common. Myxoid MFH is less common; all other types are rare. In liposarcoma, the myxoid types are by far the most common. The myxoid types of both MFH and liposarcoma may contain other elements that vary in degree and geographic distribution and that can raise the histologic grade. About 50% of liposarcomas are low-grade tumors; these are almost always purely myxoid. Low-grade myxoid liposarcoma has a much better prognosis than other types. Myxoid liposarcoma has a better prognosis than myxoid MFH. The peak incidence of MFH is in the seventh decade of life whereas that of liposarcoma is in the fifth decade. A substantial number (roughly 25% to 30%) of MFHs occur in the subcutaneous tissue. Clinically they are almost invariably mistaken for ganglion cysts. Liposarcoma, however, is likely to occur in or below the buttocks. Most are in the anterior thigh. Subcutaneous liposarcoma is extremely rare. A tumor in this area is likely to be either a more malignant myxoid MFH or one of the benign atypical lipomatous tumors. In both MFH and liposarcoma, the development of distant lesions is related to the tumor's histologic grade and size and to local recurrence. Favored metastatic sites of MFH are lung and lymph nodes. Favored sites of distant lesions in the myxoid/round cell types of liposarcoma are intra-abdominal, retroperitoneal, other soft-tissue areas (especially in the neck), and bone. Lymph node involvement is very

  10. Malignant solitary fibrous tumor of the pleura: report of a case with cytogenetic analysis.

    PubMed

    de Leval, Laurence; Defraigne, Jean-Olivier; Hermans, Gilberte; Dôme, Florence; Boniver, Jacques; Herens, Christian

    2003-04-01

    The majority of solitary fibrous tumors (SFTs) of the pleura are benign, but 10-30% locally recur or metastasize. Pathogenic factors relevant to the determinism of their biological properties are largely unknown. Cytogenetic data on SFTs of the pleura are sparse. We report herein a case of a malignant SFT of the pleura where successful karyotyping was obtained from the primary and recurrent tumors. The initial karyotype showed two abnormal clones: 48, XY; +8; +8; del(9)(q22; q32) [19] and 46, XY, t(1;16)(q25;p12) [7]. Culture of the recurrent tumor yielded one clone identical to the dominant clone of the initial karyotype. Demonstration of a recurrent abnormal karyotype largely supports its relevance to the malignant clone and suggests a role of supernumerary chromosome(s) 8 in the determinism of malignant behavior in SFT.

  11. Localized malignant pleural sarcomatoid mesothelioma misdiagnosed as benign localized fibrous tumor

    PubMed Central

    Vo, Hong-Phuc

    2016-01-01

    Localized malignant pleural mesothelioma (LMPM) is a rare tumor with good prognosis by surgical resection. We report an atypical case of malignant pleural sarcomatoid mesothelioma (SM) in an asymptomatic 65-year-old woman, who had no history of exposure to asbestos. She presented with a small pleural mass without pleural effusion and was misdiagnosed as a benign localized fibrous tumor (BLFT) on pathologic examination through a surgical tumor specimen. However, seven months later, the patient returned with serious cancerous symptoms. A large recurrent tumor mass was found within the chest wall invading at the old surgical resection site. SM, a subtype of LMPM, was confirmed with histopathogy and immunohistochemisty. In conclusion, malignant pleural mesothelioma (MPM) can present with typical radiologic finding similar to a BLFT, and has a wide histopathologic presentation in biopsy specimen. A thorough pathologic investigation should be attempted even when a pleural mass resembles benign, localized, and small on radiologic studies. PMID:27293862

  12. Localized malignant pleural sarcomatoid mesothelioma misdiagnosed as benign localized fibrous tumor.

    PubMed

    Kim, Kwan-Chang; Vo, Hong-Phuc

    2016-06-01

    Localized malignant pleural mesothelioma (LMPM) is a rare tumor with good prognosis by surgical resection. We report an atypical case of malignant pleural sarcomatoid mesothelioma (SM) in an asymptomatic 65-year-old woman, who had no history of exposure to asbestos. She presented with a small pleural mass without pleural effusion and was misdiagnosed as a benign localized fibrous tumor (BLFT) on pathologic examination through a surgical tumor specimen. However, seven months later, the patient returned with serious cancerous symptoms. A large recurrent tumor mass was found within the chest wall invading at the old surgical resection site. SM, a subtype of LMPM, was confirmed with histopathogy and immunohistochemisty. In conclusion, malignant pleural mesothelioma (MPM) can present with typical radiologic finding similar to a BLFT, and has a wide histopathologic presentation in biopsy specimen. A thorough pathologic investigation should be attempted even when a pleural mass resembles benign, localized, and small on radiologic studies. PMID:27293862

  13. A recurrent solitary fibrous tumor of the thigh with malignant transformation: A case report

    PubMed Central

    Yoshimura, Yasuo; Sano, Kenji; Isobe, Ken-ichi; Aoki, Kaoru; Kito, Munehisa; Kato, Hiroyuki

    2016-01-01

    Introduction We describe an unusual case of a uniformly high-grade malignant solitary fibrous tumor (SFT) of the thigh with recurrence after wide resection in a 31-year-old man. Presentation of case Our current case showed a long-term benign course before the operation, although the subcutaneous tumor was larger than 10 cm at presentation. The SFT was diagnosed by needle biopsy, and wide resection was performed. Histological findings showed proliferation of capillaries surrounded by masses of spindle-shaped cells without any cytologic atypia, and the percentage of MIB-1-positive nuclei was 2.1%. However, a rapidly enlarging recurrent tumor was observed 11 months after the operation. A second wide resection for the recurrent tumor was performed. Histologically, the tumor cells uniformly displayed significant cytologic atypia and pleomorphism, and had 40–50 mitoses per 10 high-power fields. The proportion of MIB-1-positive nuclei was 48%. Consequently, the tumor was diagnosed as a SFT with malignant transformation. Discussion The malignant transformation described in past studies showed high-grade areas within benign, low-grade, or intermediate-grade SFTs. Therefore, in contrast to our case, uniformly high-grade malignant histological findings at recurrence were not described. Conclusion Even if a tumor is non-malignant during the clinical course, as confirmed by tissue biopsy, the possibility of tumor progression to high-grade sarcoma at recurrence should be considered, and the treatment strategy should be determined carefully. PMID:26967903

  14. Recurrent malignant sino-nasal solitary fibrous tumor: Eliminate the enemy at the first instance.

    PubMed

    Roy, Soumyajit; Mallick, Supriya; Kakkar, Anchal; Jana, Manisha; Julka, Pramod Kumar

    2015-01-01

    Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms that usually arise in the pleura or less commonly in relation to other serosal surfaces. Infrequent case reports of extra-pleural SFTs have been described at various sub sites within the head and neck area. We report a case of recurrent sino-nasal SFTs treated with surgery followed by re-excision and adjuvant radiation on recurrence and then salvage chemotherapy on progression. Further discussed are the challenges associated with accurate histological and immunohistochemical diagnosis, the difficulty in assessing the aggressiveness and malignant potential of these lesions and the appropriate treatment and follow-up duration that these neoplasms require. To the best of our knowledge this is the first reported case of recurrent malignant sino-nasal SFT in available scientific literature in English language.

  15. Malignant solitary fibrous tumor of the thyroid: a case-report and review of the literature.

    PubMed

    Alves Filho, Wellington; Mahmoud, Renata Regina da Graça Lorencetti; Ramos, Daniel Marin; Araujo-Filho, Vergilius José Furtado de; Lima, Patricia Picciarelli de; Cernea, Claudio Roberto; Brandão, Lenine Garcia

    2014-06-01

    Solitary fibrous tumor (SFT) is an uncommon spindle-cell neoplasm that most often involves the pleura, rarely occurring in extra-thoracic locations. Twenty-six cases of SFT arising in the thyroid gland have been described. We report a case of a 60-year-old woman presenting an 8-month history of enlargement of the neck associated with dysphagia. The patient underwent a right hemithyroidectomy and SFT of the thyroid was diagnosed. Immunohistochemistry showed positivity for CD34 marker, and the high number of mitoses and the presence of cellular atypia suggested that the tumor was malignant. To our knowledge, this is the second case of malignant SFT of the thyroid gland ever reported. Due to the rarity of these tumors, the indication of adjuvant therapy and prognosis are uncertain. Long-term follow-up after surgical resection seems to be advisable.

  16. Recurrent malignant sino-nasal solitary fibrous tumor: Eliminate the enemy at the first instance.

    PubMed

    Roy, Soumyajit; Mallick, Supriya; Kakkar, Anchal; Jana, Manisha; Julka, Pramod Kumar

    2015-01-01

    Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms that usually arise in the pleura or less commonly in relation to other serosal surfaces. Infrequent case reports of extra-pleural SFTs have been described at various sub sites within the head and neck area. We report a case of recurrent sino-nasal SFTs treated with surgery followed by re-excision and adjuvant radiation on recurrence and then salvage chemotherapy on progression. Further discussed are the challenges associated with accurate histological and immunohistochemical diagnosis, the difficulty in assessing the aggressiveness and malignant potential of these lesions and the appropriate treatment and follow-up duration that these neoplasms require. To the best of our knowledge this is the first reported case of recurrent malignant sino-nasal SFT in available scientific literature in English language. PMID:26458621

  17. Postirradiation malignant fibrous histiocytoma arising in juvenile nasopharyngeal angiofibroma and producing alpha-1-antitrypsin.

    PubMed

    Spagnolo, D V; Papadimitriou, J M; Archer, M

    1984-03-01

    A fatal nasopharyngeal malignant fibrous histiocytoma developed in a young male after irradiation of juvenile nasopharyngeal angiofibroma diagnosed 5 years earlier. The sarcoma extended from the nasopharynx into the floor of the pituitary fossa and into both parasellar regions. There was no clinical evidence of any distant spread. Many of the malignant cells contained cytoplasmic granular and globular PAS-positive inclusions shown to be alpha-1-antitrypsin immunohistochemically. Ultrastructurally, this probably corresponded to electron-dense material with distinctive patterns and which had accumulated within distended ergastoplasmic cisternae of the neoplastic cells. Three previously reported case of postirradiation sarcomas arising in nasopharyngeal angiofibroma were said to be fibrosarcomas and none produced alpha-1-antitrypsin.

  18. Malignant fat-forming solitary fibrous tumor (lipomatous hemangiopericytoma) in the neck: Imaging and histopathological findings of a case.

    PubMed

    de Carvalho, Alice Duarte; Abrahão-Machado, Lucas Faria; Viana, Cristiano Ribeiro; de Castro Capuzzo, Renato; Mamere, Augusto Elias

    2013-03-01

    Fat-forming solitary fibrous tumor (SFT) is a rare variant of solitary fibrous tumor, a mesenchymal fibroblastic neoplasia with a particular branching hypervascular pattern. This tumor is usually classified as benign and only very few fat-forming SFTs with malignant histologic features have been reported. We report a histologically malignant fat-forming solitary fibrous tumor in a 61-year-old man, located in his neck. Ultrasonography examination was first performed showing a heterogeneous lesion, predominantly hyperechoic, with sound beam attenuation, containing two hypoechoic solid nodules. Magnetic resonance imaging and computed tomography examinations demonstrated a heterogeneous and predominantly adipose mass, containing post contrast enhancing solid nodules and thin septations. Treatment consisted of total removal of the lesion. Histologically, the tumor showed hypercellularity, numerous mitoses and cytological atypia, fulfilling the criteria for malignancy. The patient had no metastasis. This rare tumor may be confused with other fat-containing lesions on imaging examinations, mainly liposarcoma.

  19. Inflammatory Malignant Fibrous Histiocytoma Associated with Leukemoid Reaction or Leukocytosis: A Comprehensive Review

    PubMed Central

    Hurtado-Cordovi, Jorge; Pathak, Prajwol; Avezbakiyev, Boris; Frieri, Marianne

    2012-01-01

    Inflammatory malignant fibrous histiocytoma (IMFH) associated with leukemoid reaction (LR)/leukocytosis is a rare entity. In this paper, we search PubMed for all known cases of IMFH associated with LR/leukocytosis in an attempt to draw conclusions about this variant's response to treatments and its pathophysiology. Medline electronic database was searched using key words such as malignant fibrous histiocytoma, leukemoid reaction, and leukocytosis. A total of 16 patients were found, twelve males (75%) and 4 female (25%), with a mean age of 62.6 years, ranging from 47 to 77. The mean survival was 770 days, ranging from 14 to 6570 days. Four patients were alive at last follow-up: 6570 days, 1095 days, 335 days, and 180 days, respectively. Of the 12 patients that expired, death occurred approximately 92 days after the onset of LR or leukocytosis, ranging from 3 to 334 days. We conclude that IMFH associated with LR/leukocytosis does not completely respond to chemoradiation. Overproduction of growth factors and cytokines by IMFH cells and their interactions with the inflammatory infiltrate seem to promote immunological effector cell's dysfunction and substantiate the development and growth of this neoplasm. A clear understanding of these molecular pathways is crucial in order to identify targets for potential therapy. PMID:23097722

  20. Advanced malignant solitary fibrous tumor in pelvis responding to radiation therapy.

    PubMed

    Kawamura, Shinobu; Nakamura, Takafumi; Oya, Takeshi; Ishizawa, Shin; Sakai, Yuta; Tanaka, Tomonori; Saito, Shigeru; Fukuoka, Junya

    2007-04-01

    Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm that is benign in most cases. Although SFT was first recognized to arise only in the pleura, recent reports indicate that SFT can involve a wide range of anatomical sites. To date, 17 cases of pelvic SFT have been reported. Herein is reported a case of a 74-year-old woman with a giant malignant SFT in the pelvis. Along with massive invasion to adjacent organs and multiple lung metastases detected on radiography, biopsy from the tumor through the vaginal wall showed malignant looking spindle-cell neoplasm with increased cellularity, areas of necrosis, and high mitotic activity (5/10 high-power fields). Immunohistochemically, the tumor cells were diffusely and strongly positive for CD34, CD99, and bcl-2. Based on pathological features and clinical presentation, diagnosis of malignant SFT was made. The patient received systemic and the intra-arterial chemotherapy followed by whole pelvic radiation therapy (50 Gy). Initial chemotherapies failed to control the tumor. Afterwards, improvement was observed radiologically and pathologically in the 12 months' follow up after the radiation therapy. This is the first report related to therapeutic remarks on advanced malignant SFT.

  1. Primary hepatic solitary fibrous tumor with histologically benign and malignant areas.

    PubMed

    Silvanto, Anna; Karanjia, Nariman D; Bagwan, Izhar N

    2015-12-01

    Extrapleural solitary fibrous tumor (SFT) is an uncommon mesenchymal neoplasm, presenting most commonly in the intrathoracic sites but which has been reported at numerous extrathoracic locations. The majority of intra-thoracic SFTs are benign, but 10%-15% behave aggressively. We report a case of primary hepatic SFT with histologically benign and malignant areas. A 65-year-old man underwent an abdominal CT scan following a cerebrovascular accident, which demonstrated a sharply demarcated large liver mass with a heterogenous enhancing area and occupying most of the left lobe of the liver. Histological examination following a hemihepatectomy showed an SFT with morphological patterns ranging from benign to malignant areas, including pleomorphism, increased cellularity, herringbone pattern, necrosis and a raised mitotic count. On review of the literature, only an occasional case report with malignant areas in a hepatic SFT was identified. This case highlights that SFT should be included in the differential diagnosis of a hepatic spindle cell lesion, and that on rare occasions, malignant areas can occur in this already uncommon neoplasm. PMID:26663016

  2. Primary hepatic solitary fibrous tumor with histologically benign and malignant areas.

    PubMed

    Silvanto, Anna; Karanjia, Nariman D; Bagwan, Izhar N

    2015-12-01

    Extrapleural solitary fibrous tumor (SFT) is an uncommon mesenchymal neoplasm, presenting most commonly in the intrathoracic sites but which has been reported at numerous extrathoracic locations. The majority of intra-thoracic SFTs are benign, but 10%-15% behave aggressively. We report a case of primary hepatic SFT with histologically benign and malignant areas. A 65-year-old man underwent an abdominal CT scan following a cerebrovascular accident, which demonstrated a sharply demarcated large liver mass with a heterogenous enhancing area and occupying most of the left lobe of the liver. Histological examination following a hemihepatectomy showed an SFT with morphological patterns ranging from benign to malignant areas, including pleomorphism, increased cellularity, herringbone pattern, necrosis and a raised mitotic count. On review of the literature, only an occasional case report with malignant areas in a hepatic SFT was identified. This case highlights that SFT should be included in the differential diagnosis of a hepatic spindle cell lesion, and that on rare occasions, malignant areas can occur in this already uncommon neoplasm.

  3. Malignant solitary fibrous tumor of tandem lesions in the skull and spine.

    PubMed

    Son, Seong; Lee, Sang-Gu; Jeong, Dong-Hae; Yoo, Chan Jong

    2013-09-01

    A Solitary Fibrous Tumor (Sft) Is A Rare Neoplasm Originated From The Pleura, But They Can Occur In A Variety Of Extrathoracic Regions. Although Many Cases Of Primary Sft Have Been Reported, There Are Extremely Rare Repots To Date Of A Malignant Sft In The Spine Or Skull. A 54-year-woman Visited Our Hospital Due To Low Back Pain And Both Leg Radiating Pain. Several Imaging Studies Including Magnetic Resonance Imaging And Computed Tomography Revealed Expansive Enhanced Lesions In The Occipital Bone, T8, S1-2, And Ilium, With Neural Tissue Compression. We Performed Surgical Resection Of The Tumor In Each Site, And Postoperative Radiosurgery And Chemotherapy Were Performed. However, After Six Months, Tumors Were Recurred And Metastasized In Multiple Regions Including Whole Spine And Lung. The Authors Report Here The First Case Of Patient With Malignant Sft Of Tandem Lesions In The Various Bony Structures, Including Skull, Thoracic Spine, And Sacral Spine, With A Rapid Recurrence And Metastasis. Although Malignant Sft Is Extremely Rare, It Should Be Considered In The Differential Diagnosis And Carful Follow-up Is Needed.

  4. Malignant fibrous histiocytoma of the breast. A case report and review of the literature

    SciTech Connect

    Langham, M.R. Jr.; Mills, A.S.; DeMay, R.M.; O'Dowd, G.J.; Grathwohl, M.A.; Horsley, J.S.

    1984-08-01

    The clinicopathologic features of a malignant fibrous histiocytoma arising in a female breast are presented. Seven cases have been previously reported. These may be divided into three subgroups based on history and pathologic findings. Three of the seven occurred in women without a history of other tumors or previous irradiation. One appeared with a cystosarcoma phyllodes, and three arose following radiation for carcinoma of the breast. The patient in this study had received no radiation. Treatment has been based on surgical resection; the roles of chemotherapy and radiation are uncertain. Although resection alone in the first subgroup has lead to no reported mortality, the small number of cases and the relatively short follow-up does not allow a meaningful assessment of the prognosis in this disease.

  5. Primary malignant fibrous histiocytoma of the breast: report of one case

    PubMed Central

    Liu, Caigang; Zhao, Zuowei; Zhang, Qingfu; Wu, Yunfei; Jin, Feng

    2013-01-01

    Seven years ago, a 48-year-old female patient discovered a painless tumor in her right breast simply by chance. In the next year, the tumor increased significantly in size. At this point, the patient received a tumor resection, which was misdiagnosed as a benign mesenchymal tissue-originated tumor. Unfortunately, the tumor recurred just 10 days after resection. We subsequently resected the recurrent lesion and confirmed primary breast malignant fibrous histiocytoma. The tumor began to exhibit an unprecedented, massive, and uncontrolled growth, ulcerating soon after the operation. Treatment of the patient was limited by time. After the patient received a cycle of chemotherapy, she died of cachexia with the emergence of multiorgan metastasis 2 months after the operation. PMID:23589696

  6. [Primary malignant solitary fibrous tumor/hemangiopericytoma of the parotid gland].

    PubMed

    Messa-Botero, Oscar Alberto; Romero-Rojas, Alfredo Ernesto; Chinchilla Olaya, Sandra Isabel; Díaz-Pérez, Julio Alexander; Tapias-Vargas, Luis Felipe

    2011-01-01

    Solitary fibrous tumor (SFT) was first described in the pleura by Lietaud in 1767; later in 1870, Wagner described the localized nature of this type of tumor and Klemperer and Rabin classified pleural tumors into two types: diffuse mesotheliomas and localized mesotheliomas. Recent years have seen the redefinition of this neoplasm, due to better technology; it is now proven that this neoplasm may have multiple different extrapleural origins including the head and neck regions. This diversity of locations is related to the particular mesenchymal histogenesis of SFT which allows its development from very unusual sites such as the salivary glands (SGs). In this particular site, this neoplasm is very infrequent and most of reported cases refer to benign disease, with just one case informed so far of primary malignant SFT.

  7. Cutaneous Inflammatory Malignant Fibrous Histiocytoma Presenting with a Leukemoid Reaction: A Case Report and Review of the Literature

    PubMed Central

    Hurtado-Cordovi, Jorge; Avezbakiyev, Boris; Frieri, Marianne; Freedman, Lester; Gebre, Wondwoosen

    2012-01-01

    Malignant fibrous histiocytoma (MFH) is the most common sarcoma found in adults. We discuss a case of inflammatory MFH of dermal/epidermal origin presenting with a severe leukemoid reaction (LR). A 60 years old white male presented to hematology/oncology clinic complaining of mild shortness of breath on exertion. Past medical history was remarkable for removal of a left upper extremity necrotic mass 4.4 × 3 × 3 cm. Microscopy of the specimen showed clear surgical margin, and tumor cells restricted to the dermis without lymphovascular invasion. Immunohistochemestry was positive for CD 68 and CD 99. Chest x-ray was negative for metastatic disease. White blood cell count was 109.4 k/mm3 with 24 k/mm3 band neutrophils, and absolute neutrophil count of 69 k/mm3. CT scan of the thorax revealed numerous bilateral pulmonary nodules suspicious for metastasis. Based on these findings patient was diagnosed with metastatic cutaneous IMFH associated with a LR. Following review of medical literature, this appears to be the first reported case of inflammatory cutaneous MFH associated with LR. This histological variant is rare, and carries a poor prognosis. Thus, we would like to emphasize the need for investigating alternative therapies capable of improving the survival of these patients. PMID:22844297

  8. Loss of CD34 and high IGF2 are associated with malignant transformation in solitary fibrous tumors.

    PubMed

    Schulz, Birte; Altendorf-Hofmann, Annelore; Kirchner, Thomas; Katenkamp, Detlef; Petersen, Iver; Knösel, Thomas

    2014-02-01

    The aim of this study was to characterize the subgroups of solitary fibrous tumor (SFT) and to investigate the expression of different biomarkers including CD34 and IGF2 in malignant transformation. Two hundred and ninety-four (294) SFTs from a single German consultation center of soft tissue tumors were categorized into the new proposal of SFT designation. We found the fibrous variant in 223 (75.9%), the cellular variant in 65 (22.1%), the fat forming variant in 4 (1.4%), and the giant cell-rich variant in 2 (0.6%) cases. Anatomical location, size, mitotic index, necrosis, cellularity, collagenous ropes, and growth pattern of the vessels were recorded. Criteria of malignancy were found in 68 (23%) tumors. Expression of IGF2, IGF1R, CD34, BCL2, CD99, SMA, S100, PanCK, and Ki67 was analyzed immunohistochemically. Low expression of CD34 and high expression of IGF2 were significantly associated with malignant transformation and the metastatic rate. Moreover the presence of necrosis showed the most significant p-value (p<0.004). Of all SFTs, the fibrous variant is the most common, followed by the cellular variant. The fat-forming and giant cell-rich variants are very rare. Low expression of CD34 and high expression of IGF2 are significantly associated with malignant transformation, and might be an interesting target of individualized therapy.

  9. Solitary fibrous tumor of the pleura: apparent diffusion coefficient (ADC) value and ADC map to predict malignant transformation.

    PubMed

    Inaoka, Tsutomu; Takahashi, Koji; Miyokawa, Naoyuki; Ohsaki, Yoshinobu; Aburano, Tamio

    2007-07-01

    Solitary fibrous tumors (SFTs) of the pleura are rare soft-tissue tumors that are presumed to be of mesenchymal origin. Most SFTs are histologically benign, but up to 20% of SFTs may be malignant. In addition, malignant transformation may occur within histologically benign SFTs, though it is rare. However, it is difficult to diagnose malignant SFTs of the pleura by means of conventional computed tomography and magnetic resonance imaging (MRI). In this article we present the first case of malignant SFT of the pleura in an 81-year-old man in which the apparent diffusion coefficient (ADC) value and ADC map based on diffusion-weighted MRI were very useful for identifying malignant transformation.

  10. Abdominal malignant fibrous histiocytoma infiltrating stomach with Chilaiditi's sign manifestation (a rare case report).

    PubMed

    Nurdjanah, Siti; Bayupurnama, Putut; Maduseno, Sutanto; Ratnasari, Neneng

    2007-01-01

    The incidence and mortality rate of gastric cancer are decreasing within last 60 years. Approximately 85% of gastric cancer is carcinoma where as the rest 15% is lymphoma and sarcoma. Leiomyosarcoma is the most frequently encountered sarcoma in stomach and represents 1-3% gastric cancer. This tumor is frequently located in the anterior and posterior wall of gastric fundus accompanied by ulceration and bleeding. Malignant fibrous histiocytoma (MFH) is a soft tissue tumor with poor prognosis, rarely found in stomach. This tumor is most frequently originated from inner fascia or skeletal muscle (58-75%) and most frequently happen in the peritoneal cavity and abdomen (9-16 %). We reported a 45-year-old woman with abdominal MFH which infiltrated to the lesser curvature of stomach with Chilaiditi sign. Chilaiditi sign is a condition of intestine disposition (usually at the hepatic flexure of colon) which lies between liver and diagfragma, found in 0.1 ? 0.25% cases which is diagnosed by chest X Ray. This case is a very rare case.

  11. The RB1 gene is the target of chromosome 13 deletions in malignant fibrous histiocytoma.

    PubMed

    Chibon, F; Mairal, A; Fréneaux, P; Terrier, P; Coindre, J M; Sastre, X; Aurias, A

    2000-11-15

    Forty-four malignant fibrous histiocytomas (MFHs) were studied by comparative genomic hybridization. Among the observed imbalances, losses of the 13q14-q21 region were observed in almost all tumors (78%), suggesting that a gene localized in this region could act as a tumor suppressor gene and that its inactivation could be relevant for MFH oncogenesis and/or progression. We determined by CA repeat analyses a consensus region of deletion focusing on the RB1 region. The RB1 gene was then analyzed by protein truncation test, direct sequencing, fluorescence in situ hybridization, Southern blotting, and immunohistochemistry. RB1 mutations and/or homozygous deletions were found in 7 of the 34 tumors analyzed (20%). Among the 35 tumors with comparative genomic hybridization imbalances analyzed by immunohistochemistry, 30 (86%) did not exhibit significant nuclear labeling. The high correlation between chromosome 13 losses and absence of RB1 protein expression and the mutations detected strongly suggest that RB1 gene inactivation is a pivotal event in MFH oncogenesis. Moreover, the observation of a high incidence of MFH in patients previously treated for hereditary retinoblastoma fits well this hypothesis. PMID:11103795

  12. Malignant Fibrous Histiocytoma of the Maxillary Sinus in a Spray Painter from an Automobile Repair Shop

    PubMed Central

    2013-01-01

    Background We report a case of a spray painter who developed malignant fibrous histiocytoma (MFH) of the maxillary sinus following long-term exposure to chromium, nickel, and formaldehyde, implying that these agents are probable causal agents of MFH. Case report The patient developed right-sided prosopalgia that began twenty months ago. The symptom persisted despite medical treatment. After two months, he was diagnosed with MFH through imaging studies, surgery, and pathological microscopic findings at a university hospital in Seoul. His social, medical, and family history was unremarkable. The patient had worked for about 18 years at an automobile repair shop as a spray painter. During this period, he had been exposed to various occupational agents, such as hexavalent chromium, nickel, and formaldehyde, without appropriate personal protective equipment. He painted 6 days a week and worked for about 8 hours a day. Investigation of the patient’s work environment detected hexavalent chromium, chromate, nickel, and formaldehyde. Conclusions The study revealed that the patient had been exposed to hexavalent chromium, formaldehyde, and nickel compounds through sanding and spray painting. The association between paranasal cancer and exposure to the aforementioned occupational human carcinogens has been established. We suggest, in this case, the possibility that the paint spraying acted as a causal agent for paranasal cancer. PMID:24472378

  13. Malignant solitary fibrous tumor of the thyroid gland: report of a case and review of the literature.

    PubMed

    Ning, Shanglei; Song, Xie; Xiang, Lei; Chen, Yuxin; Cheng, Yu; Chen, Hongqiang

    2011-09-01

    Solitary fibrous tumors of the thyroid gland (T-SFT) are rarely described, with only 21 cases being reported in the English literatures, all showing benign clinical characteristics. We herein present a 76-year-old woman presenting with a 3-month history of rapidly enlarging neck masses and the CT showed masses with partial calcification in the right thyroid lobe. We performed right hemithyroidectomy and isthmectomy with negative margin under general anesthesia. Histologically, the masses consisted of pleomorphic spindle cells with high mitoses and collagen bands. Immunohistochemically, the tumor cells showed positive reactions for CD34, vimentin and bcl-2, then a diagnosis of malignant solitary fibrous tumor of the right thyroid was made. Six months postoperatively, the CT showed the recurrence of the thyroid tumor and the presence of many nodules of varying sizes throughout bilateral pulmonary lobes. To our knowledge, this is the first case of malignant solitary fibrous tumor of the thyroid gland (T-SFT) with local recurrence and pulmonary metastasis and T-SFT must be considered in the differential diagnosis of spindle cells lesions in the thyroid gland. Correct diagnosis of the malignant T-SFT plays an important role in choosing appropriate therapeutic strategies and long-term follow-up is also extremely essential for these patients.

  14. Myxoid malignant fibrous histiocytoma and pleomorphic liposarcoma share very similar genomic imbalances.

    PubMed

    Idbaih, Ahmed; Coindre, Jean-Michel; Derré, Josette; Mariani, Odette; Terrier, Philippe; Ranchère, Dominique; Mairal, Aline; Aurias, Alain

    2005-02-01

    Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma. Nevertheless, the validity of this heterogeneous pathological entity has been recurrently questioned by pathologists. Recently, analyses by comparative genomic hybridization (CGH) of a large series of MFHs suggested that MFHs consist of morphologic modulation of other poorly differentiated sarcomas like leiomyosarcomas (LMS) or dedifferentiated liposarcomas (DLPS). We report here an analysis by CGH of 22 myxoid MFHs (mMFH), one of the five histological subtypes of MFH, and of nine pleomorphic liposarcomas (pLPS), a rare poorly differentiated LPS. The chromosome imbalances encountered in the series of mMFH were very similar to those observed in the series of pLPS studied in the laboratory and in the series of 14 pLPS published in the literature. The most frequent gains involved chromosome subregions: pericentromeric regions of 1, 5p, 19p, 19q and 20q. Losses found in the chromosomal arms 1q, 2q, 3p, 4q, 10q, 11q and 13q were also recurrent. The use of a clustering software did not separate the two pathological groups (mMFH and pLPS) on the basis of genomic data. Moreover, pLPS-mMFH represented, according to the clustering software results, an entity clearly distinguished from other soft tissue sarcomas, LMS in particular, with which they share common genetic aberrations. Additional studies are needed to identify genes targeted by these genomic aberrations, and implicated in the oncogenesis of these tumor subtypes. The characterization of common gene alterations in both tumor groups would suggest a closer relationship between these two types of soft tissue sarcomas. PMID:15702084

  15. Malignant fat-forming solitary fibrous tumor (so-called "lipomatous hemangiopericytoma"): clinicopathologic analysis of 14 cases.

    PubMed

    Lee, Jen-Chieh; Fletcher, Christopher D M

    2011-08-01

    Fat-forming solitary fibrous tumor is a rare variant of solitary fibrous tumor (SFT). Generally regarded as benign, very few fat-forming SFTs with malignant histologic features have been reported. Here, we report 14 histologically malignant fat-forming SFTs to better characterize this subset. Seven patients were female and 7 were male, with ages ranging 20 to 93 years (median, 57 y). Five tumors were located in the lower limb, 3 in the trunk, 3 in abdominopelvic locations, 2 in the head and neck region, and 1 in the upper limb. The tumor size ranged from 3.4 to 20 cm (median, 8.6 cm). Histologically, all exhibited at least focal hypercellularity; 12 tumors had mitoses >4/10 high-power fields (range, 2 to 37; median, 8), 12 showed at least moderate atypia, and 8 showed necrosis. It should be noted that 7 tumors contained only mature adipose tissue, whereas 5 contained multivacuolated lipoblasts and 2 had areas resembling atypical lipomatous tumor (ALT). Immunohistochemically, CD34 and CD99 were positive in most cases (11 of 14 and 8 of 10, respectively); MDM2 and CDK4 were both negative in all 4 cases tested (including both tumors with ALT-like areas). Follow-up data from 10 cases (median duration, 47.5 mo; range, 5 to 76) showed 2 patients with multiple metastases (both to lung and bones, and 1 each to breast and to soft tissue), both of whom died of disease. In conclusion, fat-forming SFTs exhibiting malignant histologic features have potential for aggressive behavior. The presence of lipoblasts and/or ALT-like areas, although described in some "benign" examples of fat-forming SFT, seems much more common in the malignant subset and may prompt a careful search for morphologic evidence of malignancy in any case of fat-forming SFT.

  16. Adult Ocular Toxocariasis Mimicking Ciliary Body Malignancy

    PubMed Central

    Mansour, Ahmad M.; Abiad, Bachir; Boulos, Fouad I.; Alameddine, Ramzi; Maalouf, Fadi C.; Bu Ghannam, Alaa; Hamam, Rola N.

    2014-01-01

    Purpose. To discuss an unusual presentation of ocular toxocariasis. Methods. Case report. Results. A 40-year-old woman presented with decreased vision in the left eye with a long history of recurrent red eye from uveitis. Eosinophilia and positive ELISA titers for Toxocara canis favored the diagnosis of ocular toxocariasis. Over 3 months, an anterior scleral mass had a rapid growth raising the possibility of medulloepithelioma, which rarely can mimic uveitic syndromes. Surgical plan changed from local excision to enucleation. Histopathology demonstrated a large homogeneous mass of chronic inflammatory cells with inflammation of the overlying thinned out sclera, medial rectus insertion, and limbal cornea. The triad of peripheral granuloma, eosinophilia, and positive blood serology established the diagnosis of ocular toxocariasis. Conclusions. Ocular toxocariasis can mimic ocular malignancy such as medulloepithelioma in adults and rarely presents as an anterior scleral mass. PMID:25371681

  17. Adult Celiac Disease and Its Malignant Complications

    PubMed Central

    2009-01-01

    Adult celiac disease is a chronic intestinal disorder that has been estimated to affect up to 1-2% of the population in some nations. Awareness of the disease has increased, but still it remains markedly underdiagnosed. Celiac disease is a pathologically defined condition with several characteristic clinical scenarios that should lead the clinician to suspect its presence. Critical to diagnosis is a documented responsiveness to a gluten-free diet. After diagnosis and treatment, symptoms and biopsy-proven changes may recur and appear refractory to a gluten-free diet. Recurrent symptoms are most often due to poor diet compliance, a ubiquitous and unrecognized gluten source, an initially incorrect diagnosis, or an associated disease or complication of celiac disease. Some patients with persistent symptoms and biopsy-proven changes may not have celiac disease at all, instead suffering from a sprue-like intestinal disease, so-called unclassified sprue, which is a specific entity that does not appear to respond to a gluten-free diet. Some of these patients eventually prove to have an underlying malignant cause, particularly lymphoma. The risk of developing lymphoma and other malignancies is increased in celiac disease, especially if initially diagnosed in the elderly, or late in the clinical course of the disease. However, recent studies suggest that the risk of gastric and colon cancer is low. This has led to the hypothesis that untreated celiac disease may be protective, possibly due to impaired absorption and more rapid excretion of fat or fat-soluble agents, including hydrocarbons and other putative cocarcinogens, which are implicated in the pathogenesis of colorectal cancer. PMID:20431755

  18. The solitary fibrous malignant tumour of the kidney: clinical and pathological considerations on a case revisiting the literature.

    PubMed

    Marzi, M; Piras, P; D'Alpaos, M; Paiusco, A; Canessa, S; Minervini, M S; Di Zitti, P

    2011-03-01

    The solitary fibrous tumours (SFT) are rare spindle cell neoplasms which generally originate from the pleura; also described are cases of SFT in other locations, included the genital-urinary tract. Described in the ambit the kidney are 19 cases of SFT and such rarity of localisation makes rather unknown the histogenesis and the prognosis of the lesion. We report the case of a 72 year old lady who attended our Unit for a mass which was clinically palpable at the level of the left hemiabdomen. Following an abdominal ultrasound scan a neoformation was highlighted which a successive tomodensitographic test indicated as being of likely pertinence of the middle third of the left kidney; the mass had a diameter of approximately 19 cm. A radical nephrectomy has been conducted. The histological examen highlighted a solitary fibrous tumour: the presence of hypercellularity, of cellular pleiomorphism and of a high number of mitosis has led to a histopathological diagnosis of malignancy of the neoplasm under examination. Departing from this case a review of the literature is carried out. The SFT of the kidney can have an aggressive character and more the present has hystopathological characters and clinical results are still rather unknown.

  19. Histologically malignant solitary fibrous tumour of the anterior thoracic wall: a case report and review of the literature.

    PubMed

    Archontaki, Maria; Korkolis, Dimitris P; Arnogiannaki, Niki; Hatzijiannis, Stelios; Dendrinos, Panagiotis; Megapanos, Christos; Kassotakis, Dimitris; Kokkalis, Georgios

    2010-01-01

    Solitary fibrous tumour (SFT) is a rare oncological entity that most often arises in the pleura. Over the past 10 years, the tumour has been described at numerous extrapleural locations. We present the case of a 42-year-old female Caucasian patient with an extrapleural SFT located at the anterior thoracic wall for 22 years, with atypical histological characteristics and clinical features of malignancy. Management consisted of a wide surgical resection, plastic reconstruction, and postoperative radiotherapy. Although extrapleural SFT usually behaves as a benign soft tissue tumour, it can also present with a more aggressive local behavior, including locoregional recurrence or metastasis. In that case, a multidisciplinary approach is required for accurate diagnosis and proper management.

  20. Mediastinal epithelioid hemangioendothelioma with abundant spindle cells and osteoclast-like giant cells mimicking malignant fibrous histiocytoma

    PubMed Central

    2013-01-01

    Abstract Epithelioid hemangioendothelioma is a relatively uncommon lesion usually presenting in soft tissues. The occurrence in the mediastinum is exceptional rare. Histologically, this tumor is characterized by epithelioid cells with intracytoplasmic vacuoles in a hyalinized or mucinous stroma. Occasionally, spindle cells or osteoclast-like giant cells can be observed. Herein, we present a case of epithelioid hemangioendothelioma in a 38 year-old Chinese male. The tumor was predominantly composed of abundant spindle cells with marked atypia and scattered osteoclast-like giant cells reminiscent of malignant fibrous histiocytoma. The unusual histological appearance can pose a great diagnostic challenge. It may be easily misdiagnosed, especially if the specimen is limited or from fine-needle aspiration. Virtual slides http://www.diagnosticpathology.diagnomx.eu/vs/5804918529726307 PMID:23800015

  1. Postoperative Radiotherapy for the Treatment of Solitary Fibrous Tumor With Malignant Transformation of the Pelvic: A Rare Case Report With Literature Review.

    PubMed

    Gao, Chao; Zhang, Yong; Jing, Ming; Qu, Wei; Li, Jia; Zhao, Xiang-Rong; Yu, Yong-Hua

    2016-01-01

    Solitary fibrous tumor of the pelvic is an uncommon neoplasm with nonspecific symptoms. Reports of malignant transformation are especially rare. We report a case of solitary fibrous tumor in pelvic. A unique feature of our case compared with previously reported is that this patient relapsed with malignant transformation and had significant response to radiotherapy. The patient was initially treated with surgery, followed by postoperative dimensional conformal intensity modulated radiation therapy (dynamic MLC VRIAN 23EX Linac, inversely optimized by the Eclipse system) to provide a radical cure for residual tumor.In this case, there were no signs of recurrence after six and a half years of further follow-up, indicating that postoperation radiotherapy may be an effective treatment for SFT with malignant transformation in pelvic.

  2. Postoperative Radiotherapy for the Treatment of Solitary Fibrous Tumor With Malignant Transformation of the Pelvic: A Rare Case Report With Literature Review.

    PubMed

    Gao, Chao; Zhang, Yong; Jing, Ming; Qu, Wei; Li, Jia; Zhao, Xiang-Rong; Yu, Yong-Hua

    2016-01-01

    Solitary fibrous tumor of the pelvic is an uncommon neoplasm with nonspecific symptoms. Reports of malignant transformation are especially rare. We report a case of solitary fibrous tumor in pelvic. A unique feature of our case compared with previously reported is that this patient relapsed with malignant transformation and had significant response to radiotherapy. The patient was initially treated with surgery, followed by postoperative dimensional conformal intensity modulated radiation therapy (dynamic MLC VRIAN 23EX Linac, inversely optimized by the Eclipse system) to provide a radical cure for residual tumor.In this case, there were no signs of recurrence after six and a half years of further follow-up, indicating that postoperation radiotherapy may be an effective treatment for SFT with malignant transformation in pelvic. PMID:26765426

  3. Malignant solitary fibrous tumor with high-grade nuclear atypia: an alternate entity for the undetermined tumor group.

    PubMed

    Yamada, Yuichi; Kohashi, Kenichi; Bekki, Hirofumi; Ishii, Takeaki; Iura, Kunio; Maekawa, Akira; Yamamoto, Hidetaka; Iwamoto, Yukihide; Oda, Yoshinao

    2015-02-01

    Recently, a novel fusion transcript, NAB2-STAT6, and its variants have also been reported to be specific diagnostic markers for solitary fibrous tumors (SFTs). In this study, we validated the existence of the NAB2-STAT6 fusion gene in SFTs and examined its relation with the pathological features. Frozen samples from 9 tumors were assessed for fusion gene. The detected fusion genes exhibited large intron sequences and the insertion of unknown and previously unreported sequences. The fusion genes were not detected in the 2 malignant cases with high-grade nuclear atypia, nuclear pleomorphism and necrosis, that was confirmed by multiplex PCR method. In addition, 1 of the 2 NAB2-STAT6 fusion gene-negative tumors showed amplification of the MDM2 and CDK4 genes. It was suggested that a certain proportion of tumors previously diagnosed as malignant SFTs with high-grade nuclear atypia lacking NAB2-STAT6 should be categorized into a special subtype of SFT, which is genetically different from conventional SFTs, and which cannot be apparently distinguished from dedifferentiated liposarcoma or undifferentiated pleomorphic sarcoma.

  4. Malignant fibrous tumor of the pleura: case report and literature review.

    PubMed

    Toelen, C; Deleersnijder, R; Thomas, B

    2012-01-01

    Solitary fibrous tumours (SFT) of the pleura are uncommon and are incidental findings or discovered in patients with non-specific respiratory symptoms. We report a case of a 74 year old man diagnosed with a mesenchymal pleural neoplasm, associated with typical hypertrophic osteoarthropathy, referred to as Pierre-Marie-Bamberg syndrome. As reported in the literature, complete surgical resection is the gold standard for treatment of such lesions and recurrences. Radiotherapy and chemotherapy are of limited value in the curative treatment of pleural SFT. In our case surgical excision of the mass was performed. After a disease-free period of 3 years a second intervention was necessary because of recurrence. Until now our patient is free of complaints and no signs of reappearance were noted. Based on our experience and on literature findings we would like to underline the importance of regular long-term follow-up because of the substantial risk of recurrence.

  5. Pleomorphic malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma of the scrotum in a patient presenting as fournier gangrene: a case report.

    PubMed

    Guo, Juan; Zhou, Shengmei; Rao, Nagesh P; Pez, Gholam H

    2010-10-01

    Pleomorphic malignant fibrous histiocytoma (MFH), also known as undifferentiated high-grade pleomorphic sarcoma according to the latest World Health Organization classification, is a diagnosis of exclusion and extremely rare in adult scrotal/paratesticular region. Clinical presentation of scrotal/paratesticular pleomorphic MFH is usually a painless and gradual scrotal swelling. We report a case of scrotal MFH in a 63-year-old man who presented as Fournier gangrene after 10-month painful scrotal swelling and multiple procedures. The specimen of emergent debridement was submitted for pathologic and bacteriologic examination. Microscopically, the lesion had marked architectural and cytologic pleomorphism. The neoplastic cells were positive for vimentin, but negative for all lineage-specific markers. Fluorescence in-situ hybridization showed an aneuploid karyotype and negative results for lipomatous tumor abnormalities. Bacterial cultures of the specimen showed extensive growth of virulent polymicrobes. The diagnosis of scrotal/paratesticular pleomorphic MFH with concurrent Fournier gangrene was made. Thoracic computed tomography scan showed bilateral multiple pulmonary nodules. The patient died 1 month later. PMID:20505510

  6. Sorafenib in patients with progressive malignant solitary fibrous tumors: a subgroup analysis from a phase II study of the French Sarcoma Group (GSF/GETO).

    PubMed

    Valentin, T; Fournier, C; Penel, N; Bompas, E; Chaigneau, L; Isambert, N; Chevreau, C

    2013-12-01

    Malignant solitary fibrous tumors are rare soft-tissue sarcomas. They are considered as low-grade malignancies, but may display metastatic potential in 20% of the cases. In case of metastatic or locally advanced, unresectable disease, standard treatments, like anthracycline-based regimens, are poorly effective. Previous studies suggested that antiangiogenic drugs, such as sorafenib, could be efficient to treat vascular sarcomas and solitary fibrous tumors. Five patients with progressive SFT were included in this phase 2 study, and treated with sorafenib at a dose of 800 mg daily. Two patients out of the five achieved a 9 months disease control with sorafenib, while their disease had progressed within the month preceding their inclusion. Consequently, our data suggest a potential efficacy of sorafenib in SFT, Further investigation is needed to confirm these data.

  7. Malignant fibrous histiocytoma of visceral organs: clinicopathologic features and diagnostic value of ezrin and HMG-CoA reductase

    PubMed Central

    Gu, Jinyang; Zhang, Shu; Wu, Xingyu; Shi, Jiong; Zhang, Bin; Zhang, Xiaoqi; Yang, Jun; Obulkasim, Halmurat; Duan, Fei; Deng, Chao; He, Jing; Zou, Xiaoping; Ding, Yitao

    2015-01-01

    Malignant fibrous histiocytoma (MFH) of the breast and visceral organs is extremely rare. There is an incomplete understanding of the clinical pathology of the primary MFH originating from the breast and visceral organs, especially in comparison with other soft tissue sarcomas. As a consequence we searched and analyzed the clinical and pathological records of all the nine patients with diagnosed breast and visceral MFH in our hospital. Immunohistochemical staining was performed for ezrin and HMG-CoA reductase in these MFH cases and relevant mesenchymal sarcomas. The 9 MFH cases presented with nonspecific symptoms and imaging manifestations. 6 cases were classified as storiform-pleomorphic MFH, 2 cases as inflammatory MFH, and the remaining 1 case as giant cell MFH. The results showed that ezrin expression, as well as HMG-CoA reductase expression, was significantly stronger in MFH cases than other non-MFH sarcomas. Poor prognosis seemed to be associated with younger age. Certain characteristics and clinicopathologic features can help us making the diagnosis of MFH. In conclusion, our study provided the potential value of ezrin and HMG-CoA reductase for diagnosis and differential diagnosis of MFH located in the breast and visceral organs. More accurate prognostic information of this rare disease needed to be further investigated. PMID:26045796

  8. Malignant solitary fibrous tumor of the kidney: report of a case and comprehensive review of the literature.

    PubMed

    Fine, Samson W; McCarthy, Denis M; Chan, Theresa Y; Epstein, Jonathan I; Argani, Pedram

    2006-06-01

    Renal solitary fibrous tumors (SFTs) have been reported infrequently. We report a 76-year-old man with a left renal mass that had previously been shown radiographically to be stable, but was now growing. Grossly, the mass measured 12 cm, was poorly circumscribed, and invaded beyond the renal capsule. Approximately 10% of the neoplasm consisted of haphazardly arranged spindle cells admixed with dense collagenous bands, which is typical of benign SFT. However, the remainder of the mass was composed of pleomorphic, spindled sarcoma cells with frequent mitoses and foci of necrosis. Immunohistochemically, we observed CD34 labeling in the benign SFT component with loss of expression in the sarcomatous component, focal labeling for Bcl-2 protein in both areas, and absence of labeling for cytokeratin, renal cell carcinoma marker, S100 protein, CD117, and muscle markers in both areas. To our knowledge, this is the first reported case of malignant renal SFT, likely representing transformation from a histologically documented benign SFT component.

  9. [Malignant solitary fibrous tumour of the kidney: report of a case and cumulative analysis of the literature].

    PubMed

    de Martino, M; Böhm, M; Klatte, T

    2012-01-01

    We report the case of a primary metastatic renal solitary fibrous tumour (SFT) and present a cumulative analysis of the literature. A 68-year-old woman presenting with a history of flank pain was diagnosed with a 7 cm renal mass. Further staging showed liver, lung and bone metastases. The patient underwent radical nephrectomy. Microscopically, the tumour consisted of pleomorphic, high-grade spindle cells with high mitotic activity, tumour necrosis and dense collagenous bands. Immunohistochemistry showed the strong expression of CD34 and vimentin, a weak expression of bcl-2 and CD99, and no expression of smooth muscle actin, desmin, S-100, pan-cytokeratin, and epithelial membrane antigen. These findings are consistent with an SFT. For the cumulative analysis, a total of 46 renal SFTs from 35 reports were analysed. Median age at the time of surgery was 52 years and 63% of the patients were female. Sixty-two percent of the tumours were symptomatic, most commonly with flank / back pain (24%). Median tumor size was 6.4 cm. Histologically, 91% of the SFTs were benign and 9% were malignant. One patient died of the disease, while 90% are alive without evidence of disease.

  10. CyberKnife radiotherapy for malignant fibrous histiocytoma of the chest wall: A case report and review of the literature

    PubMed Central

    WANG, ZHEN; WU, XIN-HU; LI, BING; KONG, QING-TAO; SHEN, ZE-TIAN; LI, JING; LIU, ZHI-BING; ZHU, XI-XU

    2014-01-01

    Malignant fibrous histiocytoma (MFH) is the most common type of soft tissue sarcoma, but rarely originates in the chest wall. Surgical resection is considered to be the most reliable treatment, however, no consensus has been reached concerning the best treatment for unresectable MFH. The current study presents the case of a 77-year-old male with MFH of the chest wall. The patient developed a painless mass and intermittent fever over a four-month period. A computed tomography scan demonstrated a large inhomogeneous lesion in the right chest wall, which was subsequently diagnosed via biopsy as a MFH. Since the tumor was an unresectable mass, CyberKnife® radiotherapy was conducted. Following the treatment, a marked reduction in the tumor size was observed with a tolerable level of toxicity. The sequencing analysis also revealed an in-frame deletion (delE746-A750) in exon 19 of the epidermal growth factor receptor gene. Based on this result, gefitinib was administered to the patient at a dose of 250 mg/day. PMID:24932251

  11. Haploidentical Stem Cell Transplantation in Adult Haematological Malignancies

    PubMed Central

    Parmesar, Kevon; Raj, Kavita

    2016-01-01

    Haematopoietic stem cell transplantation is a well-established treatment option for both hematological malignancies and nonmalignant conditions such as aplastic anemia and haemoglobinopathies. For those patients lacking a suitable matched sibling or matched unrelated donor, haploidentical donors are an alternative expedient donor pool. Historically, haploidentical transplantation led to high rates of graft rejection and GVHD. Strategies to circumvent these issues include T cell depletion and management of complications thereof or T replete transplants with GVHD prophylaxis. This review is an overview of these strategies and contemporaneous outcomes for hematological malignancies in adult haploidentical stem cell transplant recipients. PMID:27313619

  12. Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors.

    PubMed

    Vallat-Decouvelaere, A V; Dry, S M; Fletcher, C D

    1998-12-01

    Solitary fibrous tumor (SFT), first described as a pleural lesion, has been reported at numerous extrathoracic sites over the past 10 years. About 10% to 15% of intrathoracic SFTs are histologically or clinically malignant, but such cases have very rarely been described at other locations. Among 92 cases of extrathoracic SFT in our files, we identified 10 that either had recurred (2 cases) or had a least one atypical histologic feature (8 cases). The ten tumors occurred in five men and five women, 32 to 81 years old (median 56), measured 1.9 cm to 20 cm (median 11.5 cm), and were located in the abdomen/pelvis (4 cases), retroperitoneum (3 cases), groin, trunk, and upper arm. Nuclear atypia (8 cases), markedly increased cellularity (6 cases), areas of necrosis (4 cases), and greater than 4 mitoses/10 HPFs (3 cases) were seen in addition to the typical histologic features of SFT. Six tumors had at least two of these atypical histologic features. Nine cases were positive for CD34, six were positive for O-13, and one was focally positive for smooth muscle actin. Eight were excised completely. Subsequent follow-up revealed tumor relapse in eight cases (follow up 6-180 months, median 24). Four patients had local recurrence at 12 to 168 months. Distant metastasis developed at 1 to 6 years in five cases with spread to lung (2 cases), liver (4 cases), and bone. Metastasis or local recurrence developed within 2 years in five patients. To date, no patient has died of their tumor. These findings demonstrate that nuclear atypia, hypercellularity, greater than 4 mitoses/10 HPFs, and necrosis may be seen in up to 10% of extrathoracic SFTs, and are associated with, but are not by themselves predictive of, aggressive clinical behavior. In addition, our findings confirm that the behavior of extrathoracic SFTs is unpredictable, entirely comparable to that of their better known pleural counterparts, and confirm that patients with SFTs in all locations require careful, long-term follow

  13. Establishment of a human malignant fibrous histiocytoma cell line, COMA. Characterization By conventional cytogenetics, comparative genomic hybridization, and multiplex fluorescence In situ hybridization.

    PubMed

    Mairal, A; Chibon, F; Rousselet, A; Couturier, J; Terrier, P; Aurias, A

    2000-09-01

    The human COMA cell line has been established from a storiform pleomorphic malignant fibrous histiocytoma (MFH). As expected for this tumor type, a very complex karyotype was observed after R-banding analysis. An extensive analysis by 24-color painting, comparative genomic hybridization (CGH), and fluorescence in situ hybridization (FISH) was performed. Twelve complex marker chromosomes recurrently observed were clearly identified; among them, three were systematically present in all analyzed metaphases. Amplifications detected by CGH were refined by FISH with probes specific for various candidate loci. A significant aneuploidy and numerous micronuclei were observed, which could be related to the anomalies of centriole numbers detected in a proportion of cells. Such an analysis, performed on a series of MFH cell lines, would allow the delineation of the genomic alterations specific for the oncogenesis or progression of this complex tumor type or both. PMID:11063793

  14. Indium-111-labeled leukocyte and technetium-99m-sulfur colloid uptake by a malignant fibrous histiocytoma: Phagocytosis by tumor cells

    SciTech Connect

    Palestro, C.J.; Klein, M.; Kim, C.K.; Swyer, A.J.; Goldsmith, S.J. )

    1990-09-01

    Indium-111-labeled leukocyte imaging, performed on a patient with a calcified mass in the right thigh, demonstrated labeled leukocyte accumulation in this mass. Technetium-99m-sulfur colloid imaging was performed to differentiate labeled leukocyte uptake in heterotopic bone marrow from uptake in a focus of infection. Leukocyte and sulfur colloid images were virtually identical, and the study was interpreted as without evidence of infection. Excision of the mass revealed an angiomatoid malignant fibrous histiocytoma with metaplastic bone formation. While no marrow elements were present in either the tumor or the metaplastic bone, phagocytosis of leukocytes by tumor cells was identified. Phagocytosis of leukocytes by tumor cells may be another cause of white cell accumulation in uninfected neoplasms.

  15. Loss of chromosome 13 is the most frequent genomic imbalance in malignant fibrous histiocytomas. A comparative genomic hybridization analysis of a series of 30 cases.

    PubMed

    Mairal, A; Terrier, P; Chibon, F; Sastre, X; Lecesne, A; Aurias, A

    1999-06-01

    Regional chromosome localizations of DNA copy number imbalances were studied by comparative genomic hybridization in 30 malignant fibrous histiocytomas: 13 primary tumors (2 myxoid, 9 storiform pleomorphic, and 2 with more undifferentiated phenotype) and 17 local recurrences (2 myxoid, 11 storiform pleomorphic, and 4 with more undifferentiated phenotype). Abnormal comparative genomic hybridization (CGH) profiles were observed in 25 tumors (83%). The most frequent gains (ratio > 1.2) corresponded, by order of frequency, to entire Xp, and bands 1q21, 19q13.1, 19p13, 5p13-p14, 1p31, 17p, 18p, 20q, 1p35, 17q23, and 22q12. High levels of gains (ratio > 1.5) were recurrently detected for Xp (10 cases), and in bands 1q21-q22 (8 cases), 3q27 (4 cases), 5p13-p14 (3 cases), 13q32-q34 (3 cases), 15q22-q26 (3 cases), and 17p11-p12 (3 cases). Losses of 13q12-q14 or 13q21 were observed in a large proportion of tumors (17 cases), suggesting that a gene localized in this region could act as a tumor suppressor gene. Losses of 11q23, 2q32, 11p13, 10p, 1q4, 9p2, 16q12, 4q3, 10q25, 3p23, 2p24, and 12p were also recurrently observed. Taken together, these results provide an overview of chromosome imbalances present in MFH, which could be of use for diagnostic purposes. They point to various chromosome regions which may harbor genes important for malignant fibrous histiocytomas (MFH) oncogenesis and progression. PMID:10347550

  16. Paratesticular Fibrous Pseudotumors

    PubMed Central

    Turkan, Sadi; Kalkan, Mehmet; Ekmekcioglu, Ozan; Haltas, Hacer; Sahin, Coskun

    2016-01-01

    Paratesticular fibrous pseudotumors (PFPs) are rare pathologies with quite wide and variable topographic-morphological features. It is difficult to distinguish PFPs from malignant masses. Treatment can be done by resection of the mass. We reported a young patient’s findings about this rare pathology. PMID:27441080

  17. ASK1 (MAP3K5) as a potential therapeutic target in malignant fibrous histiocytomas with 12q14-q15 and 6q23 amplifications.

    PubMed

    Chibon, Frédéric; Mariani, Odette; Derré, Josette; Mairal, Aline; Coindre, Jean-Michel; Guillou, Louis; Sastre, Xavier; Pédeutour, Florence; Aurias, Alain

    2004-05-01

    Malignant fibrous histiocytomas (MFHs) are aggressive tumors without any definable line of differentiation. We recently demonstrated that about 20% of them are characterized by high-level amplifications of the 12q14-q15 chromosome region, associated with either 1p32 or 6q23 band amplification. This genetic finding, very similar to that in well-differentiated liposarcomas, strongly suggests that these tumors actually correspond to undifferentiated liposarcomas. It also suggests that the lack of differentiation could be the consequence of amplification of target genes localized in the 1p32 or 6q23 bands. We report here the characterization by array CGH of the 6q23 minimal region of amplification. Our findings demonstrate that amplification and overexpression of ASK1 (MAP3K5), a gene localized in the 6q23 band and encoding a mitogen-activated protein kinase kinase kinase of the JNK-MAPK signaling pathway, could inhibit the adipocytic differentiation process of the tumor cells. Treatment of a cell line with specific inhibitors of ASK1 protein resulted in the bypass of the differentiation block and induction of a strong adipocytic differentiation. These observations indicate that ASK1 is a target for new therapeutic management of these aggressive tumors. PMID:15034865

  18. Distinct clinicopathological features of NAB2-STAT6 fusion gene variants in solitary fibrous tumor with emphasis on the acquisition of highly malignant potential.

    PubMed

    Akaike, Keisuke; Kurisaki-Arakawa, Aiko; Hara, Kieko; Suehara, Yoshiyuki; Takagi, Tatsuya; Mitani, Keiko; Kaneko, Kazuo; Yao, Takashi; Saito, Tsuyoshi

    2015-03-01

    The impact of NGFI-A binding protein 2 (NAB2)-signal transducer and activator of transcription 6 (STAT6) fusion on the biological behavior and the mechanism of acquisition of malignant phenotype in solitary fibrous tumor (SFT) is not well understood. We examined variations of the NAB2-STAT6 fusion gene in 40 cases of SFT using formalin-fixed, paraffin-embedded tissues and secondary genetic alterations of tumor protein p53 (TP53),, platelet-derived growth factor receptor, β polypeptide (PDGFRB), and telomerase reverse transcriptase (TERT) promoters. These gene variations were compared with the clinicopathological features. The 2-year and 5-year disease-free survival rates (DFSRs) were 91% and 83%, respectively. All 40 samples demonstrated nuclear staining for STAT6, including CD34-negative cases. Moreover, p53-positive staining was associated with a lower DFSR and was significantly associated with higher Ki-67 label index, higher mitotic rate (mitosis, >4/high-power field), and the presence of nuclear atypia/pleomorphism. NAB2-STAT6 fusions were detected in all of the cases; the NAB2 exon 4-STAT6 exon 2, the most common genotype, appeared in 18 cases, which was associated with thoracic tumor location and the less aggressive phenotype. In contrast, tumors with NAB2 exon 6-STAT6 exon 16/18 demonstrated an aggressive phenotype. Mutations in TP53 and PDGFRB were detected in 2 and 3 cases respectively, and these occurred in a mutually exclusive fashion. TERT promoter hot spot mutations were observed in 5 cases, which were associated with shorter DFSR. Two dedifferentiated SFT cases harbored both TP53 and TERT promoter mutations. TP53 mutations, which result in its overexpression, in combination with TERT promoter mutations seem to play an important role in the dedifferentiation process.

  19. Leiomyosarcomas and most malignant fibrous histiocytomas share very similar comparative genomic hybridization imbalances: an analysis of a series of 27 leiomyosarcomas.

    PubMed

    Derré, J; Lagacé, R; Nicolas, A; Mairal, A; Chibon, F; Coindre, J M; Terrier, P; Sastre, X; Aurias, A

    2001-02-01

    Twenty-seven tumor samples with a diagnosis of leiomyosarcomas (LMS) were characterized by comparative genomic hybridization. The results were compared with immunohistochemical analysis of the smooth muscle profile of the tumors and expression of the RB1 gene protein. The comparative genomic hybridization profiles suggested that 7 of the 27 tumors might have been misclassified. High levels of DNA amplification were detected in 20 different small regions and recurrently involved bands 1p34, q21, 12q13-15, 17p, and 22q. Most recurrent simple gains were noted at sites such as 1p3, 1q21, 15q12-15, 16p, 17p and 17q, 19, 20q, 22q, and Xp. Significant losses of chromosome 13 were detected in 19 of the 27 tumors with a putative common region of loss in bands 13q14-21. Losses of chromosomes 1q, 2p and 2q, 4q, 9p, 10p and 10q, 11p and 11q23, and 16q were also highly recurrent. A comparative analysis between the most frequent genomic imbalances observed in this study of LMS and the genomic imbalances observed in a large proportion of malignant fibrous histiocytomas (MFH) from a previous study demonstrated that both types of tumors had similar recurrent imbalances. Although MFH were once thought to be a separate member of the soft tissue sarcoma family, our observations support the hypothesis that MFH are a morphologic modulation in the tumoral progression of other sarcomas, particularly LMS. PMID:11232643

  20. Interleukin-6/Soluble Interleukin-6 Receptor Signaling Attenuates Proliferation and Invasion, and Induces Morphological Changes of a Newly Established Pleomorphic Malignant Fibrous Histiocytoma Cell Line

    PubMed Central

    Nakanishi, Hirofumi; Yoshioka, Kiyoko; Joyama, Susumu; Araki, Nobuhito; Myoui, Akira; Ishiguro, Shingo; Ueda, Takafumi; Yoshikawa, Hideki; Itoh, Kazuyuki

    2004-01-01

    Pleomorphic malignant fibrous histiocytoma (MFH) is occasionally associated with inflammatory paraneoplastic syndrome (PNS). Recently, we reported that interleukin (IL)-6, one of the candidate cytokines, which induces such systemic inflammatory reaction, may be a tumor-associated factor involved in the pathogenesis and its clinical manifestations of MFH. In the local microenvironment, tumor-induced inflammatory reaction may play a role favoring tumor progression. To clarify the biological relevance of IL-6 in MFH, we established a human MFH cell line, named MIPS-2, derived from a resected specimen of a patient presenting with PNS. In this patient, the serum IL-6 level ran parallel to the disease course: elevated serum IL-6 concentration normalized immediately after radical surgery, and re-elevation occurred on tumor recurrence. MIPS-2 presented pleomorphic appearance, severe nuclear abnormalities with prominent nucleoli, and tumorigenesis in nude mice. MIPS-2 expressed IL-6, IL-6 receptor (IL-6R), and glycoprotein 130 (gp130) but lacked the soluble form of IL-6R (sIL-6R), as determined by flow cytometry and reverse transcriptase-polymerase chain reaction analyses. Stimulation of MIPS-2 with IL-6 combined with exogenous sIL-6R induced phosphorylation of both signal transducer and activator of transcription 3 (STAT3) and mitogen-activated protein kinase (MAPK), decreased cell proliferation, attenuated invasion, and induced morphological changes. Collectively, these data suggested that the IL-6/sIL-6R signaling pathway plays a pivotal role for proliferation, invasion, and morphology of MFH via STAT3 and MAPK pathway as autocrine and/or paracrine manner, and proposed the therapeutic potential for the use of both anti-growth factor and proinflammatory cytokine-targeting strategies to combat devastating MFH. PMID:15277221

  1. Fibrous dysplasia of the sphenoid sinus and skull base presents in an adult with localized temporal headache.

    PubMed

    Selmani, Ziane; Aitasalo, Kalle; Ashammakhi, Nureddin

    2004-03-01

    Fibrous dysplasia (FD) of the sphenoidal sinus is a rare disease, especially during adulthood. We report a case of FD of the right sphenoidal sinus in an adult male patient who presented with nonspecific symptoms limited to headache localized to the right temporal area and to the inferior orbital rim of both sides. Magnetic resonance imaging revealed a dense mass that occupied the entire right sphenoidal sinus and skull base with typical ground-glass opacification and bony sclerosis of the whole sphenoidal wall. The diagnosis of FD was confirmed on pathological examination of a biopsy taken through sphenotomy. The patient underwent a subcranial craniotomy for tumor resection. After more than 4 years of follow-up, the patient was disease-free. On the basis of these clinical features, it is important to consider sphenoidal FD in both young and adult patients complaining of an unexplained headache, because it may present unusually with headache localized to the temporal region or the inferior orbital rim.

  2. Fibrous dysplasia

    MedlinePlus

    ... page, please enable JavaScript. Fibrous dysplasia is a bone disease that destroys and replaces normal bone with fibrous ... A.M. Editorial team. Related MedlinePlus Health Topics Bone Diseases Browse the Encyclopedia A.D.A.M., Inc. ...

  3. Comparison of survival of adolescents and young adults with hematologic malignancies in Osaka, Japan.

    PubMed

    Nakata-Yamada, Kayo; Inoue, Masami; Ioka, Akiko; Ito, Yuri; Tabuchi, Takahiro; Miyashiro, Isao; Masaie, Hiroaki; Ishikawa, Jun; Hino, Masayuki; Tsukuma, Hideaki

    2016-01-01

    The survival gap between adolescents and young adults (AYAs) with hematological malignancies persists in many countries. To determine to what extent it does in Japan, we investigated survival and treatment regimens in 211 Japanese AYAs (15-29 years) in the Osaka Cancer Registry diagnosed during 2001-2005 with hematological malignancies, and compared adolescents (15-19 years) with young adults (20-29 years). AYAs with acute lymphoblastic leukemia (ALL) had a poor 5-year survival (44%), particularly young adults (29% vs. 64% in adolescents, p = 0.01). Additional investigation for patients with ALL revealed that only 19% of young adults were treated with pediatric treatment regimens compared with 45% of adolescents (p = 0.05). Our data indicate that we need to focus on young adults with ALL and to consider establishing appropriate cancer care system and guidelines for them in Japan.

  4. The effect of replacement of methionine by homocystine on survival of malignant and normal adult mammalian cells in culture.

    PubMed

    Halpern, B C; Clark, B R; Hardy, D N; Halpern, R M; Smith, R A

    1974-04-01

    In tissue cultures of normal adult and malignant mammalian cells, homocystine has been substituted for methionine in a medium rich in folic acid and cyanocobalamin. Normal adult cells thrive. Three highly malignant cell types from three different species, including man, die.

  5. Transformation of quiescent adult oligodendrocyte precursor cells into malignant glioma through a multistep reactivation process.

    PubMed

    Galvao, Rui Pedro; Kasina, Anita; McNeill, Robert S; Harbin, Jordan E; Foreman, Oded; Verhaak, Roel G W; Nishiyama, Akiko; Miller, C Ryan; Zong, Hui

    2014-10-01

    How malignant gliomas arise in a mature brain remains a mystery, hindering the development of preventive and therapeutic interventions. We previously showed that oligodendrocyte precursor cells (OPCs) can be transformed into glioma when mutations are introduced perinatally. However, adult OPCs rarely proliferate compared with their perinatal counterparts. Whether these relatively quiescent cells have the potential to transform is unknown, which is a critical question considering the late onset of human glioma. Additionally, the premalignant events taking place between initial mutation and a fully developed tumor mass are particularly poorly understood in glioma. Here we used a temporally controllable Cre transgene to delete p53 and NF1 specifically in adult OPCs and demonstrated that these cells consistently give rise to malignant gliomas. To investigate the transforming process of quiescent adult OPCs, we then tracked these cells throughout the premalignant phase, which revealed a dynamic multistep transformation, starting with rapid but transient hyperproliferative reactivation, followed by a long period of dormancy, and then final malignant transformation. Using pharmacological approaches, we discovered that mammalian target of rapamycin signaling is critical for both the initial OPC reactivation step and late-stage tumor cell proliferation and thus might be a potential target for both glioma prevention and treatment. In summary, our results firmly establish the transforming potential of adult OPCs and reveal an actionable multiphasic reactivation process that turns slowly dividing OPCs into malignant gliomas.

  6. Transformation of quiescent adult oligodendrocyte precursor cells into malignant glioma through a multistep reactivation process.

    PubMed

    Galvao, Rui Pedro; Kasina, Anita; McNeill, Robert S; Harbin, Jordan E; Foreman, Oded; Verhaak, Roel G W; Nishiyama, Akiko; Miller, C Ryan; Zong, Hui

    2014-10-01

    How malignant gliomas arise in a mature brain remains a mystery, hindering the development of preventive and therapeutic interventions. We previously showed that oligodendrocyte precursor cells (OPCs) can be transformed into glioma when mutations are introduced perinatally. However, adult OPCs rarely proliferate compared with their perinatal counterparts. Whether these relatively quiescent cells have the potential to transform is unknown, which is a critical question considering the late onset of human glioma. Additionally, the premalignant events taking place between initial mutation and a fully developed tumor mass are particularly poorly understood in glioma. Here we used a temporally controllable Cre transgene to delete p53 and NF1 specifically in adult OPCs and demonstrated that these cells consistently give rise to malignant gliomas. To investigate the transforming process of quiescent adult OPCs, we then tracked these cells throughout the premalignant phase, which revealed a dynamic multistep transformation, starting with rapid but transient hyperproliferative reactivation, followed by a long period of dormancy, and then final malignant transformation. Using pharmacological approaches, we discovered that mammalian target of rapamycin signaling is critical for both the initial OPC reactivation step and late-stage tumor cell proliferation and thus might be a potential target for both glioma prevention and treatment. In summary, our results firmly establish the transforming potential of adult OPCs and reveal an actionable multiphasic reactivation process that turns slowly dividing OPCs into malignant gliomas. PMID:25246577

  7. Targeted Therapies in Adult B-Cell Malignancies

    PubMed Central

    Rossi, Jean-François

    2015-01-01

    B-lymphocytes are programmed for the production of immunoglobulin (Ig) after antigen presentation, in the context of T-lymphocyte control within lymphoid organs. During this differentiation/activation process, B-lymphocytes exhibit different restricted or common surface markers, activation of cellular pathways that regulate cell cycle, metabolism, proteasome activity, and protein synthesis. All molecules involved in these different cellular mechanisms are potent therapeutic targets. Nowadays, due to the progress of the biology, more and more targeted drugs are identified, a situation that is correlated with an extended field of the targeted therapy. The full knowledge of the cellular machinery and cell-cell communication allows making the best choice to treat patients, in the context of personalized medicine. Also, focus should not be restricted to the immediate effects observed as clinical endpoints, that is, response rate, survival markers with conventional statistical methods, but it should consider the prediction of different clinical consequences due to other collateral drug targets, based on new methodologies. This means that new reflection and new bioclinical follow-up have to be monitored, particularly with the new drugs used with success in B-cell malignancies. This review discussed the principal aspects of such evident bioclinical progress. PMID:26425544

  8. Primary Cardiac Solitary Fibrous Tumors

    PubMed Central

    2015-01-01

    Primary cardiac solitary fibrous tumors were reviewed. They are classified as pericardial tumors. Their incidences are very rare. Only 16 cases were reported in the literature. Basically, surgical treatments are performed. Their prognoses are generally good, although malignant cases are also reported. PMID:26156195

  9. ARSENIC AND SKIN LESION STATUS IN RELATION TO MALIGNANT AND NON-MALIGNANT LUNG DISEASE MORTALITY IN BANGLADESHI ADULTS

    PubMed Central

    Argos, Maria; Parvez, Faruque; Rahman, Mahfuzar; Rakibuz-Zaman, Muhammad; Ahmed, Alauddin; Hore, Samar Kumar; Islam, Tariqul; Chen, Yu; Pierce, Brandon L.; Slavkovich, Vesna; Olopade, Christopher; Yunus, Muhammad; Baron, John A.; Graziano, Joseph H.; Ahsan, Habibul

    2015-01-01

    Background Chronic arsenic exposure through drinking water is a public health problem affecting millions of people worldwide, including at least 30 million in Bangladesh. We prospectively investigated the associations of arsenic exposure and arsenical skin lesion status with lung disease mortality in Bangladeshi adults. Methods Data are from a population-based sample of 26,043 adults, with an average of 8.5 years of follow-up (220,157 total person-years). There were 156 non-malignant lung disease deaths and 90 lung cancer deaths ascertained through October 2013. We used Cox proportional hazards models to estimate adjusted hazard ratios and 95% confidence intervals (CIs) for lung disease mortality. Results Creatinine-adjusted urinary total arsenic was associated with non-malignant lung disease mortality, with persons in the highest tertile of exposure having a 75% increased risk for mortality (95% CI=1.15–2.66) compared with those in the lowest tertile of exposure. Persons with arsenical skin lesions were at increased risk of lung cancer mortality (hazard ratio=4.53 [95% CI=2.82–7.29]) compared with those without skin lesions. Conclusions This prospective investigation of lung disease mortality, utilizing individual-level arsenic measures and skin lesion status, confirms a deleterious effect of ingested arsenic on mortality from lung disease. Further investigations should evaluate effects on the incidence of specific lung diseases, more fully characterize dose-response, and evaluate screening and biomedical interventions to prevent premature death among arsenic-exposed populations, particularly among those who may be most susceptible to arsenic toxicity. PMID:24802365

  10. Retroperitoneal malignant solitary fibrous tumor of the small pelvis causing recurrent hypoglycemia by secretion of insulin-like growth factor 2.

    PubMed

    Wagner, Sigrid; Greco, Francesco; Hamza, Amir; Hoda, Rashid M; Holzhausen, Hans Jürgen; Fornara, Paolo

    2009-03-01

    A 28-yr-old man presented with recurrent reduced consciousness, generalized seizures of unknown etiology, recurrent hypoglycemia, psychomotor retardation, and grade 2 ectasia of the left kidney. Abdominal computed tomography (CT) and positron emission tomography (PET) scans demonstrated a well-circumscribed suprapubic pelvic mass, measuring 18 x 15 x 11 cm, with involvement of para-aortic lymph nodes and dilatation of the left ureter suggestive of an extragonadal testicular tumor. We excised the tumor by laparotomy, and it was confirmed to be a solitary fibrous tumor (SFT). After surgery and R0 tumor resection, the patient had no further evidence of hypoglycemia or of recurrence.

  11. A Rare Case of Monostotic Spinal Fibrous Dysplasia Mimicking Solitary Metastatic Lesion of Thyroid Carcinoma.

    PubMed

    Sharifudin, Mohd Ariff; Zakaria, Zamzuri; Awang, Mohamed Saufi; Mohamed Amin, Mohamed Azril; Abd Aziz, Azian

    2016-01-01

    Monostotic fibrous dysplasia of the vertebra is a rare entity. A case of a 53-year-old lady who presented with an 8 months history of pain in the thoracic spine region with paraparesis is discussed. She had a history of papillary thyroid carcinoma and had undergone total thyroidectomy one year prior to her current problem. Magnetic resonance imaging revealed isolated osteolytic lesion over the posterior element of the T12 vertebra with narrowing of the spinal canal causing compression of the cord. The diagnosis of fibrous dysplasia was made histologically. Fibrous dysplasia rarely occurs in axial bones compared with peripheral bones. This case illustrates that osteolytic lesion of the vertebrae should be evaluated with detailed radiological and histopathological examination before an empirical diagnosis of spinal metastasis is made in an adult with a background history of primary malignancy well-known to spread to the bone. PMID:27540330

  12. Fibrous dysplasia and cherubism

    PubMed Central

    Bhattacharya, Surajit; Mishra, RK

    2015-01-01

    Fibrous dysplasia (FD) is a non-malignant fibro-osseous bony lesion in which the involved bone/bones gradually get converted into expanding cystic and fibrous tissue. The underlying defect in FD is post-natal mutation of GNAS1 gene, which leads to the proliferation and activation of undifferentiated mesenchymal cells arresting the bone development in woven phase and ultimately converting them into fibro-osseous cystic tissue. Cherubism is a hereditary form of fibrous dysplasia in which the causative factor is transmission of autosomal dominant SH3BP2 gene mutation. The disease may present in two distinct forms, a less severe and limited monostotic form, and a more aggressive and more widespread polyostotic form. Polyostotic form may be associated with various endocrine abnormalities, which require active management apart from the management of FD. Management of FD is not free from controversies. While total surgical excision of the involved area and reconstruction using newer micro-vascular technique is the only definitive treatment available from the curative point of view, but this can be only offered to monostotic and very few polyostotic lesions. In polyostotic varieties on many occasions these radical surgeries are very deforming in these slow growing lesions and so their indication is highly debated. The treatment of cranio-facial fibrous dysplasia should be highly individualized, depending on the fact that the clinical behavior of lesion is variable at various ages and in individual patients. A more conservative approach in the form of aesthetic recontouring of deformed bone, orthodontic occlusal correction, and watchful expectancy may be the more accepted form of treatment in young patients. Newer generation real-time imaging guidance during recontouring surgery adds to accuracy and safety of these procedures. Regular clinical and radiological follow up is required to watch for quiescence, regression or reactivation of the disease process. Patients must be

  13. Late recurrence of a malignant hypoglycemia-inducing pelvic solitary fibrous tumor secreting high-molecular-weight insulin-like growth factor-II: A case report with protein analysis

    PubMed Central

    ISHIHARA, HIROKI; OMAE, KENJI; IIZUKA, JUNPEI; KOBAYASHI, HIROHITO; FUKUDA, IZUMI; KONDO, TSUNENORI; HIZUKA, NAOMI; NAGASHIMA, YOJI; TANABE, KAZUNARI

    2016-01-01

    The present study reports a case of recurrent malignant pelvic solitary fibrous tumor (SFT) that induced non-islet cell tumor hypoglycemia via high-molecular-weight insulin-like growth factor-II in a 72-year-old male patient. The tumor recurred ~12 years after the complete resection of the original mass. The recurrent tumor, which had directly invaded the left ureter and perirectal fat tissue, could not be completely excised due to its fragility and adhesiveness. At 13 days post-surgery, the patient presented with rectal perforation, and an urgent rectal resection and colostomy was performed. Neither recurrence of the tumor nor hypoglycemic symptoms were observed 9 months after the surgery. High molecular weight insulin-like growth factor-II was detected in the serum and tumor specimens by western blot analysis and immunohistochemistry. The present case report suggests that certain SFTs can relapse even ≥10 years after a presumed complete resection of the primary tumor, and that performing a safe and complete resection of these tumors can be challenging, due to their adhesiveness or physical presentation; therefore, the indications for surgery should be considered with caution. PMID:27347168

  14. Expanding the spectrum of malignant progression in solitary fibrous tumors: a study of 8 cases with a discrete anaplastic component--is this dedifferentiated SFT?

    PubMed

    Mosquera, Juan-Miguel; Fletcher, Christopher D M

    2009-09-01

    Dedifferentiation is a well recognized, if sometimes controversial, form of tumor progression in certain types of soft tissue and bone sarcoma, and confers a worse prognosis when compared with the low-grade counterpart. To date, dedifferentiation has not been described in solitary fibrous tumor (SFT). Among 948 cases of both intrathoracic and extrathoracic SFTs in our files accessioned between 1988 and 2008, we identified 8 cases of conventional SFT with a discrete anaplastic component, which we believe represents dedifferentiation. These occurred in 3 men and 5 women, 40 to 76 years old (median 60 y), and measured 3.4 to 20.0 cm (median 8.5 cm). Two cases were intrathoracic, 2 were located in the deep soft tissue of thigh, and single cases were located in the omentum, scalp, retroperitoneum, and abdominal wall. In addition to typical features of benign-appearing SFT there was an abrupt transition to nondistinctive high-grade sarcoma in all cases. The latter included epithelioid, round cell, and/or spindle cell components with increased mitotic activity, necrosis, and cystic degeneration. By immunohistochemistry, 7 of 8 cases were CD34 positive in the usual SFT areas, whereas 5 showed loss of CD34 in the poorly differentiated component. Six of 7 cases stained for p53 and p16 showed either negative or scattered positive cells in well-differentiated SFT areas, in contrast to positive or stronger and more diffuse staining in the high-grade component. Follow-up information available in 7 patients ranged from 1 to 58 months (mean 24 mo). Three patients with the largest tumors (9.0, 17.0, and 20.0 cm) died of disease, whereas 3 patients whose tumors measured 8.0 cm or less were treated by surgical excision only, and show no evidence of disease but with only limited follow-up. One patient with an 11.5 cm intrathoracic tumor is alive with disease at 58 months after recurrence and metastasis. We describe, apparently for the first time, what seems, at least in our view, to

  15. Prediction of Clinical Deterioration in Hospitalized Adult Patients with Hematologic Malignancies Using a Neural Network Model

    PubMed Central

    Hu, Scott B.; Wong, Deborah J. L.; Correa, Aditi; Li, Ning; Deng, Jane C.

    2016-01-01

    Introduction Clinical deterioration (ICU transfer and cardiac arrest) occurs during approximately 5–10% of hospital admissions. Existing prediction models have a high false positive rate, leading to multiple false alarms and alarm fatigue. We used routine vital signs and laboratory values obtained from the electronic medical record (EMR) along with a machine learning algorithm called a neural network to develop a prediction model that would increase the predictive accuracy and decrease false alarm rates. Design Retrospective cohort study. Setting The hematologic malignancy unit in an academic medical center in the United States. Patient Population Adult patients admitted to the hematologic malignancy unit from 2009 to 2010. Intervention None. Measurements and Main Results Vital signs and laboratory values were obtained from the electronic medical record system and then used as predictors (features). A neural network was used to build a model to predict clinical deterioration events (ICU transfer and cardiac arrest). The performance of the neural network model was compared to the VitalPac Early Warning Score (ViEWS). Five hundred sixty five consecutive total admissions were available with 43 admissions resulting in clinical deterioration. Using simulation, the neural network outperformed the ViEWS model with a positive predictive value of 82% compared to 24%, respectively. Conclusion We developed and tested a neural network-based prediction model for clinical deterioration in patients hospitalized in the hematologic malignancy unit. Our neural network model outperformed an existing model, substantially increasing the positive predictive value, allowing the clinician to be confident in the alarm raised. This system can be readily implemented in a real-time fashion in existing EMR systems. PMID:27532679

  16. High-grade microscopic hematuria in adult men can predict urothelial malignancy

    PubMed Central

    Kotb, Ahmed Fouad; Attia, Doaa

    2014-01-01

    Introduction: Microscopic hematuria in men younger than 40 is a confusing issue to urologists, especially when these men have normal radiological findings. We report our experience in looking for urologic malignancy in this group of patients. Methods: We conducted a prospective study for men with vague urological symptoms. We included men under 40 years old, men with microscopic hematuria greater than 25 red blood cells/high power field in 2 properly collected mid-stream urine samples, and men with free urine culture and normal multiphasic computed tomography abdomen and pelvis studies. All patients underwent diagnostic cystoscurethroscopy. If there were no lesions, multiple random biopsies were taken. In cases of apparently normal cystoscopic findings and associated renal colic, uretroscopy was done to the suspected side. Results: Only 20 patients fulfilled our inclusion criteria. The mean age of the patients were 34; 2 patients presented with pain. The other 18 patients were presenting with mild recurrent lower urinary tract symptoms. Cystoscopy showed small papillary low-grade tumour in 3 patients. All random biopsies were free of malignancy. Unilateral uretroscopy for the 2 cases presented with pain detected carcinoma in situ in one of them. Conclusion: Cystoscopy is highly recommended for young adult men, with significant levels of microscopic hematuria, due to the 20% incidence rate of associated urological malignancy. Random bladder biopsies, in the absence of suspicious lesions, have no diagnostic role, and should not be done. Uretroscopy is advised for patients with microscopic hematuria and loin pain, even in the absence of suspicious radiological findings. PMID:25132893

  17. Secondary malignant neoplasms following radiotherapy for primary cancer in children and young adults.

    PubMed

    Harbron, Richard W; Feltbower, Richard G; Glaser, Adam; Lilley, John; Pearce, Mark S

    2014-04-01

    A study was conducted to investigate secondary malignant neoplasm (SMN) occurrence following radiotherapy (RT) for cancer in children and young adults, to examine the spatial distribution of SMNs in relation to the irradiated field, and to evaluate a possible role of bystander effects in SMN distribution. Forty-two SMNs were identified among 7257 subjects diagnosed with cancer while living in Yorkshire, UK. Thirty-two of these occurred in patients receiving RT. Distances between SMN locations and RT field edge were estimated along with dose at SMN site. Expected radiation-induced SMN frequency in remote tissues receiving less than 0.1 Gy was predicted using risk estimates based on atomic bombing data. After a median follow-up period of 7.58 years, patients treated with RT were at a nearly five-fold increased risk of developing a subsequent primary neoplasm than the general population in the 0-29 years age range. The most common type of secondary malignancy associated with RT was of the central nervous system (28%), followed by sarcoma (25%) and leukemia (19%). Considering only solid SMNs developing 5 years or more from treatment, the spatial distribution showed a strong pattern of proximity to the irradiated field, with 68% occurring in-field or within 8 cm of the field edge. The SMN frequency in distant tissues receiving doses of less than 0.1 Gy was low but compatible with local absorbed dose.

  18. Robust Vaccine Responses in Adult and Pediatric Cord Blood Transplantation Recipients Treated for Hematologic Malignancies.

    PubMed

    Shah, Gunjan L; Shune, Leyla; Purtill, Duncan; Devlin, Sean; Lauer, Emily; Lubin, Marissa; Bhatt, Valkal; McElrath, Courtney; Kernan, Nancy A; Scaradavou, Andromachi; Giralt, Sergio; Perales, Miguel A; Ponce, Doris M; Young, James W; Shah, Monica; Papanicolaou, Genovefa; Barker, Juliet N

    2015-12-01

    Because cord blood (CB) lacks memory T and B cells and recent decreases in herd immunity to vaccine-preventable diseases in many developed countries have been documented, vaccine responses in CB transplantation (CBT) survivors are of great interest. We analyzed vaccine responses in double-unit CBT recipients transplanted for hematologic malignancies. In 103 vaccine-eligible patients, graft-versus-host disease (GVHD) most commonly precluded vaccination. Sixty-five patients (63%; engrafting units median HLA-allele match 5/8; range, 2 to 7/8) received protein conjugated vaccines, and 63 patients (median age, 34 years; range, .9 to 64) were evaluated for responses. Median vaccination time was 17 months (range, 7 to 45) post-CBT. GVHD (n = 42) and prior rituximab (n = 13) delayed vaccination. Responses to Prevnar 7 and/or 13 vaccines (serotypes 14, 19F, 23F) were seen in children and adults (60% versus 49%, P = .555). Responses to tetanus, diphtheria, pertussis, Haemophilus influenzae, and polio were observed in children (86% to 100%) and adults (53% to 89%) even if patients had prior GVHD or rituximab. CD4(+)CD45RA(+) and CD19(+) cell recovery significantly influenced tetanus and polio responses. In a smaller cohort responses were seen to measles (65%), mumps (50%), and rubella (100%) vaccines. No vaccine side effects were identified, and all vaccinated patients survived (median follow-up, 57 months). Although GVHD and rituximab can delay vaccination, CBT recipients (including adults and those with prior GVHD) have similar vaccine response rates to adult donor allograft recipients supporting vaccination in CBT recipients. PMID:26271191

  19. Altered expression of Lewis blood group and related antigens in fetal, normal adult and malignant tissues of the uterine endometrium.

    PubMed

    Inoue, M; Nakayama, M; Tanizawa, O

    1990-01-01

    The expression of the Lewis blood group and its related antigens in fetal, normal adult and malignant tissues of the uterine endometrium was examined immunohistochemically using a panel of mouse monoclonal antibodies with specificities for Lewis-a (La), Sialyl Lewis-a (SLa), Lewis-b (Lb), Lewis-X (LX), Sialyl Lewis-X (SLX) and Lewis-Y (LY) antigens. La, SLa and SLX having one fucose residue were detected in a small number of fetal tissues, while Lb and LY having two fucose residues were found in most cases. In the adult endometrium, expression of Lb and LY was considerably lower than those in fetal tissues, although expression of La and SLa was not different between these two tissues. Expression of LX and SLX was pronounced in adult when compared with fetal tissues. Malignant endometrial glands expressed La, SLa, Lb and LY, extensively, while LX and SLX were expressed less than in normal tissues. Lb and LY can thus be considered oncofetal antigens, extensively expressed in fetal and malignant tissues but not in normal adult tissues. Expression of Lb and LY was greater than that of La and SLA in carcinoma; an increase in the activity of fucose transferase might be associated with malignant transformation in the uterine endometrium.

  20. Primary and metastatic high-grade pleomorphic sarcoma/malignant fibrous histiocytoma of the gastrointestinal tract: an approach to the differential diagnosis in a series of five cases with emphasis on myofibroblastic differentiation.

    PubMed

    Agaimy, Abbas; Gaumann, Andreas; Schroeder, Josef; Dietmaier, Wolfgang; Hartmann, Arndt; Hofstaedter, Ferdinand; Wünsch, Peter H; Mentzel, Thomas

    2007-11-01

    Primary and metastatic so-called malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma (MFH) is rare in the gastrointestinal (GI) tract with approximately 50 primary and five metastatic cases reported so far. We evaluated two primary gastric and three metastatic intestinal high-grade pleomorphic sarcomas with features of storiform-pleomorphic MFH. Gastric tumours occurred in a 79-year-old man and a 68-year-old woman. One patient died post-operatively, and the other was disease-free at 6 months. Three patients presented with GI metastasis 24, 60 and 0 months after diagnosis of MFH of the heart (n = 1) and the thigh (n = 2). Metastases were located in the small (n = 1) and large bowel (n = 2) and were characteristically pedunculated and polypoid with oedematous haemorrhagic stroma. Concurrent metastases (brain, lung, bone) were present in all three cases. Tumours expressed alpha-smooth muscle actin (four of five), platelet-derived growth factor receptor (PDGFR) alpha (three of three) and PDGFRbeta (two of three) but were negative for CD117, CD34 and other lineage-specific markers. Ultrastructural examination revealed myo/fibroblastic features. Both gastric MFH were wild type for KIT and PDGFRalpha. In conclusion, primary and metastatic MFH of the GI tract commonly express PDGFRalpha and show a myo/fibroblastic phenotype. They should be distinguished from a variety of primary and metastatic pleomorphic neoplasms, in particular high-grade sarcomatous GI stromal tumours (GIST), pleomorphic leiomyosarcoma, sarcomatoid carcinoma and other mimics. PMID:17874130

  1. Solitary Fibrous Tumor of the Sigmoid Colon Masquerading as an Adnexal Neoplasm

    PubMed Central

    Bratton, Laura; Salloum, Rabih; Cao, Wenqing

    2016-01-01

    Solitary fibrous tumor is a rare, benign spindle cell neoplasm that was first described in the thoracic pleura. This tumor is now known to occur at many extrapleural sites. There are established criteria for the diagnosis of malignant solitary fibrous tumor including ≥4 mitotic figures per 10 high-power fields, increased cellularity, cytologic atypia, infiltrative margins, and/or necrosis. Although all solitary fibrous tumors have the potential to recur or metastasize, those with malignant histologic features tend to behave more aggressively. We report a case of solitary fibrous tumor, with malignant histologic features, in a 21-year-old woman which arose from the serosal surface of the sigmoid colon.

  2. Primary solitary fibrous tumor of the retroperitoneum.

    PubMed

    Charhi, Hind; Bernoussi, Zakiya; Haddan, Azzouz; Mesmoudi, Siham; Elktaibi, Abderrahim; Mansouri, Fatima; Elktaibi, Rachid; Lahlou, Khalid; Jahid, Ahmed; Mahassini, Najat

    2011-09-28

    Solitary fibrous tumor is an uncommon neoplasm affecting adults and typically located in the pleura and can also occur in a large number of other extra thoracic sites. We present the case of a solitary fibrous tumor (SFT) of the retroperitoneum and describe their histopathological and immunohistochemical features. The identification of SFT in the retroperitoneum is of importance because its clinico-pathological behaviour is still unclear. The pathologist plays a fundamental role in establishing both the positive and differential diagnosis.

  3. Solitary Fibrous Tumor of the Orbit: A Case Report and Review of the Literature.

    PubMed

    Genc, Ali; Toktas, Zafer; Azman, Coruh; Bozkurt, Suheyla Uyar; Kilic, Turker

    2015-01-01

    Solitary fibrous tumor (SFT) is a rare, benign, spindle cell tumor that is most commonly found in the visceral pleura. The orbit is one of the most common extrapleural sites of occurrence. Though they can be seen in any age, they typically present in adults with proptosis as the prominent symptom. They show no significant gender predominance. Orbital solitary fibrous tumors routinely exhibit a benign course, but malignant forms with an increased propensity for local recurrence have been reported. Histopathologically, they share similar features with hemangiopericytoma, which is much more common. The diagnosis of SFT depends on the diffuse and intense positivity of CD34 staining by immunohistochemistry (14). Here, we report a case of SFT, which presented with proptosis and double vision on lateral gaze. We describe the clinical, radiographic, histopathological, and immunohistochemical findings. We also provide a discussion on its origin and differential diagnosis in the light of relevant literature.

  4. Solitary fibrous tumor of the upper respiratory tract. A report of six cases.

    PubMed

    Witkin, G B; Rosai, J

    1991-09-01

    We report six cases of a neoplasm that arose in the upper respiratory tract and had a histological appearance indistinguishable from that of solitary fibrous tumor of the pleura (SFT, so-called fibrous mesothelioma). The patients were adults who presented with nasal obstruction. The lesions lacked the characteristic features of other recognized neoplasms that occur in this region. The tumor cells were immunoreactive for vimentin but not for keratin. The occurrence of SFT in this location further supports the argument that SFT is a tumor of mesenchymal and not mesothelial origin. None of the tumors in this series had the histologic features of malignancy described for SFT in other locations, and there was no aggressive behavior in limited follow-up. Until more cases of SFT in unusual locations have been studied, we recommend that the same criteria used for assessing aggressiveness in SFT of the pleura be applied to them.

  5. Solitary Fibrous Tumor of the Orbit: A Case Report and Review of the Literature.

    PubMed

    Genc, Ali; Toktas, Zafer; Azman, Coruh; Bozkurt, Suheyla Uyar; Kilic, Turker

    2015-01-01

    Solitary fibrous tumor (SFT) is a rare, benign, spindle cell tumor that is most commonly found in the visceral pleura. The orbit is one of the most common extrapleural sites of occurrence. Though they can be seen in any age, they typically present in adults with proptosis as the prominent symptom. They show no significant gender predominance. Orbital solitary fibrous tumors routinely exhibit a benign course, but malignant forms with an increased propensity for local recurrence have been reported. Histopathologically, they share similar features with hemangiopericytoma, which is much more common. The diagnosis of SFT depends on the diffuse and intense positivity of CD34 staining by immunohistochemistry (14). Here, we report a case of SFT, which presented with proptosis and double vision on lateral gaze. We describe the clinical, radiographic, histopathological, and immunohistochemical findings. We also provide a discussion on its origin and differential diagnosis in the light of relevant literature. PMID:26617156

  6. Malignant rhabdoid tumor of the floor of mouth: first reported case in the oral cavity of an adult.

    PubMed

    Wetzel, Stephanie L; Kerpel, Stanley; Reich, Renee F; Freedman, Paul D

    2015-06-01

    Malignant rhabdoid tumors (MRTs) are exceedingly rare lesions. To our knowledge, only 2 cases have been reported in the oral cavity, with both examples occurring in infants. The current case is the third reported case of MRT of the oral cavity and the first reported case to occur in an adult at this location. The following report describes the clinical, histologic and immunohistochemical features of this tumor.

  7. Solitary fibrous tumor of the submandibular gland.

    PubMed

    Hofmann, Thiemo; Braun, Hannes; Köle, Wolfgang; Beham, Alfred

    2002-10-01

    Solitary fibrous tumors (SFT) are generally benign, well-circumscribed soft-tissue tumors of mesenchymal origin. CD34 antigen expression is characteristic for this tumor. A rare subgroup shows malignant histological patterns with aggressive behavior. The common site of occurrence is the pleura, but various other sites, including the head and neck, have been described. We present a 56-year-old, white, female patient with a solitary fibrous tumor developing in the right submandibular salivary gland. The tumor was surgically removed, and no recurrence or metastases have occurred during the 43 months of follow-up. All solitary fibrous tumors reported in the salivary glands were benign. However, new cases should be presented and followed up carefully to monitor their biological behavior.

  8. Solitary fibrous tumor of the liver.

    PubMed

    Vennarecci, Giovanni; Ettorre, Giuseppe Maria; Giovannelli, Luca; Del Nonno, Franca; Perracchio, Letizia; Visca, Paolo; Corazza, Valerio; Vidiri, Antonello; Visco, Giuseppe; Santoro, Eugenio

    2005-01-01

    We report a new case of benign solitary fibrous tumor (SFT) of the liver. A 65-year-old man presented to our unit with upper right abdominal discomfort. On examination abdominal distension was present and palpation showed a large firm mass in the right hypochondrium and epigastrium. The patient's past medical history was not significant and laboratory tests were normal. Ultrasonography and computed tomography showed a large tumor, 20 cm in diameter, in the right lobe of the liver. An extended right hepatectomy was performed. The tumor measured 30 x 28 x 14 cm and weighed 4725 g. Microscopic evaluation showed a benign SFT of the liver with tumor cells typically positive for vimentin and CD34. The postoperative course was uneventful, and the patient is alive 30 months after surgery. This is a rare neoplasm of mesenchymal origin that occasionally involves the liver in adult patients. Most SFTs are benign, but some may have malignant histological features and recur locally or metastasize. Because of their rarity, overall experience has not been significant and little has been published concerning this tumor, Including the present one, 28 cases have been reported in the English literature. Surgery is the mainstay of treatment. Little can be said about the benefits of adjuvant radiochemotherapy in these patients. As SFT of the liver is often a benign neoplasm, chemotherapy or radiotherapy should not be necessary, and should be reserved for when resection is incomplete and/or histological examination reveals features of malignancy. Surgeons must be aware of SFT of the liver, and this neoplasm should be included in the differential diagnosis of a single large hepatic mass.

  9. A case/control study of adult haematological malignancies in relation to overhead powerlines.

    PubMed Central

    Youngson, J. H.; Clayden, A. D.; Myers, A.; Cartwright, R. A.

    1991-01-01

    A population based case control study of adult haematological malignancy and distance from, and magnetic fields associated with, overhead (OH) power lines has been carried out in the North West and Yorkshire regions of England. Three-thousand, one hundred and forty-four cases with histologically proven disease were entered into the study. One control per case, matched for age, sex, year of diagnosis and health district of residence, was selected from hospital discharges. Seven per cent of cases and controls lived near to OH power lines as defined by the study protocol. The measure of exposure used was the calculated magnetic field strength at each of these addresses due to maximum load currents carried by OH power lines in the 5 years preceding diagnosis. The odds ratio (OR) for living within 50 m of an OH line was 1.29 with a 95% Confidence Interval (CI) of 0.99-1.68 but a chi 2 test for trend with distance was not statistically significant. The analysis of calculated magnetic fields, did not produce any statistically odds ratios. The OR for magnetic fields greater than or equal to 0.1 mG was 1.03 (95% CI 0.81 1.32). Analysis of magnetic fields greater than or equal to 3.0 mG gave an OR of 1.87 (95% CI 0.79 4.42), but this result is based on small numbers. No evidence was found for confounding by the type of dwelling which was used as a partial surrogate for socio-economic status. PMID:2069855

  10. Synchronous abdominal and thoracic solitary fibrous tumour: a case report.

    PubMed

    Maassarani, F; Leroy, C; Dekeuleneer, R

    2010-01-01

    Solitary fibrous tumours (SFT), described for the first time by Klemperer and Rabin in 1931, are uncommon mesenchymal tumours that have been known to mainly affect the pleura and mediastinum. More recently, solitary fibrous tumours affecting other anatomical sites are being increasingly reported. Clinically, these tumours are asymptomatic and are discovered incidentally. Diagnosis is established at pathology by positive staining for CD34. Their prognosis depends on complete surgical resection and lack of histological signs of malignancy. We report here the case of a 63-year-old woman with double localisation of malignant solitary fibrous tumour who underwent complete removal of her lesions. To the best of our knowledge, this observation is the first description of a primary solitary fibrous tumour totally asymptomatic but already metastatic.

  11. Membranous nephropathy associated with malignant pleural mesothelioma in an adult patient: A case report

    PubMed Central

    Pu, Xinyu; Dou, Yanna; Liu, Dong; Lu, Shan; Quan, Songxia; Zhang, Xiaoxue; Ma, Shuang; Zhao, Zhanzheng

    2016-01-01

    A 23-year-old man presented to our hospital with membranous nephropathy and received a detailed examination, including pleural biopsy, due to a feeling of chest oppression. The result of the pleural biopsy was malignant pleural mesothelioma. However, the patient did not have a history of asbestos or tobacco exposure. A review of the English literature identified only 7 reported cases of concomitant malignant mesothelioma and nephrotic syndrome. Furthermore, among the 7 cases reviewed, 6 had a history of asbestos exposure, 1 had a history of prolonged tobacco exposure and in only 1 case the renal pathology results revealed the presence of membranous nephropathy. PMID:27699035

  12. Red Blood Cell Transfusion Strategies in Adult and Pediatric Patients with Malignancy.

    PubMed

    Roubinian, Nareg; Carson, Jeffrey L

    2016-06-01

    Anemia in patients with malignancy is common as a consequence of their disease and treatment. Substantial progress has been made in the management of anemia with red blood cell transfusion in acute conditions, such as bleeding and infection, through the performance of large clinical trials. These trials suggest that transfusion at lower hemoglobin thresholds (restrictive transfusion ∼7-8 g/dL) is safe and in some cases superior to higher transfusion thresholds (liberal transfusion ∼9-10 g/dL). However, additional studies are needed in patients with malignancy to understand best practice in relation to quality of life as well as clinical outcomes. PMID:27112994

  13. Membranous nephropathy associated with malignant pleural mesothelioma in an adult patient: A case report

    PubMed Central

    Pu, Xinyu; Dou, Yanna; Liu, Dong; Lu, Shan; Quan, Songxia; Zhang, Xiaoxue; Ma, Shuang; Zhao, Zhanzheng

    2016-01-01

    A 23-year-old man presented to our hospital with membranous nephropathy and received a detailed examination, including pleural biopsy, due to a feeling of chest oppression. The result of the pleural biopsy was malignant pleural mesothelioma. However, the patient did not have a history of asbestos or tobacco exposure. A review of the English literature identified only 7 reported cases of concomitant malignant mesothelioma and nephrotic syndrome. Furthermore, among the 7 cases reviewed, 6 had a history of asbestos exposure, 1 had a history of prolonged tobacco exposure and in only 1 case the renal pathology results revealed the presence of membranous nephropathy.

  14. Differences in Stylet Sheath Occurrence and the Fibrous Ring (Sclerenchyma) between xCitroncirus Plants Relatively Resistant or Susceptible to Adults of the Asian Citrus Psyllid Diaphorina citri (Hemiptera: Liviidae)

    PubMed Central

    Ammar, El-Desouky; Richardson, Matthew L.; Abdo, Zaid; Hall, David G.; Shatters, Robert G.

    2014-01-01

    The Asian citrus psyllid (ACP, Diaphorina citri, Hemiptera: Liviidae), is the principal vector of the phloem-limited bacteria strongly associated with huanglongbing (HLB), the world’s most serious disease of citrus. Host plant resistance may provide an environmentally safe and sustainable method of controlling ACP and/or HLB. Two xCitroncirus accessions (hybrids of Poncirus trifoliata and Citrus spp.), that are relatively resistant (UN-3881) or relatively susceptible (Troyer-1459) to ACP adults with regard to adult longevity, were compared in relation to ACP feeding behavior and some structural features of the leaf midrib. The settling (putative feeding/probing) sites of ACP adults on various parts of the leaf were not influenced primarily by plant accession. However, fewer ACP stylet sheaths were found in the midrib and fewer stylet sheath termini reached the vascular bundle (phloem and/or xylem) in UN-3881 compared to Troyer-1459 plants. Furthermore, in midribs of UN-3881 leaves the fibrous ring (sclerenchyma) around the phloem was significantly wider (thicker) compared to that in midribs of Troyer-1459 leaves. Our data indicate that feeding and/or probing by ACP adults into the vascular bundle is less frequent in the more resistant (UN-3881) than in the more susceptible (Troyer-1459) accessions. Our results also suggest that the thickness of the fibrous ring may be a barrier to stylet penetration into the vascular bundle, which is important for successful ACP feeding on the phloem and for transmitting HLB-associated bacteria. These results may help in the development of citrus plants resistant to ACP, which in turn could halt or slow the spread of the HLB-associated bacteria by this vector. PMID:25343712

  15. Differences in stylet sheath occurrence and the fibrous ring (sclerenchyma) between xCitroncirus plants relatively resistant or susceptible to adults of the Asian citrus psyllid Diaphorina citri (Hemiptera: Liviidae).

    PubMed

    Ammar, El-Desouky; Richardson, Matthew L; Abdo, Zaid; Hall, David G; Shatters, Robert G

    2014-01-01

    The Asian citrus psyllid (ACP, Diaphorina citri, Hemiptera: Liviidae), is the principal vector of the phloem-limited bacteria strongly associated with huanglongbing (HLB), the world's most serious disease of citrus. Host plant resistance may provide an environmentally safe and sustainable method of controlling ACP and/or HLB. Two xCitroncirus accessions (hybrids of Poncirus trifoliata and Citrus spp.), that are relatively resistant (UN-3881) or relatively susceptible (Troyer-1459) to ACP adults with regard to adult longevity, were compared in relation to ACP feeding behavior and some structural features of the leaf midrib. The settling (putative feeding/probing) sites of ACP adults on various parts of the leaf were not influenced primarily by plant accession. However, fewer ACP stylet sheaths were found in the midrib and fewer stylet sheath termini reached the vascular bundle (phloem and/or xylem) in UN-3881 compared to Troyer-1459 plants. Furthermore, in midribs of UN-3881 leaves the fibrous ring (sclerenchyma) around the phloem was significantly wider (thicker) compared to that in midribs of Troyer-1459 leaves. Our data indicate that feeding and/or probing by ACP adults into the vascular bundle is less frequent in the more resistant (UN-3881) than in the more susceptible (Troyer-1459) accessions. Our results also suggest that the thickness of the fibrous ring may be a barrier to stylet penetration into the vascular bundle, which is important for successful ACP feeding on the phloem and for transmitting HLB-associated bacteria. These results may help in the development of citrus plants resistant to ACP, which in turn could halt or slow the spread of the HLB-associated bacteria by this vector. PMID:25343712

  16. Differences in stylet sheath occurrence and the fibrous ring (sclerenchyma) between xCitroncirus plants relatively resistant or susceptible to adults of the Asian citrus psyllid Diaphorina citri (Hemiptera: Liviidae).

    PubMed

    Ammar, El-Desouky; Richardson, Matthew L; Abdo, Zaid; Hall, David G; Shatters, Robert G

    2014-01-01

    The Asian citrus psyllid (ACP, Diaphorina citri, Hemiptera: Liviidae), is the principal vector of the phloem-limited bacteria strongly associated with huanglongbing (HLB), the world's most serious disease of citrus. Host plant resistance may provide an environmentally safe and sustainable method of controlling ACP and/or HLB. Two xCitroncirus accessions (hybrids of Poncirus trifoliata and Citrus spp.), that are relatively resistant (UN-3881) or relatively susceptible (Troyer-1459) to ACP adults with regard to adult longevity, were compared in relation to ACP feeding behavior and some structural features of the leaf midrib. The settling (putative feeding/probing) sites of ACP adults on various parts of the leaf were not influenced primarily by plant accession. However, fewer ACP stylet sheaths were found in the midrib and fewer stylet sheath termini reached the vascular bundle (phloem and/or xylem) in UN-3881 compared to Troyer-1459 plants. Furthermore, in midribs of UN-3881 leaves the fibrous ring (sclerenchyma) around the phloem was significantly wider (thicker) compared to that in midribs of Troyer-1459 leaves. Our data indicate that feeding and/or probing by ACP adults into the vascular bundle is less frequent in the more resistant (UN-3881) than in the more susceptible (Troyer-1459) accessions. Our results also suggest that the thickness of the fibrous ring may be a barrier to stylet penetration into the vascular bundle, which is important for successful ACP feeding on the phloem and for transmitting HLB-associated bacteria. These results may help in the development of citrus plants resistant to ACP, which in turn could halt or slow the spread of the HLB-associated bacteria by this vector.

  17. Effect of methionine replacement by homocystine in cultures containing both malignant rat breast carcinosarcoma (Walker-256) cells and normal adult rat liver fibroblasts.

    PubMed

    Halpern, B C; Ezzell, R; Hardy, D N; Clark, B R; Ashe, H; Halpern, R M; Smith, R A

    1975-01-01

    When malignant W-256 rat breast carcinosarcoma cells are mixed with an equal number of normal adult rat liver fibroblasts and allowed to grow in a medium containing sufficient L-methionine and an excess of vitamin B12 and of folic acid, the malignant cells outgrow the normal cells, and within 2 weeks the tissue culture flasks contain only neoplastic cells. However, when ample DL-homocystine or homocysteine replaces methionine in the medium containing the same amount of vitamin B12 and folic acid, and seeded with the same type and number of malignant and normal cells, the malignant cells die and the normal cells thrive. Substantiating this conclusion are the results of injections into rats of comparable numbers of cells from each group after 3 weeks of growth in tissue culture. Fatal malignancies are produced by the homocystein-cultivated cells.

  18. [The experience of disease and treatments among teenagers and young adults with hematological malignancies].

    PubMed

    Polomeni, Alice

    2011-10-01

    We conducted an exploratory study of young patients (< 25 years) in remission from hematologic malignancies to better understand their experiences of care, the impact of disease and therapeutics on their quality of life (assessed by the FACT-BMT) and on their mental state (assessed by the HADS). On the plan of research, the findings of this study confirm the relevance of the qualitative approach; on the plan of care, the need of interdisciplinary support for these patients in remission.

  19. Hematologic malignancies

    SciTech Connect

    Hoogstraten, B.

    1986-01-01

    The principle aim of this book is to give practical guidelines to the modern treatment of the six important hematologic malignancies. Topics considered include the treatment of the chronic leukemias; acute leukemia in adults; the myeloproliferative disorders: polycythemia vera, essential thrombocythemia, and idiopathic myelofibrosis/agnogenic myeloid metaplasia; Hodgkin's Disease; non-Hodgkin's lymphoma; and Multiple Myeloma.

  20. Phase II Trial of Talampanel, a Glutamate Receptor Inhibitor, for Adults with Recurrent Malignant Gliomas

    PubMed Central

    Iwamoto, Fabio M.; Kreisl, Teri N.; Kim, Lyndon; Duic, J. Paul; Butman, John A.; Albert, Paul S.; Fine, Howard A.

    2010-01-01

    Background: Glioma cells secrete glutamate and also express AMPA glutamate receptors, which contribute to proliferation, migration and neurotoxicity of malignant gliomas. Talampanel is an oral AMPA receptor inhibitor with excellent CNS penetration and good tolerability in clinical trials for epilepsy and other neurological disorders. Methods: We conducted a phase II trial to evaluate the efficacy of talampanel in patients with recurrent malignant glioma as measured by 6-month progression free survival (PFS6). Results: Thirty patients (22 glioblastomas [GBM], 8 anaplastic gliomas [AG]; 63% men) with median age of 51 years (range, 20 to 67) and median KPS of 80 were included. Patients tolerated treatment well and most adverse events were mild and reversible; the most common toxicities were fatigue (27%), dizziness (23%) and ataxia (17%). There was only one partial response (5%) in the GBM stratum and none among AG patients. With a median follow-up of 13 months, 28 patients (93%) had died. The PFS6 was 4.6% for the initial 22 GBM patients and the study was terminated early due to treatment futility; PFS6 was 0% for 8 AG patients. Median PFS was 5.9 weeks for GBM and 8.9 weeks for AG patients. Median overall survival was 13 weeks for GBM and 14 months for AG patients. Conclusion: Talampanel was well tolerated but had no significant activity as a single agent in unselected recurrent malignant gliomas. PMID:20143438

  1. Intraorbital metastasis from solitary fibrous tumor.

    PubMed

    Patel, Mrinali M; Jakobiec, Frederick A; Zakka, Fouad R; Du, Rose; Annino, Donald J; Borboli-Gerogiannis, Sheila; Daniels, Anthony B

    2013-01-01

    Solitary fibrous tumor (SFT) is a rare spindle cell tumor of mesenchymal origin that usually arises from pleura or pericardium but can also arise from many extraserosal sites. Although more than 50 cases of primary SFT of the orbit have been reported, there are no reports to date of a malignant nonophthalmic SFT metastasizing in the orbital soft tissues (although sphenoid wing bony involvement has been reported). The authors report here the first case of a patient with intraorbital metastasis of a CD34-positive malignant SFT. The patient was a 57-year-old man with a history of malignant pleural SFT and a prior kidney metastasis. He presented with the rapid appearance of proptosis and massive conjunctival chemosis preventing eyelid closure, and he was found to have a well-circumscribed metastasis to his lateral rectus muscle. Surgical excision cured his ocular symptoms, although he died 3 months later from brain and widespread metastases.

  2. Solitary fibrous tumor of the kidney. Case report.

    PubMed

    Bozkurt, Suheyla Uyar; Ahiskali, Rengin; Kaya, Handan; Demir, Aslan; Ilker, Yalcin

    2007-03-01

    Solitary fibrous tumors are rare spindle cell neoplasms usually arising in the pleura. They have, however, also been reported at extrapleural locations. Solitary fibrous tumor (SFT) of the kidney is rare. Despite its rarity, histological diagnosis of solitary fibrous tumor is crucial to avoid misdiagnosis with other more aggressive tumors arising in the kidney. We report a solitary fibrous tumor of the left kidney that presented as a malignant tumor in a 51-year-old woman, and include clinical and radiographic findings. The tumor was well circumscribed and composed of spindle cells in a collagenous stroma. Immunohistochemistry showed reactivity for vimentin, CD 34, BCL-2 protein and CD99. Immunohistochemical stains for cytokeratin, S-100, desmin, alpha-smooth muscle actin and HMB-45 were negative. A diagnosis of SFT was made based on light microscopy and immunohistochemistry.

  3. A case report of an intracaval extrathoracic solitary fibrous tumour

    PubMed Central

    Tiong, HY; Wang, S; Madhavan, K

    2013-01-01

    Solitary fibrous tumours are infrequent neoplasms based in the pleura that are predominantly benign with malignant pathology and behaviour described in 10–36% of cases. Extrathoracic solitary fibrous tumours (ESFTs) have been considered separately to their intrathoracic counterparts and comprise a third of all solitary fibrous tumours. The extrathoracic location was identified as an adverse prognostic factor for local recurrence but not for metastatic disease. So far, there have not been any reports of solitary fibrous tumours demonstrating caval infiltration. We present a case of a benign ESFT infiltrating into the perirenal inferior vena cava. Together with extrauterine leiomyomas, ESFTs should also be considered as a differential diagnosis for the rare benign lesions invading the inferior vena cava. PMID:23676804

  4. Pediatric Salivary Gland Malignancies.

    PubMed

    Ord, Robert A; Carlson, Eric R

    2016-02-01

    Pediatric malignant salivary gland tumors are extremely rare. The percentage of malignant tumors is higher than that seen in adults, although the outcomes in terms of survival are better in pediatric patients. The mainstay of treatment is surgical excision with negative margins. This article reviews current concepts in demographics, etiology, management, and outcomes of malignant salivary tumors in children.

  5. Mesothelioma - benign-fibrous

    MedlinePlus

    ... fibroma; Solitary fibrous tumor of the pleura Images Respiratory system References Broaddus VC, Robinson BWS. Tumors of the pleura. In: Mason RJ, Broaddus VC, Martin TR, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine . 5th ed. Philadelphia, PA: Elsevier Saunders; 2010: ...

  6. Gastric calcifying fibrous tumour

    PubMed Central

    Attila, Tan; Chen, Dean; Gardiner, Geoffrey W; Ptak, Theadore W; Marcon, Norman E

    2006-01-01

    Intramucosal gastric tumours are most commonly found to be gastrointestinal stromal tumours or leiomyomas (smooth muscle tumours); however, a variety of other uncommon mesenchymal tumours can occur in the stomach wall. A rare benign calcifying fibrous tumour is reported and the endoscopic appearance, ultrasound findings and morphology are documented. A review of the literature found only two similar cases. PMID:16858502

  7. Solitary fibrous tumor of the skin.

    PubMed

    Cowper, S E; Kilpatrick, T; Proper, S; Morgan, M B

    1999-06-01

    Solitary fibrous tumor (SFT) is an uncommon mesenchymal tumor that typically arises in the pleural cavity. Comprised of spindled cells characteristically arranged in diverse architectural patterns, SFT histologically simulates a variety of benign and malignant mesenchymal tumors. The diagnosis of SFT has been refined by the availability of newer immunohistochemical markers such as CD-34 and factor XIIIa, facilitating the identification of SFTs arising in multiple extrapleural sites, including the skin. We describe three cases of primary cutaneous SFT, review the literature, and discuss the histologic and immunohistochemical differential of other cutaneous tumors that SFT can mimic.

  8. [Retroperitoneal solitary fibrous tumor: a case report].

    PubMed

    Nukui, Akinori; Ochi, Masanori; Suzuki, Kazumi; Yuzawa, Masayuki; Morita, Tatsuo

    2009-08-01

    A 63-year-old man with a retroperitoneal tumor found incidentally was referred to our hospital. Computed tomography showed a tumor ventrally adjacent to urinary bladder and prostate. Pathological examination of retroperitoneal tumor specimens obtained by percutaneous needle biopsy revealed hypercellularity of spindle cells positive for CD 34. Under the suspicion of solitary fibrous tumor (SFT) or stromal tumors of uncertain malignant potential (STUMP), we performed en bloc resection of tumor, urinary bladder and prostate because tumor was firmly fixed to urinary bladder and prostate. The final diagnosis of retroperitoneal tumor was SFT because pathological findings of the surgical specimen were the same as those of the biopsy specimens.

  9. [Extraconal solitary fibrous tumor of the orbit].

    PubMed

    von Lovenberg, E; Kedziora, O; Wolf, H K; Bankfalvi, A

    2013-04-01

    Solitary fibrous tumors (SFT) are rare spindle cell neoplasms derived from specialized fibroblasts. This tumor was first described in the pleura and later in the whole body including the orbit. Although an SFT is generally a benign tumor malignant transformation and metastasization have also been observed in a few cases. Complete excision is the therapy of choice. Here we report on a 50-year-old male patient whose orbital SFT was removed by transconjunctival anterior orbitotomy and 1.5 years after the operation the patient is recurrence and complaint-free.

  10. Chondroblastoma of the acromion mimicking fibrous dysplasia.

    PubMed

    Gebert, Carsten; Hardes, Jendrik; Streitbürger, Arne; Vieth, Volker; Bürger, Horst; Winkelmann, Winfried; Gosheger, Georg

    2004-12-01

    The authors report the case of a 65-year-old man who presented with an expansive osteolytic lesion in the right acromion, mimicking cystic fibrous dysplasia. Magnetic resonance imaging showed a lesion with intermediate-signal intensity on T1-weighted images and a high-signal intensity on fat suppressed T2-weighted images. The biopsy led to the diagnosis of chondroblastoma. This tumour is rare in flat bones, and may mimic other benign or malignant lesions. It is therefore essential to perform a biopsy in order to obtain a definite diagnosis. The acromion was excised, and replaced with an iliac crest graft. PMID:15669467

  11. Solitary Fibrous Tumor of the Sigmoid Colon Masquerading as an Adnexal Neoplasm

    PubMed Central

    Bratton, Laura; Salloum, Rabih; Cao, Wenqing

    2016-01-01

    Solitary fibrous tumor is a rare, benign spindle cell neoplasm that was first described in the thoracic pleura. This tumor is now known to occur at many extrapleural sites. There are established criteria for the diagnosis of malignant solitary fibrous tumor including ≥4 mitotic figures per 10 high-power fields, increased cellularity, cytologic atypia, infiltrative margins, and/or necrosis. Although all solitary fibrous tumors have the potential to recur or metastasize, those with malignant histologic features tend to behave more aggressively. We report a case of solitary fibrous tumor, with malignant histologic features, in a 21-year-old woman which arose from the serosal surface of the sigmoid colon. PMID:27672467

  12. Solitary Fibrous Tumor of the Sigmoid Colon Masquerading as an Adnexal Neoplasm.

    PubMed

    Bratton, Laura; Salloum, Rabih; Cao, Wenqing; Huber, Aaron R

    2016-01-01

    Solitary fibrous tumor is a rare, benign spindle cell neoplasm that was first described in the thoracic pleura. This tumor is now known to occur at many extrapleural sites. There are established criteria for the diagnosis of malignant solitary fibrous tumor including ≥4 mitotic figures per 10 high-power fields, increased cellularity, cytologic atypia, infiltrative margins, and/or necrosis. Although all solitary fibrous tumors have the potential to recur or metastasize, those with malignant histologic features tend to behave more aggressively. We report a case of solitary fibrous tumor, with malignant histologic features, in a 21-year-old woman which arose from the serosal surface of the sigmoid colon. PMID:27672467

  13. [About a case of calcifying fibrous tumor of the pleura].

    PubMed

    Rocas, Delphine; Thivolet-Béjui, Françoise; Tronc, François; Chalabreysse, Lara

    2015-12-01

    Calcifying fibrous tumor is a rare soft tissue benign tumor (OMS 2002). Some pleural localisations are described, which affect slightly older individuals than the other soft tissue forms. The calcifying fibrous tumor is included in the 2004 World Health Organization classification of pleural tumors. A pleural tumor located in the right inferior pulmonary lobe is diagnosed in a 59-year-old man. This pleural tumor is macroscopically well-circumscribed. Histologically, the rare spindle tumoral cells are located between bundles of a collagenous tissue, sometimes hyalinized, with psammomatous or dystrophic calcifications. The tumoral cells have a fibrohistiocytic origin. They stain positively for antibodies against vimentin, factor XIIIa, CD68, CD163, CD34. Antibodies against smooth muscle actin, desmin, PS100, ALK1 and EBV are negative. Main differencial diagnoses are other benign pleural tumors (solitary fibrous tumor, inflammatory myofibroblastique tumor), some malignant tumors (desmoplastic malignant pleural mesothelioma) and pleural pseudotumors (calcified pleural plaques, chronic fibrous pleuritis, amylose, hyalinizing granuloma). Our case is the 15th pleural calcifying fibrous tumor being reported. PMID:26608111

  14. Recurrent solitary fibrous tumor of the pleura with malignant transformation and non-islet cell tumor-induced hypoglycemia due to paraneoplastic overexpression and secretion of high-molecular-weight insulin-like growth factor II.

    PubMed

    Tominaga, Naoto; Kawarasaki, Chiaki; Kanemoto, Keiko; Yokochi, Akio; Sugino, Keishi; Hatanaka, Kazuhito; Uekusa, Toshimasa; Fukuda, Izumi; Aiba, Motohiko; Hizuka, Naomi; Uda, Susumu

    2012-01-01

    A 41-year-old man was diagnosed with a solitary fibrous tumor (SFT) of the pleura in the posterior mediastinum. Despite two surgeries for excision, the SFT recurred and progressed with direct invasion of the chest wall and bone metastases. He was hospitalized because of cerebral infarction and presented with recurrent severe hypoglycemia fourteen years later. High-molecular-weight (HMW) insulin-like growth factor II (IGF-II) was identified in the serum and tumor using Western blotting and immunohistochemistry. These findings suggested that the cause of the recurrent severe hypoglycemia was SFT production of HMW IGF-II, a mediator of non-islet cell tumor-induced hypoglycemia (NICTH).

  15. Advances in conditioning regimens for older adults undergoing allogeneic stem cell transplantation to treat hematologic malignancies.

    PubMed

    William, Basem M; de Lima, Marcos

    2013-06-01

    Allogeneic stem cell transplantation (SCT) is a potentially curative treatment for patients with hematological malignancies. These diseases, however, have their peak incidence in the sixth to eighth decades of life. Historically, elderly patients have been considered unsuitable candidates for SCT because of high treatment-related mortality (TRM). Over the past 15 years, the use of reduced-intensity conditioning (RIC) regimens before SCT has allowed patients in the sixth and seventh decades of life to be routinely transplanted. Despite major differences among transplant centers in the intensity and composition of the conditioning regimen and immunosuppression, choice of graft source, postgraft immunomodulation, and supportive care, there has been a dramatic decrease in TRM, allowing safer delivery of SCT. Major obstacles to SCT in elderly patients include donor availability, graft-versus-host disease, delayed immune recovery, multiple comorbidities, and chemo refractoriness. Here we review the current results of SCT in elderly patients, focusing on the role of RIC, and using myeloid diseases as the model for discussion.

  16. Synchronous solitary fibrous tumor of the pleura and lung cancer.

    PubMed

    Watanabe, Shun-Ichi; Nakamura, Yoshihiro; Sakasegawa, Koh-Ichi; Kariatsumari, Kota; Yotsumoto, Daisuke; Sakata, Ryuzo; Gezima, Kentaro

    2003-01-01

    We report herein on a 57-year-old woman with comorbid malignant solitary fibrous tumor (SFT) of the pleura and adenocarcinoma of the lung. To the best of our knowledge, this is the first report of a patient presenting with these two pathological entities simultaneously. The patient was treated successfully for both diseases via a one-stage operation through median sternotomy with good results. Although the incidence of multiple primary malignancy is rare, clinicians should be cautious not to discount the possibility of two coexisting primary malignancies.

  17. Solitary fibrous tumor of the mediastinum.

    PubMed

    Weidner, N

    1991-01-01

    A 62-year-old woman presented with an asymptomatic anterior mediastinal mass. Clinically considered to be a thymoma, the tumor was solid, firm, and composed of hypocellular dense collagen and cytologically bland, spindled fibroblastlike cells growing in a patternless pattern. There was no apparent connection to pleura or pericardium, yet the clinicopathologic features clearly fit with solitary fibrous tumor (SFT) of mediastinum. SFTs occur most commonly in pleura but have been reported in other locations, including the mediastinum, where aggressive behavior has been more common when these tumors are compared to those occurring in pleura. Although it is difficult to predict behavior for all cases of SFT occurring in the mediastinum by cytologic features alone, morphologic criteria for benign and malignant forms have been described. Roughly half the malignant forms will progress, yet the single most important indicator of clinical outcome is whether the tumor can be initially totally excised.

  18. Subcutaneous solitary fibrous tumor.

    PubMed

    Yoshida, Yuichi; Kubota, Yumiko; Yamaguchi, Takahiro; Iwasaki, Hiroshi; Nakayama, Juichiro

    2004-12-01

    We describe a unique case of subcutaneous solitary fibrous tumor (SFT) in a 56-year-old female patient. The patient had been aware of a painless soft mass in her back for ten years. The lesion was surgically excised. Histological examination revealed that the well-defined mass was composed of a proliferation of spindle-shaped fibroblastic cells and polygonal cells embedded in a fibrous matrix corresponding to the so-called "patternless pattern". A prominent pericytomatous pattern (hemangiopericytoma-like structures), focal myxoid changes, and thick hyalinized collagen fibers were also observed. Immunohistochemical stainings for CD34 and bcl-2 were positive in the tumor cells. These features are compatible with SFT. We suggest that SFT should be included in the differential diagnosis of subcutaneous spindle cell tumors.

  19. [Intrapulmonary Solitary Fibrous Tumor].

    PubMed

    Komori, Kazuyuki; Tabata, Toshiharu; Katsumata, Hiroshi; Minowa, Muneo; Fujimura, Shigefumi

    2015-08-01

    We report a case of intrapulmonary solitary fibrous tumor( SFT). A 34-year-old woman was referred to our hospital due to an abnormal shadow on a chest roentgenogram without symptom. Computed tomography showed a circumscribed intrapulmonary tumor with mild uptake on fluorodeoxyglucose (FDG)-positron emission tomography( PET) in the left lower lobe( S6). Frozen examination revealed a mesenchymal tumor. Based on the pathological and immunohistochemical findings, the tumor was diagnosed as intrapulmonary SFT.

  20. [Intrapulmonary Solitary Fibrous Tumor].

    PubMed

    Komori, Kazuyuki; Tabata, Toshiharu; Katsumata, Hiroshi; Minowa, Muneo; Fujimura, Shigefumi

    2015-08-01

    We report a case of intrapulmonary solitary fibrous tumor( SFT). A 34-year-old woman was referred to our hospital due to an abnormal shadow on a chest roentgenogram without symptom. Computed tomography showed a circumscribed intrapulmonary tumor with mild uptake on fluorodeoxyglucose (FDG)-positron emission tomography( PET) in the left lower lobe( S6). Frozen examination revealed a mesenchymal tumor. Based on the pathological and immunohistochemical findings, the tumor was diagnosed as intrapulmonary SFT. PMID:26329708

  1. Solitary fibrous tumour of thyroid: report of two cases with immunohistochemical features and literature review.

    PubMed

    Santeusanio, Giuseppe; Schiaroli, Stefania; Ortenzi, Angela; Mulè, Antonino; Perrone, Giuseppe; Fadda, Guido

    2008-09-01

    Solitary fibrous tumour (SFT) is a rare tumour principally found in adults in the pleural cavity. Extrapleural occurrences are rare. Two cases of SFT of the thyroid gland are described in this paper showing their distinctive microscopical architecture, namely "patternless growth pattern". It is characterized by a bland spindle-cell proliferation alternating hyper- and hypo-cellular areas, keloid-like hyalinization and a focal hemangiopericytoma-like vascular pattern. Tumour cells revealed a diffuse strong positivity for CD34, CD99, bcl-2 and Vimentin, but negativity for Desmin, EMA, AE1/AE3, SMA, S-100 and CD31 antibodies. The differential diagnosis of thyroid SFT includes different types of spindle cell proliferation, benign and malignant mesenchymal tumours, medullary thyroid carcinoma, fasciitis-like papillary carcinoma, and undifferentiated (anaplastic) carcinoma. However, the morphologic and immunohistochemical findings of SFT are so characteristic that this diagnosis seldom represent a difficulty.

  2. Effects of Allogeneic Hematopoietic Stem Cell Transplantation Plus Thymus Transplantation on Malignant Tumors: Comparison Between Fetal, Newborn, and Adult Mice

    PubMed Central

    Zhang, Yuming; Hosaka, Naoki; Cui, Yunze; Shi, Ming

    2011-01-01

    We have recently shown that allogeneic intrabone marrow–bone marrow transplantation + adult thymus transplantation (TT) is effective for hosts with malignant tumors. However, since thymic and hematopoietic cell functions differ with age, the most effective age for such intervention needed to be determined. We performed hematopoietic stem cell transplantation (HSCT) using the intrabone marrow method with or without TT from fetal, newborn, and adult B6 mice (H-2b) into BALB/c mice (H-2d) bearing Meth-A sarcoma (H-2d). The mice treated with all types of HSCT + TT showed more pronounced regression and longer survival than those treated with HSCT alone in all age groups. Those treated with HSCT + TT showed increased numbers of CD4+ and CD8+ T cells but decreased numbers of Gr-1/Mac-1 myeloid suppressor cells and decreased percentages of FoxP3 cells in CD4+ T cells, compared with those treated with HSCT alone. In all mice, those treated with fetal liver cell (as fetal HSCs) transplantation + fetal TT or with newborn liver cell (as newborn HSCs) transplantation (NLT) + newborn TT (NTT) showed the most regression, and the latter showed the longest survival. The number of Gr-1/Mac-1 cells was the lowest, whereas the percentage of CD62L−CD44+ effector memory T cells and the production of interferon γ (IFN-γ) were highest in the mice treated with NLT + NTT. These findings indicate that, at any age, HSCT + TT is more effective against cancer than HSCT alone and that NLT + NTT is most effective. PMID:20672991

  3. Hepatic solitary fibrous tumor: report of a rare case.

    PubMed

    Patra, Sushma; Vij, Mukul; Venugopal, K; Rela, Mohamed

    2012-01-01

    Solitary fibrous tumor (SFT) of the liver is an extremely rare neoplasm of mesenchymal origin. In the English literature, less than 40 cases of SFTs of the liver have been reported. The present case concerns a 34-year-old female who presented to us with complaint of dyspepsia. On examination, there was hepatomegaly. On ultrasound examination, an SOL in the liver was detected. Large tumor measuring 14.5 × 10 × 8 cm was resected. Microscopic evaluation of the tumor showed a well-circumscribed, low to moderately cellular tumor demonstrating spindle- and fibroblast-like cells within the collagenous stroma. Immunohistochemistry revealed diffuse strong cytoplasmic immunopositivity of CD34, Bcl2, and vimentin. A diagnosis of a benign SFT was given. The patient remained well 4 years after surgery. SFT is a rare mesenchymal neoplasm that occasionally involves the liver in adult patients. Most SFTs are benign, but some may have malignant histological features. With less than 40 reported cases in the literature, little can be said regarding its natural history or the benefits of adjuvant radio chemotherapy. Complete surgical resection remains the cornerstone of its treatment.

  4. Haploidentical hematopoietic stem cell transplantation to adults with hematologic malignancies: analysis of 66 cases at a single Japanese center.

    PubMed

    Kurokawa, Toshiro; Ishiyama, Ken; Ozaki, Jun; Yamashita, Yumiko; Iwaki, Noriko; Saito, Chizuru; Arahata, Masahisa; Kaya, Hiroyasu; Yoshida, Takashi

    2010-05-01

    Sixty-six adult patients with hematologic malignancies underwent haploidentical hematopoietic stem cell transplantation (haplo-HSCT) without T cell depletion. The patients were preconditioned with a reduced intensity regimen, and tacrolimus was used for graft-versus-host disease (GVHD) prophylaxis. Successful engraftment occurred in 60 patients (90.1%) and graft rejection in only 4 patients (6.1%). Among the 60 engrafted patients, only 5 developed severe (grade III or IV) acute GVHD. Twenty patients, including 19 relapse-free patients were alive at a median follow-up of 48 months (range 6-77 months). The overall survival (OS) at 6 years was 29.3%. The OS of 45 patients < 60 years of age was 43.6%, which was superior to that of 21 patients who were 60 years of age and older (9.5%) (P < 0.01). The OS of 11 patients from human leukocyte antigen (HLA) 1 locus-mismatched donors (63.6%) was higher than that of 28 patients from HLA 3 loci-mismatched donors (12.5%) (P < 0.01). Organ injury and infection were the main causes of mortality. Notably, immunosuppressive therapy could be successfully stopped in 9 patients transplanted from HLA 2 or 3 loci-mismatched donors with a median duration of 45 months (range 5-71 months). These data suggest that haplo-HSCT is a promising treatment for patients who need urgent allogeneic transplantation but lack HLA-identical family donors.

  5. Craniofacial fibrous dysplasia.

    PubMed

    Ricalde, Pat; Magliocca, Kelly R; Lee, Janice S

    2012-08-01

    Despite recent advances in the understanding of the natural history and molecular abnormalities, many questions remain surrounding the progression and management of fibrous dysplasia (FD). In the absence of comorbidities, the expected behavior of craniofacial FD (CFD) is to be slow growing and without functional consequence. Understanding of the pathophysiologic mechanisms contributing to the various phenotypes of this condition, as well as the predictors of the different behaviors of FD lesions, must be improved. Long-term follow-up of patients with CFD is vital because spontaneous recovery is unlikely, and the course of disease can be unpredictable. PMID:22771278

  6. Intramasseteric solitary fibrous tumor.

    PubMed

    Dogan, Ersoy; Erdag, Taner Kemal; Ikiz, Ahmet Omer; Guneri, Ataman; Sarioglu, Sulen

    2013-03-01

    Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm that usually arises from the pleura. SFTs occurring within the head and neck region are uncommon. Recently, it has been described in various head and neck sites such as oral cavity, nasal cavity, paranasal sinuses, salivary glands, thyroid, buccal space, and larynx. Here, we report a case of SFT originating in the masseter muscle of a 27-year-old woman. To our knowledge, this is the first description of a SFT of the head and neck region, arising within the masseter muscle. We present the clinical history, radiologic and histopathologic findings as well as immunoreactivity of this tumor.

  7. Solitary fibrous tumors: loss of chimeric protein expression and genomic instability mark dedifferentiation.

    PubMed

    Dagrada, Gian P; Spagnuolo, Rosalin D; Mauro, Valentina; Tamborini, Elena; Cesana, Luca; Gronchi, Alessandro; Stacchiotti, Silvia; Pierotti, Marco A; Negri, Tiziana; Pilotti, Silvana

    2015-08-01

    Solitary fibrous tumors, which are characterized by their broad morphological spectrum and unpredictable behavior, are rare mesenchymal neoplasias that are currently divided into three main variants that have the NAB2-STAT6 gene fusion as their unifying molecular lesion: usual, malignant and dedifferentiated solitary fibrous tumors. The aims of this study were to validate molecular and immunohistochemical/biochemical approaches to diagnose the range of solitary fibrous tumors by focusing on the dedifferentiated variant, and to reveal the genetic events associated with dedifferentiation by integrating the findings of array comparative genomic hybridization. We studied 29 usual, malignant and dedifferentiated solitary fibrous tumors from 24 patients (including paired samples from five patients whose tumors progressed to the dedifferentiated form) by means of STAT6 immunohistochemistry and (when frozen material was available) reverse-transcriptase polymerase chain reaction and biochemistry. In addition, the array comparative genomic hybridization findings were used to profile 12 tumors from nine patients. The NAB2/STAT6 fusion was detected in all of the tumors, but immunohistochemistry and western blotting indicated that chimeric protein expression was atypical or absent in 9 out of 11 dedifferentiated tumors. The comparative genomic hybridization results revealed that the usual and malignant solitary fibrous tumors had a simple profile, whereas the genome of the dedifferentiated tumors was complex and unstable, and suggested that 13q and 17p deletions and TP53 mutations may be present in malignant lesions before the full expression of a dedifferentiated phenotype. Solitary fibrous tumor dedifferentiation is associated with the loss of chimeric oncoprotein expression, genomic instability, and cell decommitment and reprogramming. The assessment of dedifferentiated solitary fibrous tumors is based on the presence of the fusion transcripts and, in principle, negative

  8. Giant localized fibrous tumours of the pleura: report of three subsequent cases.

    PubMed

    Breda, Cristiano; Zuin, Andrea; Marulli, Giuseppe; Galligioni, Alessandra; Rea, Federico

    2006-05-01

    Localized fibrous tumours of the pleura (LFTP) represent clinical entities rarely encountered, especially in giant forms. We report of three cases of giant localized fibrous tumours of the pleura found last year in a short time. The patients underwent surgery by thoracotomy. All tumours arose from visceral pleura and were radically excised by a wedge resection of the lung. The patients discharged from the hospital after a short postoperative period without complications. Two of three giant localized fibrous tumours were classified as benign forms; in one case a malignant characteristic was found. PMID:16580088

  9. Recent insights into the molecular mechanisms involved in aging and the malignant transformation of adult stem/progenitor cells and their therapeutic implications.

    PubMed

    Mimeault, Murielle; Batra, Surinder K

    2009-04-01

    Recent advancements in tissue-resident adult stem/progenitor cell research have revealed that enhanced telomere attrition, oxidative stress, ultraviolet radiation exposure and oncogenic events leading to severe DNA damages and genomic instability may occur in these immature and regenerative cells during chronological aging. Particularly, the alterations in key signaling components controlling their self-renewal capacity and an up-regulation of tumor suppressor gene products such as p16(INK4A), p19(ARF), ataxia-telangiectasia mutated (ATM) kinase, p53 and/or the forkhead box O (FOXOs) family of transcription factors may result in their dysfunctions, growth arrest and senescence or apoptotic death during the aging process. These molecular events may culminate in a progressive decline in the regenerative functions and the number of tissue-resident adult stem/progenitor cells, and age-related disease development. Conversely, the telomerase re-activation and accumulation of numerous genetic and/or epigenetic alterations in adult stem/progenitor cells with advancing age may result in their immortalization and malignant transformation into highly leukemic or tumorigenic cancer-initiating cells and cancer initiation. Therefore, the cell-replacement and gene therapies and molecular targeting of aged and dysfunctional adult stem/progenitor cells including their malignant counterpart, cancer-initiating cells, hold great promise for treating and even curing diverse devastating human diseases. These diseases include premature aging diseases, hematopoietic, cardiovascular, musculoskeletal, pulmonary, ocular, urogenital, neurodegenerative and skin disorders and aggressive and recurrent cancers.

  10. Laryngeal solitary fibrous tumour.

    PubMed

    Stomeo, Francesco; Padovani, Davide; Bozzo, Corrado; Pastore, Antonio

    2007-09-01

    Solitary fibrous tumours (SFT) are rare neoplasms, with an uncommon laryngeal involvement. Only five cases of laryngeal localization have been described in literature. The following is a case of a 75-year-old man with a supraglottic neoplasm of the larynx; after the biopsy immunohistochemical study demonstrated a strong positivity for vimentin, CD34 and Bcl-2. The neoplasm was consequently classified as a SFT. CO(2) laser surgery of the supraglottic larynx, with a wide excision of the neoplasm, was performed. Twenty-four months on, the patient is alive, well and free of disease. Surgical resection is the treatment of choice for laryngeal SFT, but tumour-free resection margins must be achieved to prevent the possibility of local recurrence. Endoscopic resection by means of the CO(2) laser must be accurately planned with MRI or CT imaging to confirm of this kind of surgery.

  11. Solitary fibrous tumor.

    PubMed

    Yilmaz, Cem; Kabatas, Serdar; Ozen, Ozlem Isiksacan; Gulsen, Salih; Caner, Hakan; Altinors, Nur

    2009-12-01

    Intracranial solitary fibrous tumors (SFT) are typically dural based, CD34-positive neoplasms of mesenchymal origin. Since they were first described in 1996 at the meninges, fewer than 100 SFT had been reported in both cranial and spinal compartments of the central nervous system. SFT can resemble other spindle cell tumors both radiologically and histopathologically, and differentiation can be best achieved through viewing their ultrastructure and using immunohistochemical techniques. In this report, we present four patients with SFT. Upon diagnosing two patients with SFT located in the cerebellopontine angle and parasagittal areas, we reviewed our pathological files and found two more patients; one having a parasagittal tumor and the other having a convexity tumor, that had been diagnosed with hemangiopericytoma. These tumours proved to be SFT after an immunohistochemical re-examination.

  12. Solitary fibrous tumor.

    PubMed

    Bruzzone, Andrea; Varaldo, Marco; Ferrarazzo, Claudia; Tunesi, Gianni; Mencoboni, Manlio

    2010-01-01

    Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm which may be found everywhere in the body. It is now distinguished into two forms, pleural and extrapleural, which morphologically resemble each other. Abdominal localizations are quite rare, with 10 cases only reported in bladder; rarely they can be source of paraneoplastic syndromes (i.e., hypoglycemia secondary to insulin-like growth factor). In April 2006 a 74-year-old white male presented with chills, diaphoresis and acute abdominal pain with hematuria. At admission in emergency he underwent an abdominal Xray (no pathological findings) and an ultrasound examination of the kidneys and urinary tract, which revealed a pelvic hyperechogenic neoformation measuring approximately 10×8×7 cm, compressing the bladder. Blood chemistry at admission revealed only a mild neutrophilic leucocytosis (WBC 16600, N 80%, L 11%), elevated fibrinogen and ESR, and hypoglycemia (38 mg/dL). Macro scopic hematuria was evident, while urinocolture was negative. Contrast enhanced CT scan of the abdomen and pelvic region revealed a large round neoformation dislocating the bladder, with an evident contrast-enhanced periphery and a central necrotic area. Continuous infusion of glucose 5% solution was necessary in order to maintain blood glucose levels above 50 mg/dL. The patient underwent complete surgical resection of an ovoidal mass coated by adipose tissue, with well delimited margins; histological findings were consistent with solitary fibrous tumor (SFT). Hypoglycemia resolved completely with removal of the growth. In this case report we describe a SFT growing in the bladder, a quite rare localization, which presented a unique hypoglycemia. In contrast to the majority of cases reported in the literature, the behavior of this SFT was not aggressive, and, since the patient is still alive, surgical resection was considered conclusive.

  13. Giant Solitary Fibrous Tumor of Orbit.

    PubMed

    Tenekeci, Goktekin; Sari, Alper; Vayisoglu, Yusuf; Serin, Onur

    2015-07-01

    Solitary fibrous tumors (SFTs) have been reported in various locations in the body. Solitary fibrous tumors are extremely rare tumors, especially when located in the orbit. Diagnosis of SFT cannot be made based on histopathology only because it exhibits a variable microscopic appearance, and necessitates immunohistochemistry to confirm the diagnosis. A 51-year-old man was admitted to our clinic for the evaluation of a mass bulging in his left eye. Clinical examination revealed a painless mass extruding out of the orbital cavity with dimensions of 8 × 7  cm. Exenteration of the left eye including the upper and lower eyelid and reconstruction of the orbital cavity using a temporoparietal fascia flap and a temporal muscle flap was performed. SFT of orbital region is known as a slow growing and painless tumor. Based on previous studies, increased mitotic rate of the tumor gives the impression that the tumor has a malignant nature. Until now a small number or orbital SFTs were reported and none of them presented with a giant mass protruding out of the orbital cavity. We present a unique case of orbital SFT filling the whole orbital cavity and protruding outward as a giant mass. This case has been reported to expand our knowledge in this debated entity. PMID:26102546

  14. Giant Solitary Fibrous Tumor of Orbit.

    PubMed

    Tenekeci, Goktekin; Sari, Alper; Vayisoglu, Yusuf; Serin, Onur

    2015-07-01

    Solitary fibrous tumors (SFTs) have been reported in various locations in the body. Solitary fibrous tumors are extremely rare tumors, especially when located in the orbit. Diagnosis of SFT cannot be made based on histopathology only because it exhibits a variable microscopic appearance, and necessitates immunohistochemistry to confirm the diagnosis. A 51-year-old man was admitted to our clinic for the evaluation of a mass bulging in his left eye. Clinical examination revealed a painless mass extruding out of the orbital cavity with dimensions of 8 × 7  cm. Exenteration of the left eye including the upper and lower eyelid and reconstruction of the orbital cavity using a temporoparietal fascia flap and a temporal muscle flap was performed. SFT of orbital region is known as a slow growing and painless tumor. Based on previous studies, increased mitotic rate of the tumor gives the impression that the tumor has a malignant nature. Until now a small number or orbital SFTs were reported and none of them presented with a giant mass protruding out of the orbital cavity. We present a unique case of orbital SFT filling the whole orbital cavity and protruding outward as a giant mass. This case has been reported to expand our knowledge in this debated entity.

  15. Computed tomography of fibrous dysplasia

    SciTech Connect

    Daffner, R.H.; Kirks, D.R.; Gehweiler, J.A. Jr.; Heaston, D.K.

    1982-11-01

    Skeletal fibrous dysplasia produces changes that are usually readily recognized on plain radiographs. Occasionally, routine radiography may not demonstrate the characteristic appearance of the disease. The density of abormal bone in craniofacial fibrous dysplasia may preclude adequate assessment of areas where soft-tissue impingement may occur. Computed tomography (CT) is useful in demonstrating the amorphous ''ground-glass'' texture of the lesion and in defining the extent of craniofacial disease including impingement upon orbital structures. CT was useful in five patients with fibrous dysplasia in whom the nature or extent of involvement was not entirely clear.

  16. Solitary fibrous tumor of the mediastinum.

    PubMed

    Suehisa, Hiroshi; Yamashita, Motohiro; Komori, Eisaku; Sawada, Shigeki; Teramoto, Norihiro

    2010-04-01

    An 18-year-old man was referred to our hospital for further evaluation of a right anterior mediastinal tumor that measured 6 cm in diameter. Computed tomography-guided transcutaneous aspiration biopsy was performed, but no definitive diagnosis could be obtained. Because the tumor did not appear to be a high-grade malignant tumor, we undertook resection of the tumor to obtain a definitive diagnosis and provide appropriate treatment. Total thymectomy with tumor resection was performed through a median sternotomy. The tumor was solid, measuring 5.2 x 4.2 x 3.5 cm. The histological diagnosis was solitary fibrous tumor (SFT) arising from the mediastinum. Most extrathoracic SFTs appear to pursue a benign course, although careful long-term follow-up of these patients is necessary because the tumors have been reported to recur or metastasize in some cases.

  17. [Solitary fibrous tumor of the liver].

    PubMed

    Lehmann, C; Mourra, N; Tubiana, J-M; Arrivé, L

    2006-02-01

    Solitary fibrous tumor (SFT) is commonly found on serosal surfaces, and is rarely localized in the liver. There are benign and malignant variants of hepatic SFT. We report a new case of benign SFT. Our patient, a 63-year old woman, who has been followed for 5 years for an asymptomatic liver mass, was admitted for abdominal pain. Ultrasonography (US), CT, MR Imaging and angiography showed the liver mass with typical imaging features, situated in the right hepatic lobe with blood supply from the hepatic artery. Histopathological examination demonstrated a highly vascularized tumor, composed of short spindle cells alternating with hypocellular collagenous regions, with a hemangiopericytoma-like vascular pattern. The immunohistochemical staining was positive for CD 34. Tumor resection was performed. Follow-up 8 years after the resection showed no tumor recurrence or metastasis, thus confirming the initial diagnosis of benign SFT.

  18. Case of retroperitoneal solitary fibrous tumor.

    PubMed

    Yamashita, Shinichi; Tochigi, Tatsuo; Kawamura, Sadafumi; Aoki, Hiroshi; Tateno, Hiroo; Kuwahara, Masaaki

    2007-07-01

    Solitary fibrous tumor (SFT) of the retroperitoneal space is rare. We report a case of retroperitoneal tumor, diagnosed as SFT. A 69-year-old woman presented with right lower abdominal swelling, and was referred to our hospital with suspicion of right renal tumor. Abdominal ultrasound and computerized tomography (CT) showed a mass (about 15 x 14 x 10 cm) in the right abdomen. The tumor was thought to be right renal rumor, and right radical nephrectomy was performed. In the excised specimen the tumor was not connected to gastrointestinal tract, peritoneum, or right kidney. The histological and immunohistochemical examination of the specimen revealed SFT. The tumor has malignant potential with partially increased mitotic activity and cellularity in the histological examination. The patient is healthy and without evidence of recurrence or metastasis 26 months from surgery.

  19. Solitary fibrous tumor of the pancreas.

    PubMed

    Baxter, Andrew R; Newman, Elliot; Hajdu, Cristina H

    2015-01-01

    Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms of fibroblastic origin. Most commonly they affect the pleura but they been described in other viscera. SFT of the pancreas is extremely rare, and only eight cases have been reported to date. We perform a literature review and report a ninth case. The patient is a 54-year-old African-American female who presented with several months of abdominal pain. Abdominal radiography demonstrated a lesion in the head of the pancreas, and she underwent a Whipple operation. Pathology demonstrated SFT of the pancreas. She is alive and well 1 year post-operatively. SFT of the pancreas predominately affects middle-aged women. These tumors are difficult to distinguish radiologically from neuroendocrine tumors. While SFT of the pancreas tend to have an indolent course, there is the potential for malignancy. We recommend complete surgical excision.

  20. Solitary fibrous tumor of the pancreas.

    PubMed

    Baxter, Andrew R; Newman, Elliot; Hajdu, Cristina H

    2015-01-01

    Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms of fibroblastic origin. Most commonly they affect the pleura but they been described in other viscera. SFT of the pancreas is extremely rare, and only eight cases have been reported to date. We perform a literature review and report a ninth case. The patient is a 54-year-old African-American female who presented with several months of abdominal pain. Abdominal radiography demonstrated a lesion in the head of the pancreas, and she underwent a Whipple operation. Pathology demonstrated SFT of the pancreas. She is alive and well 1 year post-operatively. SFT of the pancreas predominately affects middle-aged women. These tumors are difficult to distinguish radiologically from neuroendocrine tumors. While SFT of the pancreas tend to have an indolent course, there is the potential for malignancy. We recommend complete surgical excision. PMID:26628714

  1. Ewing Sarcoma of the Pelvis with an Atypical Radiographic Appearance: A Mimicker of Non-malignant Etiologies

    PubMed Central

    Caram, Anthony; Derrick, Edward; Reith, John D; Bancroft, Laura; Scherer, Kurt

    2016-01-01

    Ewing sarcoma (ES) is a primary malignant bone tumor which most commonly arises in children and young adults. The common clinical presentation with ES includes nighttime pain or pain related to activity, though patients may also present with a combination of localized swelling, a palpable mass, pathologic fracture, and constitutional symptoms. Clinical diagnosis may be delayed when a patient presents with clinical or imaging findings that overlap with non-malignant etiologies, such as fibrous dysplasia (FD) or osteomyelitis. Furthermore, multimodality imaging, including computed tomography (CT), magnetic resonance imaging (MRI), and nuclear medicine may prove inconclusive in particular cases. Suspicion for malignancy should not be overlooked. A biopsy must be considered, unless the diagnosis is evident, such as a clinical response to antibiotics in the setting of osteomyelitis.  PMID:27774356

  2. Angiomatoid fibrous histiocytoma: case report and review of the literature.

    PubMed

    Tataroğlu, Canten; Çulhacı, Nil; Çeçen, Emre

    2015-01-01

    Angiomatoid fibrous histiocytoma is a rare soft tissue tumor of uncertain differentiation and low metastatic potential, which occurs predominantly in children and young adults. It occurs mostly within the extremities, trunk, head and neck. It can be associated with systemic manifestations such as anemia, pyrexia and malaise. Its morphology is distinct, with an outer shell of lymphoid tissue, sheets of dendritic-like tumor cells with bland nuclei and blood-filled cystic cavities. Herein, we present a case of angiomatoid fibrous histiocytoma with systemic symptoms before any mass was clinically detectable, arising in the scalp of a 10-year-old girl. PMID:26613231

  3. Intrarenal solitary fibrous tumor of the kidney report of a case with emphasis on the differential diagnosis in the wide spectrum of monomorphous spindle cell tumors of the kidney.

    PubMed

    Magro, Gaetano; Cavallaro, Vincenzo; Torrisi, Antonietta; Lopes, Maria; Dell'Albani, Marcello; Lanzafame, Salvatore

    2002-01-01

    Solitary fibrous tumor (SFT) is a neoplasm that can occur in the urogenital tract, and is also reported occurring in the spermatic cord, seminal vesicles, urinary bladder, prostate, and kidney. Furthermore, it is most important to consider its existence in the kidney, because it is usually diagnosed as renal cell carcinoma pre-operatively. To our knowledge, only 10 cases of SFT have been reported in the kidney to date. We report the clinico-pathological features of an intrarenal SFT occurring in a 31-year-old woman. The tumor, measuring 8.6 cm in its greatest diameter, completely replaced the cortex and the medulla of the middle region of the right kidney, compressing the pelvis. Radiological imaging was consistent with a renal cell carcinoma. Histologically, the tumor was composed of a proliferation of bland-looking vimentin+, CD34+, bcl2+ and CD99+ spindle cells exhibiting a haphazard to storiform growth pattern, pushing borders, and a low mitotic rate (2 mitoses x 10 HPF). We placed emphasis on the differential diagnostic problems, i.e., its differentiation from other primary monomorphous benign and malignant spindle cell tumors of the kidney, such as fibroma, benign fibrous histiocytoma, hemangiopericytoma, inflammatory myofibroblastic (pseudo-)tumor, leiomyoma, angiomyolipoma with predominant spindle cell smooth muscle component, benign peripheral nerve sheath tumors, renal mixed epithelial/stromal tumors, adult type mesoblastic nephroma, fibrous type monophasic synovial sarcoma, malignant peripheral nerve sheath tumors, fibrosarcoma, and low-grade fibromyxoid sarcoma.

  4. Trends in malignant intraductal papillary mucinous neoplasm in US adults from 1990 to 2010: a SEER database analysis

    PubMed Central

    McCarty, Thomas R.; Njei, Basile

    2016-01-01

    Background: Intraductal papillary mucinous neoplasms (IPMNs) are precancerous lesions with a well-described adenoma-carcinoma sequence. Although the risk of malignant transformation has been well studied, data on trends in long-term survival and important prognostic factors associated with survival in malignant IPMN are lacking. Methods: The Surveillance, Epidemiology, and End Results (SEER) database was queried to identify patients with confirmed malignant IPMN based upon pathologic diagnosis or radiographic evidence concerning for malignant potential. Median survival and age-adjusted incidence were calculated. Cox proportional hazard regression was used to determine independent mortality factors. Results: Based upon the SEER database query, 2651 patients were diagnosed with malignant IPMN between 1990 and 2010. The age-adjusted incidence of IPMN in 1990 was 0.361 per 100 000 persons (95% confidence interval [CI]: 0.285–0.451) with a steady decline observed through 2010 (0.135 per 100 000 persons, 95% CI: 0.098–0.186). A total of 564 patients (21.3%) underwent a surgical procedure, though the number of patients who underwent surgery from 1990 to 2010 also decreased (1990–1995, n = 132 to 2006–2010, n = 96, respectively). The overall median survival was 4 months and remained relatively stable from 1990 to 2010. Performance of surgery (HR: 0.45, 95% CI: 0.40–0.53, P < 0.001) was associated with a decreased risk of death. Conclusion: A significant decrease in the incidence of malignant IPMN was seen from 1990 to 2010. There was also no improvement observed in long-term survival. The small percentage of eligible cases receiving surgical treatment suggests that there is room for further improvement in survival, with increased utilization of surgery. PMID:26818977

  5. RO4929097, Temozolomide, and Radiation Therapy in Treating Patients With Newly Diagnosed Malignant Glioma

    ClinicalTrials.gov

    2015-09-28

    Acoustic Schwannoma; Adult Anaplastic (Malignant) Meningioma; Adult Anaplastic Astrocytoma; Adult Anaplastic Ependymoma; Adult Brain Stem Glioma; Adult Choroid Plexus Neoplasm; Adult Craniopharyngioma; Adult Diffuse Astrocytoma; Adult Ependymoblastoma; Adult Ependymoma; Adult Giant Cell Glioblastoma; Adult Glioblastoma; Adult Gliosarcoma; Adult Grade I Meningioma; Adult Grade II Meningioma; Adult Medulloblastoma; Adult Mixed Glioma; Adult Myxopapillary Ependymoma; Adult Oligodendroglioma; Adult Papillary Meningioma; Adult Pilocytic Astrocytoma; Adult Pineal Gland Astrocytoma; Adult Pineoblastoma; Adult Pineocytoma; Adult Primary Melanocytic Lesion of Meninges; Adult Subependymal Giant Cell Astrocytoma; Adult Subependymoma; Adult Supratentorial Primitive Neuroectodermal Tumor; Malignant Adult Intracranial Hemangiopericytoma

  6. Calcifying Fibrous Tumor

    PubMed Central

    Chorti, Angeliki; Papavramidis, Theodossis S.; Michalopoulos, Antonios

    2016-01-01

    Abstract Calcifying fibrous tumor (CFT) is a benign lesion characterized by its specific histological findings and is found as solitary or multiple lesions in several locations of the human body. The aim of the present systematic review is to give a detailed account of all reported cases of CFT in the literature and to analyze the available data, to completely characterize the entity from epidemiological, medical, and surgical aspects. A bibliographic research was performed from 1988 until 2015. A database with the patients’ characteristics was made, including sex, age, location of the tumor, symptoms, symptoms duration, size of the tumor, diagnostic methods, treatment, metastasis, and follow-up. A total of 104 articles were identified, reporting 157 cases of CFT. Mean age of patients was 33.58 years and the ratio between men and women was 1:1.27. The most common locations of CFT were stomach (18%), small intestine (8.7%), pleura (9.9%), mesentery (5%), and peritoneum (6.8%). Mean diameter of the tumor was estimated 4.6 cm. The correlations proceeded showed that as age increases, size decreases (P = 0.001) and that the tumor is larger in females (P = 0.027). Kruskal-Wallis test showed that the larger tumors appear in the neck and adrenal gland (P = 0.001). The percentage of asymptomatic patients was 30.57%. Computed tomography and biopsy were the most common tests for the diagnosis of CFT. Open surgical procedure was performed in the majority of cases. The median hospitalization was 6.06 days and the mean follow-up period was 29.97 months. Recurrences were mentioned in 10 of 96 patients with available data. No deaths owing to CFT were mentioned in the literature. CFT should be included in the differential diagnosis of enlarging mass revealed by clinical or imaging examination either incidentally or after specific acute or chronic symptomatology. PMID:27196478

  7. Laminin alpha 5, a major transcript of normal and malignant rat liver epithelial cells, is differentially expressed in developing and adult liver.

    PubMed

    Seebacher, T; Medina, J L; Bade, E G

    1997-11-25

    The laminin family of extracellular matrix glycoproteins plays a major role in cell migration and differentiation and in tumor cell invasion. As previously shown, the laminin deposited by normal and malignant rat liver epithelial cells in their extracellular matrix (ECM) and into their ECM migration tracks does not contain a typical (EHS-like) alpha 1 heavy chain. By RT-PCR screening we have now identified two alpha chains among a total of five additional laminin chains produced by these cells. Three of the newly identified chains were not previously known for the rat. Their sequences have been deposited in the EMBL nucleotide sequence data bank. The alpha 5 chain now identified is expressed at comparably high levels by both the normal and the malignant liver epithelial cells. The chain is also expressed in fetal liver together with the alpha 2 and beta 2 chains, but it is only vestigially expressed in the mature organ as shown by RT-PCR. These results suggest for alpha 5 a role in development and production of the chain by only a small subset of cells in adult liver. At the level of detection used, no changes were observed in regenerating liver after partial hepatectomy. In addition to the alpha 5 chain, the cultured cells express the beta 1 and beta 2 light chains, indicating the expression of more than one laminin isoform by the same cell line. The expression of the alpha 5 chain and of the other new non-EHS isoform chains was also analyzed in various tissues. The malignant liver epithelial cells, but not their nontumorigenic parental cells, also express, in addition to the alpha 5 chain the alpha 2 chain, which is expressed at high level by the NBT II bladder carcinoma cell line, suggesting a relationship with malignancy. PMID:9417868

  8. [A case of retroperitoneal solitary fibrous tumor].

    PubMed

    Anchi, Takashi; Tamura, Kenji; Inoue, Keiji; Fukata, Satoshi; Nishikawa, Hiroshi; Moriki, Toshiaki; Shuin, Taro

    2009-07-01

    Solitary fibrous tumor (SFT) is a common neoplasm of the pleura and rarely arises from the retroperitoneal space. We report a case of retroperitoneal SFT. A 54-year-old woman was admitted to the department of dermatology in our hospital for examination and treatment of malignant melanoma of the right lower leg. Computerized tomography (CT) showed the left adrenal mass measuring 6.0 x 4.5 x 10 cm. Because the tumor was thought to be left adrenal tumor or left adrenal metastasis of the malignant melanoma, she admitted to our department. Left radical adrenalectomy was performed. The histological examination of the specimen revealed that the spindle or oval cells proliferated patternless with hyalinized collagen cells and there were few mitotic figures. The tumor was not connected to the adrenal gland. The immunohistochemical examination showed that the tumor cells were diffusely positive for CD34, bcl-2 and vimentin. According to these findings, we diagnosed SFT arising from the retroperitoneum. At 5 years follow up she had no evidence of local recurrence and no distant metastasis.

  9. Solitary fibrous tumors of the pleura: clinicopathological and immunohistochemical examination.

    PubMed

    Hiraoka, Kei; Morikawa, Toshiaki; Ohbuchi, Toshiro; Katoh, Hiroyuki

    2003-03-01

    The purpose of this work was to study clinical and biological characteristics of solitary fibrous tumor (SFT) of the pleura. We reviewed the clinicopathological and immunohistochemical features of 12 patients who underwent surgical resection for SFT. Ten cases were histologically defined as benign; two were found to be malignant. CD34 negativity and strong expression of p53 could be observed in a patient with fatal outcome. Ki-67 expression was increased in malignant cases, as compared with benign. We also found that Bcl-2 expression inversely correlated with a tumor diameter. As the development of malignant SFT might be associated with these molecular statuses, immunohistochemical staining should be performed in all cases to identify the biological characteristics of the tumor.

  10. Pedunculated hemangiopericytoma-like tumor: peculiar fibroepithelial polyp or fibrous histiocytoma variant.

    PubMed

    Brown, Jameel Ahmad; Morgan, Michael B

    2008-08-01

    Pathologists are continually challenged with the difficult task of discriminating between innocuous disease processes and potentially malignant entities. Apropos of this concern, we present a series of three cutaneous polypoid lesions that simulated fibroepithelial polyp, yet upon close scrutiny yielded histologic features of solitary fibrous tumor (SFT) or hemangiopericytoma. These pedunculated lesions showed a storiform pattern of spindled cells with interspersed gaping vascular channels reminiscent of SFT or hemangiopericytoma. Interestingly, the immunohistochemical staining profile of these lesions was negative for CD34 and positive for bcl-2 and factor XIIIa. These findings were discordant with SFT and suggest a relationship with fibrous histiocytoma. We propose that this entity represents a hitherto described variant of fibrous histiocytoma known as pedunculated hemangiopericytoma-like fibrous histiocytoma.

  11. Clinical guidelines for the management of craniofacial fibrous dysplasia.

    PubMed

    Lee, J S; FitzGibbon, E J; Chen, Y R; Kim, H J; Lustig, L R; Akintoye, S O; Collins, M T; Kaban, L B

    2012-05-24

    Fibrous dysplasia (FD) is a non-malignant condition caused by post-zygotic, activating mutations of the GNAS gene that results in inhibition of the differentiation and proliferation of bone-forming stromal cells and leads to the replacement of normal bone and marrow by fibrous tissue and woven bone. The phenotype is variable and may be isolated to a single skeletal site or multiple sites and sometimes is associated with extraskeletal manifestations in the skin and/or endocrine organs (McCune-Albright syndrome). The clinical behavior and progression of FD may also vary, thereby making the management of this condition difficult with few established clinical guidelines. This paper provides a clinically-focused comprehensive description of craniofacial FD, its natural progression, the components of the diagnostic evaluation and the multi-disciplinary management, and considerations for future research. PMID:22640797

  12. [Solitary fibrous tumor and haemangiopericytoma: what is new?].

    PubMed

    Knösel, T; Schulz, B; Katenkamp, K; Katenkamp, D; Petersen, I

    2010-03-01

    Soft-tissue tumors with haemangiopericytoma (HPC)-like growth patterns can now be divided into three categories: (1) The solitary fibrous tumour (SFT) group with its variants; (2) lesions showing clear evidence of myoid/pericytic differentiation and corresponding to "true" HPCs (myopericytoma/glomangiopericytoma and a subset of sinonasal HPCs); (3) neoplasms that occasionally display HPC-like features (e.g. synovial sarcoma). In this study 268 intrathoracic and extrathoracic SFTs from the German consultation and reference center of soft tissue tumors in Jena were evaluated and analyzed immunohistochemically with antibodies CD34, Bcl-2, CD99, SMA, S100, PanCK and Ki-67. Furthermore, SFTs were categorized into the newly proposed SFT designation: Fibrous variant, cellular variant (more than 90% hypercellularity), fat-forming variant, giant cell-rich variant and malignant SFTs. This article should provide insights into the diagnosis of this entity with emphasis on the new international standard.

  13. Solitary fibrous tumor of the greater omentum mimicking an ovarian tumor in a young woman.

    PubMed

    Rodriguez Tarrega, Elisabet; Hidalgo Mora, Juan Jose; Paya Amate, Vicente; Vega Oomen, Olivia

    2016-08-01

    We report a case of solitary fibrous tumor (SFT) of greater omentum in a young woman. SFT arising from the greater omentum can mimic a gynecologic neoplasm. SFTs are generally benign but some of them are malignant and have uncertain prognosis. An adequate follow-up is essential in these patients.

  14. Solitary fibrous tumor of the greater omentum mimicking an ovarian tumor in a young woman.

    PubMed

    Rodriguez Tarrega, Elisabet; Hidalgo Mora, Juan Jose; Paya Amate, Vicente; Vega Oomen, Olivia

    2016-08-01

    We report a case of solitary fibrous tumor (SFT) of greater omentum in a young woman. SFT arising from the greater omentum can mimic a gynecologic neoplasm. SFTs are generally benign but some of them are malignant and have uncertain prognosis. An adequate follow-up is essential in these patients. PMID:27354994

  15. Treatment Option Overview (Osteosarcoma and Malignant Fibrous Histiocytoma of Bone)

    MedlinePlus

    ... tomography, computerized tomography, or computerized axial tomography. MRI (magnetic resonance imaging) : A procedure that uses a magnet, radio waves , ... the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). A biopsy is done to diagnose osteosarcoma. ...

  16. Solitary Fibrous Tumour of the Clavicle: A Rare Case Report.

    PubMed

    Srinivas, Dileep Krishnamoorty; Ballal, Arjun; Pai, Mukta; Subbiah, Kushalappa; Rai, H Ravindranath

    2016-06-01

    A Solitary Fibrous Tumour (SFT) is the preferred term by most of the pathologists than "haemangiopericytoma". SFT is a heterogeneous group of benign and malignant neoplasms along a morphologic continuum. Here we report a case of SFT of the clavicle in a 26-year-old male patient, who presented to us with complaints of pain and swelling over the dominant shoulder. No signs of metastasis were noted clinically and radiologically. He underwent surgical resection of swelling. At 6 months after resection and after 22 cycles of radiotherapy, he was noted to have excellent prognosis with satisfactory shoulder function.

  17. Metastatic melanoma mimicking solitary fibrous tumor: report of two cases.

    PubMed

    Bekers, Elise M; van Engen-van Grunsven, Adriana C H; Groenen, Patricia J T A; Westdorp, Harm; Koornstra, Rutger H T; Bonenkamp, Johannes J; Flucke, Uta; Blokx, Willeke A M

    2014-02-01

    Malignant melanomas are known for their remarkable morphological variation and aberrant immunophenotype with loss of lineage-specific markers, especially in recurrences and metastases. Hot spot mutations in BRAF, NRAS, GNAQ, and GNA11 and mutations in KIT are oncogenic events in melanomas. Therefore, genotyping can be a useful ancillary diagnostic tool. We present one case each of recurrent and metastatic melanoma, both showing histological and immunohistochemical features of solitary fibrous tumor (SFT). Mutational analysis detected BRAF and NRAS mutations in the primary and secondary lesions, respectively. This result confirmed the diagnosis of recurrent/metastastic melanoma.

  18. Solitary Fibrous Tumour of the Clavicle: A Rare Case Report.

    PubMed

    Srinivas, Dileep Krishnamoorty; Ballal, Arjun; Pai, Mukta; Subbiah, Kushalappa; Rai, H Ravindranath

    2016-06-01

    A Solitary Fibrous Tumour (SFT) is the preferred term by most of the pathologists than "haemangiopericytoma". SFT is a heterogeneous group of benign and malignant neoplasms along a morphologic continuum. Here we report a case of SFT of the clavicle in a 26-year-old male patient, who presented to us with complaints of pain and swelling over the dominant shoulder. No signs of metastasis were noted clinically and radiologically. He underwent surgical resection of swelling. At 6 months after resection and after 22 cycles of radiotherapy, he was noted to have excellent prognosis with satisfactory shoulder function. PMID:27504363

  19. Solitary Fibrous Tumour of the Clavicle: A Rare Case Report

    PubMed Central

    Srinivas, Dileep Krishnamoorty; Pai, Mukta; Subbiah, Kushalappa; Rai, H. Ravindranath

    2016-01-01

    A Solitary Fibrous Tumour (SFT) is the preferred term by most of the pathologists than “haemangiopericytoma”. SFT is a heterogeneous group of benign and malignant neoplasms along a morphologic continuum. Here we report a case of SFT of the clavicle in a 26-year-old male patient, who presented to us with complaints of pain and swelling over the dominant shoulder. No signs of metastasis were noted clinically and radiologically. He underwent surgical resection of swelling. At 6 months after resection and after 22 cycles of radiotherapy, he was noted to have excellent prognosis with satisfactory shoulder function. PMID:27504363

  20. Solitary fibrous tumor of the orbit--two cases and a review of the literature.

    PubMed

    Romer, M; Bode, B; Schuknecht, B; Schmid, S; Holzmann, D

    2005-02-01

    Solitary fibrous tumors of the orbit (SFT) are mesenchymal lesions that can develop either as malignant or benign neoplasias. We describe the histological features leading to the diagnosis in two females and review the current literature. Diagnosis of SFT only can be performed by histological examination, since clinical signs and radiological features are not specific enough. Even a malignant or benign course cannot be predicted, since clinical and radiological features do not correlate with histological signs of malignancy and vice versa. Radical resection is the treatment of choice, since no other treatment option has been proven to be efficient.

  1. Solitary fibrous tumour of the kidney with sarcomatous overgrowth. Case report and review of the literature.

    PubMed

    Magro, Gaetano; Emmanuele, Carmela; Lopes, Maria; Vallone, Giuseppe; Greco, Paolo

    2008-11-01

    Solitary fibrous tumour (SFT) rarely occurs in the kidney, with only one case exhibiting malignant behaviour. We report the case of a typical SFT of the kidney with sarcomatous overgrowth in a 34-year-old woman. This malignant component, grossly apparent as a nodular area arising in the context of the main tumour mass, consisted of CD34+ mitotically active atypical plump spindle- to epithelioid-shaped cells, including pleomorphic multinucleated giant cells. A novel immunohistochemical finding was diffuse and strong S-100 protein expression by sarcomatous cells. This should be kept in mind by pathologists to avoid confusion with other S-100 protein-positive malignant neoplasms.

  2. Metastatic solitary fibrous tumor of the meninges. Case report.

    PubMed

    Ng, H K; Choi, P C; Wong, C W; To, K F; Poon, W S

    2000-09-01

    Solitary fibrous tumor (SFT) is a unique tumor composed of interstitial dendritic cells that was first described in the thorax and subsequently reported in diverse organs. Extrathoracic SFTs are predominantly benign but rare malignant cases have been documented. In the nervous system, SFT has been described as a meningeal lesion although all 14 previously reported cases were benign. The authors report the first case of a meningeal SFT occurring in a 55-year-old woman. The tumor first presented as a meningeal lesion that after three recurrences over a 10-year period metastasized to the soft tissues and lungs. The potentially malignant nature of cranial SFTs, especially those with atypical histological features and high mitotic counts, should be recognized.

  3. [Solitary fibrous tumor of the pelvis: a rare extrathoracic manifestation].

    PubMed

    Gessmann, J; Seybold, D; Helwing, M; Muhr, G; Schildhauer, T A

    2009-07-01

    Solitary fibrous tumors (SFT) are rare spindle cell neoplasms. To date only very few cases of pelvic SFT have been reported in the literature. SFT are characterized by unique microscopic and immunohistochemical findings. Complete local resection is the treatment of choice. Recurrence and metastasis may be related to infrequent malignant histological features, but histology is not always a reliable predictor for prognosis. Therefore long-term follow-up is necessary.We report about a male patient with a malignant pelvic SFT. After complete resection the tumor recurred after a short period of 6 months posterior to the original location in the pelvis. The differential diagnoses and the therapy options are discussed with a review of the present literature.

  4. [Solitary fibrous tumor of the epicardium].

    PubMed

    Flemming, P; Maschek, H; Werner, M; Kreft, A; Graeter, T; Georgii, A

    1996-03-01

    A giant Solitary Fibrous Tumor (SFT) arising in the pericardium is described. A 53 year old woman was suspected to have a lung tumor and thoracotomy was performed. Intraoperatively the child-head sized mass was found to be localized in the pericardial sac. The histopathological interpretation of a small wedge-biopsy was 'endothelioma of uncertain malignancy' and heart-transplantation was performed two months later. The patient died of postoperative infection. Post mortem examination could exclude tumor rest or metastasis. The explanted heart revealed a large bulk of 2800 grams arising from the epicardium of the left chamber and enveloping the heart without invading the underlying myocardium. The histopathological pattern varied between cell-rich and -poor fibromatous areas and well capillarized endothelioma-like zones. Immunohistochemistry revealed positive reactions with monoclonal antibodies against Vimentin and CD 34 and no reactions against Cytokeratins and Faktor VIII. This pattern was confirmed in 7 SFT's of the pleura from our archives. A second control group of 7 mesotheliomas was positive for cytokeratins and vimentin but not for CD 34 and Faktor VIII. Diagnosis of SFT might be difficult because of its variability in histopathology, sometimes mimicking a hemangiopericytoma or endothelioma. Recent reports of tumor localizations devoiding serosal surfaces illustrate the diagnostic and histogenetic dilemma of this tumor. The differentiation of SFT from mesothelioma and endothelioma can be achieved by immunophenotyping including CD 34.

  5. Solitary fibrous tumour of the supraglottic larynx.

    PubMed

    Grammatica, A; Bolzoni Villaret, A; Ravanelli, M; Nicolai, P

    2016-06-01

    Solitary fibrous tumour (SFT) is a rare, benign, mesenchymal neoplasm that usually arises in the pleura, but rarely involves other sites outside the serosal space (mediastinum, lung, liver, thyroid gland); larynx involvement is very rare with only sporadic cases reported in the literature. We report a case of SFT in a 41-year-old woman with supraglottic laryngeal invovlement; symptoms included dysphonia and mild odynophagia lasting 2 years, and fibre-optic laryngeal evaluation showed a sub-mucosal mass involving the left supraglottis and medial wall of the pyriform sinus. MRI represents the gold standard tool for differential diagnosis (with schwannoma, paraganglioma and haemangioma) and correct staging, while immunohistochemical and cytomorphologic analysis (bcl-2 and CD34 positivity in 90% of cases) is needed for definitive diagnosis. Surgery is the main treatment (endoscopic and open conservative technique), and its goal is a balance between safe oncological resection and good preservation of laryngeal functions; in this particular case an open laryngeal approach was scheduled due to the size of the tumour. Prognosis is good and in only a few cases (especially in pleural SFT) does the biological behaviour take a malignant course. PMID:27070539

  6. Solitary fibrous tumour of the supraglottic larynx.

    PubMed

    Grammatica, A; Bolzoni Villaret, A; Ravanelli, M; Nicolai, P

    2016-06-01

    Solitary fibrous tumour (SFT) is a rare, benign, mesenchymal neoplasm that usually arises in the pleura, but rarely involves other sites outside the serosal space (mediastinum, lung, liver, thyroid gland); larynx involvement is very rare with only sporadic cases reported in the literature. We report a case of SFT in a 41-year-old woman with supraglottic laryngeal invovlement; symptoms included dysphonia and mild odynophagia lasting 2 years, and fibre-optic laryngeal evaluation showed a sub-mucosal mass involving the left supraglottis and medial wall of the pyriform sinus. MRI represents the gold standard tool for differential diagnosis (with schwannoma, paraganglioma and haemangioma) and correct staging, while immunohistochemical and cytomorphologic analysis (bcl-2 and CD34 positivity in 90% of cases) is needed for definitive diagnosis. Surgery is the main treatment (endoscopic and open conservative technique), and its goal is a balance between safe oncological resection and good preservation of laryngeal functions; in this particular case an open laryngeal approach was scheduled due to the size of the tumour. Prognosis is good and in only a few cases (especially in pleural SFT) does the biological behaviour take a malignant course.

  7. Solitary fibrous tumor of the breast: a case report and review of the literature.

    PubMed

    Rhee, Sun Jung; Ryu, Jung Kyu; Han, Sang-Ah; Won, Kyu Yeoun

    2016-01-01

    Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms that can be benign or malignant. SFTs have been most often documented in the pleura. Recently, involvement of extrapleural sites such as the abdomen, musculoskeletal soft tissue, upper respiratory tract, mediastinum, and head and neck were reported. Less than 15 cases of SFT of the breast have been reported. Here, we report a case of a pathologically proven SFT of the breast and review the literature on the radiologic findings. US imaging showed an oval, well-circumscribed, hypoechoic solid mass. A solitary fibrous tumor of the breast is a very rare lesion.

  8. Solitary fibrous tumor of the breast: a case report and review of the literature.

    PubMed

    Rhee, Sun Jung; Ryu, Jung Kyu; Han, Sang-Ah; Won, Kyu Yeoun

    2016-01-01

    Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms that can be benign or malignant. SFTs have been most often documented in the pleura. Recently, involvement of extrapleural sites such as the abdomen, musculoskeletal soft tissue, upper respiratory tract, mediastinum, and head and neck were reported. Less than 15 cases of SFT of the breast have been reported. Here, we report a case of a pathologically proven SFT of the breast and review the literature on the radiologic findings. US imaging showed an oval, well-circumscribed, hypoechoic solid mass. A solitary fibrous tumor of the breast is a very rare lesion. PMID:26703178

  9. Paratesticular fibrous pseudotumour: Intraoperative frozen section analysis can help prevent unnecessary orchiectomy

    PubMed Central

    DeCoste, Ryan C.; Carter, Michael D.; Bagnell, Scott; Merrimen, Jennifer

    2015-01-01

    Paratesticular fibrous pseudotumours are rare intrascrotal lesions, most frequently affecting the testicular tunics. They are benign in nature; however, their pathogenesis is not completely understood. Presenting features are similar to testicular malignancy, which may result in unnecessary radical surgery. It has been suggested that additional diagnostic imaging combined with frozen section analysis may help prevent orchiectomy in these patients. We describe a case of paratesticular fibrous pseudotumour in a 40-year-old male treated with testicle-sparing surgery aided by intraoperative frozen section analysis. PMID:26664509

  10. Orbital fibrous hamartoma of infancy.

    PubMed

    Choi, Se Hyun; Hwang, Jeong-Min; Kim, Namju

    2016-02-01

    Fibrous hamartoma of infancy is a rare, benign lesion of the superficial soft tissue, typically presenting within the first 2 years of life, and usually located on the upper extremities, axilla, and upper back. Lesions affecting the orbit have not been reported previously. We report the case of a 7-month-old boy with orbital fibrous hamartoma of infancy. He had a right hypertropia and a limitation of infraduction in the right eye. A mass on the right anteroinferior orbit involving the inferior rectus and inferior oblique muscles was seen on magnetic resonance imaging. Hamartoma of infancy was confirmed through pathologic examination. Right hypertropia and limitation of infraduction moderately improved after surgery.

  11. Protease degradable electrospun fibrous hydrogels

    PubMed Central

    Wade, Ryan J.; Bassin, Ethan J.; Rodell, Christopher B.; Burdick, Jason A.

    2015-01-01

    Electrospun nanofibers are promising in biomedical applications to replicate features of the natural extracellular matrix (ECM). However, nearly all electrospun scaffolds are either non-degradable or degrade hydrolytically, whereas natural ECM degrades proteolytically, often through matrix metalloproteinases (MMPs). Here, we synthesize reactive macromers that contain protease-cleavable and fluorescent peptides and are able to form both isotropic hydrogels and electrospun fibrous hydrogels through a photoinitiated polymerization. These biomimetic scaffolds are susceptible to protease-mediated cleavage in vitro in a protease dose dependent manner and in vivo in a subcutaneous mouse model using transdermal fluorescent imaging to monitor degradation. Importantly, materials containing an alternate and non-protease-cleavable peptide sequence are stable in both in vitro and in vivo settings. To illustrate the specificity in degradation, scaffolds with mixed fiber populations support selective fiber degradation based on individual fiber degradability. Overall, this represents a novel biomimetic approach to generate protease-sensitive fibrous scaffolds for biomedical applications. PMID:25799370

  12. Intraventricular etoposide safety and toxicity profile in children and young adults with refractory or recurrent malignant brain tumors.

    PubMed

    Pajtler, Kristian W; Tippelt, Stephan; Siegler, Nele; Reichling, Stefanie; Zimmermann, Martina; Mikasch, Ruth; Bode, Udo; Gnekow, Astrid; Pietsch, Torsten; Benesch, Martin; Rutkowski, Stefan; Fleischhack, Gudrun

    2016-07-01

    Systemic administration of etoposide is effective in treating metastatic, recurrent or refractory brain tumors, but penetration into the cerebrospinal fluid is extremely poor. This study was designed to determine the safety and toxicity profile of intraventricular etoposide administration and was affiliated with the prospective, multicenter, nonblinded, nonrandomized, multi-armed HIT-REZ-97 trial. The study enrolled 68 patients, aged 1.1-34.6 (median age 11 years). Adverse events that could possibly be related to intraventricular etoposide therapy were documented and analyzed. Intraventricular etoposide was simultaneously administered with either oral or intravenous chemotherapy in 426 courses according to three major schedules varying in dosing (0.25-1 mg), frequency of administration (bolus injection, every 12 or 24 h), course duration (5-10 days) and length of interval between courses (2-5 weeks). Potential treatment-related adverse effects included transient headache, seizures, infection of the reservoir, nausea and neuropsychological symptoms. Hematological side effects were not observed. One patient, with history of multiple prior therapies, who received long-term intraventricular and oral etoposide treatment developed acute myeloid leukemia as a secondary malignancy. Overall intraventricular etoposide is well tolerated. The results of this study have warranted a phase II trial to determine the effectiveness of this regimen in disease stages with very limited therapeutic options. PMID:27147083

  13. How to manage lung infiltrates in adults suffering from haematological malignancies outside allogeneic haematopoietic stem cell transplantation.

    PubMed

    Maschmeyer, Georg; Donnelly, J Peter

    2016-04-01

    Pulmonary complications affect up to 40% of patients with severe neutropenia lasting for more than 10 d. As they are frequently associated with fever and elevation of C-reactive protein or other signs of inflammation, they are mostly handled as pneumonia. However, the differential diagnosis is broad, and a causative microbial agent remains undetected in the majority of cases. Pulmonary side effects from cytotoxic treatment or pulmonary involvement by the underlying malignancy must always be taken into account and may provide grounds for invasive diagnostic procedures in selected patients. Pneumocystis jirovecii (in patients not receiving co-trimoxazole as prophylaxis), multi-resistant gram-negative bacilli, mycobacteria or respiratory viruses may be involved. High-risk patients may be infected by filamentous fungi, such as Aspergillus spp., but these infections are seldom proven when treatment is initiated. Microorganisms isolated from cultures of blood, bronchoalveolar lavage or respiratory secretions need careful interpretation as they may be irrelevant for determining the aetiology of pulmonary infiltrates, particularly when cultures yield coagulase-negative staphylococci, enterococci or Candida species. Non-culture based diagnostics for detecting Aspergillus galactomannan, beta-D-glucan or DNA from blood, bronchoalveolar lavage or tissue samples can facilitate the diagnosis, but must always be interpreted in the context of clinical and imaging findings. Systemic antifungal treatment with mould-active agents, given in combination with broad-spectrum antibiotics, improves clinical outcome when given pre-emptively. Co-trimoxazole remains the first-line treatment for Pneumocystis pneumonia, while cytomegalovirus pneumonia will respond to ganciclovir or foscarnet in most cases. The clinical outcome of acute respiratory failure can also be successful with proper intensive care, when indicated. PMID:26729577

  14. Risk of second primary malignancies in a population-based study of adult patients with essential thrombocythemia

    PubMed Central

    Shrestha, Rajesh; Giri, Smith; Pathak, Ranjan; Bhatt, Vijaya Raj

    2016-01-01

    AIM To determine the risk of second primary malignancy (SPM) and survival of patients with essential thrombocythemia (ET). METHODS We identified all patients with ET diagnosed during 2001 to 2011 from the Surveillance, Epidemiology and End Results (SEER) 18 database. Actuarial and relative survival methods were used to calculate the survival statistics. We utilized the SEER 13 database to calculate SPM. We used multiple primary standardized incidence ratio (SIR) session of the SEER*Stat software (version 8.1.5) to calculate SIR and excess risk of SPM for ET patients. RESULTS Age standardized five-year cause-specific survival was greater for patients < 50 years vs those ≥ 50 years (99.4% vs 93.5%, P < 0.01). Five-year cause-specific survival was lower for men vs women (70.2% vs 79.7%). A total of 201 patients (2.46%) developed SPM at a median age of 75 years. SPMs occurred at an observed/expected (O/E) ratio of 1.26 (95%CI: 1.09-1.45, P = 0.002) with an absolute excess risk (AER) of 37.44 per 10000 population. A significantly higher risk was noted for leukemia (O/E 3.78; 95%CI: 2.20-6.05, P < 0.001; AER 11.28/10000). CONCLUSION ET patients have an excellent cause-specific five-year survival but are at an increased risk of SPM, particularly leukemia, which may contribute to excess deaths. PMID:27579252

  15. Aggressive CD34-positive fibrous scalp lesion of childhood: extrapulmonary solitary fibrous tumor.

    PubMed

    Ramdial, P K; Madaree, A

    2001-01-01

    Although solitary fibrous tumor (SFT) was originally described as a pleural tumor, an increasing number of extrapleural sites of SFTs have been documented. This has been attributed not only to the heightened awareness of the spectrum of histopathological features that characterizes SFTs but also to the recognition of the role of CD34 immunostaining in soft tissue tumors in general, and in SFTs in particular. Despite the large number of documented extrapleural SFTs in adults, cranial SFTs are rare, having been documented in the meninges, scalp, and infratemporal fossa. Extrapleural SFTs are, to date, an unrecognized entity in children. We document an aggressive fibrous scalp lesion in a 30-month-old female child that demonstrated features common to benign cranial fasciitis and SFT. However, based on bright, diffuse CD34 antigen immunopositivity, a diagnosis of SFT was made. The need to include the CD34 antigen stain in a panel of immunohistochemical markers used to assess spindle cell lesions of childhood is emphasized.

  16. Osteogenic sarcoma and soft tissue myxoma in a patient with fibrous dysplasia and hemoglobins JBaltimore and S.

    PubMed

    Witkin, G B; Guilford, W B; Siegal, G P

    1986-03-01

    A 41-year-old man with recognized polyostotic fibrous dysplasia since late childhood developed fibroblastic osteogenic sarcoma in the left tibia. Four months after the initial diagnosis, an intramuscular myxoma was discovered in the left thigh. Twenty years previously he had been found to be heterozygous for hemoglobins JBaltimore and S. Malignant transformation in fibrous dysplasia is unusual and may be associated in some individuals with prior irradiation. Soft tissue myxomas associated with fibrous dysplasia are even rarer. To the best of the authors' knowledge the occurrence of both of these lesions in a patient with fibrous dysplasia has been reported only once before. Patients with both fibrous dysplasia and myxomas may be at greater risk for malignant transformation than are individuals with only one of these lesions. There is no well-recognized association between hemoglobinopathies and either fibrous dysplasia or bone tumors. It is therefore probable that the rare constellation of findings is in this patient a stochastic event. PMID:3456858

  17. Is 4q13 a recurring breakpoint in solitary fibrous tumors?

    PubMed

    Debiec-Rychter, M; de Wever, I; Hagemeijer, A; Sciot, R

    2001-11-01

    Solitary fibrous tumor (SFT) is a mesenchymal neoplasm found predominantly in the subpleural region but also in many other body sites. We report a malignant solitary fibrous tumor of the peritoneum with a 47,XY,t(4;9)(q13;p23),+5 karyotype. The chromosome 4q13 breakpoint in the presented and previously published case of pleural solitary fibrous tumor with a 46,XY,t(4;15)(q13;q26) karyotype was further characterized by fluorescence in situ hybridization analysis and localized within the 5-cM interval that was flanked by regions specific to YAC clones 761A7 and 886C11. Chromosome translocations involving chromosome 4q13 may characterize a separate cytogenetic subgroup of SFT.

  18. Polyostotic fibrous dysplasia with gigantism and huge pelvic tumor: a rare case of McCune-Albright syndrome.

    PubMed

    Sakayama, Kenshi; Sugawara, Yoshifumi; Kidani, Teruki; Fujibuchi, Taketsugu; Kito, Katsumi; Tanji, Nozomu; Nakamura, Atsushi

    2011-06-01

    We report a rare case of polyostotic fibrous dysplasia on endocrine hyperfunction with elevated human growth hormone and normal serum level of prolactin. There were some differential points of gender, gigantism, endocrine function, and GNAS gene from McCune-Albright syndrome. Malignant transformation was suspected in the pelvic tumor from imaging because rapid growth of the tumor by imaging was observed; however, no malignant change occurred in this case. PMID:20878436

  19. Polyostotic fibrous dysplasia with gigantism and huge pelvic tumor: a rare case of McCune-Albright syndrome.

    PubMed

    Sakayama, Kenshi; Sugawara, Yoshifumi; Kidani, Teruki; Fujibuchi, Taketsugu; Kito, Katsumi; Tanji, Nozomu; Nakamura, Atsushi

    2011-06-01

    We report a rare case of polyostotic fibrous dysplasia on endocrine hyperfunction with elevated human growth hormone and normal serum level of prolactin. There were some differential points of gender, gigantism, endocrine function, and GNAS gene from McCune-Albright syndrome. Malignant transformation was suspected in the pelvic tumor from imaging because rapid growth of the tumor by imaging was observed; however, no malignant change occurred in this case.

  20. Modelling fracture in fibrous microstructures

    SciTech Connect

    Beyerlein, I.

    1998-04-01

    This work describes some complementary studies directed towards micromechanical modeling and simulation of the statistical fracture process in composites with fibrous microstructures. A few studies involve combining efficient computational stress analyses and piezospectroscopic measurement techniques to quantify interface deformation around a single break in model composites. It is shown how estimated interface parameters can be used to predict activity around more complex break arrangement in much larger composites. The final studies involve incorporating these experimentally refined stress analyses into large scale simulation for statistical predictions and subsequent analytical modeling of composite fracture.

  1. Flame resistant fibrous structures development

    NASA Technical Reports Server (NTRS)

    Coskren, Robert J.

    1992-01-01

    The purpose of the current program was (1) to investigate potentially useful new polymers, both for fire safety and mechanical properties, (2) to produce fibers from these polymers if necessary, and (3) to produce sufficient quantities of qualified fibrous structures, composites, or laminates for use in various areas of the Space Shuttle and Space Station Programs. During the past six years, development efforts have been expended in several major areas in support of Space Shuttle missions and Space Station Freedom projects. The summarized results of several of these major efforts are included in this report.

  2. T cell–depleted stem-cell transplantation for adults with hematologic malignancies: sustained engraftment of HLA-matched related donor grafts without the use of antithymocyte globulin

    PubMed Central

    Small, Trudy N.; Young, James W.; Kernan, Nancy A.; Castro-Malaspina, Hugo; Hsu, Katherine C.; Perales, Miguel-Angel; Collins, Nancy; Cisek, Christine; Chiu, Michelle; van den Brink, Marcel R. M.; O'Reilly, Richard J.; Papadopoulos, Esperanza B.

    2007-01-01

    Antithymocyte globulin (ATG) has been used in allogeneic stem-cell transplantation to prevent graft rejection and graft-versus-host disease (GvHD). Its use, however, has been associated with delayed T-cell reconstitution and prolonged susceptibility to opportunistic infections (OIs) especially in patients undergoing T cell–depleted (TCD) transplantation. Recently, a prospective trial was conducted in 52 adult patients (median age, 47 years) with various hematologic malignancies undergoing TCD transplantation from HLA-matched related donors without the use of ATG. The cytoreductive regimen consisted of hyperfractionated total body irradiation (HFTBI), thiotepa, and fludarabine. The preferred source of the graft was peripheral blood stem cells (PBSCs). No additional graft rejection or GvHD prophylaxis was given. All evaluable patients engrafted without any immune-mediated graft rejections. Disease-free survival (DFS) at 3 years was 61% in all patients, and 70% in patients with standard-risk disease. Acute GvHD was limited to grade 2 in 8% and chronic GvHD in 9% of patients. Life-threatening OIs occurred in 3 of 52 patients and was fatal in 1. This study demonstrates durable engraftment with a low incidence of GvHD despite the lack of ATG, as well as the curative potential of this regimen. PMID:17717135

  3. Post-Thaw Viable CD45+ Cells and Clonogenic Efficiency are Associated with Better Engraftment and Outcomes after Single Cord Blood Transplantation in Adult Patients with Malignant Diseases.

    PubMed

    Castillo, Nerea; García-Cadenas, Irene; Barba, Pere; Martino, Rodrigo; Azqueta, Carmen; Ferrà, Christelle; Canals, Carme; Sierra, Jorge; Valcárcel, David; Querol, Sergio

    2015-12-01

    The quantity of cells is widely accepted as the main factor influencing the outcome after umbilical cord blood transplantation (UCBT) however, the quality of the cord blood units (CBUs) has been less studied. In order to determine the impact of qualitative variables in UCBT outcomes, we conducted a multicenter retrospective study in adult patients with hematological malignancies who underwent single UCBT after a common myeloablative conditioning regimen. One hundred and ten patients from 3 institutions [median age, 35 years (range 18-55)] were included. Quantitative (TNC and total CD34+cells) and qualitative variables [viable CD45+ (vCD45+), vCD34+ and clonogenic efficiency [(CLONE), quotient of post-thaw colony-forming units (CFU)] and pre-freeze CD34+ cells predicted engraftment in univariate analysis however, only 2 qualitative variables remained significant in the multivariate analysis. Infusion of more than 2 × 10(7) post-thaw vCD45+ cells per kilogram was significantly associated with faster neutrophil (P = .01), platelet engraftment (P = .01), higher disease-free (P = .01) and overall survival (0.02). In addition, CLONE ≥ 20% predicted a faster neutrophil (P = .005), platelet engraftment (P = .01) and contributed to decrease the non-relapse mortality (P = .02). Our study suggests that the vCD45+ cells dose and CLONE are powerful surrogate markers of graft quality and can potentially help on CBUs selection if tested with representative reference samples.

  4. Malignant adenolymphoma.

    PubMed

    Moosavi, H; Ryan, C; Schwartz, S; Donnelly, J A

    1980-01-01

    Adenolymphoma (Warthin's tumor) is a well studied benign tumor of the salivary gland. Malignant transformation of such a tumor is rare and not well documented in the literature. The light microscopic and ultrastructural features of an undifferentiated carcinoma arising in an adenolymphoma in the parotid gland of a middle aged male are described, and the relevant literature is reviewed. Similarities between the benign adenolymphoma and the undifferentiated malignant tumor, such as the presence of interstitial lymphoplasmacytic cell infiltrates, dark and light epithelial cells, similar cytoplasmic organelles, and nuclear morphology, suggest a malignant transformation of a previously existing benign adenolymphoma.

  5. Systemic therapy for selected skull base sarcomas: Chondrosarcoma, chordoma, giant cell tumour and solitary fibrous tumour/hemangiopericytoma.

    PubMed

    Colia, Vittoria; Provenzano, Salvatore; Hindi, Nadia; Casali, Paolo G; Stacchiotti, Silvia

    2016-01-01

    This review highlights the data currently available on the activity of systemic therapy in chondrosarcoma, chordoma, giant cell tumour of the bone (GCTB) and solitary fibrous tumour, i.e., four rare sarcomas amongst mesenchymal malignancy arising from the skull base.

  6. Systemic therapy for selected skull base sarcomas: Chondrosarcoma, chordoma, giant cell tumour and solitary fibrous tumour/hemangiopericytoma.

    PubMed

    Colia, Vittoria; Provenzano, Salvatore; Hindi, Nadia; Casali, Paolo G; Stacchiotti, Silvia

    2016-01-01

    This review highlights the data currently available on the activity of systemic therapy in chondrosarcoma, chordoma, giant cell tumour of the bone (GCTB) and solitary fibrous tumour, i.e., four rare sarcomas amongst mesenchymal malignancy arising from the skull base. PMID:27330421

  7. Primary malignancy, secondary malignancy and semimalignancy of bone tumors.

    PubMed

    Uehlinger, E

    1976-01-01

    1. Bone tumors in contrast to tumors in soft tissue, show a wide variety of clinical behavior qualified by the expressions semimalignancy, low grade of malignancy, sarcomatous degeneration and primarily benign bone tumors and bone lesions. 2. The term semimalignancy is characterized by local invasive and destructive tumor growth with a tendency to recur locally but no hematogeneous spreading. Semimalignancy requires wide en-bloc resection of amputation. 3. The term low grade malignancy is used to describe a tumor of very slow growth and with very late metastasis. Low-grade malignancy requires resection with careful preservation of functional structures. 4. The term secondary malignancy means the sarcomatous degeneration of a primarily benign lesion or bone tumor. This transformation is enhanced by irradiation and probably by acceleration of the normal turnover of bone tissue. In Paget's disease sarcomatous degeneration is to be expected in 2 percent of cases and in fibrous dysplasia in 0.5 percent of cases. 5. Sarcomatous degeneration of bone infarcts is rare, but an increase is to be expected due to an increased frequency of bone infarcts caused by long-term treatment with cortisone. 6. Primary bone tumors and recurrences show the same structure and cytology. In a minority of cases the recurrences are less differentiated; in a very few cases the recurrences are more highly differentiated and have a better prognosis than the initial lesion. PMID:1070716

  8. Malignant hyperthermia.

    PubMed

    Brockhouse, R T

    1979-04-01

    A case has been presented that illustrates successful managment of a patient with suspected malignant hyperthermia. The causes of this disorder are uncertain. If screening procedures identify a patient as susceptible to this disorder, careful planning in the preoperative stage is indicated. Preparedness during the operative procedure for any emergency is mandatory. Early and effective treatment seems to be the only method of preventing mortality with patients experiencing malignant hyperthermia. PMID:285135

  9. Malignant oncocytoma.

    PubMed

    Laurian, N; Zohar, Y; Kende, L

    1977-09-01

    A case of malignant oncocytoma of the parotid gland in a 32-year-old male is presented. Ten months after parotidectomy an undifferentiated carcinoma, in which oncocytes still could be recognized, developed in the operated area. According to the literature available to us, this is the second reported case in which malignant transformation in a benign oncocytoma of the salivary gland has been observed.

  10. [Solitary fibrous tumor in the tongue: case report and review of the literature].

    PubMed

    Nkenke, E; Fenner, M; Lell, M; Vairaktaris, E; Neukam, F W; Faller, G

    2007-04-01

    Solitary fibrous tumors (SFT) are rare, mostly fibroblastic tumors usually situated in the pleura. Extrapleural manifestations have been described. However, the oral cavity is an uncommon localisation of this tumor. We report the very unusual case of an SFT affecting the tongue that could be removed completely because of its clear delineation. Intraoperative incisional biopsies were used to exclude malignancy. For definitive classification of the tumor, additional histopathologic examinations had to be carried out. Because SFT exhibit malignant behavior only in exceptional cases and their recurrence after complete removal has never been encountered, surgery can focus on the preservation of undisturbed function of the tongue.

  11. Solitary Fibrous Tumor of the Kidney Developing Local Recurrence.

    PubMed

    Usuba, Wataru; Sasaki, Hideo; Yoshie, Hidekazu; Kitajima, Kazuki; Kudo, Hiroya; Nakazawa, Ryuto; Sato, Yuichi; Takagi, Masayuki; Chikaraishi, Tatsuya

    2016-01-01

    Solitary fibrous tumor (SFT) of the kidney is a rare entity and usually displays a favorable prognosis. We herein report a second case of renal SFT developing local recurrence. A 50-year-old man was referred to our hospital because of a left renal mass. An abdominal CT detected a large renal tumor and radical nephrectomy was performed with a possible diagnosis of renal cell carcinoma. The resected tumor size was measured at 17 × 11 × 8 cm. Grossly, necrosis was observed in central lesion of the tumor but hemorrhage was not observed. Microscopically, the tumor consisted of spindle-shaped cells with scant cytoplasm accompanied by hyalinized collagenous tissue, which displayed hemangiopericytomatous patterns. The cellularity was normal and nuclear pleomorphism was not observed. Ki-67 labeling index was less than 3%. The pathological diagnosis of SFT was made without obvious malignant findings. Three years after the surgery, a follow-up CT scan detected a mass lesion in the tumor bed. Surgical resection was performed and the resected tumor was compatible with local recurrence of the SFT without obvious malignant findings. Renal SFT should be carefully monitored even in the absence of obvious malignant findings. PMID:27239363

  12. Solitary Fibrous Tumor of the Kidney Developing Local Recurrence

    PubMed Central

    Usuba, Wataru; Sasaki, Hideo; Yoshie, Hidekazu; Kitajima, Kazuki; Kudo, Hiroya; Nakazawa, Ryuto; Sato, Yuichi; Takagi, Masayuki; Chikaraishi, Tatsuya

    2016-01-01

    Solitary fibrous tumor (SFT) of the kidney is a rare entity and usually displays a favorable prognosis. We herein report a second case of renal SFT developing local recurrence. A 50-year-old man was referred to our hospital because of a left renal mass. An abdominal CT detected a large renal tumor and radical nephrectomy was performed with a possible diagnosis of renal cell carcinoma. The resected tumor size was measured at 17 × 11 × 8 cm. Grossly, necrosis was observed in central lesion of the tumor but hemorrhage was not observed. Microscopically, the tumor consisted of spindle-shaped cells with scant cytoplasm accompanied by hyalinized collagenous tissue, which displayed hemangiopericytomatous patterns. The cellularity was normal and nuclear pleomorphism was not observed. Ki-67 labeling index was less than 3%. The pathological diagnosis of SFT was made without obvious malignant findings. Three years after the surgery, a follow-up CT scan detected a mass lesion in the tumor bed. Surgical resection was performed and the resected tumor was compatible with local recurrence of the SFT without obvious malignant findings. Renal SFT should be carefully monitored even in the absence of obvious malignant findings. PMID:27239363

  13. Solitary Fibrous Tumor of the Kidney Developing Local Recurrence.

    PubMed

    Usuba, Wataru; Sasaki, Hideo; Yoshie, Hidekazu; Kitajima, Kazuki; Kudo, Hiroya; Nakazawa, Ryuto; Sato, Yuichi; Takagi, Masayuki; Chikaraishi, Tatsuya

    2016-01-01

    Solitary fibrous tumor (SFT) of the kidney is a rare entity and usually displays a favorable prognosis. We herein report a second case of renal SFT developing local recurrence. A 50-year-old man was referred to our hospital because of a left renal mass. An abdominal CT detected a large renal tumor and radical nephrectomy was performed with a possible diagnosis of renal cell carcinoma. The resected tumor size was measured at 17 × 11 × 8 cm. Grossly, necrosis was observed in central lesion of the tumor but hemorrhage was not observed. Microscopically, the tumor consisted of spindle-shaped cells with scant cytoplasm accompanied by hyalinized collagenous tissue, which displayed hemangiopericytomatous patterns. The cellularity was normal and nuclear pleomorphism was not observed. Ki-67 labeling index was less than 3%. The pathological diagnosis of SFT was made without obvious malignant findings. Three years after the surgery, a follow-up CT scan detected a mass lesion in the tumor bed. Surgical resection was performed and the resected tumor was compatible with local recurrence of the SFT without obvious malignant findings. Renal SFT should be carefully monitored even in the absence of obvious malignant findings.

  14. Fibrous-Ceramic/Aerogel Composite Insulating Tiles

    NASA Technical Reports Server (NTRS)

    White, Susan M.; Rasky, Daniel J.

    2004-01-01

    Fibrous-ceramic/aerogel composite tiles have been invented to afford combinations of thermal-insulation and mechanical properties superior to those attainable by making tiles of fibrous ceramics alone or aerogels alone. These lightweight tiles can be tailored to a variety of applications that range from insulating cryogenic tanks to protecting spacecraft against re-entry heating. The advantages and disadvantages of fibrous ceramics and aerogels can be summarized as follows: Tiles made of ceramic fibers are known for mechanical strength, toughness, and machinability. Fibrous ceramic tiles are highly effective as thermal insulators in a vacuum. However, undesirably, the porosity of these materials makes them permeable by gases, so that in the presence of air or other gases, convection and gas-phase conduction contribute to the effective thermal conductivity of the tiles. Other disadvantages of the porosity and permeability of fibrous ceramic tiles arise because gases (e.g., water vapor or cryogenic gases) can condense in pores. This condensation contributes to weight, and in the case of cryogenic systems, the heat of condensation undesirably adds to the heat flowing to the objects that one seeks to keep cold. Moreover, there is a risk of explosion associated with vaporization of previously condensed gas upon reheating. Aerogels offer low permeability, low density, and low thermal conductivity, but are mechanically fragile. The basic idea of the present invention is to exploit the best features of fibrous ceramic tiles and aerogels. In a composite tile according to the invention, the fibrous ceramic serves as a matrix that mechanically supports the aerogel, while the aerogel serves as a low-conductivity, low-permeability filling that closes what would otherwise be the open pores of the fibrous ceramic. Because the aerogel eliminates or at least suppresses permeation by gas, gas-phase conduction, and convection, the thermal conductivity of such a composite even at

  15. bcl-2 expression in pleural and extrapleural solitary fibrous tumours.

    PubMed

    Chilosi, M; Facchettti, F; Dei Tos, A P; Lestani, M; Morassi, M L; Martignoni, G; Sorio, C; Benedetti, A; Morelli, L; Doglioni, C; Barberis, M; Menestrina, F; Viale, G

    1997-04-01

    This study evaluated the immunoreactivity for bcl-2, a molecule involved in the control of programmed cell death, in cases of pleural (14) and extrapleural (2) solitary fibrous tumour (SFT), malignant mesotheliomas of different histological types, and a variety of extrapleural CD34-positive and CD34-negative spindle-cell tumours. In all SFTs, strong and diffuse immunostaining was demonstrated with anti-bel-2 antibody, sharply contrasting with the complete lack of staining observed in all mesotheliomas. The specificity of immunodetection of bcl-2 in SFT was confirmed by immunoblot analysis, showing a band consistent with the bcl-2 protein. At extrapleural locations, strong bcl-2 immunoreactivity was observed in Schwannoma (2/3 cases), synovial sarcoma (4/4 cases), and all cases of CD34-positive gastrointestinal stromal tumour (GIST; 10/10 cases). Most sarcomas were bcl-2-negative. Lack of bcl-2 expression was demonstrated in tumours which can pose problems in the differential diagnosis of SFT and can exhibit haemangiopericytoma-like features, including haemangiopericytoma (3 cases), dermatofibrosarcoma protuberans (16 cases), and deep-seated fibrous histiocytoma (3 cases). The constitutive expression of bcl-2 in SFT widens the spectrum of available markers for these tumours, providing a useful adjunct to their differential diagnosis in difficult cases at pleural and extrapleural sites, and contributing to the understanding of their histogenesis and molecular pathogenesis.

  16. Solitary fibrous tumour and haemangiopericytoma: evolution of a concept.

    PubMed

    Gengler, C; Guillou, L

    2006-01-01

    Haemangiopericytoma (HPC) was described in 1942 by Stout and Murray as a distinctive soft tissue neoplasm, presumably of pericytic origin, exhibiting a characteristic well-developed "staghorn" branching vascular pattern. Over the years, it appeared that this growth pattern was a non-specific one, shared by numerous, unrelated benign and malignant lesions, and that HPC was better considered as a diagnosis of exclusion. Three categories of lesion may now be individualized within the heterogeneous group of HPC-like neoplasms. The first category corresponds to those non-HPC neoplasms that occasionally display HPC-like features (e.g. synovial sarcoma). Lesions belonging to the second category show clear evidence of myoid/pericytic differentiation and correspond to true HPCs. They generally show a benign clinical course, and include glomangiopericytoma/myopericytoma, infantile myofibromatosis (previously called infantile HPC), and a subset of sinonasal HPCs. The third category is the solitary fibrous tumour (SFT) lesional group, which includes fibrous-to-cellular SFTs, and related lesions such as giant cell angiofibromas and lipomatous HPCs. In practice, any HPC-like lesion can be allocated to one of these categories, leaving the ill-defined "haemangiopericytoma" category empty.

  17. Gastric calcifying fibrous tumor removed by endoscopic submucosal dissection

    PubMed Central

    Ogasawara, Naotaka; Izawa, Shinya; Mizuno, Mari; Tanabe, Atsushi; Ozeki, Tomonori; Noda, Hisatsugu; Takahashi, Emiko; Sasaki, Makoto; Yokoi, Toyoharu; Kasugai, Kunio

    2013-01-01

    The World Health Organization describes calcifying fibrous tumors (CFTs) as rare, benign lesions characterized by hypocellular, densely hyalinized collagenization with lymphoplasmacytic infiltration. These tumors rarely involve the gastrointestinal (GI) tract. A routine endoscopic upper gastrointestinal screen detected a 10-mm submucosal tumor (SMT) in the lesser curvature of the lower corpus of the stomach of an apparently healthy, 37-year-old woman with no history of Helicobacter pylori infection. Endoscopic ultrasonography (EUS) localized the internally isoechoic, homogeneous SMT mainly within the submucosa. Malignancy was ruled out using endoscopic submucosal dissection (ESD). A pathological examination confirmed complete resection of the SMT, and defined a hypocellular, spindle-cell tumor with a densely hyalinized, collagenous matrix, scattered lymphoplasmacytic aggregates as well as a few psammomatous, dystrophic calcified foci. The mass was immunohistochemically positive for vimentin and negative for CD117 (c-kit protein), CD34, desmin, smooth muscle actin (SMA) and S100. Therefore, the histological findings were characteristic of a CFT. To date, CFT resection by ESD has not been described. This is the first case report of a gastric calcifying fibrous tumor being completely resected by ESD after endoscopic ultrasonography. PMID:24044047

  18. Pleural malignancies.

    PubMed

    Vargas, F S; Teixeira, L R

    1996-07-01

    Carcinoma of the lung, metastatic breast carcinoma, and lymphoma are responsible for approximately 75% of all malignant pleural effusions. The presence of malignant cells in the pleural fluid or in the parietal pleura confirms the diagnosis. Recently, several authors have proposed the combination of morphometric procedures and quantitative analysis of nucleolar organizer regions stained by silver nitrate. Videothoracoscopy is recommended for patients suspected of having a malignant pleural effusion in whom the diagnosis is not established after two cytologic studies of the fluid and one needle biopsy. The standard treatment is the intrapleural instillation of a chemical agent to produce a pleurodesis. The recommended sclerosant is talc, a tetracycline derivative, or Corynebacterium parvum where it is available. When a patient is not an ideal candidate for chemical pleurodesis, the options include symptomatic treatment, serial thoracentesis, implantation of a pleuroperitoneal shunt, and pleurectomy. PMID:9363162

  19. [Solitary fibrous tumor of the meninges].

    PubMed

    Gentil Perret, A; Mosnier, J F; Duthel, R; Brunon, J; Barral, F; Boucheron, S

    1999-12-01

    We report a case of solitary fibrous tumor (SFT) of the meninges. SFTs have been initially described in the pleura. SFTs show similar histological findings as in other locations. SFTs show a diffuse positive staining for vimentin and CD34. Meningeal SFTs have usually a favourable outcome. These tumors have to be essentially distinguished from hemangiopericytomas and fibrous meningiomas. Immunostaining for CD34 is of value for this purpose. CD34 expression is often patchy and weaker in hemangiopericytomas whereas it is rarely observed in fibrous meningiomas. It is of great interest to isolate SFTs from hemangiopericytomas because of their favourable outcome.

  20. A Case of Craniofacial Polyostotic Fibrous Dysplasia

    PubMed Central

    Clark, Justin; Carson, William

    2010-01-01

    We present the case of a patient with craniofacial polyostotic fibrous dysplasia. Polyostotic fibrous dysplasia is relatively rare and usually presents in late childhood/early adulthood. It is occasionally associated with endocrine disorders such as McCune-Albright syndrome. The benign pathology of this bone tumor belies its implications in the region of the skull base. Craniofacial polyostotic fibrous dysplasia can have devastating complications depending on which ostia are involved, including vision loss. Our patient was already beginning to experience visual field deficits from ischemic neuropathy. He was treated surgically with optic nerve decompression; however, the efficacy of this approach is currently being debated. PMID:22470752

  1. CD34-negative solitary fibrous tumour resistant to imatinib.

    PubMed

    Watanabe, Koichiro; Otsu, Satoshi; Morinaga, Ryotaro; Shirao, Kuniaki

    2013-01-01

    A 75-year-old man presented to our hospital with multifocal thickening of the left pleura and left pleural effusion. Histology of the pleura showed uniform and bipolar spindle cells with moderate mitosis in a collagenised stroma. It further showed abundant blood vessels in a haemangiopericytoma-like pattern. These findings were strongly suggestive of malignant solitary fibrous tumour (SFT). The tumour showed negative staining for CD34. The loss of CD34 expression could imply histologically high-grade tumour, as reported previously. Imatinib, a multityrosine kinase inhibitor with targets, including platelet-derived growth factor receptor (PDGFR)-α and PDGFR-β, has antitumour activity in some patients with SFT. Unfortunately, imatinib treatment failed to control disease progression in the present case that expressed PDGFR-β, but not PDGFR-α. This report described a case of CD34-negative SFT resistant to imatinib.

  2. [Intraabdominal mass with difficult diagnosis: Solitary fibrous tumor].

    PubMed

    Alonso, Inmaculada; Hernández-Guerra, Manuel; González, Yanira; Gimeno-García, Antonio; Méndez, Rafael; Malagón, Antonio; Quintero Carrión, Enrique

    2010-04-01

    Solitary fibrous tumor (SFT) is a rare neoplasm of mesenchymal origin. The most commonly reported locations are the pleura and meninges. Less frequently, SFT manifests as an asymptomatic mass in the pancreas, liver, peritoneum or kidney. Clinical and radiological findings have failed to provide any specific diagnostic pattern but allow malignant development to be suspected due to infiltration or metastasis. In addition, preoperative cytology often yields inconclusive or misleading results. Therefore the definitive diagnosis is achieved after both surgical resection and immunohistochemical analysis, with markers such as CD34, vimentin and desmin. We present a case of SFT, which was difficult to diagnose, even after an extensive battery of tests based on imaging techniques.

  3. Optical spectroscopy of benign and malignant breast tissues

    NASA Astrophysics Data System (ADS)

    Yang, Yuanlong; Katz, Alvin; Celmer, Edward J.; Zurawska-Szczepaniak, Margaret; Alfano, Robert R.

    1996-04-01

    Fluorescence spectroscopy was applied to characterize normal, malignant and adipose breast tissues. Excitation, emission, and synchronized diffusive reflectance spectral scans were measured on over one hundred specimens for the purpose of developing an improved spectroscopic diagnostic technique. These techniques were able to successfully distinguish malignant tissue from adipose glandular fibrous and normal tissue. A sensitivity of 91% for fifty-six (56) malignant specimens with specificity of 91% for forty-six (46) benign tissue specimens has been achieved, using pathology as the golden standard.

  4. Malignant hyperthermia.

    PubMed

    Taiclet, L

    1985-01-01

    Despite numerous reviews and clinical reports, much remains to be learned about the cause, treatment, and prevention of malignant hyperthermia.Among the most worrisome concerns of the clinician administering anesthesia is the malignant hyperthermia crisis. When it arises, it is always frightening-and sometimes fatal. Usually occurring very suddenly and without warning, malignant hyperthermia is considered to be a hypercatabolic crisis; the condition is known to affect humans and certain breeds of pigs. The exact triggering mechanisms of malignant hyperthermia (MH) in humans are not known, but a crisis can be initiated by volatile general anesthetics, neuromuscular blocking agents, and amide local anesthetics. Although a history of an MH crisis is a diagnostic aid, previous uneventful exposure to anesthesia does not guarantee the safety of the patient in subsequent anesthetic procedures.(1) For these reasons, it is important for the anesthesiologist to be aware of the initial signs of MH and to be prepared to provide immediate treatment to reverse such a crisis. PMID:3865561

  5. Malignant hyperthermia.

    PubMed Central

    Ben Abraham, R.; Adnet, P.; Glauber, V.; Perel, A.

    1998-01-01

    Malignant hyperthermia is a rare autosomal dominant trait that predisposes affected individuals to great danger when exposed to certain anaesthetic triggering agents (such as potent volatile anaesthetics and succinylcholine). A sudden hypermetabolic reaction in skeletal muscle leading to hyperthermia and massive rhabdomyolysis can occur. The ultimate treatment is dantrolene sodium a nonspecific muscle relaxant. Certain precautions should be taken before anaesthesia of patients known to be susceptible to malignant hyperthermia. These include the prohibition of the use of triggering agents, monitoring of central body temperature and expired CO2, and immediate availability of dantrolene. In addition, careful cleansing of the anaesthesia machine of vapours of halogenated agents is recommended. If these measures are taken, the chances of an MH episode are greatly reduced. When malignant hyperthermia-does occur in the operating room, prompt recognition and treatment usually prevent a potentially fatal outcome. The most reliable test to establish susceptibility to malignant hyperthermia is currently the in vitro caffeine-halothane contracture test. It is hoped that in the future a genetic test will be available. PMID:9538480

  6. Fibrous capsule formation around titanium and copper.

    PubMed

    Suska, Felicia; Emanuelsson, Lena; Johansson, Anna; Tengvall, Pentti; Thomsen, Peter

    2008-06-15

    Previous studies suggest that implant material properties influence the quality and quantity of fibrous capsule around the implant. However, the precise relation between material surface chemistry, early inflammatory response, and fibrous subsequent repair outcome is still unknown. Titanium (Ti) and copper (Cu), surfaces with different inflammatory potential, were implanted subcutaneously in rats and retrieved fibrous capsules were analyzed after 28 and 56 days. Histological examinations show pronounced differences in capsule morphology. The fibrous capsule around Ti was thinner than that around Cu, with less number of the inflammatory cells in the layer close to the implant surface, and less and smaller blood vessels. The capsule around Cu was thick, with a large number of the inflammatory cells, particularly macrophages and giant cells, and increased number of blood vessels. Our study suggests that material surface properties, which initiate early, multiple cellular inflammatory events, are also associated with increased fibrosis and angiogenesis during repair phase. PMID:17896778

  7. Fibrous dysplasia of the temporal bone.

    PubMed

    Mallina, S; Philip, R; Chong, A W; Gurdeep, S

    2007-06-01

    Fibrous dysplasia is a benign disease characterized by a progressive replacement of normal bone elements with fibrous tissue and the temporal bone involvement is uncommon. It has a male:female ratio of 2:1 and is seen more commonly in the first two decades of life. Diagnosis is made based on radiological findings and the modality of treatment is mainly conservative. However, surgery is reserved for preserving function and preventing complications. Fibrous dysplasia in the region of craniofacial bones is of particular interest to the otolaryngologist as it causes deformity and dysfunction that can be debilitating. We present a 49 year old Chinese gentleman with complaints of chronic dizziness over the last one year and had no obvious hearing impairment. Computed tomography of the mastoid revealed fibrous dysplasia of the right temporal bone. This case is of particular interest due to the late presentation as it is more commonly seen in the first two decades of life.

  8. General Information about Ovarian Low Malignant Potential Tumors

    MedlinePlus

    ... Malignant Potential Tumors Treatment (PDQ®)–Patient Version General Information About Ovarian Low Malignant Potential Tumors Go to ... the PDQ Adult Treatment Editorial Board . Clinical Trial Information A clinical trial is a study to answer ...

  9. [Solitary fibrous tumours of the kidney].

    PubMed

    Gres, Pascal; Avances, Christophe; Ben Naoum, Kamel; Chapuis, Héliette; Costa, Pierre

    2004-02-01

    Solitary fibrous tumours (SFT) are mesenchymal tumours that usually arise from the pleura. Renal SFT are exceptional (9 cases reported in the literature). The authors report a new case discovered during assessment of HT and treated by radical right nephrectomy. The histological appearance is characteristic: a tumour with a fibrous centre, composed of a monomorphic proliferation of spindle cells, with positive CD 34, CD 99, and bcl 2 labelling. The prognosis after complete resection is generally favourable.

  10. Solitary fibrous tumor of filum terminale.

    PubMed

    Wu, Yanping; Huang, Biao; Liang, Changhong

    2012-01-01

    Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that most commonly arises from the visceral or parietal pleura. Solitary fibrous tumor with a primary site in the filum terminale has not been reported previously in the literature. Here we report a case of SFT occurring in the filum terminale. The characteristic imaging feature of this tumor is hypointensity on T2-weighted images. Even though rare, SFT should be considered in the differential diagnosis of tumors occurring intraspinally, even in the filum terminale.

  11. Variable Solitary Fibrous Tumor Locations

    PubMed Central

    Zhanlong, Ma; Haibin, Shi; Xiangshan, Fang; Jiacheng, Song; Yicheng, Ni

    2016-01-01

    Abstract The aim of the study is to describe the radiological imaging features of different solitary fibrous tumors (SFTs) locations and present histopathological correlations. From 2007 to 2013, 20 cases of histologically confirmed that SFTs were retrospectively analyzed with computed tomography (CT; 9/20), magnetic resonance imaging (MRI; 5/20), or both CT and MRI (6/20). All 20 SFTs were well defined, lobular, soft-tissue masses, and 60% were located outside of the pleura. One pleural case invaded to the 10th thoracic vertebra and had lung metastases. Images revealed 11 heterogeneous lesions that exceeded 3.0 ± 0.203 cm along the greatest axis with patchy necrotic foci, and 9 homogeneous lesions <3.0 ± 0.203. Microscopically, all SFTs were proliferative spindle cells with varying degrees of fibrosis and interspersed vessel branching. Cells were strongly immunopositive for CD34. Here we review variable imaging findings of SFTs, which can be within the pleura as well as within other serosal tissues such as the meninges and postperitoneum. SFTs > 3.0 ± 0.203 cm along the greatest axis appeared to be mixed patterns, whereas SFTs < 3.0 ± 0.203 cm had isodense appearances. SFTs cells were CD34 immunopositive and surgery was a first-line treatment choice. PMID:27043668

  12. A structure-based extracellular matrix expansion mechanism of fibrous tissue growth.

    PubMed

    Kalson, Nicholas S; Lu, Yinhui; Taylor, Susan H; Starborg, Tobias; Holmes, David F; Kadler, Karl E

    2015-01-01

    Embryonic growth occurs predominately by an increase in cell number; little is known about growth mechanisms later in development when fibrous tissues account for the bulk of adult vertebrate mass. We present a model for fibrous tissue growth based on 3D-electron microscopy of mouse tendon. We show that the number of collagen fibrils increases during embryonic development and then remains constant during postnatal growth. Embryonic growth was explained predominately by increases in fibril number and length. Postnatal growth arose predominately from increases in fibril length and diameter. A helical crimp structure was established in embryogenesis, and persisted postnatally. The data support a model where the shape and size of tendon is determined by the number and position of embryonic fibroblasts. The collagen fibrils that these cells synthesise provide a template for postnatal growth by structure-based matrix expansion. The model has important implications for growth of other fibrous tissues and fibrosis. PMID:25992598

  13. Meningeal solitary fibrous tumor: report of a case and literature review.

    PubMed

    Deniz, Kemal; Kontas, Olgun; Tucer, Bulent; Kurtsoy, Ali

    2005-01-01

    Solitary fibrous tumor is a rare neoplasm that most often involves the pleura. The increasing numbers of this neoplasm have also been reported to date in extrapleural sites. We report a case of a twenty-four-year-old female with right frontal mass. Histologically, the tumor composed of spindle cell proliferation. Tumor cells were found to be positive for CD34 and CD117 with immunohistochemical studies. Ten months follow-up was uneventful. Seventy seven cases of meningeal solitary fibrous tumor from the literature are analysed and pathological, immunohistochemical and clinical features are discussed. Solitary fibrous tumor has a slight female predominance, with a male to female ratio of 1:1.5. Age distribution is similar to meningioma ranging from 7-81 years. Approximately 23% of cases originate in the spine which is the most common meningeal location. Histopathologic examination shows uniform spindle cell proliferation with various amount of collagen. CD34-positivity usually allows discrimination from schwannomas, meningiomas and hemangiopericytomas. A differential diagnosis is important because most of the solitary fibrous tumors usually behave in a benign fashion. In this study, we also showed CD117 (Kit) expression in a case of meningeal SFT. CD117-positivity can be a good strategy for treatment in malignant and recurrent cases. Further investigations are necessary for therapeutic implication of CD117-positivity in SFT.

  14. Denosumab Treatment for Fibrous Dysplasia

    PubMed Central

    Boyce, AM; Chong, WH; Yao, J; Gafni, RI; Kelly, MH; Chamberlain, CE; Bassim, C; Cherman, N; Ellsworth, M; Kasa-Vubu, JZ; Farley, FA; Molinolo, AA; Bhattacharyya, N; Collins, MT

    2012-01-01

    Fibrous dysplasia (FD) is a skeletal disease caused by somatic activating mutations of the cAMP-regulating protein, Gsα. These mutations lead to replacement of normal bone by proliferative osteogenic precursors, resulting in deformity, fracture, and pain. Medical treatment has been ineffective in altering the disease course. RANK ligand (RANKL) is a cell surface protein involved in many cellular processes, including osteoclastogenesis, and is reported to be overexpressed in FD-like bone cells. Denosumab is a humanized monoclonal antibody to RANKL approved for treatment of osteoporosis and prevention of skeletal-related events from bone metastases. We present the case of a 9-year-old boy with severe FD who was treated with denosumab for a rapidly expanding femoral lesion. Immunohistochemical staining on a pre-treatment bone biopsy specimen revealed marked RANKL expression. He was started on monthly denosumab, with an initial starting dose of 1 mg/kg and planned 0.25 mg/kg dose escalations every three months. Over seven months of treatment he showed marked reduction in pain, bone turnover markers, and tumor growth rate. Denosumab did not appear to impair healing of a femoral fracture that occurred while on treatment. With initiation of treatment he developed hypophosphatemia and secondary hyperparathyroidism, necessitating supplementation with phosphorus, calcium and calcitriol. Bone turnover markers (BTM) showed rapid and sustained suppression. With discontinuation there was rapid and dramatic rebound of BTM) with CTX (reflecting osteoclast activity) exceeding pre-treatment levels, and accompanied by severe hypercalcemia. In this child, denosumab lead to dramatic reduction of FD expansion and FD-related bone pain. Denosumab was associated with clinically significant disturbances of mineral metabolism both while on treatment and after discontinuation. Denosumab treatment of FD warrants further study to confirm efficacy and determine potential morbidity, as well as

  15. Characteristic endobronchial ultrasound image of hemangiopericytoma/solitary fibrous tumor.

    PubMed

    Chen, Fengshi; Yoshizawa, Akihiko; Okubo, Kenichi; Date, Hiroshi

    2010-09-01

    Hemangiopericytomatous pattern is characteristic of hemangiopericytoma/solitary fibrous tumor (HPC/SFT) and certain histological features might indicate a malignant potential, but the behavior of HPC/SFT is unpredictable. Endobronchial ultrasound (EBUS) is a useful diagnostic device in that the ultrasonographic image can be viewed and the EBUS-transbronchial needle aspiration can obtain a biopsied sample. We herein report a patient undergoing multiple surgical resections of recurrent HPC/SFT. A 74-year-old man had undergone right upper lobectomy for HPC/SFT 15 years ago. He received a partial resection of the left lung and a resection of the anterior mediastinal mass for its recurrences 13 years and six years ago, respectively. He had also undergone surgery for gastric carcinoma two years ago. He then presented with a tumor measuring 3 x 4 cm in the subcarinal area. Preoperative EBUS revealed a tumor with abundant thin-walled vessel-like structures, which was consistent with HPC/SFT. The tumor was completely resected and was finally diagnosed as low-grade malignant HPC/SFT.

  16. Complex cellular composition of solitary fibrous tumor of the prostate.

    PubMed

    Gharaee-Kermani, Mehrnaz; Mehra, Rohit; Robinson, Dan R; Wei, John T; Macoska, Jill A

    2014-03-01

    Solitary fibrous tumors (SFTs) of the prostate are a rare type of spindle cell neoplasm that can demonstrate either a benign or malignant phenotype. SFTs represent a clinical challenge along with other spindle cell lesions of the prostate in terms of both diagnosis and treatment. The present study shows, for the first time, that SFTs of the prostate and other organs can comprise a mixed population of fibroblast, myofibroblast, and smooth muscle cell types. The highly proliferative component demonstrated a fibroblastic phenotype that readily underwent myofibroblast differentiation on exposure to profibrotic stimuli. Consistent with other recent studies, the prostatic SFTs demonstrated NAB2-STAT6 gene fusions that were also present in the fibroblast, myofibroblast, and smooth muscle cell types of the SFT. The results of these studies suggest that benign and malignant prostatic tumors of mesenchymal origin may be distinguished at the molecular and cellular levels, and that delineation of such defining characteristics may help elucidate the etiology and prognosis of such tumors.

  17. Malignant Myopericytoma of Shoulder: A Rare Lesion

    PubMed Central

    Moghadam, Reza Nafisi; Shabani, Masoud; Mortazavizadeh, Mohammad Reza; Zare, Saeedeh

    2016-01-01

    Myopericytoma is a soft tissue tumor with perivascular myoid differentiation. It accounts for 1% of the vascular tumors and involves mostly cutaneous or subcutaneous tissue of the limbs in adults. Malignant myopericytoma is exceedingly rare. A 15-year old girl presented with slowly progressive mass over left shoulder region. Histopathology and immunohistochemistry after complete excision revealed it as malignant myopericytoma. PMID:27398322

  18. Recurrent Monostotic Fibrous Dysplasia in the Mandible.

    PubMed

    Alves, Nilton; de Oliveira, Reinaldo José; Takehana, Denise; Deana, Naira Figueiredo

    2016-01-01

    Fibrous dysplasia (FD) is a condition in which normal bone marrow is replaced by an abnormal proliferation of new fibrous connective tissue. Female patient, white, 20 years old, attended the dental clinic reporting a slow increase in volume in the right mandible region over the last 5 years. She was examined by imaging: the panoramic X-ray revealed a lesion with the appearance of ground glass while the cone-beam computed tomography showed an extensive lesion in the region of the right hemimandible. The histopathological examination was compatible with fibrous dysplasia. Bone gammagraphy was indicated, plus an endocrinological study to eliminate polyostotic forms, which produced a negative result. Monostotic fibrous dysplasia in the right hemimandible was diagnosed. Conservative surgery was carried out and after 1 year recurrence of the tumour was observed. We may conclude that conservative surgery might not be the best choice for treatment for monostotic fibrous dysplasia in the mandible and that other options must be considered, such as radical surgery or the use of bisphosphonates. In our study, we may also conclude that it is very important to explain to the patient the possibility of recurrence of the lesion and the need for monitoring with periodic imaging studies. PMID:27340572

  19. Recurrent Monostotic Fibrous Dysplasia in the Mandible

    PubMed Central

    de Oliveira, Reinaldo José; Takehana, Denise; Deana, Naira Figueiredo

    2016-01-01

    Fibrous dysplasia (FD) is a condition in which normal bone marrow is replaced by an abnormal proliferation of new fibrous connective tissue. Female patient, white, 20 years old, attended the dental clinic reporting a slow increase in volume in the right mandible region over the last 5 years. She was examined by imaging: the panoramic X-ray revealed a lesion with the appearance of ground glass while the cone-beam computed tomography showed an extensive lesion in the region of the right hemimandible. The histopathological examination was compatible with fibrous dysplasia. Bone gammagraphy was indicated, plus an endocrinological study to eliminate polyostotic forms, which produced a negative result. Monostotic fibrous dysplasia in the right hemimandible was diagnosed. Conservative surgery was carried out and after 1 year recurrence of the tumour was observed. We may conclude that conservative surgery might not be the best choice for treatment for monostotic fibrous dysplasia in the mandible and that other options must be considered, such as radical surgery or the use of bisphosphonates. In our study, we may also conclude that it is very important to explain to the patient the possibility of recurrence of the lesion and the need for monitoring with periodic imaging studies. PMID:27340572

  20. Malignant hyperthermia.

    PubMed

    Cantin, R Y; Poole, A; Ryan, J F

    1986-10-01

    The increasing use of intravenous and inhalation sedation in the dental office has the potential of increasing the incidence of malignant hyperthermia (MH) in susceptible subjects. The object of this article is to present two cases of MH and to discuss its pathophysiology, its clinical picture, and its management in the light of the current literature. Stringent screening procedures should be adopted and maintained in order to channel suspected cases to appropriate centers for expert consultation and management. It is further advocated that a program of education for patients and their families be instituted, as it is an essential prerequisite of effective prophylaxis. PMID:2946013

  1. Solitary fibrous tumor of the kidney with massive retroperitoneal recurrence. A case presentation.

    PubMed

    Sfoungaristos, S; Papatheodorou, M; Kavouras, A; Perimenis, P

    2012-01-01

    Solitary fibrous tumor is an unusual spindle cell neoplasm that usually occurs in the pleura but has also been described in other sites. Renal presentation is rare and only 38 cases of SFT of the kidney have been described until now. Up to 90% of the tumors have benign characteristics. Local retroperitoneal recurrence is extremely rare. We report a case of a large malignant solitary fibrous tumor recurrence after the surgical excision of the primary tumor. Histological examination of the specimen confirmed the diagnosis by revealing highly positive reaction of the neoplasmatic cells for vimentin, CD34, bcl-2 and moderately positive for actin. The rate of Ki67 lied between 2-7%. No adjuvant therapy was given to the patient and he is disease-free with no imaging findings of recurrence or metastasis 9 months after the re-operation.

  2. A rare solitary fibrous tumour of kidney.

    PubMed

    Pathak, Tilak Bahadur; Nepal, Umesh

    2013-01-01

    A solitary fibrous tumour is an unusual spindle cell neoplasm. It frequently arises from the serosal surface of pleural cavity but has recently been described in diverse extrapleural sites. Urogenital localization is rare and only 36 cases of solitary fibrous tumours of the kidney have been described on published report. We report a case of a large solitary fibrous tumour clinically and radiologically thought to be renal cell carcinoma arising in the kidney of a 30 year old female. The radical nephrectomy was performed. The tumour was a well- circumscribed, solid mass attached to the renal pelvis without necrosis and haemorrhage. Histopathologically, a spindle cell neoplasia with alternating hypo and hypercellular areas, storiform, fascicular and hemangipericytoma like growth pattern and less cellular dense collagen deposits were observed. Immunohistochemical studies revealed reactivity for CD34, CD99 and Bcl-2 protein. PMID:24362666

  3. Solitary fibrous tumor of the orbit

    PubMed Central

    Gupta, Sumiti; Verma, Renuka; Sen, Rajeev; Singh, Ishwar; Marwah, Nisha; Kohli, Rachneet

    2016-01-01

    Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm originating from the mesenchyme. It was originally thought to occur exclusively in the intrathoracic region but has been recently described in extrapleural sites including the orbit. SFT of the orbit is a rare lesion, which can be misdiagnosed as hemangiopericytoma, fibrous histiocytoma, meningioma, or neurofibroma. Immunohistochemistry plays an important role. We report an orbital SFT in a 39-year-old female presented with painless, progressive proptosis, and diminished vision in the right eye for the duration of 6 months. Magnetic resonance imaging demonstrated well-defined enhancing mass lesion. The patient underwent complete tumor removal through a right fronto-orbital approach, and a pathological diagnosis of the solitary fibrous tumor was made. Postoperatively, the patient was symptom-free. Clinical and pathological findings including immunohistochemistry are presented along with a brief discussion of literature. PMID:26889300

  4. Solitary fibrous tumor of the orbit.

    PubMed

    Gupta, Sumiti; Verma, Renuka; Sen, Rajeev; Singh, Ishwar; Marwah, Nisha; Kohli, Rachneet

    2016-01-01

    Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm originating from the mesenchyme. It was originally thought to occur exclusively in the intrathoracic region but has been recently described in extrapleural sites including the orbit. SFT of the orbit is a rare lesion, which can be misdiagnosed as hemangiopericytoma, fibrous histiocytoma, meningioma, or neurofibroma. Immunohistochemistry plays an important role. We report an orbital SFT in a 39-year-old female presented with painless, progressive proptosis, and diminished vision in the right eye for the duration of 6 months. Magnetic resonance imaging demonstrated well-defined enhancing mass lesion. The patient underwent complete tumor removal through a right fronto-orbital approach, and a pathological diagnosis of the solitary fibrous tumor was made. Postoperatively, the patient was symptom-free. Clinical and pathological findings including immunohistochemistry are presented along with a brief discussion of literature.

  5. Solitary fibrous tumor of the orbit.

    PubMed

    Gupta, Sumiti; Verma, Renuka; Sen, Rajeev; Singh, Ishwar; Marwah, Nisha; Kohli, Rachneet

    2016-01-01

    Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm originating from the mesenchyme. It was originally thought to occur exclusively in the intrathoracic region but has been recently described in extrapleural sites including the orbit. SFT of the orbit is a rare lesion, which can be misdiagnosed as hemangiopericytoma, fibrous histiocytoma, meningioma, or neurofibroma. Immunohistochemistry plays an important role. We report an orbital SFT in a 39-year-old female presented with painless, progressive proptosis, and diminished vision in the right eye for the duration of 6 months. Magnetic resonance imaging demonstrated well-defined enhancing mass lesion. The patient underwent complete tumor removal through a right fronto-orbital approach, and a pathological diagnosis of the solitary fibrous tumor was made. Postoperatively, the patient was symptom-free. Clinical and pathological findings including immunohistochemistry are presented along with a brief discussion of literature. PMID:26889300

  6. Malignant hyperthermia

    PubMed Central

    2012-01-01

    Malignant hyperthermia (MH) is an uncommon, life-threatening pharmacogenetic disorder of the skeletal muscle. It presents as a hypermetabolic response in susceptible individuals to potent volatile anesthetics with/without depolarizing muscle relaxants; in rare cases, to stress from exertion or heat stress. Susceptibility to malignant hyperthermia (MHS) is inherited as an autosomally dominant trait with variable expression and incomplete penetrance. It is known that the pathophysiology of MH is related to an uncontrolled rise of myoplasmic calcium, which activates biochemical processes resulting in hypermetabolism of the skeletal muscle. In most cases, defects in the ryanodine receptor are responsible for the functional changes of calcium regulation in MH, and more than 300 mutations have been identified in the RYR1 gene, located on chromosome 19q13.1. The classic signs of MH include increase of end-tidal carbon dioxide, tachycardia, skeletal muscle rigidity, tachycardia, hyperthermia and acidosis. Up to now, muscle contracture test is regarded as the gold standard for the diagnosis of MHS though molecular genetic test is used, on a limited basis so far to diagnose MHS. The mortality of MH is dramatically decreased from 70-80% to less than 5%, due to an introduction of dantrolene sodium for treatment of MH, early detection of MH episode using capnography, and the introduction of diagnostic testing for MHS. This review summarizes the clinically essential and important knowledge of MH, and presents new developments in the field. PMID:23198031

  7. Maxillofacial fibrous dysplasia: a diagnostic challenge.

    PubMed

    Markov, Peter; Syed, Ali Zakir; Markova, Christiana; Mendes, Rui Amaral

    2016-01-01

    A 30-year-old woman presented for orthodontic treatment, with a chief symptom of a 'shifting bite' and concurrent facial asymmetry with aesthetic concerns. The patient had previously received treatment from several general dentists and several specialists, without accurate diagnosis. Radiological investigation coupled with biopsy confirmed a diagnosis of fibrous dysplasia. Proper diagnosis led to changes in the treatment plan and gave the patient realistic expectations about the options she had for the outcome of treatment. Prompt diagnosis by dental practitioners is critical to patient satisfaction and successful outcome; therefore, it is important to familiarise ourselves with the signs, symptoms and proper course of management of fibrous dysplasia. PMID:27358102

  8. Benign fibrous xanthoma of the parotid gland: a case report.

    PubMed

    Nilsen, R; Lind, O

    1978-11-01

    A fibrous xanthoma of the parotid gland is reported, the literature is reviewed with a discussion of the classification of such tumours. The present case is possibly the first report of a typical benign fibrous xanthoma of the parotid gland.

  9. Consolidation and densification methods for fibrous monolith processing

    SciTech Connect

    Sutaria, Manish P.; Rigali, Mark J.; Cipriani, Ronald A.; Artz, Gregory J.; Mulligan, Anthony C.

    2006-06-20

    Methods for consolidation and densification of fibrous monolith composite structures are provided. Consolidation and densification of two- and three-dimensional fibrous monolith components having complex geometries can be achieved by pressureless sintering. The fibrous monolith composites are formed from filaments having at least a first material composition generally surrounded by a second material composition. The composites are sintered at a pressure of no more than about 30 psi to provide consolidated and densified fibrous monolith composites.

  10. Malignant mesothelioma

    PubMed Central

    Ahmed, Ishtiaq; Ahmed Tipu, Salman; Ishtiaq, Sundas

    2013-01-01

    Malignant Mesothelioma (MM) is a rare but rapidly fatal and aggressive tumor of the pleura and peritoneum with limited knowledge of its natural history. The incidence has increased in the past two decades but still it is a rare tumor. Etiology of all forms of mesothelioma is strongly associated with industrial pollutants, of which asbestos is the principal carcinogen. Mesothelioma is an insidious neoplasm arising from mesothelial surfaces i.e., pleura (65%-70%), peritoneum (30%), tunica vaginalis testis, and pericardium (1%-2%). The diagnosis of peritoneal and Pleural mesothelioma is often delayed, due to a long latent period between onset and symptoms and the common and nonspecific clinical presentation. The definite diagnosis can only be established by diagnostic laparoscopy or open surgery along with biopsy to obtain histological examination and immunocytochemical analysis. Different treatment options are available but Surgery can achieve a complete or incomplete resection and Radical resection is the preferred treatment. Chemotherapy has an important role in palliative treatment. Photodynamic therapy is also an option under trial. Patients who successfully underwent surgical resection had a considerably longer median survival as well as a significantly higher 5-year survival. Source of Data/Study Selection: The data were collected from case reports, cross-sectional studies, Open-label studies and phase –II trials between 1973-2012. Data Extraction: Web sites and other online resources of American college of surgeons, Medline, NCBI and Medscape resource centers were used to extract data. Conclusion: Malignant Mesothelioma (MM) is a rare but rapidly fatal and aggressive tumor with limited knowledge of its natural history. The diagnosis of peritoneal and Pleural mesothelioma is often delayed, so level of index of suspicion must be kept high. PMID:24550969

  11. Cryptococcus neoformans infection in malignancy.

    PubMed

    Schmalzle, Sarah A; Buchwald, Ulrike K; Gilliam, Bruce L; Riedel, David J

    2016-09-01

    Cryptococcosis is an opportunistic invasive fungal infection that is well described and easily recognised when it occurs as meningitis in HIV-infected persons. Malignancy and its treatment may also confer a higher risk of infection with Cryptococcus neoformans, but this association has not been as well described. A case of cryptococcosis in a cancer patient is presented, and all cases of coincident C. neoformans infection and malignancy in adults published in the literature in English between 1970 and 2014 are reviewed. Data from these cases were aggregated in order to describe the demographics, type of malignancy, site of infection, clinical manifestations, treatment and outcomes of cryptococcosis in patients with cancer. Haematologic malignancies accounted for 82% of cases, with lymphomas over-represented compared to US population data (66% vs. 53% respectively). Cryptococcosis was reported rarely in patients with solid tumours. Haematologic malignancy patients were more likely to have central nervous system (P < 0.001) or disseminated disease (P < 0.001), receive Amphotericin B as part of initial therapy (P = 0.023), and had higher reported mortality rates than those with solid tumours (P = 0.222). Providers should have heightened awareness of the possibility of cryptococcosis in patients with haematologic malignancy presenting with infection. PMID:26932366

  12. Solitary fibrous tumor of the meninges: a lesion distinct from fibrous meningioma. A clinicopathologic and immunohistochemical study.

    PubMed

    Carneiro, S S; Scheithauer, B W; Nascimento, A G; Hirose, T; Davis, D H

    1996-08-01

    Seven solitary fibrous tumors (SFTs) of the meninges are presented and their clinicopathologic features are compared with those of 64 fibrous meningiomas (FM). Patients with SFT included 5 females and 2 males age 47 to 73 years. The dura-based tumors involved the parasagittal region (1), tentorium (2), cerebellopontine angle (2), and spinal region (2). One each showed invasion of brain and of a spinal nerve root. Of four SFTs with at least 1-year follow-up, one subtotally resected example recurred. No tumors metastasized. All consisted of spindle cells disposed in fascicles between prominent, eosinophilic bands of collagen. Whorls and storiform cell arrangements were lacking. Mitoses ranged from 1 to 7/10 400 x fields. MIB-1 labeling indices ranged from 1% to 18% (mean 4%). All were PAS negative and showed strong immunoreactivity for vimentin and CD34. Of cases studied, half were estrogen and all were progesterone receptor immunopositive. The majority (72%) of FMs occurred in females and most (72%) were supratentorial. Recurrence was noted in 15%. Mitotic activity varied from 0 to 3 mitoses per 10 400 x fields (mean < 1). MIB-1 labeling indices ranged from 1% to 5% (mean 1.5%). Unlike SFT, FMs were glycogen-containing and variously exhibited a storiform pattern (13 of 20), psammoma body formation (9 of 20), and calcification of collagen (4 of 20). Immunoreactivities included vimentin (100%), focal to patchy EMA (80%), S-100 protein (80%), collagen IV (25%), and patchy, mild-to-moderate CD34 staining (60%). Of cases studied, nearly half were estrogen and all were progesterone receptor staining positive. Meningeal SFTs represent a distinct morphologic entity, the morphologic and immunohistochemical features of which differ from those of FM and suggest a histogenetic relationship to pleural SFT. Although a minority histologically appear to be low grade malignant, our limited experience suggests that they behave in a benign fashion. The classification of mesenchymal

  13. Solitary fibrous tumour of the vagus nerve.

    PubMed

    Scholsem, Martin; Scholtes, Felix

    2012-04-01

    We describe the complete removal of a foramen magnum solitary fibrous tumour in a 36-year-old woman. It originated on a caudal vagus nerve rootlet, classically described as the 'cranial' accessory nerve root. This ninth case of immunohistologically confirmed cranial or spinal nerve SFT is the first of the vagus nerve.

  14. Solitary fibrous tumor of the vagina.

    PubMed

    Vadmal, M S; Pellegrini, A E

    2000-02-01

    We report of a solitary fibrous tumor (SFT) of the vagina and discuss the differential diagnosis. This is the first SFT documented, to our knowledge. SFTs should be included in the differential diagnosis of fibroblastic, myofibroblastic, and neural lesions of the skin, subcutaneous tissue, and mucosa and can be distinguished from other spindle cell neoplasms at those sites.

  15. Solitary fibrous tumor of the thyroid gland.

    PubMed

    Papi, Giampaolo; Corrado, Stefania; Uberti, Ettore Degli; Roti, Elio

    2007-02-01

    Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm more commonly involving the pleura, but recognized also in other tissues. Nineteen patients with SFT arising from the thyroid gland have been reported in the literature. The present report reviews these cases and discusses epidemiology, etio-pathogenesis, clinical-pathologic characteristics, differential diagnosis, therapy, and prognosis of thyroid SFT.

  16. Research priorities for advanced fibrous composites

    NASA Technical Reports Server (NTRS)

    Baumann, K. J.; Swedlow, J. L.

    1981-01-01

    Priorities for research in advanced laminated fibrous composite materials are presented. Supporting evidence is presented in two bodies, including a general literature survey and a survey of aerospace composite hardware and service experience. Both surveys were undertaken during 1977-1979. Specific results and conclusions indicate that a significant portion of contemporary published research diverges from recommended priorites.

  17. Development of oxide fibrous monolith systems.

    SciTech Connect

    Goretta, K. C.

    1999-03-02

    Fibrous monolithic ceramics generally have a cellular structure that consists of a strong cell surrounded by a weaker boundary phase [1-5]. Fibrous monoliths (FMs) are produced from powders by conventional ceramic fabrication techniques, such as extrusion [1,2]. When properly engineered, they exhibit fail gracefully [3-5]. Several compositions of ceramics and cermets have been processed successfully in fibrous monolithic form [4]. The most thoroughly investigated fibrous monolith consists of Si{sub 3}N{sub 4} cells and a BN cell-boundary phase [3-5]. Through appropriate selection of initial powders and extrusion and hot-pressing parameters, very tough final products have been produced. The resultant high toughness is due primarily to delamination during fracture along textured platelike BN grains. The primary objectives of our program are to develop: (1) Oxide-based FMs, including new systems with improved properties; (2) FMs that can be pressureless sintered rather than hot-pressed; (3) Techniques for continuous extrusion of FM filaments, including solid freeform fabrication (SFF) for net-shape fabrication of FMs; (4) Predictive micromechanical models for FM design and performance; and (5) Ties with industrial producers and users of FMs.

  18. Steam Reformer With Fibrous Catalytic Combustor

    NASA Technical Reports Server (NTRS)

    Voecks, Gerald E.

    1987-01-01

    Proposed steam-reforming reactor derives heat from internal combustion on fibrous catalyst. Supplies of fuel and air to combustor controlled to meet demand for heat for steam-reforming reaction. Enables use of less expensive reactor-tube material by limiting temperature to value safe for material yet not so low as to reduce reactor efficiency.

  19. Clinical Characteristics and Outcomes for Solitary Fibrous Tumor (SFT): A Single Center Experience.

    PubMed

    DeVito, Nicholas; Henderson, Evita; Han, Gang; Reed, Damon; Bui, Marilyn M; Lavey, Robert; Robinson, Lary; Zager, Jonathan S; Gonzalez, Ricardo J; Sondak, Vernon K; Letson, G Douglas; Conley, Anthony

    2015-01-01

    Solitary fibrous tumor (SFT) is a mesenchymal neoplasm of fibrous origin. The 2013 WHO classification of soft tissue tumors defines malignant forms as hypercellular, mitotically active (>4 mitosis/10 high-power fields), with cytological atypia, tumor necrosis, and/or infiltrative margins. With an IRB-approved protocol, we investigated patient records and clinicopathologic data from our Sarcoma Database to describe the clinical characteristics of both benign and malignant SFT. All pathology specimens were reviewed by two pathologists. Descriptive statistics and univariate/multivariate survival analysis were performed. Patient records and Social Security Death Index were used to evaluate vital status. Of 82 patients, 47 (57%) were women and 73 (89%) were Caucasian. Median age was 62 years (range, 20 to 89). Thirty-two (39%) patients succumbed to the disease. Primary tumor site was lung/pleura in 28 (34%), abdomen/pelvis in 23 (28%), extremity in 13 (16%), and head/neck in 9 (11%) patients. Pathology was described as benign in 42 (51%) and malignant in 40 (49%) patients. Compared to benign SFT, malignant histology is associated with larger tumor size, higher mitotic counts, metastatic disease at diagnosis, and greater use of chemotherapy and radiation therapy. Gender, age, and tumor site were not significantly different between benign and malignant subtypes. By univariate analysis, only benign vs. malignant variant and complete resection positively impacted overall survival (P = 0.02 and P<0.0001, respectively). In the multivariable analysis of overall survival, receiving chemotherapy or not receiving surgery were two variables significantly associated with higher failure rate in overall survival: patients with chemotherapy vs. no chemotherapy (P = 0.003, HR = 4.55, with 95% CI: 1.68-12.34) and patients without surgery vs. with surgery (P = 0.005, HR = 25.49, with 95% CI: 2.62-247.57). Clear survival differences exist between benign and malignant SFT. While surgery

  20. Clinical Characteristics and Outcomes for Solitary Fibrous Tumor (SFT): A Single Center Experience.

    PubMed

    DeVito, Nicholas; Henderson, Evita; Han, Gang; Reed, Damon; Bui, Marilyn M; Lavey, Robert; Robinson, Lary; Zager, Jonathan S; Gonzalez, Ricardo J; Sondak, Vernon K; Letson, G Douglas; Conley, Anthony

    2015-01-01

    Solitary fibrous tumor (SFT) is a mesenchymal neoplasm of fibrous origin. The 2013 WHO classification of soft tissue tumors defines malignant forms as hypercellular, mitotically active (>4 mitosis/10 high-power fields), with cytological atypia, tumor necrosis, and/or infiltrative margins. With an IRB-approved protocol, we investigated patient records and clinicopathologic data from our Sarcoma Database to describe the clinical characteristics of both benign and malignant SFT. All pathology specimens were reviewed by two pathologists. Descriptive statistics and univariate/multivariate survival analysis were performed. Patient records and Social Security Death Index were used to evaluate vital status. Of 82 patients, 47 (57%) were women and 73 (89%) were Caucasian. Median age was 62 years (range, 20 to 89). Thirty-two (39%) patients succumbed to the disease. Primary tumor site was lung/pleura in 28 (34%), abdomen/pelvis in 23 (28%), extremity in 13 (16%), and head/neck in 9 (11%) patients. Pathology was described as benign in 42 (51%) and malignant in 40 (49%) patients. Compared to benign SFT, malignant histology is associated with larger tumor size, higher mitotic counts, metastatic disease at diagnosis, and greater use of chemotherapy and radiation therapy. Gender, age, and tumor site were not significantly different between benign and malignant subtypes. By univariate analysis, only benign vs. malignant variant and complete resection positively impacted overall survival (P = 0.02 and P<0.0001, respectively). In the multivariable analysis of overall survival, receiving chemotherapy or not receiving surgery were two variables significantly associated with higher failure rate in overall survival: patients with chemotherapy vs. no chemotherapy (P = 0.003, HR = 4.55, with 95% CI: 1.68-12.34) and patients without surgery vs. with surgery (P = 0.005, HR = 25.49, with 95% CI: 2.62-247.57). Clear survival differences exist between benign and malignant SFT. While surgery

  1. Endonasal endoscopic resection of an ethmoidal solitary fibrous tumor.

    PubMed

    Eloy, P H; Nollevaux, M C; Watelet, J B; Van Damme, J P; Collet, S T; Bertrand, B

    2006-09-01

    Solitary fibrous tumor (SFT) is an uncommon spindle cell tumor that typically arises at the level of the pleura in adults. However, SFT has also been reported in various extrapleural sites including orbit, meninges, liver, lung, salivary glands, retroperitoneum, mediastinum. In the head and neck region, SFT has been documented in the external auditory canal, larynx, thyroid, sublingual gland, tongue, parapharyngeal space and the infratemporal fossa. The nose and the paranasal sinuses are a rare site for SFT with only 14 publications in the world literature. We present an additional case of a SFT arising at the level of the right ethmoid sinus successfully removed in one piece endoscopically and review the corresponding literature.

  2. Improving vaccine efficacy against malignant glioma.

    PubMed

    Ladomersky, Erik; Genet, Matthew; Zhai, Lijie; Gritsina, Galina; Lauing, Kristen L; Lulla, Rishi R; Fangusaro, Jason; Lenzen, Alicia; Kumthekar, Priya; Raizer, Jeffrey J; Binder, David C; James, C David; Wainwright, Derek A

    2016-08-01

    The effective treatment of adult and pediatric malignant glioma is a significant clinical challenge. In adults, glioblastoma (GBM) accounts for the majority of malignant glioma diagnoses with a median survival of 14.6 mo. In children, malignant glioma accounts for 20% of primary CNS tumors with a median survival of less than 1 y. Here, we discuss vaccine treatment for children diagnosed with malignant glioma, through targeting EphA2, IL-13Rα2 and/or histone H3 K27M, while in adults, treatments with RINTEGA, Prophage Series G-100 and dendritic cells are explored. We conclude by proposing new strategies that are built on current vaccine technologies and improved upon with novel combinatorial approaches. PMID:27622066

  3. Fibrocartilaginous Dysplasia of the Bone: A Rare Variant of Fibrous Dysplasia

    PubMed Central

    Vaishya, Raju; Gupta, Nishint; Vijay, Vipul

    2016-01-01

    Fibrocartilaginous dysplasia (FCD) is a rare variant of fibrous dysplasia (FD) which frequently involves the long bones, and the proximal femur is the most commonly affected site. This benign, lytic, and expansile bone lesion causes progressive deformity in the bones and may lead to pathological fracture. Radiologically, this lesion may mimic cartilaginous benign and malignant bone tumors. Therefore, histopathological differentiation of FCD from other cartilaginous tumors is of the utmost importance. The treatment is often surgical, in the form of curettage and bone grafting or corrective osteotomy, to treat progressive deformity in the long bones. The risk of pathological fracture is high in FCD with bony deformity and often requires surgery. PMID:26918216

  4. [Pleural solitary fibrous tumor from diaphragm, being suspected of liver invasion; report of a case].

    PubMed

    Kita, Yusuke

    2012-04-01

    We report a case of solitary fibrous tumor (SFT) from diaphragmatic pleura. A 71-year-old female was admitted to our hospital because of an abnormal shadow on chest X-ray. A preoperative imaging study revealed a mass on her right diaphragm. At thoracoscopic exploration, the tumor was found to originate from diaphragm and was suspected of liver invasion. The conversion to open thoracotomy was introduced to perform appropriate surgical procedure. Pathological examination revealed proliferation of spindle cells and collagen fibers. Immuno-histochemical findings showed positive immunostaining for cell differentiation (CD) 34 and vimentin. The tumor was diagnosed as SFT with malignant potential.

  5. Solitary fibrous tumor arising slowly in the vulva over 10 years: case report and review.

    PubMed

    Taki, Mana; Baba, Tsukasa; Mandai, Masaki; Suzuki, Ayako; Mikami, Yoshiki; Matsumura, Noriomi; Konishi, Ikuo

    2012-05-01

    Solitary fibrous tumor (SFT), which was first described as a pleural lesion by Klemperer and Rabin in 1931, is a rare, slow-growing neoplasm characterized by the proliferation of fibroblast-like spindle cells arranged in a patternless manner with few nuclear atypia. Extrathoracic SFT have increasingly been recognized, but those in the genital tract are extremely rare. Gynecological SFT behave as tumors with low malignant potential to recur, metastasize, or affect nearby organs resulting in infertility. We report a case of vulvar SFT slowly growing for 10 years, and propose a strategy for the diagnosis and treatment of gynecological SFT.

  6. [Solitary fibrous tumor of the orbit: Possibly recurrent in the long-run].

    PubMed

    Rougeot, A; Barnoud, R; Ferri, J; Béziat, J-L

    2013-12-01

    Solitary fibrous tumor (SFT) is a rare etiology of progressive unilateral exophthalmia. The tumor is of mesenchymal origin and it is usually well defined. But recurrences can occur despite of complete surgical resection. Metastases have been observed. Tumors of the SFT spectrum are considered as benign or low-grade malignant. Histological features do not currently allow any prognosis. The most important prognostic factor is complete surgical resection. Craniofacial approaches provide a good view of the tumor extensions and orbital contents. Recurrent tumors must be surgically removed when possible. Complementary treatments have not proved effective. A very long-term follow-up is mandatory.

  7. Solitary fibrous tumor arising in the mons pubis: a case report.

    PubMed

    Lee, D H

    2016-01-01

    Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms of fibroblastic origin that mainly arise from the pleura. Although SFTs arising at numerous extrapleural locations have been reported, extrapleural soft tissue SFTs are extremely rare. The diagnosis of SFTs is based on histologic findings. However, given the histological variability of SFTs, immunohistochemical examination becomes important in their diagnosis. Complete surgical resection is the only and a very important prognostic factor and is recommended for the treatment of both benign and malignant SFTs with a curative intent. Here, the author reports what he believes to be the first case of an SFT originating in the mons pubis.

  8. Solitary fibrous tumor arising in the mons pubis: a case report.

    PubMed

    Lee, D H

    2016-01-01

    Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms of fibroblastic origin that mainly arise from the pleura. Although SFTs arising at numerous extrapleural locations have been reported, extrapleural soft tissue SFTs are extremely rare. The diagnosis of SFTs is based on histologic findings. However, given the histological variability of SFTs, immunohistochemical examination becomes important in their diagnosis. Complete surgical resection is the only and a very important prognostic factor and is recommended for the treatment of both benign and malignant SFTs with a curative intent. Here, the author reports what he believes to be the first case of an SFT originating in the mons pubis. PMID:27352579

  9. [Malignant hyperthermia].

    PubMed

    Metterlein, T; Schuster, F; Graf, B M; Anetseder, M

    2014-12-01

    Malignant hyperthermia (MH) is a rare hereditary, mostly subclinical myopathy. Trigger substances, such as volatile anesthetic agents and the depolarizing muscle relaxant succinylcholine can induce a potentially fatal metabolic increase in predisposed patients caused by a dysregulation of the myoplasmic calcium (Ca) concentration. Mutations in the dihydropyridine ryanodine receptor complex in combination with the trigger substances are responsible for an uncontrolled release of Ca from the sarcoplasmic reticulum. This leads to activation of the contractile apparatus and a massive increase in cellular energy production. Exhaustion of the cellular energy reserves ultimately results in local muscle cell destruction and subsequent cardiovascular failure. The clinical picture of MH episodes is very variable. Early symptoms are hypoxia, hypercapnia and cardiac arrhythmia whereas the body temperature rise, after which MH is named, often occurs later. Decisive for the course of MH episodes is a timely targeted therapy. Following introduction of the hydantoin derivative dantrolene, the previously high mortality of fulminant MH episodes could be reduced to well under 10 %. An MH predisposition can be detected using the invasive in vitro contracture test (IVCT) or mutation analysis. Few elaborate diagnostic procedures are in the developmental stage. PMID:25384957

  10. Low-grade central osteosarcoma of distal femur, resembling fibrous dysplasia

    PubMed Central

    Vasiliadis, Haris S; Arnaoutoglou, Christina; Plakoutsis, Sotiris; Doukas, Michalis; Batistatou, Anna; Xenakis, Theodoros A

    2013-01-01

    We report a case of a 32 year-old male, admitted for a lytic lesion of the distal femur. One month after the first X-ray, clinical and imaging deterioration was evident. Open biopsy revealed fibrous dysplasia. Three months later, the lytic lesion had spread to the whole distal third of the femur reaching the articular cartilage. The malignant clinical and imaging features necessitated excision of the lesion and reconstruction with a custom-made total knee arthroplasty. Intra-operatively, no obvious soft tissue infiltration was evident. Nevertheless, an excision of the distal 15.5 cm of the femur including 3.0 cm of the surrounding muscles was finally performed. The histological examination of the excised specimen revealed central low-grade osteosarcoma. Based on the morphological features of the excised tumor, allied to the clinical findings, the diagnosis of low-grade central osteosarcoma was finally made although characters of a fibrous dysplasia were apparent. Central low-grade osteosarcoma is a rare, well-differentiated sub-type of osteosarcoma, with clinical, imaging, and histological features similar to benign tumours. Thus, initial misdiagnosis is usual with the condition commonly mistaken for fibrous dysplasia. Central low-grade osteosarcoma is usually treated with surgery alone, with rare cases of distal metastases. However, regional recurrence is quite frequent after close margin excision. PMID:24147271

  11. Nature and extent of the exposure to fibrous amphiboles in Biancavilla.

    PubMed

    Bruni, B M; Pacella, A; MazziottiTagliani, S; Gianfagna, A; Paoletti, L

    2006-10-15

    An epidemiological and environmental study in the Biancavilla area (Sicily, Italy) was recently prompted by an impressively high incidence of malignant pleural mesothelioma. Epidemiology suggested an environmental contamination by amphibole fibres rather than risks related to a specific occupational activity. The aim of this study is to describe the diffusion of fibrous amphiboles in the area and identify their source. Fibrous amphiboles were found in the products from the local quarries, which had been used for years to build houses. After sampling all around Biancavilla, three sites were detected and they were characterized by an abundant presence of mineral fibres. Fibrous amphiboles were also recovered from building materials (mortar and plasters) and airborne particulates sampled in urban sites with high dust emissions due mainly to unpaved roads. Moreover, amphibole fibres were detected in the lung tissue of a woman who died of pleural mesothelioma. The results of this study suggest that the amphibole fibre diffusion in the Biancavilla environment lasted for many years and had been maximum during the sixties and the seventies with the uncontrolled development of the local building industry. Today, the environmental situation results to be changed following both the closing of the stone quarries and the urbanization works after 2001, above all the asphalting of dusty roads. Anyway sporadic mesothelioma cases have still to be expected in the next years.

  12. A case of extranodal NK/T-cell lymphoma, nasal type mimicking typical manifestations of adult-onset Still's disease (AOSD) with hemophagocytic syndrome: diagnostic consideration between malignant lymphoma without lymphadenopathy and AOSD.

    PubMed

    Kato, Takashi; Tanabe, Juichi; Kanemoto, Motoko; Kobayashi, Chiharu; Morita, Sho; Karahashi, Taro

    2009-01-01

    A 25-year-old Japanese man was suffering from high fever, sore throat, arthralgia, and macular salmon-pink eruption. The superficial lymph node was not palpable, and computed tomographic scans from the neck to pelvis demonstrated hepatosplenomegaly without apparent lymphadenopathy. Therefore, the possibility of malignant lymphoma was considered to be extremely low. Serology for Epstein Barr virus (EBV) and cytomegalovirus showed a postinfectious state, and blood culture was negative. Serum rheumatoid factor and antinuclear antibody were negative. Leukocytopenia (2.4 x 10(3)/mul) was observed, and thus a diagnosis of adult-onset Still's disease (AOSD) with hemophagocytic syndrome (HPS) was made. Fifty-five milligrams of prednisolone daily improved his symptoms and leukocytopenia promptly, but high fever with severe and progressive thrombocytopenia occurred 12 days later. Bone marrow aspiration revealed the presence of lymphoma cells and hemophagocytosis, and the CD45 gating analysis showed expanding population of CD2(+), CD3(-), and CD56(+) cells. Further, mucosal ulceration in the nasal cavity was detected. Therefore, a diagnosis of extranodal natural killer (NK)/T-cell lymphoma, nasal type, concomitant with HPS was made, and treatment with dexamethasone, etoposide, ifosfamide, carboplatin (DeVIC) regimen ameliorated his symptoms and platelet transfusion dependency. Later, a high titer of serum EBV-DNA was detected, which supported the diagnosis. Diagnosing AOSD, extranodal presentation of malignant lymphoma such as extranodal NK/T-cell lymphoma, nasal type, should be carefully considered. PMID:19609486

  13. Malignant pheochromocytoma in a young adult forming the structure simulating Homer Wright rosette: differentiation from neuroblastoma on repeating fluorescence in situ hybridization.

    PubMed

    Mori, Hiroki; Nagata, Masao; Nishijima, Nariaki; Nagura, Kiyoko; Igarashi, Hisaki; Hamazaki, Minoru; Ozono, Seiichiro; Sugimura, Haruhiko

    2008-08-01

    A peculiar adrenal tumor was analyzed using immunohistochemistry, electron microscopy, and fluorescence in situ hybridization (FISH) with multiple bacterial artificial chromosome (BAC) probes. The patient was a 34-year-old woman with a mass above the left kidney and multiple metastases. Her serum and urine dopamine level were elevated, and a diagnosis of malignant pheochromocytoma was made. The patient died approximately 3 years after her first visit. On post-mortem an adrenal tumor composed of small round cells forming Homer Wright rosette-like structures, a feature rarely observed in pheochromocytoma, was found. Immunohistochemistry was positive for chromogranin A and synaptophysin, and negative for cytokeratin, vimentin and neurofilaments. Because these results did not rule out a diagnosis of neuroblastoma, the tumor was further characterized on FISH with multiple BAC probes for loci known to be altered in neuroblastoma or pheochromocytoma, according to information in the literature that was for the most part obtained using comparative genomic hybridization. FISH demonstrated loss of heterozygosity at 11p, and gains at 16p, 19p, and 19q, a profile that favored a diagnosis of malignant pheochromocytoma over neuroblastoma. This case demonstrates that repeating FISH is useful for differential diagnosis.

  14. A case of solitary fibrous tumor arising from the palatine tonsil.

    PubMed

    Kanazawa, Takeharu; Kodama, Kozue; Nokubi, Mitsuhiro; Gotsu, Kazuo; Shinnabe, Akihiro; Hasegawa, Masayo; Kusaka, Gen; Iino, Yukiko

    2015-03-01

    Solitary fibrous tumor (SFT) is a distinctive, relatively uncommon soft-tissue neoplasm that usually arises from the pleura. It occurs at various sites; head and neck lesions are very rare. While most of these tumors have a benign course, a small number have malignant potential. We describe a rare case of SFT arising from the left palatine tonsil in a 66-year-old Japanese woman. The mass was completely resected. Immunohistochemical studies were strongly positive for CD34 and bcl-2, mildly positive for phosphorylated protein kinase B and phosphorylated extracellular signal-regulated kinase 1/2, and negative for platelet-derived growth factor receptor alpha and p53. These findings suggested that this tumor was benign. The patient showed no evidence of recurrence during 2 years of follow-up. We believe that the candidate prognostic marker should be checked to distinguish malignant from benign SFTs.

  15. Solitary Fibrous Tumor of the Lower Leg: A Rare and Difficult Diagnosis.

    PubMed

    D'Arpa, Salvatore; Rossi, Matteo; Montesano, Luigi; Florena, Ada Maria; Moschella, Francesco; Cordova, Adriana

    2015-10-01

    Solitary fibrous tumor (SFT) is a rare neoplasm that commonly originates in the pleura. Extrapleural locations are rare and for this reason sometimes difficult to diagnose. Malignant forms with local recurrence or distant metastases have been reported, also as a consequence of inappropriate treatment. In this article, we report the case of an SFT of the lower leg in a 37-year-old man. Leg SFT is a rare occurrence, and differential diagnosis may be difficult because they can mimic a variety of benign and malignant mesenchymal tumors; immunohistochemical analysis for CD34, CD99, vimentin, and Bcl-2 is necessary. Misdiagnosis carries a significant risk of inadequate removal with subsequent increased risk of recurrence and distant metastases.

  16. Solitary Fibrous Tumor of the Lower Leg: A Rare and Difficult Diagnosis.

    PubMed

    D'Arpa, Salvatore; Rossi, Matteo; Montesano, Luigi; Florena, Ada Maria; Moschella, Francesco; Cordova, Adriana

    2015-10-01

    Solitary fibrous tumor (SFT) is a rare neoplasm that commonly originates in the pleura. Extrapleural locations are rare and for this reason sometimes difficult to diagnose. Malignant forms with local recurrence or distant metastases have been reported, also as a consequence of inappropriate treatment. In this article, we report the case of an SFT of the lower leg in a 37-year-old man. Leg SFT is a rare occurrence, and differential diagnosis may be difficult because they can mimic a variety of benign and malignant mesenchymal tumors; immunohistochemical analysis for CD34, CD99, vimentin, and Bcl-2 is necessary. Misdiagnosis carries a significant risk of inadequate removal with subsequent increased risk of recurrence and distant metastases. PMID:26579334

  17. A case of solitary fibrous tumor arising from the palatine tonsil.

    PubMed

    Kanazawa, Takeharu; Kodama, Kozue; Nokubi, Mitsuhiro; Gotsu, Kazuo; Shinnabe, Akihiro; Hasegawa, Masayo; Kusaka, Gen; Iino, Yukiko

    2015-03-01

    Solitary fibrous tumor (SFT) is a distinctive, relatively uncommon soft-tissue neoplasm that usually arises from the pleura. It occurs at various sites; head and neck lesions are very rare. While most of these tumors have a benign course, a small number have malignant potential. We describe a rare case of SFT arising from the left palatine tonsil in a 66-year-old Japanese woman. The mass was completely resected. Immunohistochemical studies were strongly positive for CD34 and bcl-2, mildly positive for phosphorylated protein kinase B and phosphorylated extracellular signal-regulated kinase 1/2, and negative for platelet-derived growth factor receptor alpha and p53. These findings suggested that this tumor was benign. The patient showed no evidence of recurrence during 2 years of follow-up. We believe that the candidate prognostic marker should be checked to distinguish malignant from benign SFTs. PMID:25738718

  18. Integrally calcified solitary fibrous tumor in the retroperitoneum: a case report and review of the literature.

    PubMed

    Maki, Takehiro; Fujino, Syotaro; Misu, Kenjiro; Kaneko, Hiroyuki; Inomata, Hitoshi; Omi, Makoto; Tateno, Masatoshi; Nihei, Kazuyoshi

    2016-12-01

    Solitary fibrous tumor (SFT) is a rare stromal neoplasm and usually occurs in the thoracic cavity. We here report a case of retroperitoneal SFT with prominent calcification. A 64-year-old man presented with an incidentally detected retroperitoneal mass in the right upper abdomen. Imaging tests indicated an integrally calcified mass. The lesion was observed for 2 years and laparoscopically resected according to the patient's wish. Microscopically, the mass was mostly occupied by calcification and proliferous spindle cells were scattered with positive CD34 expression. We diagnosed morphologically benign SFT and the patient remained disease-free 1 year after the excision. There has been no report of such integrally calcified SFT. Retroperitoneal SFT is difficult to make a preoperative diagnosis, and careful follow-up after the excision is recommended because morphological malignancy does not always correspond to clinical malignancy. PMID:26943690

  19. Integrally calcified solitary fibrous tumor in the retroperitoneum: a case report and review of the literature.

    PubMed

    Maki, Takehiro; Fujino, Syotaro; Misu, Kenjiro; Kaneko, Hiroyuki; Inomata, Hitoshi; Omi, Makoto; Tateno, Masatoshi; Nihei, Kazuyoshi

    2016-12-01

    Solitary fibrous tumor (SFT) is a rare stromal neoplasm and usually occurs in the thoracic cavity. We here report a case of retroperitoneal SFT with prominent calcification. A 64-year-old man presented with an incidentally detected retroperitoneal mass in the right upper abdomen. Imaging tests indicated an integrally calcified mass. The lesion was observed for 2 years and laparoscopically resected according to the patient's wish. Microscopically, the mass was mostly occupied by calcification and proliferous spindle cells were scattered with positive CD34 expression. We diagnosed morphologically benign SFT and the patient remained disease-free 1 year after the excision. There has been no report of such integrally calcified SFT. Retroperitoneal SFT is difficult to make a preoperative diagnosis, and careful follow-up after the excision is recommended because morphological malignancy does not always correspond to clinical malignancy.

  20. [Successful operation on giant solitary fibrous tumor of the pleura].

    PubMed

    Harada, T; Nakayama, K; Kitano, T; Sakaguchi, H

    1997-11-01

    A 68-year-old man began complaining of dizziness and dyspnea on exertion 2 months ago. Chest X-ray and CT scan taken on admission revealed a giant tumor occupying almost all of the right thoracic cavity. The mediastinum, especially RA and IVC, was markedly oppressed and shifted by the tumor. MRI showed no invasive lesions of the tumor into adjacent organs. An operation was performed under the tentative diagnosis of solitary fibrous tumor (SFT) of the pleura by transcutaneous needle biopsy. The tumor was encapsulated and resected from the lower lobe of the right lung using a surgical stapler. Then the tumor was carefully dissected from the chest wall, pericardium and diaphragma, and removed completely. It weighed 3920 g and measured 22 x 20 x 20 cm. Various immunohistochemical properties of the specimen were morphologically compatible with SFT. The postoperative course was uneventful and the patient is now free of complaints. Because malignant cases of SFT have been reported a careful follow-up will be performed.

  1. [Solitary Fibrous Tumors of the Nasal and Paranasal Sinuses].

    PubMed

    Minami, Kazuhiko; Kuba, Kiyomi; Matsumura, Satoko; Hayashi, Takahiro; Nakahira, Mitsuhiko; Sugasawa, Masashi; Yamaguchi, Hiroshi; Takayanagi, Natsuko

    2015-07-01

    Solitary fibrous tumors (SFT) are uncommon neoplasm that arises in most cases from the pleura. SFT has been rarely observed in the head and neck, but SFT of the paranasal sinuses is especially rare, with 39 previously reported cases to date including those reported in this abstract. Herein we describe three cases of SFT in the paranasal sinuses that were successfully treated endoscopically. Two of the three cases involved patients with no previous history of SFT. The lesion of one of the patients was pathologically diagnosed as SFT preoperatively, but the other was diagnosed as an angiogenic tumor without any biopsies. The tumors were completely resected after arterial embolization by a transnasal endoscopic procedure. The third case involved a 43-year old man, who had undergone medial maxillectomy through a lateral rhinotomy incision to resect SFT four years and seven months before. The tumor relapsed intracranially and, therefore, a craniotomy procedure followed by endoscopic skull base surgery was performed. Radiation therapy was performed postoperatively because the recurrent tumor was pathologically identified as malignant SFT, which had been classified benign at the time of the first resection. All three patients are presently alive with no evidence of disease. PMID:26427128

  2. [Solitary Fibrous Tumors of the Nasal and Paranasal Sinuses].

    PubMed

    Minami, Kazuhiko; Kuba, Kiyomi; Matsumura, Satoko; Hayashi, Takahiro; Nakahira, Mitsuhiko; Sugasawa, Masashi; Yamaguchi, Hiroshi; Takayanagi, Natsuko

    2015-07-01

    Solitary fibrous tumors (SFT) are uncommon neoplasm that arises in most cases from the pleura. SFT has been rarely observed in the head and neck, but SFT of the paranasal sinuses is especially rare, with 39 previously reported cases to date including those reported in this abstract. Herein we describe three cases of SFT in the paranasal sinuses that were successfully treated endoscopically. Two of the three cases involved patients with no previous history of SFT. The lesion of one of the patients was pathologically diagnosed as SFT preoperatively, but the other was diagnosed as an angiogenic tumor without any biopsies. The tumors were completely resected after arterial embolization by a transnasal endoscopic procedure. The third case involved a 43-year old man, who had undergone medial maxillectomy through a lateral rhinotomy incision to resect SFT four years and seven months before. The tumor relapsed intracranially and, therefore, a craniotomy procedure followed by endoscopic skull base surgery was performed. Radiation therapy was performed postoperatively because the recurrent tumor was pathologically identified as malignant SFT, which had been classified benign at the time of the first resection. All three patients are presently alive with no evidence of disease.

  3. CELLULAR FIBROUS DERMATOFIBROMA OF THE SOLE.

    PubMed

    Wollina, U; Schönlebe, J; Nowak, A

    2016-07-01

    Cellular fibrous dermatofibroma is a rare variant of dermatofibroma/histiocytoma. We present a 61 years old female with a slow-growing, firm tumor on the sole of her right foot. The tumor was removed by slow Mohs surgery within 2 cm negative margin. Histopathologic investigation revealed a nodular encapsulated tumor composed of spindle and some epithelioid cells in a storiform growth pattern. Minimal mitotic activity was reported, however without evidence of atypical mitoses. Tumor cells expressed CD10, focally smooth muscle antigen and desmin, but remained negative for S100 protein and CD34. The diagnosis of cellular fibrous dermatofibroma was confirmed. The defect was closed by full thickness skin graft. PMID:27661268

  4. Solitary fibrous tumor of the orbit.

    PubMed

    Ing, E B; Kennerdell, J S; Olson, P R; Ogino, S; Rothfus, W E

    1998-01-01

    Solitary fibrous tumor (SFT) of the orbit is a very rare lesion that may be misdiagnosed as fibrous histiocytoma, hemangiopericytoma, or other orbital tumors. We present a 62-year-old man who presented with painless proptosis, 20 years following left eye enucleation for a presumed neurofibroma. On T2-weighted magnetic resonance imaging (MRI), a hypointense tumor almost filled his entire left orbit. There was no intracranial extension. The specimen obtained at orbital exenteration was consistent with the histologic, immunohistochemical, and electron microscopic findings of SFT. The tumor was positive for vimentin and CD34 staining but negative for S-100 protein and epithelial membrane antigen. Only nine other cases of SFT of the orbit have been documented in the literature. Recognition of SFT of the orbit as a distinct pathologic entity and further follow-up of published cases are needed to determine the prognosis of this rare lesion.

  5. Protease-degradable electrospun fibrous hydrogels

    NASA Astrophysics Data System (ADS)

    Wade, Ryan J.; Bassin, Ethan J.; Rodell, Christopher B.; Burdick, Jason A.

    2015-03-01

    Electrospun nanofibres are promising in biomedical applications to replicate features of the natural extracellular matrix (ECM). However, nearly all electrospun scaffolds are either non-degradable or degrade hydrolytically, whereas natural ECM degrades proteolytically, often through matrix metalloproteinases. Here we synthesize reactive macromers that contain protease-cleavable and fluorescent peptides and are able to form both isotropic hydrogels and electrospun fibrous hydrogels through a photoinitiated polymerization. These biomimetic scaffolds are susceptible to protease-mediated cleavage in vitro in a protease dose-dependent manner and in vivo in a subcutaneous mouse model using transdermal fluorescent imaging to monitor degradation. Importantly, materials containing an alternate and non-protease-cleavable peptide sequence are stable in both in vitro and in vivo settings. To illustrate the specificity in degradation, scaffolds with mixed fibre populations support selective fibre degradation based on individual fibre degradability. Overall, this represents a novel biomimetic approach to generate protease-sensitive fibrous scaffolds for biomedical applications.

  6. First description of Phanerozoic radiaxial fibrous dolomite

    NASA Astrophysics Data System (ADS)

    Richter, D. K.; Heinrich, F.; Geske, A.; Neuser, R. D.; Gies, H.; Immenhauser, A.

    2014-05-01

    The petrographic analysis and crystallographic analysis of concretionary carbonate cements ("coal balls") from Carboniferous paralic swamp deposits reveal the presence of (length fast) radiaxial fibrous dolomite (RFD), a fabric not previously reported from the Phanerozoic. This finding is of significance as earlier reports of Phanerozoic radiaxial fibrous carbonates are exclusively of calcite mineralogy. Dolomite concretions described here formed beneath marine transgressive intervals within palustrine coal seams. This is of significance as seawater was arguably the main source of Mg2 + ions for dolomite formation. Here, data from optical microscopy, cathodoluminescence, electron backscattered diffraction, X-ray diffraction and geochemical analyses are presented to characterize three paragenetic dolomite phases and one calcite phase in these concretions. The main focus is on the earliest diagenetic, non-stoichiometric (degree of order: 0.41-0.46) phase I, characterized by botryoidal dolomite constructed of fibres up to 110 μm wide with a systematic undulatory extinction and converging crystal axes. Petrographic and crystallographic evidence clearly qualifies phase I dolomite as radiaxial fibrous. Conversely, fascicular optical fabrics were not found. Carbon-isotope ratios (δ13C) are depleted (between - 11.8 and - 22.1‰) as expected for carbonate precipitation from marine pore-fluids in organic-matter-rich, paralic sediment. Oxygen isotope (δ18O) ratios range between - 1.3 and - 6.0‰. The earliest diagenetic nature of these cements is documented by the presence of ubiquitous, non-compacted fossil plant remains encased in phase I dolomite as well as by the complex zoned luminescence patterns in the crystals and is supported by crystallographic and thermodynamic considerations. It is argued that organic matter, and specifically carboxyl groups, reduced thermodynamic barriers for dolomite formation and facilitated Mg/CaCO3 precipitation. The data shown here

  7. Chromosomal aberrations in oral solitary fibrous tumor.

    PubMed

    Manor, Esther; Bodner, Lipa

    2007-04-15

    The results of cytogenetic analysis of a solitary fibrous tumor (SFT) of the oral cavity in a 43-year-old man is reported. The abnormal cells carried a complex translocation with the karyotype 46,XY [15 cells]/46,XYt(1;17;18)(p13;q11.2;q21)[5 cells]. This is the first case reporting chromosomal aberrations in an oral SFT.

  8. Solitary fibrous tumor of the kidney.

    PubMed

    Tritschler, P; Coulier, B; Gielen, I

    2014-01-01

    Solitary fibrous tumor (SFT) is an unusual spindle cell neoplasm rarely described in the kidney. Usually occurring in the pleura, it has also been described in various extrapleural sites. We report a rare case of SFT of the kidney fortuitously found in a 55-year-old patient. The imaging features are illustrated. The definite diagnosis was made through histological and immunohistochemical study after radical nephrectomy.

  9. Solitary fibrous tumour of the tongue.

    PubMed

    Piattelli, A; Fioroni, M; Rubini, C

    1998-09-01

    Solitary fibrous tumour (SFT) is a neoplasm most often localised in the pleura and peritoneum. The tumour is composed of spindled fibroblastic cells arranged in a haphazard way. Recently SFT has been described in many locations. Only one case of oral SFT has been described in the cheek: this is the second case of an oral SFT located in the tongue. The differential diagnosis must be made from many soft tissue tumours. SFTs stain strongly, in almost all cases, for CD34.

  10. Cervical intra-/extramedullary solitary fibrous tumour.

    PubMed

    Ogungbo, B; Prakash, S; Kulkarni, G; Bradey, N; Marks, S M; Scoones, D

    2005-06-01

    A 53-year-old man presented with a 9-month history of symptoms of right-sided weakness, tingling and hypersentivity to clothes on both sides of the body. MRI revealed a large intraspinal intradural tumour at the level of C3-C4 in the cervical cord. The final histology was a solitary fibrous tumour (SFT) of the cervical spinal cord. The radiological diagnosis, surgical management and histology are reviewed.

  11. Solitary fibrous tumor (SFT) of the pelvis.

    PubMed

    Wat, Shiu Yan J; Sur, Monalisa; Dhamanaskar, Kavita

    2008-01-01

    Solitary fibrous tumors (SFTs) are well recognized in the pleura, but their occurrence at other sites has only become appreciated in recent years, as a consequence of which extrapleural examples often go unrecognized and misdiagnosed. Because of their rarity, overall experience concerning this tumor has not been significant and reports detailing radiological findings are few. We herein report an unusual case of a large retroperitoneal pelvic SFT with features of high vascularity negating successful surgical resection.

  12. Monostotic fibrous dysplasia with Raynaud's phenomenon.

    PubMed

    Kumar, K V S Hari; Aravinda, K; Narayanan, K

    2015-01-01

    Fibrous dysplasia (FD) is a benign bone disorder characterized by alteration in bone morphology. Monostotic FD is the commonest variant and affects the craniofacial bones. Raynaud's phenomenon is recurrent vasospasm of the fingers and toes due to cold exposure. The disease is usually idiopathic or secondary to connective tissue disorders. Raynaud's phenomenon is not described previously with FD. We recently encountered two interesting patients of craniofacial monostotic FD with Raynaud's phenomenon and report the same in this report.

  13. Fibrous zinc anodes for high power batteries

    NASA Astrophysics Data System (ADS)

    Zhang, X. Gregory

    This paper introduces newly developed solid zinc anodes using fibrous material for high power applications in alkaline and large size zinc-air battery systems. The improved performance of the anodes in these two battery systems is demonstrated. The possibilities for control of electrode porosity and for anode/battery design using fibrous materials are discussed in light of experimental data. Because of its mechanical integrity and connectivity, the fibrous solid anode has good electrical conductivity, mechanical stability, and design flexibility for controlling mass distribution, porosity and effective surface area. Experimental data indicated that alkaline cells made of such anodes can have a larger capacity at high discharging currents than commercially available cells. It showed even greater improvement over commercial cells with a non-conventional cell design. Large capacity anodes for a zinc-air battery have also been made and have shown excellent material utilization at various discharge rates. The zinc-air battery was used to power an electric bicycle and demonstrated good results.

  14. Permeability of Rigid Fibrous Refractory Insulations

    NASA Technical Reports Server (NTRS)

    Marschall, J.; Milos, F. S.; Rasky, Daniel J. (Technical Monitor)

    1996-01-01

    Rigid fibrous refractory insulations (TPS tiles) are integral components of many spacecraft thermal protection systems. These materials are composed of refractory fibers With diameters on the order of 1 to 15 micrometers. They are lightweight and have an open, highly porous microstructure. Typical densities are less than 500 kilograms per cubic meters, and porosities generally exceed 0.8. Because of their open porosity, these materials are permeable to gas glow. There are numerous instances in which internal gas transport in a thermal protection system could be important; examples include the penetration of hot boundary-layer gases into the insulation, the flow of decomposition (pyrolysis) products from the interior, the use of convective flows to mitigate ice formation caused by cryopumping, and the design of refractory vents for pressure equilibration during atmospheric entry. Computational analysis of gas flow through porous media requires values of permeability which have not previously been available for the rigid fibrous insulations used in thermal protection systems. This paper will document measurements of permeability for a variety of insulations from NASA's LI, FRCI, and AETB families of lightweight ceramic ablators. The directional anisotropy of permeability and its dependence on gas pressure and material density will be presented. It will be shown that rarified-flow effects are significant in the flow through such materials. Connections will be drawn between the insulation microstructure and permeability. The paper will also include representative computations of flow through rigid fibrous insulations.

  15. Solitary fibrous tumor of the liver: report of two cases and review of the literature.

    PubMed

    Debs, Tarek; Kassir, Radwan; Amor, Imed Ben; Martini, Francesco; Iannelli, Antonio; Gugenheim, Jean

    2014-12-01

    A solitary fibrous tumor (SFT) of the liver is a rare neoplasm of mesenchymal origin. 59 cases have been reported in the literature. We report 2 patients who presented with a hepatic solitary fibrous tumor. The first case is a 65-year-old man who presented with an accidental finding of a large mass in the left liver. Biopsy revealed an SFT and left hepatectomy was performed. The diagnosis was confirmed by histopathology. The second case is an 87-year-old woman who presented with disturbances in her liver function tests. A Computed Tomography (CT) scan showed a large mass in the right liver. Surgery was contraindicated because of the patient's poor general condition. A biopsy was done and SFT was diagnosed histopathologically. SFT are usually benign but the risk of malignant transformation always exists, which mandates surgical resection as the optimal management of these tumors. However, because of the small sample size and the rarity of the entity, it is difficult to define the evolution, the risk factors and the malignant potential of these tumors.

  16. [Large solitary fibrous tumors of the pleura as incidental finding. 2 case reports of a rare tumor entity].

    PubMed

    Schwab, R; Schneider, C; Junge, K; Stumpf, M; Becker, H P; Schumpelick, V

    2004-02-01

    The solitary fibrous tumor (SFT) is a very rare and usually benign neoplasm. This tumor is mostly located in the thoracic cavity, but it has also been reported in numerous sites including liver, skin, and meninges. Due to its rarity diagnosis is often difficult. Accordingly, histopathological differentiation is of major importance. Solitary fibrous tumors consistently express CD 34 and react negatively to epithelial markers. This absence of expression of epithelial markers, especially cytokeratin, is also useful to distinguish these rare entities from sarcomatous mesotheliomas. The treatment of choice for solitary fibrous tumors is extensive surgical resection. Up to now there is no evidence that radiation and chemotherapy are effective. The local recurrence or onset of metastases depends on histologic parameters. In cases of benign classification (60-80%), the recurrence rate was only 2% after surgical excision. One-half of the patients with malignant tumors can be cured; the rest develop recurrences and metastases more often. Especially in those cases, emphasis should be placed on continual follow-up examination. We report two cases of a 65- and a 75-year-old male patient with huge intrathoracic solitary fibrous subpleural tumors, discovered incidentally on routine chest X-ray.

  17. Extracranial metastasizing solitary fibrous tumors (SFT) of meninges: histopathological features of a case with long-term follow-up.

    PubMed

    Gessi, Marco; Gielen, Gerrit H; Roeder-Geyer, Eva-Dorette; Sommer, Clemens; Vieth, Michael; Braun, Veit; Kuchelmeister, Klaus; Pietsch, Torsten

    2013-02-01

    Extrapleural solitary fibrous tumors are uncommon mesenchymal neoplasms frequently observed in middle-aged adults and are classified, according to the WHO classification of soft tissue tumors, as part of the hemangiopericytoma tumor group. However, these two entities remain separated in the WHO classification of tumors of the central nervous system. In fact, meningeal solitary fibrous tumors are believed to be benign lesion and only in a minority of cases local relapses have been described, although detailed survival clinical studies on solitary fibrous tumors of meninges are rare. In contrast to hemangiopericytoma, which frequently shows distant extracranial metastases, such an event is exceptional in patients with meningeal solitary fibrous tumors and has been clinically reported in a handful of cases only and their histopathological features have not been investigated in detail. In this report, we describe the detailed clinico-pathological features of a meningeal solitary fibrous tumor presenting during a 17-year follow-up period, multiple intra-, extracranial relapses and lung metastases.

  18. Conjunctival fibrous histiocytoma in an 8-year-old boy.

    PubMed

    Knapp, Austen N; Samara, Wasim A; Shields, Carol L; Shields, Jerry A; Eagle, Ralph C

    2016-08-01

    An 8-year-old healthy boy underwent surgery for excision of a painless, enlarging vascularized conjunctival tumor. Histopathology disclosed a mass comprised of interweaving spindle cells and scattered histiocytes in a fibrous matrix, consistent with benign fibrous histiocytoma. This rare tumor can resemble several conditions, including scleritis/episcleritis, inflamed pterygium, juvenile xanthogranuloma, foreign body granuloma, solitary fibrous tumor, amelanotic melanoma, and squamous cell carcinoma.

  19. Application of Reverse Engineering Template for the Correction of Asymmetric Deformity of Maxillofacial Fibrous Dysplasia.

    PubMed

    Pan, Jianwei; Ye, Bin; Hu, Jing; Li, Xiang; Zhang, Yiqun; Li, Jihua

    2016-03-01

    Facial asymmetry deformity is the most common symptom of patients with fibrous dysplasia. This study provides a novel method based on computed tomography scan data, mirror-imaged reverse engineering and rapid prototyping for design and manufacture of an individual template guiding accurately the extent and quantity of partial resection of hyperplastic tissues to reshape the affected bones during operation. Ten adult patients with unilateral facial fibrous dysplasia accepted these treatments, the postoperative appearances showed that the protrusions were effectively reduced; bilateral faces were basically symmetric with no serious complications. This method shorts operation time, decreases surgical risk, and guarantees the aesthetic symmetry. Apparent recurrence was not observed during the follow-up period, and the final outcomes were satisfactory for both surgeons and patients.

  20. Consolidation and densification methods for fibrous monolith processing

    DOEpatents

    Sutaria, Manish P.; Rigali, Mark J.; Cipriani, Ronald A.; Artz, Gregory J.; Mulligan, Anthony C.

    2004-05-25

    Methods for consolidation and densification of fibrous monolith composite structures are provided. Consolidation and densification of two- and three-dimensional fibrous monolith components having complex geometries can be achieved by pressureless sintering. The fibrous monolith composites are formed from filaments having at least a first material composition generally surrounded by a second material composition. The composites are sintered in an inert gas or nitrogen gas at a pressure of no more than about 30 psi to provide consolidated and densified fibrous monolith composites.

  1. Solitary Fibrous Tumor of the Uterus Presenting With Lung Metastases: A Case Report.

    PubMed

    Strickland, Kyle C; Nucci, Marisa R; Esselen, Katharine M; Muto, Michael G; Chopra, Sameer; George, Suzanne; Howitt, Brooke E

    2016-01-01

    We describe the case of an 81-yr-old woman who presented with bilateral pulmonary nodules in the setting of a large uterine mass, concerning for a gynecologic malignancy such as leiomyosarcoma. However, fine-needle aspiration of a lung nodule revealed a spindle cell neoplasm consistent with solitary fibrous tumor (SFT), a rare mesenchymal neoplasm characterized by a patternless architecture of spindle cells and branching ectatic vessels. Total abdominal hysterectomy demonstrated a primary SFT of the uterus. Both the lung lesion and uterine mass were positive for STAT6, a sensitive and specific biomarker for SFT. SFT infrequently metastasizes and only rarely occurs in the uterus. These tumors are considered to have uncertain malignant potential, and the diagnosis of "malignant" SFT requires the presence of >4 mitoses per 10 high-power fields. The uterine SFT we report did not meet this criterion for malignancy, emphasizing that this entity can behave aggressively even without increased mitoses or atypical histology. To our knowledge, this is the first reported case of a uterine SFT with metastasis to the lung. We discuss the differential diagnosis for the finding of multiple pulmonary spindle cell lesions in the setting of a uterine mass. PMID:26107564

  2. Solitary Fibrous Tumor of the Uterus Presenting With Lung Metastases: A Case Report.

    PubMed

    Strickland, Kyle C; Nucci, Marisa R; Esselen, Katharine M; Muto, Michael G; Chopra, Sameer; George, Suzanne; Howitt, Brooke E

    2016-01-01

    We describe the case of an 81-yr-old woman who presented with bilateral pulmonary nodules in the setting of a large uterine mass, concerning for a gynecologic malignancy such as leiomyosarcoma. However, fine-needle aspiration of a lung nodule revealed a spindle cell neoplasm consistent with solitary fibrous tumor (SFT), a rare mesenchymal neoplasm characterized by a patternless architecture of spindle cells and branching ectatic vessels. Total abdominal hysterectomy demonstrated a primary SFT of the uterus. Both the lung lesion and uterine mass were positive for STAT6, a sensitive and specific biomarker for SFT. SFT infrequently metastasizes and only rarely occurs in the uterus. These tumors are considered to have uncertain malignant potential, and the diagnosis of "malignant" SFT requires the presence of >4 mitoses per 10 high-power fields. The uterine SFT we report did not meet this criterion for malignancy, emphasizing that this entity can behave aggressively even without increased mitoses or atypical histology. To our knowledge, this is the first reported case of a uterine SFT with metastasis to the lung. We discuss the differential diagnosis for the finding of multiple pulmonary spindle cell lesions in the setting of a uterine mass.

  3. Treatment of malignant gliomas and brain metastases in adults with a combination of adriamycin, VM 26, and CCNU. Results of a phase II trail.

    PubMed

    Pouillart, P; Mathe, G; Thy, T H; Lheritier, J; Poisson, M; Huguenin, P; Gauthier, H; Morin, P; Parrot, R

    1976-11-01

    Forty-three patients with inoperable or recurring malignant gliomas, and 30 patients with multiple recurring brain metastases were treated with a combination of Adriamycin (45 mg/m2) and 4-dimethyl-epipodophyllotoxin D-thenylidene (VM 26) (60 mg/m2 for 2 days) with 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU) (60 mg/m2 for 2 days). These cycles of treatment were repeated as soon as the hematologic restoration was complete. The treatment was well tolerated and the clinical condition of 31 of 43 glioblastoma patients improved during the 2 months after the beginning of the treatment. Six of eight patients with breast cancer metastases, one of 13 with bronchial cancer matastases, and three of nine with other types of cancer metastases also benefitted from the treatment. Examination of the results obtained revealed the following characteristics: 1) This combination had a low degree of efficiency in the treatment of metastases to brain, except for breast cancer metastases; 2) there was no complete correlation between the clinical results observed and the cinegammagraphic developments; 3) the results obtained were similar, independent of the initial localization; and a 6-month median survival period was established, with 10 patients now in a state of apparently complete remission, 180 to 506 days after beginning of the treatment. PMID:1033028

  4. Adult intussusception.

    PubMed Central

    Azar, T; Berger, D L

    1997-01-01

    OBJECTIVE: The objectives were to review adult intussusception, its diagnosis, and its treatment. SUMMARY BACKGROUND DATA: Adult intussusception represents 1% of all bowel obstructions, 5% of all intussusceptions, and 0.003%-0.02% of all hospital admissions. Intussusception is a different entity in adults than it is in children. METHODS: The records of all patients 18 years and older with the postoperative diagnosis of intussusception at the Massachusetts General Hospital during the years 1964 through 1993 were reviewed retrospectively. The 58 patients were divided into those with benign enteric, malignant enteric, benign colonic, and malignant colonic lesions associated with their intussusception. The diagnosis and treatment of each were reviewed. RESULTS: In 30 years at the Massachusetts General Hospital, there are 58 cases of surgically proven adult intussusception. The patients' mean age was 54.4 years. Most patients presented with symptoms consistent with bowel obstruction. There were 44 enteric and 14 colonic intussusceptions. Ninety-three percent of the intussusceptions were associated with a pathologic lesion. Forty-eight percent of the enteric lesions were malignant and 52% were benign. Forty-three percent of the colonic lesions were malignant and 57% were benign. CONCLUSIONS: Intussusception occurs rarely in adults. It presents with a variety of acute, intermittent, and chronic symptoms, thus making its preoperative diagnosis difficult. Computed tomography scanning proved to be the most useful diagnostic radiologic method. The diagnosis and treatment of adult intussusception are surgical. Surgical resection of the intussusception without reduction is the preferred treatment in adults, as almost half of both colonic and enteric intussusceptions are associated with malignancy. PMID:9296505

  5. Elasto-capillarity in fibrous materials

    NASA Astrophysics Data System (ADS)

    Monaenkova, Daria

    Current advances in the manufacture of nanoporous and nanofibrous materials with high absorption capacity open up new opportunities for the development of fiber-based probes and sensors. Pore structures of these materials can be designed to provide high suction pressure and fast wicking. During wicking, due to the strong capillary action, the liquids exert stresses on the fiber network, thus the stressed state of dry and wet parts of the material differs. In this work the effect of stress reduction in fibrous materials due to the presence of wetting liquid in the pore structure is studied in details for both static and dynamic cases. It is suggested that this effect can be used for liquid monitoring and the examples of one and two dimensional probes are provided. To open a discussion an illustrative example of a single capillary is considered and the effect of a moving meniscus on the stress distribution along capillary walls is demonstrated. Then the similar effects are analyzed in yarns and fabrics. A yarn that can capture an aerosol droplet is considered as a promising sensing element that could monitor the stresses caused by wetting fronts. It is shown that the stress transfer between dry and wet parts of the yarn upon liquid wicking significantly depends on the boundary conditions. The stress distribution in the yarn with clamped ends is discussed. The elasto-capillary problem is resolved for 2-D case of a freely suspended self-reconfigurable material. It is shown that the classical Bernoulli problem of a freely suspended fabric can be used for the analysis of stresses in the fibrous matrix. The theoretical conclusions on elasto-capillarity are supported by experimental results on tensile testing of fibrous materials. The results show that the elasto-capillary effect is pronounced in the porous samples with the pore sizes smaller than 10 microm.

  6. Irradiation of the potential cancer stem cell niches in the adult brain improves progression-free survival of patients with malignant glioma

    PubMed Central

    2010-01-01

    Background Glioblastoma is the most common brain tumor in adults. The mechanisms leading to glioblastoma are not well understood but animal studies support that inactivation of tumor suppressor genes in neural stem cells (NSC) is required and sufficient to induce glial cancers. This suggests that the NSC niches in the brain may harbor cancer stem cells (CSCs), Thus providing novel therapy targets. We hypothesize that higher radiation doses to these NSC niches improve patient survival by eradicating CSCs. Methods 55 adult patients with Grade 3 or Grade 4 glial cancer treated with radiotherapy at UCLA between February of 2003 and May of 2009 were included in this retrospective study. Using radiation planning software and patient radiological records, the SVZ and SGL were reconstructed for each of these patients and dosimetry data for these structures was calculated. Results Using Kaplan-Meier analysis we show that patients whose bilateral subventricular zone (SVZ) received greater than the median SVZ dose (= 43 Gy) had a significant improvement in progression-free survival if compared to patients who received less than the median dose (15.0 vs 7.2 months PFS; P = 0.028). Furthermore, a mean dose >43 Gy to the bilateral SVZ yielded a hazard ratio of 0.73 (P = 0.019). Importantly, similarly analyzing total prescription dose failed to illustrate a statistically significant impact. Conclusions Our study leads us to hypothesize that in glioma targeted radiotherapy of the stem cell niches in the adult brain could yield significant benefits over radiotherapy of the primary tumor mass alone and that damage caused by smaller fractions of radiation maybe less efficiently detected by the DNA repair mechanisms in CSCs. PMID:20663133

  7. Solitary fibrous tumor of the thyroid.

    PubMed

    Cameselle-Teijeiro, J; Varela-Duran, J; Fonseca, E; Villanueva, J P; Sobrinho-Simoes, M

    1994-04-01

    A case of solitary fibrous tumor (SFT) of the thyroid in a 43-year-old woman with a multinodular goiter is reported. This is the first case of SFT described in the thyroid. On histologic, immunohistochemical, and ultrastructural examination, the tumor was identical to SFT of the pleura and other organs. Despite its rarity, SFT should be included in the differential diagnosis of spindle-cell tumors of the thyroid, along with anaplastic carcinoma, spindle-cell medullary carcinoma, and several types of mesenchymal tumors.

  8. Solitary fibrous tumor of the auditory canal.

    PubMed

    Rezk, Sherif; Yousef, Mohammad; Zamansky, Marshall; Khan, Ashraf

    2004-12-01

    Solitary fibrous tumor (SFT) is an uncommon spindle cell neoplasm of increasing incidence that was originally described to be of pleural origin; however, more recently, SFT has been reported in extrapleural sites, including the orbit, liver, salivary glands, tongue, nose, paranasal sinuses, larynx, retroperitoneum, meninges, and thyroid. The increase in the number of SFTs does not necessarily mean increased incidence of this tumor but rather an increased understanding of this tumor, especially recognition of this tumor in extrapleural locations, which has been aided by immunohistochemical analysis. We report a case of SFT in the auditory canal, which to our knowledge has not been previously reported, as evident by morphologic findings and immunophenotype.

  9. Extrapericardial solitary fibrous tumour of the pericardium.

    PubMed

    Andreani, S M; Tavecchio, L; Giardini, R; Bedini, A V

    1998-07-01

    Solitary fibrous tumour (SFT) occurs most commonly in the pleura and is extremely rare in the pericardium. The authors report a case of a 60-year-old man in whom a large mediastinal mass was accidentally discovered. Computed tomography showed involvement of the left anterosuperior mediastinum with displacement of the trachea, large vessels and oesophagus; histopathological findings after complete resection of the neoplasia demonstrated an SFT of the pericardium, the first reported case with extrapericardial pattern of growth. A review of the literature on SFTs of the pericardium is provided.

  10. Atypical solitary fibrous tumor of the vulva.

    PubMed

    Fukunaga, M

    2000-04-01

    An atypical solitary fibrous tumor (SFT) was encountered as a slow-growing, 15-cm, well-demarcated, vulvar tumor in a 70-year-old woman. The tumor was highly cellular and composed predominantly of hemangiopericytomatous and capillary hemangioma-like proliferations and short fascicular arrangements of spindled cells. Multinucleated giant cells and tumor necrosis also were present. The tumor cells were positive for vimentin, CD34, progesterone receptors, and bcl-2 and were diploid by flow cytometry. The patient was well without disease 9 months after surgery. Awareness of the occurrence of atypical SFT in the vulva is important so that confusion with other neoplasms can be avoided.

  11. Solitary fibrous tumor of the periosteum.

    PubMed

    O'Connell, J X; Logan, P M; Beauchamp, C P

    1995-04-01

    We report a solitary fibrous tumor (SFT) of the leg that presented as a pedunculated encapsulated soft tissue mass attached to the periosteum of the posterior tibia. To our knowledge this is the first reported case of an SFT in an extremity. In addition to having the typical gross and microscopic appearance of a usual SFT, this SFT also contained abundant elastic tissue, suggesting an origin from periosteal fibroblasts. This report further extends the anatomic range of the SFT, a neoplasm once thought to be of mesothelial origin but now recognized as a mesenchymal tumor of probable fibroblastic lineage. To date the behavior of the this periosteal SFT has been benign.

  12. [Single fibrous tumor of the parapharyngeal space].

    PubMed

    Galera-Ruiz, H; Martínez-Pozo, A; Alos, L L; Cardesa, A; Traserra, J

    2000-01-01

    A solitary fibrous tumor (SFT) of the parapharyngeal space presented with local symptoms (hearing loss, nasal obstruction, and paralysis of the soft palate and tongue). SFT, originally described as a mesothelial tumor of the pleura, now is recognized as a mesenchymal tumor that occurs in different locations. In the head and neck region, about 50 cases have been reported. This is the fifth published report of an SFT of the parapharyngeal space. The diagnosis was confirmed by immunohistochemical (positivity for vimentin, CD34, and CD99) and ultrastructural markers (fibroblastic characteristics).

  13. Solitary fibrous tumour of the pleura.

    PubMed

    Sikri, V; Chawla, R

    2013-01-01

    Solitary fibrous tumour (SFT) of the pleura is a rare, usually benign primary tumour of the pleura. Spectrum of presentation can vary from an incidental finding on chest radiograph done for some other purpose, features of compression of surrounding structures to symptoms resulting from the tumour per se. We report a case of a female who presented with complaints of cough and chest pain in whom a diagnosis of SFT was confirmed on tru-cut biopsy and immunohistochemistry studies. The patient underwent thoracotomy and successful removal of the tumour.

  14. Fibrous composites comprising carbon nanotubes and silica

    DOEpatents

    Peng, Huisheng; Zhu, Yuntian Theodore; Peterson, Dean E.; Jia, Quanxi

    2011-10-11

    Fibrous composite comprising a plurality of carbon nanotubes; and a silica-containing moiety having one of the structures: (SiO).sub.3Si--(CH.sub.2).sub.n--NR.sub.1R.sub.2) or (SiO).sub.3Si--(CH.sub.2).sub.n--NCO; where n is from 1 to 6, and R.sub.1 and R.sub.2 are each independently H, CH.sub.3, or C.sub.2H.sub.5.

  15. Solitary fibrous tumor surrounding the carotid sheath.

    PubMed

    Gómez-Oliveira, Guillermo; Alvarez-Flores, Modesto; Arribas-García, Ignacio; Martínez-Gimeno, Carlos

    2010-03-01

    Solitary fibrous tumors (SFTs) are rare spindle cell neoplasms that are mostly found arising from the pleura. Although SFTs recently have been reported in other regions, they are rare in the head and neck and have often been misdiagnosed due to their rarity. SFTs are benign in most cases. Clinically, SFTs usually manifest as well-circumscribed, slow-growing, smooth and painless masses. Symptoms are often minimal, although they may include sore throat, difficulty in swallowing, change of voice or trismus. CT-Scan and MRI are the most sensitive imaging procedures used. The treatment of choice is complete surgical excision of the lesion. Because recurrences have been noted up to 30 years after surgery, long-term follow up is mandatory. In this article, we present a case of a Solitary Fibrous Tumor arising in the parapharyngeal space in a 20-year-old man, involving the carotid sheath, treated by surgical excision with no recurrence after 1 year. The clinical presentation, surgical management and pathological findings are described.

  16. [Polyostotic fibrous dysplasia. A clinical case report].

    PubMed

    Gallesio, C; Tagliabue, M; Mazzeo, R; De Gioanni, P P

    1996-11-01

    The authors present a severe case of polyostotic fibrous dysplasia in which there was considerable involvement of cranial bone and facial skeleton. Numerous lesions were present at the level of the long bones of limbs. Endocrine dysfunction was also present in the form of a hypophyseal adenoma secreting prolactin and ACTH. The concomitance of acromegaly or gigantism and/or hyperprolactinemia and polyostotic fibrous dysplasia has only been reported to date in a few cases in literature. The authors describe the appearance of the subject, correlating clinical photographs with X-rays. They report the clinical excursus of the patient characterised by the gradual increase in deformities which seriously jeopardized the patient's relational life, in particular the appearance of a bulk on the forehead and checks and the deformation of the symphyseal portion of the mandible with presence of interdental diastemata. The patient also complained diplopia, difficulty in chewing owing to the mobilisation of teeth, and increasing bone pain probably due to nerve compression by exuberant bone. It was not possible to perform corrective surgery owing to the patient's overall poor health conditions. In fact, dilatative cardiomyopathy which continued to worsen in spite of numerous forms of medical treatment resulted in the patient's death owing to cardiac decompensation. Even the attempt to treat the patient's primary endocrine dysfunction using bromocryptine and subsequently octreotide failed to produce positive results owing to the onset of collateral effects which led to the early suspension of treatment.

  17. Self-Organization of Bioinspired Fibrous Surfaces

    NASA Astrophysics Data System (ADS)

    Kang, Sung Hoon

    Nature uses fibrous surfaces for a wide range of functions such as sensing, adhesion, structural color, and self-cleaning. However, little is known about how fiber properties enable them to self-organize into diverse and complex functional forms. Using polymeric micro/nanofiber arrays with tunable properties as model systems, we demonstrate how the combination of mechanical and surface properties can be harnessed to transform an array of anchored nanofibers into a variety of complex, hierarchically organized dynamic functional surfaces. We show that the delicate balance between fiber elasticity and surface adhesion plays a critical role in determining the shape, chirality, and hierarchy of the assembled structures. We further report a strategy for controlling the long-range order of fiber assemblies by manipulating the shape and movement of the liquid-vapor interface. Our study provides fundamental understanding of the pattern formation by self-organization of bioinspired fibrous surfaces. Moreover, our new strategies offer a foundation for designing a vast assortment of functional surfaces with adhesive, optical, water-repellent, capture and release, and many more capabilities with the structural and dynamic sophistication of their biological counterparts.

  18. Craniofacial fibrous dysplasia: Surgery and literature review

    PubMed Central

    Menon, Suresh; Venkatswamy, Srihari; Ramu, Veena; Banu, Khurshida; Ehtaih, Sham; Kashyap, Vinay M.

    2013-01-01

    Objective: To highlight the clinical and radiologic features and management of craniofacial fibrous dysplasia with review of literature. Materials and Methods: A retrospective review of 6 patients who underwent surgical treatment in a tertiary healthcare centre was done using the parameters of patients' details, clinical features, radiological findings, management and postoperative review. Results: Of the six patients, 3 females and 2 males were in the 2nd decade of life and 1 male in the 1st decade of life. The disease was restricted to maxilla in 3 patients, involved the temporal and frontal bones in addition to maxilla in one, involved the frontal bone in one patient and involved frontal and parietal bones in one patient. The primary reason for seeking treatment in all the 6 cases was facial deformity. There was absence of pain in all 6 cases. For surgical treatment in all three cases involving the maxilla, the approach was intraoral while bicoronal approach was used for the other three cases. Treatment consisted of surgical contouring and reshaping the area. All cases were followed up over a period of 2 years with no signs of recurrence. Conclusion: Treatment of craniofacial fibro-osseous lesions is highly individualized. Most cases of craniofacial fibrous dysplasia manifest as swellings that cause facial deformity and surgical recontouring after cessation of growth seems to provide the best results. PMID:23662263

  19. [Polyostotic fibrous dysplasia. A clinical case report].

    PubMed

    Gallesio, C; Tagliabue, M; Mazzeo, R; De Gioanni, P P

    1996-11-01

    The authors present a severe case of polyostotic fibrous dysplasia in which there was considerable involvement of cranial bone and facial skeleton. Numerous lesions were present at the level of the long bones of limbs. Endocrine dysfunction was also present in the form of a hypophyseal adenoma secreting prolactin and ACTH. The concomitance of acromegaly or gigantism and/or hyperprolactinemia and polyostotic fibrous dysplasia has only been reported to date in a few cases in literature. The authors describe the appearance of the subject, correlating clinical photographs with X-rays. They report the clinical excursus of the patient characterised by the gradual increase in deformities which seriously jeopardized the patient's relational life, in particular the appearance of a bulk on the forehead and checks and the deformation of the symphyseal portion of the mandible with presence of interdental diastemata. The patient also complained diplopia, difficulty in chewing owing to the mobilisation of teeth, and increasing bone pain probably due to nerve compression by exuberant bone. It was not possible to perform corrective surgery owing to the patient's overall poor health conditions. In fact, dilatative cardiomyopathy which continued to worsen in spite of numerous forms of medical treatment resulted in the patient's death owing to cardiac decompensation. Even the attempt to treat the patient's primary endocrine dysfunction using bromocryptine and subsequently octreotide failed to produce positive results owing to the onset of collateral effects which led to the early suspension of treatment. PMID:9026699

  20. Elasticity of fibrous networks under uniaxial prestress.

    PubMed

    Vahabi, Mahsa; Sharma, Abhinav; Licup, Albert James; van Oosten, Anne S G; Galie, Peter A; Janmey, Paul A; MacKintosh, Fred C

    2016-06-14

    We present theoretical and experimental studies of the elastic response of fibrous networks subjected to uniaxial strain. Uniaxial compression or extension is applied to extracellular networks of fibrin and collagen using a shear rheometer with free water in/outflow. Both uniaxial stress and the network shear modulus are measured. Prior work [van Oosten, et al., Sci. Rep., 2015, 6, 19270] has shown softening/stiffening of these networks under compression/extension, together with a nonlinear response to shear, but the origin of such behaviour remains poorly understood. Here, we study how uniaxial strain influences the nonlinear mechanics of fibrous networks. Using a computational network model with bendable and stretchable fibres, we show that the softening/stiffening behaviour can be understood for fixed lateral boundaries in 2D and 3D networks with comparable average connectivities to the experimental extracellular networks. Moreover, we show that the onset of stiffening depends strongly on the imposed uniaxial strain. Our study highlights the importance of both uniaxial strain and boundary conditions in determining the mechanical response of hydrogels. PMID:27174568

  1. STAT6 rabbit monoclonal antibody is a robust diagnostic tool for the distinction of solitary fibrous tumour from its mimics.

    PubMed

    Cheah, Alison L; Billings, Steven D; Goldblum, John R; Carver, Paula; Tanas, Munir Z; Rubin, Brian P

    2014-08-01

    Recurrent NAB2-STAT6 gene fusions have recently been identified in solitary fibrous tumour by next generation sequencing. Our aim was to examine the sensitivity and specificity of STAT6 immunohistochemistry for solitary fibrous tumour versus other morphologically similar soft tissue tumours. STAT6 expression was evaluated in 54 solitary fibrous tumours of various sites and 99 soft tissue tumours in the histological differential diagnosis. We used a rabbit monoclonal STAT6 antibody (1:100), which has not been reported by others, on formalin fixed, paraffin embedded whole sections and tissue microarray slides. Only nuclear staining of STAT6 was considered positive. Distribution of staining was scored as: 0 (no staining), 1+ (1-25%), 2+ (26-50%), 3+ (>50%). Intensity was scored as weak, moderate or strong. Nuclear STAT6 staining was present in all SFT cases tested (54/54, sensitivity 100%), regardless of histology, anatomical site or CD34 status. The majority of cases showed 3+ and strong staining. All tested cases of cellular angiofibroma (0/9), myofibroblastoma (0/10), spindle cell lipoma (0/10), benign fibrous histiocytoma (0/13), dermatofibrosarcoma protruberans (0/9), low-grade fibromyxoid sarcoma (0/7), schwannoma (0/8), desmoid-type fibromatosis (0/8), monophasic synovial sarcoma (0/11), malignant peripheral nerve sheath tumour (0/7), and mesenchymal chondrosarcoma (0/7) were negative for STAT6 (specificity 100%). Our study further supports the utility of STAT6 immunohistochemistry as an adjunct in the diagnosis of solitary fibrous tumour.

  2. Fibrous microcapsules and methods of assembly and use thereof

    DOEpatents

    Stupp, Samuel; Rozkiewicz, Dorota

    2015-01-27

    The present invention relates to assembly of peptide amphiphiles and biopolymers into fibrous microcapsules, and uses thereof. In particular, the present invention provides devices, compositions, and methods for interfacial self-assembly of peptide amphiphiles and biopolyments into fibrous microcapsules, and uses thereof.

  3. Fibrous monoliths: Economic ceramic matrix composites from powders [Final report

    SciTech Connect

    Rigali, Mark; Sutaria, Manish; Mulligan, Anthony; Creegan, Peter; Cipriani, Ron

    1999-05-26

    The project was to develop and perform pilot-scale production of fibrous monolith composites. The principal focus of the program was to develop damage-tolerant, wear-resistant tooling for petroleum drilling applications and generate a basic mechanical properties database on fibrous monolith composites.

  4. Ceramic-Fibrous-Insulation Thermal-Protection System

    NASA Technical Reports Server (NTRS)

    Leiser, Daniel; Churchward, Rex; Katvala, Victor; Stewart, David; Balter, Aliza

    1992-01-01

    New composite thermal-protection system developed in which glass-ceramic impregnated into surface of fibrous insulation. Called TUFI for toughened unipiece fibrous insulation developed as replacement for tiles with reaction-cured-glass (RCG) coating. Impregnation of glass-ceramic results in thermal protection system with insulating properties comparable to existing system but with 20 to 100 times more resistance to impact.

  5. Regulating inflammation using acid-responsive electrospun fibrous scaffolds for skin scarless healing.

    PubMed

    Yuan, Ziming; Zhao, Jingwen; Chen, Yigang; Yang, Zhili; Cui, Wenguo; Zheng, Qi

    2014-01-01

    Skin injury in adult mammals brings about a series of events and inflammation in the wounded area is initiated first and provides lots of inflammatory factors, which is critical for the final scar formation. While the postinjured skin of fetus and nude mice heals scarlessly owing to the absence of inflammation or immunodeficient, we designed a feasible acid-responsive ibuprofen-loaded poly(L-lactide) (PLLA) fibrous scaffolds via doping sodium bicarbonate to prevent excessive inflammation and achieve scarless healing finally. The morphological results of in vivo experiments revealed that animals treated with acid-responsive ibuprofen-loaded PLLA fibrous scaffolds exhibited alleviative inflammation, accelerated healing process, and regulated collagen deposition via interference in the collagen distribution, the α-smooth muscle actin (α-SMA), and the basic fibroblast growth factor (bFGF) expression. The lower ratios of collagen I/collagen III and TGF-β1/TGF-β3 and higher ratio of matrix metalloproteinase-1 (MMP-1)/tissue inhibitor of metalloproteinase-1 (TIMP-1) in acid-responsive ibuprofen-loaded PLLA fibrous scaffolds group were confirmed by real-time qPCR as well. These results suggest that inhibiting the excessive inflammation will result in regular collagen distribution and appropriate ratio between the factors, which promote or suppress the scar formation, then decrease the scar area, and finally achieve the scarless healing. PMID:24795507

  6. Regulating inflammation using acid-responsive electrospun fibrous scaffolds for skin scarless healing.

    PubMed

    Yuan, Ziming; Zhao, Jingwen; Chen, Yigang; Yang, Zhili; Cui, Wenguo; Zheng, Qi

    2014-01-01

    Skin injury in adult mammals brings about a series of events and inflammation in the wounded area is initiated first and provides lots of inflammatory factors, which is critical for the final scar formation. While the postinjured skin of fetus and nude mice heals scarlessly owing to the absence of inflammation or immunodeficient, we designed a feasible acid-responsive ibuprofen-loaded poly(L-lactide) (PLLA) fibrous scaffolds via doping sodium bicarbonate to prevent excessive inflammation and achieve scarless healing finally. The morphological results of in vivo experiments revealed that animals treated with acid-responsive ibuprofen-loaded PLLA fibrous scaffolds exhibited alleviative inflammation, accelerated healing process, and regulated collagen deposition via interference in the collagen distribution, the α-smooth muscle actin (α-SMA), and the basic fibroblast growth factor (bFGF) expression. The lower ratios of collagen I/collagen III and TGF-β1/TGF-β3 and higher ratio of matrix metalloproteinase-1 (MMP-1)/tissue inhibitor of metalloproteinase-1 (TIMP-1) in acid-responsive ibuprofen-loaded PLLA fibrous scaffolds group were confirmed by real-time qPCR as well. These results suggest that inhibiting the excessive inflammation will result in regular collagen distribution and appropriate ratio between the factors, which promote or suppress the scar formation, then decrease the scar area, and finally achieve the scarless healing.

  7. Regulating Inflammation Using Acid-Responsive Electrospun Fibrous Scaffolds for Skin Scarless Healing

    PubMed Central

    Yuan, Ziming; Zhao, Jingwen; Chen, Yigang; Yang, Zhili; Zheng, Qi

    2014-01-01

    Skin injury in adult mammals brings about a series of events and inflammation in the wounded area is initiated first and provides lots of inflammatory factors, which is critical for the final scar formation. While the postinjured skin of fetus and nude mice heals scarlessly owing to the absence of inflammation or immunodeficient, we designed a feasible acid-responsive ibuprofen-loaded poly(L-lactide) (PLLA) fibrous scaffolds via doping sodium bicarbonate to prevent excessive inflammation and achieve scarless healing finally. The morphological results of in vivo experiments revealed that animals treated with acid-responsive ibuprofen-loaded PLLA fibrous scaffolds exhibited alleviative inflammation, accelerated healing process, and regulated collagen deposition via interference in the collagen distribution, the α-smooth muscle actin (α-SMA), and the basic fibroblast growth factor (bFGF) expression. The lower ratios of collagen I/collagen III and TGF-β1/TGF-β3 and higher ratio of matrix metalloproteinase-1 (MMP-1)/tissue inhibitor of metalloproteinase-1 (TIMP-1) in acid-responsive ibuprofen-loaded PLLA fibrous scaffolds group were confirmed by real-time qPCR as well. These results suggest that inhibiting the excessive inflammation will result in regular collagen distribution and appropriate ratio between the factors, which promote or suppress the scar formation, then decrease the scar area, and finally achieve the scarless healing. PMID:24795507

  8. Fibrous calcite from the Middle Ordovician Holston Formation (east Tennessee)

    SciTech Connect

    Tobin, K.J.; Walker, K.R. . Dept. of Geological Sciences)

    1993-03-01

    Fibrous calcite from buildups, which occur near the top of the Middle Ordovician Holston Formation, were examined from two localities near Knoxville, TN (Alcoa Highway and Deanne Quarry). Buildups at these localities were deposited under open-marine conditions, slightly down-slope from the platform edge. Fibrous calcite (mainly radiaxial fibrous) occur most commonly as cements in mainly stromatactis structures present in bioherms and intergranular porosity in beds that flank bioherms. Fibrous calcite is interpreted to have been precipitated in a marine setting. Fibrous calcite is uniformly turbid or banded with interlayered turbid and clearer cement. Fibrous calcite most commonly shows patchy or blotchy dull-non-luminescence under cathodoluminescence. Bands of uniformly non-luminescent and relatively bright luminescent calcite are present. [delta][sup 13]C compositions of fibrous calcite vary little (0.6 to 1.0%) but [delta][sup 18]O values are highly variable ([minus]4.8 to [minus]7.1%). Post-marine cement consists of ferroan and non-ferroan, dull luminescent equant calcite ([delta][sup 13]C = 0.3 to 0.8; [delta][sup 18]O = [minus]8.6 to [minus]11.5) and is interpreted as precipitated in a deep meteoric or burial setting. Depleted [delta][sup 18]O compositions of fibrous calcite reflect addition of post-depositional calcite during stabilization. Most enriched [delta][sup 13]C and [delta][sup 18]O fibrous calcite composition are similar to enriched values from other Middle Ordovician southern Appalachian buildups (other localities of Holston (TN) and Effna (VA) formations) ([delta][sup 13]C = 0.3 to 0.8; [delta][sup 18]O = [minus]3.9 to [minus]4.8) and may reflect fibrous calcite precipitated in isotopic equilibrium with Middle Ordovician sea water.

  9. Incidence of antibody to adult T-cell leukemia-virus-associated antigen among T-cell malignancies in the Kyoto District, with a report of two unusual cases.

    PubMed

    Kita, K; Nasu, K; Kamesaki, H; Fukuhara, S; Nishikori, M; Uchino, H; Hanaoka, M

    1983-01-01

    Titration of antibody to adult T-cell leukemia (ATL)-virus-associated antigen (ATLA) is of much help for diagnosing ATL, because almost all patients with ATL are seropositive even in an ATL-nonendemic area such as Kyoto. In T-cell lymphoma, anti-ATLA antibody was thought to be related to the birthplace of the patients and the epidermotropism of their skin lesions, but it was not confirmative because the number of cases was so small. We present here two curious cases of anti-ATLA-negative T-cell leukemia/lymphoma. A 53-yr-old man, born in a nonendemic area, had manifestations similar to those of ATL except for the lack of skin involvement, but the morphology of his leukemic cells was less like that of ATL cells than that of prolymphocytic leukemia cells. Therefore, his leukemia was not diagnosed as ATL. A 52-yr-old woman, whose parents' hometown was in an endemic area, showed typical manifestations of nonleukemic T-cell lymphoma, and her biopsied lymph node was compatible with diffuse, pleomorphic lymphoma histologically. In the latter patient, the negative anti-ATLA finding might be due to titration sensitivity. Therefore, the clinical and hematologic features are still informative for distinguishing ATL from other T-cell malignancies.

  10. Life satisfaction in young adults 10 or more years after hematopoietic stem cell transplantation for childhood malignant and nonmalignant diseases does not show significant impairment compared with healthy controls: a case-matched study.

    PubMed

    Uderzo, Cornelio; Corti, Paola; Pappalettera, Marco; Baldini, Valentina; Lucchini, Giovanna; Meani, Dario; Rovelli, Attilio

    2012-11-01

    Patients undergoing hematopoietic stem cell transplantation (HSCT) may experience physical and psychological deterioration that impairs their life satisfaction (LS). This study focused on LS in long-term survivors at 10 or more years after HSCT. Fifty-five patients (39 males, median age 25 years) undergoing allogeneic HSCT for childhood malignant (n = 52) or nonmalignant diseases (n = 3) were enrolled. A control group of 98 young adults (59 males, median age 24 years) was considered. A questionnaire with a modified Satisfaction Life Domain Scale was administered. We assessed such domains as education, employment, leisure time, social relationships, and perception of physical status with a 30-item questionnaire. To investigate the association between the domains and the probability of diminished LS, we performed a logistical procedure using the maximum likelihood method. Predictive factors of LS were adjusted for sociodemographic variables. In the multivariate analysis, the participant's level of LS was not significantly correlated with sociodemographic factors or with HSCT status. The same analysis showed a slight trend in favor of the control group (P = .06) for body perception. Our data suggest that the patients who undergo HSCT in childhood have no significant difference in long-term LS compared with healthy controls.

  11. [Solitary fibrous tumor of the nasopharynx. Apropos of a case].

    PubMed

    Ferrario, F; Piantanida, R; Spriano, G; Cerati, M; Maffioli, M; Roselli, R

    1997-01-01

    Solitary fibrous tumor (SFT) is a rare neoplasm, arising in the adult and more commonly in the pleura. For many years it has been referred to the serous surfaces of the body, namely pleura, peritoneum and pericardium; recently cases arising in mesenchymal organs such as lung, mediastinum, liver and paranasal sinuses were reported. We present a SFT of the nasopharynx, observed in a 41 year-old patient complaining a 6 months history of aural fullness at the right ear. Clinical examination revealed the presence of secretory otitis media and swelling of the lateral wall of the nasopharynx. CT scan and MR showed the presence of a solid mass, with scarce vascularization, extending from the right side of the nasopharynx to the infratemporal and pterigoid fossae. The surgical approach consisted in a facial translocation by the rotation of a maxillary-check flap through different osteotomies; a firm whitish mass not invading the surrounding tissues was identified and enucleated. A definitive diagnosis cannot be made at frozen sections, requiring more accurate processing and immunohistochemical staining. Literature reports fourteen cases of SFT of the upper aero-digestive tracts (nasopharynx, paranasal sinuses, larynx) and some 8 more cases in the head and neck area (thyroid, salivary glands and parapharyngeal spaces). This case report adds a further contribution to support the mesenchymal origin of the SFT.

  12. A Phase 1 Study of 7-t-butyldimethylsilyl-10-hydroxycamptothecin (AR-67) in Adult Patients with Refractory or Metastatic Solid Malignancies

    PubMed Central

    Arnold, Susanne M.; Rinehart, John J.; Tsakalozou, Eleftheria; Eckardt, John R.; Fields, Scott Z.; Shelton, Brent J.; DeSimone, Philip A.; Kee, Bryan K.; Moscow, Jeffrey A.; Leggas, Markos

    2009-01-01

    Purpose AR-67 is a novel third generation camptothecin selected for development based on the blood stability of its pharmacologically active lactone form and high potency in preclinical models. Here we report the initial phase I experience with intravenous AR-67 in adults with refractory solid tumors. Experimental Design and Methods AR-67 was infused over 1 hour daily × 5, every 21-days, using an accelerated titration trial design. Plasma was collected on the 1st and 4th day of cycle 1 to determine pharmacokinetic parameters. Results Twenty six patients were treated at 9 dosage levels (1.2–12.4mg/m2/day). Dose limiting toxicities (DLTs) were observed in 5 patients and consisted of grade 4 febrile neutropenia, grade 3 fatigue, and grade 4 thrombocytopenia. Common toxicities included: leukopenia (23%), thrombocytopenia (15.4%), fatigue (15.4%), neutropenia (11.5%), and anemia (11.5%). No diarrhea was observed. The maximum tolerated dosage (MTD) was 7.5 mg/m2/day. The lactone form was the predominant species in plasma (>87% of AUC) at all dosages. No drug accumulation was observed on day 4. Clearance was constant with increasing dosage and hematologic toxicities correlated with exposure (p<0.001). A prolonged partial response was observed in one subject with non-small cell lung cancer (NSCLC). Stable disease was noted in patients with small cell lung cancer (SCLC), NSCLC, and duodenal cancer. Conclusions AR-67 is a novel, blood stable camptothecin with a predictable toxicity profile and linear pharmacokinetics. The recommended phase II dosage is 7.5mg/m2/day ×5 q 21 days. PMID:20068096

  13. Histological and immunohistochemical study of estrogen and progesterone receptors in normal human breast tissue in adult age groups vulnerable to malignancy.

    PubMed

    Goyal, R; Gupta, T; Gupta, R; Aggarwal, A; Sahni, D; Singh, G

    2016-09-01

    Analysis of receptor status has become standard procedure for assessing breast cancer patients. Estrogen causes epithelial proliferation in breast tissue via the estrogen receptor (ER). The progesterone receptor (PR) is also regulated by the estrogen gene. Analyzing ER and PR together gives information regarding the likely response of carcinoma patients to hormonal therapy. The aim of the present study was to record the expression patterns of ER and PR in normal mammary tissue in different age groups to provide reference data to facilitate histological diagnosis. Breast tissues from the upper outer quadrant of each side of 27 adult female cadavers were examined after H & E staining. ER and PR were identified and examined by immunohistochemistry. The percentage area occupied by parenchyma relative to stromal tissue was calculated in different age groups and was about 4:6, 3.5:6.5, 3:7, 2:8, and 1.5:8.5 in the 3rd, 4th and 5th, 6th, 7th, 8th and 9th, and 10th decades of life, respectively. Both ER and PR were present in all age groups and the numbers of both receptors were maximal during the 4th decade. The distribution and staining patterns for both ER and PR were recorded in different age groups. The contiguous pattern of ER, which is considered pathognomonic of breast carcinoma, was not seen except in one case in the 6th decade. Moderately stained ER and PR receptor sites predominated throughout. The study of normal breast tissue of similar age might provide comparisons that will help histopathologists to make clinical diagnoses from breast biopsies. Clin. Anat. 29:729-737, 2016. © 2016 Wiley Periodicals, Inc. PMID:27038435

  14. Malignant tumors in an ancient Egyptian population.

    PubMed

    Zink, A; Rohrbach, H; Szeimies, U; Hagedorn, H G; Haas, C J; Weyss, C; Bachmeier, B; Nerlich, A G

    1999-01-01

    Since it is still an open debate whether malignant tumors are mainly influenced by environmental factors, the frequency of such malignant tumors in historic populations with different living conditions is of particular interest. In the present study, we investigated the occurrence of malignant tumors affecting bone tissue in a population of mumrnies and skeletons, which had been excavated from the large necropolis of Thebes-West, Upper Egypt. Our study material comprised a series of at least 415 individuals (thereof 325 adults) dating from approx. 1500-500 B.C. All individuals had been mummified, but were severely damaged and partially broken by grave robbers, so that often only parts of the mummies/skeletons were available for investigation. The available specimens were subjected to careful macroscopic examination, while isolated findings were radiologically analyzed. Using this approach, we identified at least 4 cases showing malignant tumors affecting the skeleton. In two cases, multiple mixed osteolytic-osteoblastic lesions suggested multiple metastases from carcinomas. Two further individuals presented with multiple osteolyses (vertebra, pelvis, skull) most suggestive of multiple myeloma. The observation of at least 4 cases of malignant tumors with osseous manifestation in a series of 325 adult individuals provides clear evidence that malignant tumors were not a rare event in the ancient Egyptian study population, particularly when the limitations of a study of tumors manifested only in osseous remnants are taken into consideration. A calculation of the age- and sex-adjusted tumor frequency in our material in comparison with a recent model for such a material by Waldron (1996) indicates that the rate of malignant tumors with bone affection in our series is higher than in an English population from 1901-1905, although lower than in a comparable present day population. This clearly indicates that important factors affecting malignant tumors were effective even

  15. Solitary fibrous tumor with pseudo-lipoblasts involving the sublingual gland: report of a case and review of the literature.

    PubMed

    Ayad, Tareck; Ghannoum, Julien

    2007-01-01

    Solitary fibrous tumors (SFTs) are mesenchymal neoplasms uncommonly occurring in the salivary glands. In rare instances, SFTs can contain mature fat, atrophic fat, or vacuolated cells previously termed 'pseudo-lipoblasts', which may be misinterpreted as a feature of malignancy. We report an unusual tumor with pseudo-lipoblasts occurring in the sublingual gland. The tumor exhibited a prominent hemangiopericytic pattern, bland cytology, and immunohistochemical and morphologic features consistent with that of an SFT. A review of 15 cases of SFTs of the salivary glands is presented. Emphasis is laid upon the histologic differential diagnosis and the clinical features of these tumors.

  16. Solitary fibrous tumour of the urinary bladder in a young woman presenting with haemodynamic-relevant gross haematuria.

    PubMed

    Heinzelbecker, Julia; Becker, Frank; Pflugmann, Thomas; Friemann, Johannes; Walz, Peter H

    2008-11-01

    A 24-yr-old woman presented with gross haematuria and a huge tumour of the right bladder wall. At transurethral resection, a solid tumour was seen covered with normal mucosa. The pathological evaluation revealed a solitary fibrous tumour (SFT) of the urinary bladder. For final treatment, a partial cystectomy was performed; tumour-free margins were ensured by frozen-section analysis. This is the first case in the literature presenting intravesically in a young woman. Due to the difficulty in discriminating between malignant and benign growth pattern of this tumour entity, a regular follow-up after conservative treatment is mandatory.

  17. Fibrous lung tumor: a peculiar case.

    PubMed

    Barrettara, Barbara; Napoli, Gaetano; Lacitignola, Angelo; Sardelli, Paolo

    2013-08-01

    Solitary fibrous tumor (SFT) of the pleura and the lung is an uncommon spindle cell neoplasm arising from the visceral pleura in the majority of the cases. However there are some extrapleural sites including the lung. Current considerations were raised by a peculiar recent case: an 81-year-old female, no smoker, presented with undefined left thoracic pain. Radiographic findings of a large solid lung mass (10 cm × 9 cm). Computed tomography (CT) confirmed the thoracic mass showing characteristics of a well defined mass with capsule, the position of the mass in proximity of the postero-basal and lateral-basal wall. No secondary lesions were found. Through a left inferior lobectomy and ilo-mediastinal lymph node sampling, the entire mass was resected. Histopathological examination revealed a SFT. In conclusion STF is a rare lesion and this case showed a peculiar extremely large lesion never described before in literature. PMID:23991334

  18. Solitary fibrous tumor of the skin.

    PubMed

    Okamura, J M; Barr, R J; Battifora, H

    1997-10-01

    Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm that most commonly involves the pleura, but is increasingly more often observed in extrapleural locations. A 37-year-old woman presented with an SFT involving the skin and subcutaneous tissue of the scalp. Histologically, SFT is well circumscribed and composed of uniform spindle cells arranged in interlacing fascicles. It exhibits alternating hypercellular and hypocellular areas with abundant thick, often keloid-like, hyalinized collagen. Hemangiopericytoma-like areas are frequently prominent. Immunohistochemical markers for smooth muscle, neural, and epithelial differentiation are negative, but generalized positivity for CD-34 is characteristic. Because of the expanding spectrum of anatomic involvement of SFT, it is not surprising that on rare occasions this tumor may involve the skin.

  19. Fibrous lung tumor: a peculiar case.

    PubMed

    Barrettara, Barbara; Napoli, Gaetano; Lacitignola, Angelo; Sardelli, Paolo

    2013-08-01

    Solitary fibrous tumor (SFT) of the pleura and the lung is an uncommon spindle cell neoplasm arising from the visceral pleura in the majority of the cases. However there are some extrapleural sites including the lung. Current considerations were raised by a peculiar recent case: an 81-year-old female, no smoker, presented with undefined left thoracic pain. Radiographic findings of a large solid lung mass (10 cm × 9 cm). Computed tomography (CT) confirmed the thoracic mass showing characteristics of a well defined mass with capsule, the position of the mass in proximity of the postero-basal and lateral-basal wall. No secondary lesions were found. Through a left inferior lobectomy and ilo-mediastinal lymph node sampling, the entire mass was resected. Histopathological examination revealed a SFT. In conclusion STF is a rare lesion and this case showed a peculiar extremely large lesion never described before in literature.

  20. A solitary fibrous tumor of the kidney.

    PubMed

    Abeygunasekera, Anuruddha M; Ginige, Anusha P; Liyanage, Indika S H; Hareendra, Kalana

    2015-01-01

    A solitary fibrous tumor (SFT) is an uncommon spindle cell neoplasm that usually occurs in the pleura, but may occur in extrapleural sites. Its occurrence in the kidney is rare. We report a SFT, clinically thought to be a renal cell carcinoma arising in the kidney of a 68-year-old female. The tumor was well-circumscribed and composed of a mixture of spindle cells and dense collagenous bands. Immunohistochemical studies revealed reactivity for CD34, CD99, and Bcl-2 protein, with no staining for keratin or muscle markers, confirming the diagnosis. The immunohistochemical study was the key to diagnosis. Several younger members of her family had colorectal and lung cancers suggesting the possibility of a familial or genetic susceptibility.

  1. Chromosomal imbalances in meningeal solitary fibrous tumors.

    PubMed

    Martin, Andrew J; Summersgill, Brenda M; Fisher, Cyril; Shipley, Janet M; Dean, Andrew F

    2002-06-01

    We present the results of a comparative genomic hybridization (CGH) analysis of three meningeal solitary fibrous tumors (SFT). One case showed loss of chromosome 3 and two tumors had deletions of the region 3p21-p26. Other chromosomal losses included 4p15, 8q22-q24, 10, 11q14-q25, 17q11- q23, 20, and 21 in one case each. In addition, there were gains of 18p11-p13 in one case, and 1p11-p36 and 20q11-q13 in another. To our knowledge, there are no previous CGH or cytogenetic data on meningeal SFT, and loss of material on chromosome 3 has not been described in SFT at other sites. Our findings are discussed in relation to published molecular genetic and cytogenetic data on meningioma and hemangiopericytoma, the two lesions with which meningeal SFT are most likely to be confused.

  2. Solitary fibrous tumour of the renal peripelvis.

    PubMed

    Fukunaga, M; Nikaido, T

    1997-05-01

    Solitary fibrous tumours (SFTs) are rare spindle cell neoplasms generally associated with the serosal surface, especially the pleura. This report describes two SFTs arising in the renal peripelvis, occurring in 33- and 36-year-old females. The lesions lacked the characteristic features of other recognized neoplasms that occur in the kidney. Immunohistochemically, the tumour cells were diffusely and strongly positive for vimentin and CD34, and some tumour cells expressed alpha-smooth muscle actin. Both tumours were diploid by flow cytometry. Both patients have had benign clinical courses with 7.5- and 1-year follow-up. The findings suggest that the SFTs may originate from peripelvic mesenchymal cells, a new location for SFT. SFT should be included in the differential diagnosis of spindle cell tumours arising in the renal pelvis and peripelvis.

  3. Solitary fibrous tumor of the pancreas.

    PubMed

    Sugawara, Yoshifumi; Sakai, Shinya; Aono, Shoji; Takahashi, Tadaaki; Inoue, Takeshi; Ohta, Koji; Tanada, Minoru; Teramoto, Norihiro

    2010-07-01

    A solitary fibrous tumor (SFT) originating in the pancreas is rare. We report a 55-year-old woman with an asymptomatic pancreatic mass incidentally discovered on abdominal ultrasonography. Contrast-enhanced computed tomography (CT) showed a well-demarcated exophytic mass in the pancreatic head with prolonged and delayed enhancement. The mass showed hypointensity on T1-weighted images and heterogeneous hypointensity with spotty hyperintensity foci on T2-weighted images. Fluorodeoxyglucose-positron emission tomography (FDG-PET)/CT showed no significant FDG uptake. The resected mass was composed of spindle cells that were positive for CD34; and hemangiopericytomatous vessels were focally detected. The mass was finally diagnosed as an SFT of the pancreas.

  4. Solitary fibrous tumor of renal pelvis.

    PubMed

    Yazaki, T; Satoh, S; Iizumi, T; Umeda, T; Yamaguchi, Y

    2001-09-01

    A 70-year-old Japanese man was referred because of a right renal mass of 2 years in duration. Imaging studies, including magnetic resonance imaging, revealed an ovoid mass, with relatively abundant vascularity, in the right renal pelvis. Right radical nephrectomy was done and a tumor measuring 6.0 x 4.5 x 4.0 cm was found in the renal pelvis. Solitary fibrous tumor (SFT) was highly suspected by histology. Immunohistochemical study using a monoclonal antibody directed against the human hematopoietic progenitor cell antigen (CD34) stain confirmed SFT. This is the first case of SFT of the renal pelvis. Although SFT is extremely rare in urogenital organs, this tumor must be included in the differential diagnosis when we encounter urogenital tumors consisting of mesenchymal elements.

  5. Solitary fibrous tumor of the orbit.

    PubMed

    Luo, Shyh-Hwa; Kao, Shine C S; Pan, Chih-Shin

    2003-10-01

    Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that most frequently arises in the pleura and uncommonly involves the orbit. We report the clinical and pathological features of an orbital SFT in a 50-year-old woman. The patient presented with an 8-year history of painless, slow-growing, visible mass in the right caruncle. Computed tomography showed a well-circumscribed, homogenous, soft tissue mass in the inferomedial aspect of the orbit. The tumor was totally excised via anterior orbitotomy. Histopathologic and immunohistochemical evaluation identified the mass as an SFT. The patient was free of tumor recurrence at 1-year follow-up. Orbital SFT can present as a visible mass in the caruncle and may recur if not excised completely. Long-term follow-up of patients is necessary because SFT may recur many years after operation.

  6. Solitary fibrous tumor of the skin.

    PubMed

    Hardisson, David; Cuevas-Santos, Jesús; Contreras, Félix

    2002-02-01

    Solitary fibrous tumor (SFT) is an uncommon tumor initially reported in the pleura but recently described in other sites of the body. We report a SFT of the skin that presented as painless nodule located in the right cheek in a 56-year-old woman. Histologically, the tumor was composed of spindle-shaped cells arranged in a patternless pattern of short and narrow fascicles with interspersed bundles of thick collagen, and numerous blood vessels with a focally hemangiopericytoma-like appearance. Immunohistochemically, the tumor cells strongly expressed vimentin, CD34 and bcl-2. The lesion was excised and the patient remains well at 16-month follow-up. This case presented some diagnostic difficulty because of its unusual location, and had to be distinguished from other superficial soft tissue tumors. Recognition of SFT in the skin is important to avoid possible confusion with a variety of spindle cell neoplasms with different biologic potential.

  7. Parasagittal solitary fibrous tumor resembling hemangiopericytoma.

    PubMed

    Shidoh, Satoka; Yoshida, Kazunari; Takahashi, Satoshi; Mikami, Shuji; Mukai, Makio; Kawase, Takeshi

    2010-04-01

    Solitary fibrous tumor (SFT) is a rare mesenchymal tumor in the central nervous system, and the clinical behavior of this tumor is similar to that of meningioma. We report the case of a Japanese woman with parasagittal SFT that resembled hemangiopericytoma (HPC). Histological examination revealed that the tumor was highly cellular, with cells containing oval- or spindle-shaped nuclei arranged in sheets or a pattern-less growth mode. Focal vascular proliferation was also observed. Some areas showed intercellular stroma containing remarkable eosinophilic collagens. Tumor cells showed a strong immunoreactivity for CD34 but were negative for S-100 protein and epithelial membrane antigen. MIB-1 labeling index of the tumor was 6.6%. Owing to the high cellularity, high MIB-1 labeling index, and focal vascular proliferation, it was difficult to distinguish this lesion from HPC. However, the tumor was finally diagnosed as SFT on the basis of the strong immunostaining for CD34 and absence of pericellular reticulin.

  8. Solitary fibrous tumor in the mental region.

    PubMed

    Hirano, M; Tanuma, J; Shimoda, T; Sugihara, K; Tsuneyoshi, M; Kitano, M

    2001-11-01

    Solitary fibrous tumor (SFT) is a rare, benign, soft tissue tumor that most commonly occurs in the pleura; however, it has recently been described in other sites of the body. To date, eight examples of oral SFT have been reported. This paper is a description of the first case of an SFT occurring as a soft tissue tumor in the mental region. Histologically, the tumor was composed predominantly of rather uniform spindle-shaped fibroblastic cells arranged in vague fascicles or in a haphazard fashion, intermingled with abundant collagen fibers. Immunohistochemically, the tumor cells were positive for CD34 and vimentin, and weakly positive for muscle actin and alpha-smooth muscle actin. The diagnosis of SFT may be difficult as this tumor shares a number of histological features with other soft tissue tumors. Awareness of its occurrence in the oral cavity is important so that confusion with other spindle cell neoplasms can be avoided.

  9. Insulin receptor activation in solitary fibrous tumours.

    PubMed

    Li, Y; Chang, Q; Rubin, B P; Fletcher, C D M; Morgan, T W; Mentzer, S J; Sugarbaker, D J; Fletcher, J A; Xiao, S

    2007-04-01

    Solitary fibrous tumours (SFTs) are known to overexpress insulin-like growth factor 2 (IGF-2). The down-stream oncogenic pathways of IGF-2, however, are not clear. Here we report uniform activation of the insulin receptor (IR) pathway in SFTs, which are mesenchymal tumours frequently associated with hypoglycaemia. Whereas the IR and its downstream signalling pathways were constitutively activated in SFTs, insulin-like growth factor 1 receptor (IGF-1R) was not expressed in these tumours. We also find that SFT cells secrete IGF-2 and proliferate in serum-free medium, consistent with an IGF-2/IR autocrine loop. The aetiological relevance of IGF-2 is supported by expression of IR-A, the IR isoform with high affinity for IGF-2, in all SFTs. Our studies suggest that IR activation plays an oncogenic role in SFTs.

  10. Solitary fibrous tumor of the abdominal wall.

    PubMed

    Migita, Kazuhiro; Watanabe, Akihiko; Nakagawa, Kenji; Ohyama, Takao; Sekigawa, Susumu

    2009-12-01

    Solitary fibrous tumors (SFTs) are uncommon neoplasms of mesenchymal origin that usually arise from the pleura. SFTs of the abdominal wall are extremely rare, and only 12 cases have been reported in the English language literature. This report presents a new case of SFT of the abdominal wall in a 74-year-old female. Positron emission tomography demonstrated the heterogeneous 18F-fluorodeoxyglucose uptake of the tumor (the maximum standardized uptake value was 2.8). Histologically, the mitotic count was 1 to 2/10 high-power fields. The patient is alive without recurrence at 10 months after undergoing a surgical excision. We discuss the clinicopathological features and differential diagnosis and present a review of the pertinent literature.

  11. Solitary fibrous tumor of the uterine cervix.

    PubMed

    Rahimi, Kurosh; Shaw, Patricia A; Chetty, Runjan

    2010-03-01

    A 68-year-old woman with stage pT1b1 N0 cervical squamous carcinoma had an incidental cervical polyp. The polyp measured 1.7 cm in maximal diameter and histologic evaluation showed it to be composed of spindle-shaped cells with hypercellular and hypocellular foci. The stroma was collagenized and contained several dilated vascular channels that imparted a hemangiopericytic pattern to the lesion. Immunohistochemistry showed the spindle cells to be positive for vimentin, CD99, CD34, bcl-2, ER, PR, and beta-catenin (cytoplasmic) but negative for EMA, S100, factor XIIIa, AE1/AE3, caldesmon, desmin, CD31, and smooth muscle actin. The morphology and immunophenotype was in keeping with a diagnosis of a solitary fibrous tumor (SFT). SFT shares several histologic features of a superficial cervicovaginal myofibroblastoma; the cellular variability, pattern and distribution of vessels, stromal collagenization, and desmin negativity favors SFT.

  12. Solitary fibrous tumor in the pelvic space.

    PubMed

    Ishikawa, Tomomoto; Kawabata, Gaku; Terakawa, Tomoaki; Kamidono, Sadao; Fujisawa, Masato

    2004-02-01

    A case of a solitary fibrous tumor (SFT) of the pelvic space in a 64-year-old man is reported herein. Computed tomography (CT) of the pelvis showed a large mass enhanced heterogeneously left paracentral and posterior to the bladder and intimately associated with prostate. The site of origin of the mass could not be defined on CT because margins blended with the bladder, prostate, and rectum. A tumorectomy was performed and has remained well with no evidence of recurrence during the last 3 months. The tumor was 12.5 x 9.5 x 8.3 cm in size, solid with a fibromuscular capsule, and gray-tan in color. Histologically, the neoplasms were well circumscribed and composed of short spindle cells arranged without an obvious pattern. Immunohistochemically, these cells were strongly positive for CD 34 and negative for S-100, alpha SMA, and AE1/AE3.

  13. Solitary fibrous tumour: a diagnostic dilemma.

    PubMed

    Ghosh, Sharmila; Shet, Tanuja M; Chinoy, R F; Kane, S V

    2007-07-01

    Solitary fibrous tumour (SFT) is a rare spindle cell neoplasm arising at pleural and extrapleural sites. Five cases of SFT diagnosed at our institution over a five year period were reviewed. Haematoxylin and eosin stained histological sections, immuno-histochemical markers including CD34 and electron microscopy were the different methods used to study these tumours. Three histological features were consistently observed in all the tumours: the tumours were composed of short spindle cells separated by dense collagen bands and arranged in alternate hypocellular and hypercellular areas. CD34 positivity was seen in all the cases. SFT's have been reported to behave in an unpredictable fashion and hence prolonged follow up is essential. Histology, CD34 positivity and electron microscopy are useful tools in diagnosing SFT. While the pleural tumours can be diagnosed based on histology, this must be substantiated by ancillary techniques in case of extrapleural tumours.

  14. A solitary fibrous tumor of the kidney.

    PubMed

    Abeygunasekera, Anuruddha M; Ginige, Anusha P; Liyanage, Indika S H; Hareendra, Kalana

    2015-01-01

    A solitary fibrous tumor (SFT) is an uncommon spindle cell neoplasm that usually occurs in the pleura, but may occur in extrapleural sites. Its occurrence in the kidney is rare. We report a SFT, clinically thought to be a renal cell carcinoma arising in the kidney of a 68-year-old female. The tumor was well-circumscribed and composed of a mixture of spindle cells and dense collagenous bands. Immunohistochemical studies revealed reactivity for CD34, CD99, and Bcl-2 protein, with no staining for keratin or muscle markers, confirming the diagnosis. The immunohistochemical study was the key to diagnosis. Several younger members of her family had colorectal and lung cancers suggesting the possibility of a familial or genetic susceptibility. PMID:26458681

  15. Method of manufacturing fibrous hemostatic bandages

    SciTech Connect

    Larsen, Gustavo; Spretz, Ruben; Velarde-Ortiz, Raffet

    2012-09-04

    A method of manufacturing a sturdy and pliable fibrous hemostatic dressing by making fibers that maximally expose surface area per unit weight of active ingredients as a means for aiding in the clot forming process and as a means of minimizing waste of active ingredients. The method uses a rotating object to spin off a liquid biocompatible fiber precursor, which is added at its center. Fibers formed then deposit on a collector located at a distance from the rotating object creating a fiber layer on the collector. An electrical potential difference is maintained between the rotating disk and the collector. Then, a liquid procoagulation species is introduced at the center of the rotating disk such that it spins off the rotating disk and coats the fibers.

  16. Acoustical properties of highly porous fibrous materials

    NASA Technical Reports Server (NTRS)

    Lambert, R. F.

    1979-01-01

    Highly porous, fibrous bulk sound absorbing materials are studied with a view toward understanding their acoustical properties and performance in a wide variety of applications including liners of flow ducts. The basis and criteria for decoupling of acoustic waves in the pores of the frame and compressional waves in the frame structure are established. The equations of motion are recast in a form that elucidates the coupling mechanisms. The normal incidence surface impedance and absorption coefficient of two types of Kevlar 29 and an open celled foam material are studied. Experimental values and theoretical results are brought into agreement when the structure factor is selected to provide a fit to the experimental data. A parametric procedure for achieving that fit is established. Both a bulk material quality factor and a high frequency impedance level are required to characterize the real and imaginary part of the surface impedance and absorption coefficient. A derivation of the concepts of equivalent density and dynamic resistance is presented.

  17. [A case of retroperitoneal solitary fibrous tumor].

    PubMed

    Tadokoro, Akira; Kawaguchi, Makoto; Koike, Hiroshi

    2010-06-01

    A 96-year-old female visited our hospital with a complaint of right flank pain. She was under treatment for diabetes mellitus, hypertension and moderate renal dysfunction in the department of internal medicine. Computer tomographic (CT) scan showed a mass 12 cm in diameter compressing the right kidney and inferior vena cava. We diagnosed the right renal cyst. Eight weeks later, CT scan revealed a gradual increase of the mass. We punctured the tumor in order to reduce her complaint and investigate the rumor. Because contrast material could not be infused into the mass, needle biopsy was performed. Pathological examination revealed hypercellularity of spindle cells, that showed positive for CD34, vimentin, desmin and Mic-2, and negative for S100, alphaSMA, c-kit, AE1/3, p53 and bcl-2. The tumor was finally diagnosed as solitary fibrous tumor.

  18. Mediastinal solitary fibrous tumor with right diaphragm invasion: report of a case.

    PubMed

    Xue, Xingyang; Chen, Jun; Ma, Wei; Zhu, Daxing; Zhang, Weiguo; Chen, Gang; Wei, Sen; Zhou, Qinghua

    2009-01-01

    Mediastinal solitary fibrous tumors (SFTs) are rarely found in adults and there are few reports describing primary mediastinal SFT invading the diaphragm. We report the case of a 47-year-old woman with a large right inferior mediastinal SFT. Magnetic resonance imaging showed the tumor invading the right lower lobe of the lung and the right hemidiaphragm, with displacement of the inferior vena cava (IVC) and right lobe of the liver. Angiogram showed IVC stenosis. To our knowledge, this is the first report of complete resection of the tumor combined with right lower lobectomy of the lung and partial resection and reconstruction of the right diaphragm with a Dacron flap.

  19. Pediatric renal solitary fibrous tumor: report of a rare case and review of the literature.

    PubMed

    Wu, William W; Chu, Julia T; Romansky, Stephen G; Shane, Lisa

    2015-02-01

    Solitary fibrous tumors (SFTs) are unusual spindle cell neoplasms initially described in the pleura but have since been discovered in many extrapleural locations. SFT of the kidney is extremely rare, the majority occurring in middle-aged adults. To date, only two pediatric cases of renal SFT have been reported. We report a case of large SFT in the kidney of a 3-year-old boy that was clinically and radiologically thought to be a nephroblastoma. This case is the first pediatric renal SFT to be reported with detailed histopathologic and cytogenetic analyses. SFT should be included in the differential diagnosis of pediatric renal tumors.

  20. Solitary Fibrous Tumor in the Lacrimal Gland Fossa: A Case Report

    PubMed Central

    Mupas-Uy, Jacqueline; Kitaguchi, Yoshiyuki; Takahashi, Yasuhiro; Takahashi, Emiko; Kakizaki, Hirohiko

    2016-01-01

    Solitary fibrous tumors (SFTs) are benign, spindle-cell tumors of mesenchymal origin that are usually seen in the superior orbital area in adults. We report a rare case of SFT in the lacrimal gland fossa that developed in a young female. A 25-year-old woman had a 6-month history of a progressive painless mass in the left upper eyelid accompanied by proptosis. Computed tomography and magnetic resonance imaging showed an ovoid, demarcated mass with distinct margins in the lacrimal gland region without bone invasion. Excision biopsy with immunohistochemical study, specifically with positive signal transducer and activator of transcription 6, confirmed the diagnosis. PMID:27721790

  1. Cutaneous balloon cell dermatofibroma (fibrous histiocytoma).

    PubMed

    Tran, Tien Anh; Hayner-Buchan, Alida; Jones, David M; McRorie, Duane; Carlson, J Andrew

    2007-04-01

    Dermatofibroma (DF) or cutaneous fibrous histiocytoma is a common benign skin tumor that exhibits multiple, distinct histologic variants. Although clear cell DF has been described in the literature, balloon cell degeneration causing a clear cell DF phenotype has been not been reported to date. Herein, we describe the clinicopathologic findings of balloon cell DF arising on the heel of a 43-year-old man. Clinically, it presented as enlarging tan-white, ulcerated, firm 1.5 cm nodule, clinically suspected to be pyogenic granuloma. Excisional biopsy revealed a circumscribed fibrous tumor populated by mostly clear and spindle cells. A zonal arrangement separated the varied tumor cells where the most superficial, polypoid area showed large, clear polygonal balloon cells; the mid-dermal zone demonstrated a transition between balloon cells, epithelioid cells, and spindle cells; and the deep dermal zone had storiform arrangement of spindle cells, with the fascicles separated by coarse collagen bundles. A CD10+ > CD68+ > Factor XIIIa+ immunophenotype was identified with negative immunolabeling for S-100 protein, HMB-45, cytokeratin AE1/AE3, desmin, smooth muscle actin, lysozyme, and leukocyte common antigen (LCA). Ultrastructurally, the clear tumor cells were filled with multiple, empty, nonmembrane bound vacuoles of varying size. No recurrence has been described after complete excision and 7 months of follow up. DF with balloon cell change, likely secondary to persistent irritation, should be added to the differential diagnosis of cutaneous primary and metastatic neoplasms showing balloon cell degeneration such as balloon cell melanocytic nevi and renal cell carcinoma, respectively.

  2. Isolated subpulmonic fibrous ring, mirror-image dextrocardia and situs solitus in a young lady unreported and a near miss.

    PubMed

    Yousif, Musaab; Elhassan, Nezar B; Ali, Sulafa K M; Ahmed, Yassir

    2013-12-01

    Congenital diseases causing obstruction of the right ventricular outflow tract (RVOT) are common, but the isolated subpulmonary membrane/ring is extremely rare and can be difficult to diagnose precisely, especially in adults. We report a case of surgically resected isolated subpulmonic fibrous ring in a lady with mirror-image dextrocardia and abdominal situs solitus that was misdiagnosed by echocardiography as a subaortic membrane.

  3. [Solitary fibrous tumor of the kidney. A propos of a case].

    PubMed

    Marzi, M; D'Alpaos, M; Piras, P; Paiusco, A; Minervini, M S; Di Zitti, P

    2009-01-01

    Solitary Fibrous Tumors (SFT) are rare spindle cell neoplasm that typically originate from the pleura. However, cases of the SFT are described with origin in other organs, including the urinary and genital apparatus. Within the kidney, except from the renal pelvis, only 19 cases of SFT are described and such rarity of localization makes the histogenesis and the prognosis of the tumor rather unknown. We report the case of a 72-year-old lady who attended our Unit for a mass that was clinically palpable at the level of the left hemiabdomen. The tomodensitographic test indicated a 19cm-diameter mass of likely pertinence of the middle bystender of the left kidney. She had undergone left radical nephrectomy. The histological examination highlighted a solitary fibrous tumor (SFT): the presence of hypercellularity, of cellular pleiomorphism and of a high number of mitosis has led to a histological diagnosis of malignancy for the neoplasm analyzed. The SFT are of rare clinical comparison: this does not allow for a deep knowledge of the lesion histogenesis and prognosis; moreover, the clinical behavior should be more precisely defined.

  4. Solitary fibrous tumor of the thyroid gland: report of seven cases.

    PubMed

    Rodriguez, I; Ayala, E; Caballero, C; De Miguel, C; Matias-Guiu, X; Cubilla, A L; Rosai, J

    2001-11-01

    Solitary fibrous tumor is a soft tissue neoplasm initially described in the pleura but subsequently reported in a wide variety of locations. The clinical behavior is usually benign, but the existence of aggressive cases has been documented both in the pleura and in extrapleural sites. In this report clinical and pathologic features of seven solitary fibrous tumors of the thyroid gland are presented. Patients' ages ranged from 43 to 64 years (mean 52 years), and tumor sizes varied from 2 to 6 cm. Grossly, the tumors were white-tan and well circumscribed. Microscopically, there was a variegated, wavy, storiform, hemangiopericytic or desmoid-like arrangement of spindle cells. Trapped thyroid follicles within the tumor and peripheral jagged tumor infiltration among follicles were common. There was immunohistochemical reactivity for CD34, CD99, and bcl-2, and ultrastructural analysis of one tumor was consistent with a fibroblastic lineage. The differential diagnosis included other benign and malignant mesenchymal tumors of the thyroid, spindle cell follicular adenoma, Riedel's thyroiditis, the spindle cell, and paucicellular variants of anaplastic carcinoma, papillary thyroid carcinoma with exuberant nodular fasciitis-like stroma, and the spindle epithelial tumor with thymus-like differentiation. The cumulative data of 13 cases (comprised of the seven present cases and the six previously reported) suggest a benign clinical behavior for thyroid SFT.

  5. Pedunculated solitary fibrous tumours arising from the pleura.

    PubMed

    Poyraz, A; Kilic, D; Hatipoglu, A; Bakirci, T; Bilezikci, B

    2006-09-01

    Solitary fibrous tumour (SFT) is one of the rare tumours which arise from visceral pleura. Klemperer and Rabin first described SFT as a distinct clinical entity among primary pleural tumoUrs in 1931. Approximately 820 cases have been reported in literature to date. The management of patients with SFT is complete resection of the tumour and follow up of the patient to detect any possible late recurrence. In the present paper, we report two cases of pedunculated solitary fibrous tumours of the pleura that appeared as a wandering chest nodule to which surgical resection undertaken at our hospital. The aim is to summarise our experience in the management of solitary fibrous tumour.

  6. Solitary Fibrous Tumor of the Cerebello-Pontine Angle

    PubMed Central

    Biggs, Nigel D.; Fagan, Paul A.; Turner, Jennifer J.; Doust, Bruce

    1999-01-01

    A case is presented of solitary fibrous tumor occurring in the cerebello-pontine angle. There have been only two other reported cases of a solitary fibrous tumors in this region. Imaging studies showed the tumor to be characteristic in shape and position of an acoustic tumor. However, at surgery the tumor was found to have a “rock hard” consistency. Solitary fibrous tumor differs from acoustic schwannoma and meningioma in its histopathological features and in this case, regrowth, after incomplete excision, was extremely rapid. ImagesFigure 2Figure 3Figure 4Figure 5Figure 6 PMID:17171119

  7. Surgical resection using retroperitoneal approach for solitary fibrous tumor in the pelvis.

    PubMed

    Shoji, Sunao; Nakano, Mayura; Yamamoto, Shinya; Okada, Naotaka; Nagata, Yoshihiro; Sekido, Yasutomo; Terayama, Hayato; Ito, Masahiro; Uchida, Toyoaki

    2011-07-01

    This report concerns a case of solitary fibrous tumor (SFT) for which surgical resection was performed using a retroperitoneal approach. A 41-year-old man was referred to our hospital with urinary retention. Abdominal ultrasound sonography (US) and computed tomography (CT) showed a hypervascular mass lesion in the pelvis. Transrectal biopsy showed SFT. Surgical resection was carried out using a retroperitoneal approach and preserving the neural network related to urinary and erectile functions. Based on immunohistochemical findings, the tumor was diagnosed to be malignant SFT in the pelvic cavity. Urinary function improved post-operation. There was no change to IIEF-5 and it continued to function well. The patient showed no sign of recurrence 12 months after surgery and required no additional therapy.

  8. Solitary fibrous tumor of the liver expressing CD34 and vimentin: a case report.

    PubMed

    Korkolis, Dimitris-P; Apostolaki, Katerina; Aggeli, Chrysanthi; Plataniotis, George; Gontikakis, Emmanuel; Volanaki, Dimitra; Sebastiadou, Maria; Dimitroulopoulos, Dimitris; Xinopoulos, Dimitris; Zografos, George-N; Vassilopoulos, Perikles-P

    2008-10-28

    A case of a successfully treated solitary fibrous tumor (SFT) of the liver is reported. An 82-year-old female presented with left upper abdominal discomfort, a firm mass on palpation, and imaging studies revealed a large tumor, 15 cm in diameter, arising from the left lobe of the liver. A formal left hepatectomy was performed. Microscopic evaluation showed spindle and fibroblast-like cells within the collagenous stroma. Immunohistochemistry disclosed diffuse CD34 and positive vimentin, supporting the diagnosis of a benign SFT. The patient remained well 21 months after surgery. SFT of the liver is a very rare neoplasm of mesenchymal origin. In most cases it is a benign lesion, although some may have malignant histological features and recur locally or metastasize. With less than 30 reported cases in the literature, little can be said regarding its natural history or the benefits of adjuvant radiochemotherapy. Complete surgical resection remains the cornerstone of its treatment.

  9. Recurrent solitary fibrous tumor of the pleura: significant response to radiotherapy.

    PubMed

    Saynak, Mert; Bayir-Angin, Gulden; Kocak, Zafer; Oz-Puyan, Fulya; Hayar, Murat; Cosar-Alas, Rusen; Karamustafaoglu, Altemur; Yurut-Caloglu, Vuslat; Caloglu, Murat; Yoruk, Yener

    2010-03-01

    Solitary fibrous tumor (SFT) of the pleura is an uncommon neoplasm with non-specific symptoms and non-pathognomonical radiological findings. Surgery allows establishment of a definitive diagnosis as well as a cure of the disease. The role of radiotherapy or chemotherapy in the management of the disease is unclear because of the rarity of the disease and the successful results of the surgical treatment. Long-term clinical follow-up may be useful for the patients with SFT because of the potential adverse biological behavior of this tumor, which may lead to repeated recurrences and/or malignant transformation. We reported a 66-year-old woman with recurrence of SFT in the right lung, which had significant response to external thoracic radiotherapy.

  10. Potentially disseminated solitary fibrous tumor of the pleura: a case report.

    PubMed

    Nakano, Tomoyuki; Endo, Shunsuke; Tsubochi, Hiroyoshi; Tetsuka, Kenji; Nokubi, Mitsuhiro

    2015-10-01

    We report a case involving a female patient with frequent relapse, pleural dissemination, and port site recurrence (PSR) of a pleural solitary fibrous tumor (SFT). At the age of 55 years, she underwent tumor resection via video-assisted thoracoscopic surgery (VATS). The tumor arose from the mediastinal pleura; it was 7 cm in diameter and well demarcated. Histological examination showed neither hemorrhage nor necrosis, but moderate cellularity was present, and the Ki-67 labeling index was 15%. Despite complete resection, the tumor relapsed in the ipsilateral thoracic cavity 3 years postoperatively, and thoracoscopic complete tumor resection was performed; however, pleural lavage cytology (PLC) showed the presence of tumor cells. Multiple pleural dissemination and PSR developed 7 years after the initial surgery. The port site recurrent tumor was resected with the intercostal muscle via VATS. This case illustrates that a SFT may disseminate despite the fact that histological examination shows no evidence of malignancy.

  11. Is a Solitary Fibrous Tumor in the External Auditory Canal Benign?

    PubMed

    Lee, Chi-Kyou; Lee, Hyun Ju

    2016-09-01

    A solitary fibrous tumor (SFT) is an extremely rare, distinct otological soft-tissue tumor. Only two such tumors in the external auditory canal have been reported. A SFT related to hemangiopericytomas (HPC), which commonly arise in the central nervous system. HPCs act malignant in many cases, while SFTs at other sites are mainly benign. A 25-year-old female presented with highly vascular tumor at the right external auditory meatus and bleeding from the mass when a crust was removed from its surface. After excisional biopsy followed by pre-operative embolization, this tumor confirmed with SFT. The recurrence rate of SFT is very low after complete resection, with a slightly increased risk of recurrence with extrathoracic tumors. We describe the third case of SFT in the external auditory canal and review the literature. PMID:27626087

  12. Potentially disseminated solitary fibrous tumor of the pleura: a case report.

    PubMed

    Nakano, Tomoyuki; Endo, Shunsuke; Tsubochi, Hiroyoshi; Tetsuka, Kenji; Nokubi, Mitsuhiro

    2015-10-01

    We report a case involving a female patient with frequent relapse, pleural dissemination, and port site recurrence (PSR) of a pleural solitary fibrous tumor (SFT). At the age of 55 years, she underwent tumor resection via video-assisted thoracoscopic surgery (VATS). The tumor arose from the mediastinal pleura; it was 7 cm in diameter and well demarcated. Histological examination showed neither hemorrhage nor necrosis, but moderate cellularity was present, and the Ki-67 labeling index was 15%. Despite complete resection, the tumor relapsed in the ipsilateral thoracic cavity 3 years postoperatively, and thoracoscopic complete tumor resection was performed; however, pleural lavage cytology (PLC) showed the presence of tumor cells. Multiple pleural dissemination and PSR developed 7 years after the initial surgery. The port site recurrent tumor was resected with the intercostal muscle via VATS. This case illustrates that a SFT may disseminate despite the fact that histological examination shows no evidence of malignancy. PMID:26623132

  13. Is a Solitary Fibrous Tumor in the External Auditory Canal Benign?

    PubMed Central

    Lee, Hyun Ju

    2016-01-01

    A solitary fibrous tumor (SFT) is an extremely rare, distinct otological soft-tissue tumor. Only two such tumors in the external auditory canal have been reported. A SFT related to hemangiopericytomas (HPC), which commonly arise in the central nervous system. HPCs act malignant in many cases, while SFTs at other sites are mainly benign. A 25-year-old female presented with highly vascular tumor at the right external auditory meatus and bleeding from the mass when a crust was removed from its surface. After excisional biopsy followed by pre-operative embolization, this tumor confirmed with SFT. The recurrence rate of SFT is very low after complete resection, with a slightly increased risk of recurrence with extrathoracic tumors. We describe the third case of SFT in the external auditory canal and review the literature. PMID:27626087

  14. Is a Solitary Fibrous Tumor in the External Auditory Canal Benign?

    PubMed

    Lee, Chi-Kyou; Lee, Hyun Ju

    2016-09-01

    A solitary fibrous tumor (SFT) is an extremely rare, distinct otological soft-tissue tumor. Only two such tumors in the external auditory canal have been reported. A SFT related to hemangiopericytomas (HPC), which commonly arise in the central nervous system. HPCs act malignant in many cases, while SFTs at other sites are mainly benign. A 25-year-old female presented with highly vascular tumor at the right external auditory meatus and bleeding from the mass when a crust was removed from its surface. After excisional biopsy followed by pre-operative embolization, this tumor confirmed with SFT. The recurrence rate of SFT is very low after complete resection, with a slightly increased risk of recurrence with extrathoracic tumors. We describe the third case of SFT in the external auditory canal and review the literature.

  15. Is a Solitary Fibrous Tumor in the External Auditory Canal Benign?

    PubMed Central

    Lee, Hyun Ju

    2016-01-01

    A solitary fibrous tumor (SFT) is an extremely rare, distinct otological soft-tissue tumor. Only two such tumors in the external auditory canal have been reported. A SFT related to hemangiopericytomas (HPC), which commonly arise in the central nervous system. HPCs act malignant in many cases, while SFTs at other sites are mainly benign. A 25-year-old female presented with highly vascular tumor at the right external auditory meatus and bleeding from the mass when a crust was removed from its surface. After excisional biopsy followed by pre-operative embolization, this tumor confirmed with SFT. The recurrence rate of SFT is very low after complete resection, with a slightly increased risk of recurrence with extrathoracic tumors. We describe the third case of SFT in the external auditory canal and review the literature.

  16. Rheumatic Diseases and Malignancies

    PubMed Central

    BOJINCA, Violeta; JANTA, Iustina

    2012-01-01

    ABSTRACT There are many studies which demonstrate a higher risk for malignancy in patients with rheumatic diseases. There have been a number of possible explanations for the differences in the risk of certain malignancies in patients with rheumatic disease, compared with general population, but a clear mechanism is difficult to identify. Rheumatoid syndromes may be associated with malignancy as paraneoplastic conditions, which can antedate the neoplasm diagnosis. On the other hand, autoimmune rheumatic diseases have a higher risk of malignancy by themselves or because of the immunosuppressant treatments. PMID:23482881

  17. Method for distributing chemicals through a fibrous material using low-headspace dielectric heating

    DOEpatents

    Banerjee, Sujit; Malcolm, Earl

    2002-01-01

    System and method for diffusing chemicals rapidly and evenly into and through fibrous material, such as wood. Chemicals are introduced into the fibrous material by applying the chemicals to the fibrous material. After treating the fibrous material with the chemicals, the fibrous material is maintained under low-headspace conditions. Thermal energy or dielectric heating, such as microwave or radio frequency energy, is applied to the fibrous material. As a result, the chemicals are able to distribute evenly and quickly throughout the fibrous material.

  18. [Undifferentiated high grade pleomorphic sarcoma/ malignant fibrous histiocytoma associated a gouty tophus. a case report].

    PubMed

    Szlabi, Susana; Flores, Jorge O; Diller, Ana L de; Cabrera, Marta

    2012-01-01

    Antecedentes: La gota es una enfermedad metabólica por depósito de cristales de ácido úrico, que compromete articulaciones y tejidos blandos tanto en sus etapas agudas como crónicas. Constituye un suceso poco común la aparición de un tumor en el sitio propio de la lesión. Objetivo: presentar un caso de asociación infrecuente entre tofo gotoso y sarcoma. Material y métodos: hombre de 83 años que consultó por tumoración en codo izquierdo de aproximadamente 40 años de evolución, que comenzó a doler espontáneamente. Con la presunción diagnóstica de tofo gotoso se trató quirúrgicamente. La lesión recidivó a los 60 días, se realizó reintervención y radioterapia por diagnóstico de tumor mesenquimal maligno asociado a tofo gotoso. A los 10 meses desarrolló metástasis ganglionar homolateral, falleció antes de los 2 años de la consulta inicial. Resultados: El diagnóstico de la primer biopsia fue tofo gotoso. En el material de reintervención se diagnosticó tofo gotoso asociado a neoplasia mesenquimal fusocelular de alto grado; la inmunohistoquímica reveló: vimentina +/+, MYO D1 -/-, ASMA -/-, FVIII -/-, A1ATT -/-, CD68-/-, S100-/- con resultado diagnóstico final de sarcoma pleomórfico indiferenciado de alto grado. Conclusión: Es infrecuente que los tofos gotosos se asocien a otras enfermedades y menos que lo hagan a tumores. En la bibliografía se han reportado tres casos previos concurrentes con neoplasias, las cuales fueron angiosarcoma, tumor de células gigantes y fibrohistiocitoma maligno. Estos últimos tienen una alta tendencia a recidivar y poseen capacidad de dar metástasis, especialmente a pulmones y ganglios regionales. Palabras clave: tofo gotoso, fibrohistiocitoma maligno, sarcoma pleomórfico indiferenciado.

  19. Reflective Coating on Fibrous Insulation for Reduced Heat Transfer

    NASA Technical Reports Server (NTRS)

    Hass, Derek D.; Prasad, B. Durga; Glass, David E.; Wiedemann, Karl E.

    1997-01-01

    Radiative heat transfer through fibrous insulation used in thermal protection systems (TPS) is significant at high temperatures (1200 C). Decreasing the radiative heat transfer through the fibrous insulation can thus have a major impact on the insulating ability of the TPS. Reflective coatings applied directly to the individual fibers in fibrous insulation should decrease the radiative heat transfer leading to an insulation with decreased effective thermal conductivity. Coatings with high infrared reflectance have been developed using sol-gel techniques. Using this technique, uniform coatings can be applied to fibrous insulation without an appreciable increase in insulation weight or density. Scanning electron microscopy, Fourier Transform infrared spectroscopy, and ellipsometry have been performed to evaluate coating performance.

  20. Pancreatic solitary fibrous tumor causing ectopic adrenocorticotropic hormone syndrome.

    PubMed

    Murakami, Keigo; Nakamura, Yasuhiro; Felizola, Saulo J A; Morimoto, Ryo; Satoh, Fumitoshi; Takanami, Kentaro; Katakami, Hideki; Hirota, Seiichi; Takeda, Yoshiyu; Meguro-Horike, Makiko; Horike, Shin-Ichi; Unno, Michiaki; Sasano, Hironobu

    2016-11-15

    Solitary fibrous tumors occasionally present with hypoglycemia because of the excessive release of insulin-like growth factor II. We report the first case of pancreatic solitary fibrous tumor causing ectopic adrenocorticotropic hormone syndrome. An 82-year-old Japanese man presented with lower limb edema, uncontrolled hypertension, hypokalemia, and baseline hypercortisolism. Distal pancreatectomy was performed after the clinical diagnosis of a neuroendocrine tumor with ectopic secretion of adrenocorticotropic hormone. On histological examination, the tumor showed spindle cells in a fascicular arrangement. The diagnosis of the solitary fibrous tumor was confirmed by the identification of the NAB2-STAT6 fusion gene and positive immuno-histochemical staining for STAT6 and CD34. Using quantitative real-time polymerase chain reaction, mRNA that encoded proopiomelanocortin, precursor of adrenocorticotropic hormone, was detected. Proopiomelanocortin production through the demethylation of the promoter region Domain IV was detected. Pancreatic solitary fibrous tumors represent a new cause of ectopic adrenocorticotropic hormone syndrome. PMID:27585487

  1. Localised fibrous mesothelioma arising in an intralobar pulmonary sequestration.

    PubMed Central

    Paksoy, N; Demircan, A; Altiner, M; Artvinli, M

    1992-01-01

    A localised fibrous mesothelioma arising from an intralobar lung sequestration occurred in a 64 year old Turkish woman. This appears to be the first report of a mesothelioma occurring within a pulmonary sequestration. Images PMID:1481189

  2. Failure Analysis and Mechanisms of Failure of Fibrous Composite Structures

    NASA Technical Reports Server (NTRS)

    Noor, A. K. (Compiler); Shuart, M. J. (Compiler); Starnes, J. H., Jr. (Compiler); Williams, J. G. (Compiler)

    1983-01-01

    The state of the art of failure analysis and current design practices, especially as applied to the use of fibrous composite materials in aircraft structures is discussed. Deficiencies in these technologies are identified, as are directions for future research.

  3. PET/CT in paediatric malignancies - An update.

    PubMed

    Padma, Subramanyam; Sundaram, Palaniswamy Shanmuga; Tewari, Anshu

    2016-01-01

    (18)F-fluorodeoxyglucose positron emission tomography (FDG-PET) is a well-established imaging modality in adult oncological practice. Its role in childhood malignancies needs to be discussed as paediatric malignancies differ from adults in tumor subtypes and they have different tumor biology and FDG uptake patterns. This is also compounded by smaller body mass, dosimetric restrictions, and physiological factors that can affect the FDG uptake. It calls for careful planning of the PET study, preparing the child, the parents, and expertise of nuclear physicians in reporting pediatric positron emission tomography/computed tomography (PET/CT) studies. In a broad perspective, FDG-PET/CT has been used in staging, assessment of therapy response, identifying metastases and as a follow-up tool in a wide variety of pediatric malignancies. This review outlines the role of PET/CT in childhood malignancies other than hematological malignancies such as lymphoma and leukemia. PMID:27688605

  4. PET/CT in paediatric malignancies - An update

    PubMed Central

    Padma, Subramanyam; Sundaram, Palaniswamy Shanmuga; Tewari, Anshu

    2016-01-01

    18F-fluorodeoxyglucose positron emission tomography (FDG-PET) is a well-established imaging modality in adult oncological practice. Its role in childhood malignancies needs to be discussed as paediatric malignancies differ from adults in tumor subtypes and they have different tumor biology and FDG uptake patterns. This is also compounded by smaller body mass, dosimetric restrictions, and physiological factors that can affect the FDG uptake. It calls for careful planning of the PET study, preparing the child, the parents, and expertise of nuclear physicians in reporting pediatric positron emission tomography/computed tomography (PET/CT) studies. In a broad perspective, FDG-PET/CT has been used in staging, assessment of therapy response, identifying metastases and as a follow-up tool in a wide variety of pediatric malignancies. This review outlines the role of PET/CT in childhood malignancies other than hematological malignancies such as lymphoma and leukemia. PMID:27688605

  5. PET/CT in paediatric malignancies - An update

    PubMed Central

    Padma, Subramanyam; Sundaram, Palaniswamy Shanmuga; Tewari, Anshu

    2016-01-01

    18F-fluorodeoxyglucose positron emission tomography (FDG-PET) is a well-established imaging modality in adult oncological practice. Its role in childhood malignancies needs to be discussed as paediatric malignancies differ from adults in tumor subtypes and they have different tumor biology and FDG uptake patterns. This is also compounded by smaller body mass, dosimetric restrictions, and physiological factors that can affect the FDG uptake. It calls for careful planning of the PET study, preparing the child, the parents, and expertise of nuclear physicians in reporting pediatric positron emission tomography/computed tomography (PET/CT) studies. In a broad perspective, FDG-PET/CT has been used in staging, assessment of therapy response, identifying metastases and as a follow-up tool in a wide variety of pediatric malignancies. This review outlines the role of PET/CT in childhood malignancies other than hematological malignancies such as lymphoma and leukemia.

  6. Monostotic Fibrous Dysplasia of the Rib: A Case Report

    PubMed Central

    Mahadevappa, Asha; Patel, Sapna; Ravishankar, Sunila; Manjunath, Gubbanna V.

    2012-01-01

    Fibrous dysplasia is a noninherited bone disease in which abnormal differentiation of osteoblasts leads to replacement of normal marrow and cancellous bone by immature bone with fibrous stroma. Monostotic fibrous dysplasia accounts for 28% in the ribs. It is often asymptomatic and incidentally detected on radiographs. As with many bone abnormalities, it can be superimposed by the formation of aneurysmal bone cysts. We report a case of a 70-year-old lady who presented with swelling on the chest wall of 20-ear duration and sudden increase in size for 8 months. Radiologically, X-ray and CT scan showed an expansible lesion of the medullary cavity with a ground-glass centre and thinning of cortex of the 5th rib. The resected lesion was a firm, well-defined solid, grey-white expansile mass replacing the medullary cavity. Histopathologically, benign fibrous spindle areas with disorganized irregular bony trabeculae were seen. Hemorrhagic spaces lined by osteoclast-like multinucleated giant cells were also noted. The diagnosis was fibrous dysplasia with aneurysmal bone cyst changes. Although fibrous dysplasia with aneurysmal bone cyst is rare, it should be taken into account in differential diagnosis of the rapidly growing solitary rib lesion. PMID:23304592

  7. Primary malignant melanoma

    PubMed Central

    Mısır, A. Ferhat; Durmuşlar, Mustafa C.; Zerener, Tamer; Gün, Banu D.

    2016-01-01

    Malignant melanomas (MM) of the oral cavity are extremely rare, accounting for 0.2% to 8.0% of all malignant melanomas. Malignant melanomas is more frequently seen at the level of the hard palate and gingiva. Early diagnosis and treatment are important for reducing morbidity. Malignant melanoma cells stain positively with antibodies to human melanoma black 45, S-100 protein, and vimentin; therefore, immunohistochemistry can play an important role in evaluating the depth of invasion and the location of metastases. A 76-year-old man developed an oral malignant melanoma, which was originally diagnosed as a bluish reactive denture hyperplasia caused by an ill-fitting lower denture. The tumor was removed surgically, and histopathological examination revealed a nodular-type MM. There was no evidence of recurrence over a 4-year follow-up period. PMID:27052289

  8. Solitary fibrous tumor arising in gastric serosa.

    PubMed

    Lee, Won-Ae; Lee, Mi-Kyung; Jeen, Yoon Mi; Kie, Jeong-Hae; Chung, Jae-Joon; Yun, Seong Hyeon

    2004-06-01

    The solitary fibrous tumor (SFT) of peritoneum, especially arising in gastric serosa, is extremely rare. A case of SFT arising in gastric serosa of a 70-year-old man is reported. On abdominal computed tomography scan, an approximate 8.0 x 5.3 cm-sized solid mass with multiple nodular calcifications was noted in the omentomesentery of right upper abdomen, which was abutting to the gastric antral wall. At laparotomy the tumor was tightly attached to the gastric serosa and was completely excised by wedge resection of the stomach. The cut surfaces of the tumor were pale brown to pale yellow, firm, with multifocal yellowish, gritty, stony-hard nodules and cystic changes. Microscopically, the tumor was composed of mildly cellular benign-appearing spindle-shaped cells and bands of hyalinized collagen in varying proportions. The tumor revealed predominantly sclerosing pattern with extensive hyalinized fibrosis and multifocal dystrophic calcifications. The tumor was mainly located at the gastric serosa and subserosa, and its growth pattern was expansile with entrapping of muscularis propria and submucosa in the periphery of the tumor. By immunohistochemistry, the tumor cells were diffusely positive for CD34 and vimentin but negative for cytokeratin, desmin, smooth muscle actin, CD99 and c-kit. Although its occurrence is rare, SFT should be considered in the differential diagnosis of mesenchymal tumors of the stomach.

  9. Solitary fibrous tumor of the vagina.

    PubMed

    Akiyama, Y; Nabeshima, K; Koita, H; Yamanaka, M; Koono, M

    2000-04-01

    The solitary fibrous tumor (SFT) is a rare tumor that most commonly arises in the pleura. Recent evidence has indicated that this tumor originates from mesenchymal, probably fibroblastic, cells and is not restricted to the pleura. However, its occurrence in the female genital tract is extremely rare. We report a case of primary SFT that originated from the vagina in a 34-year-old female. It was a pedunculated polypoid tumor and occurred at the site of scar tissue, caused by laceration during her last labor 7 years previously. Histologically, the tumor was predominantly composed of a random proliferation of spindle cells, intimately admixed with collagen. Immunohistochemically, the cells were strongly positive for CD34, vimentin and bcl-2, but were negative for S-100 protein, neuron-specific enolase, smooth muscle actin, desmin, CD68, cytokeratins and epithelial membrane antigen. To the best of our knowledge, this is the first reported case of a primary vaginal SFT in the English literature. Our report suggests to include SFT in the differential diagnosis of a spindle cell neoplasm originating from the vagina.

  10. Solitary fibrous tumour of the chest wall.

    PubMed

    Mohtarrudin, N; Nor Hanipah, Z; Mohd Dusa, N

    2016-04-01

    Extrapleural solitary fibrous tumours (SFTs) are rare tumours characterized by patternless spindle cells with haemangiopericytoma-like vascular spaces. Previously the tumours have been classified as haemangiopericytoma, an entity that is now considered obsolete. We report a case of extrapleural SFT arising in the soft tissue of the chest wall. The patient was a 31-year-old Malay lady presenting with a mobile swelling of the right chest wall for more than five years. During excision the tumour was noted to be well-circumscribed and yellowish in colour, giving an impression of lipoma. Microscopically, the tumour had patternless architecture, characterized by hypocellular and hypercellular areas. It was composed of uniform, spindle-shaped cells displaying oval nuclei, inconspicuous nucleoli, pale cytoplasm and indistinct cell borders. The mitotic count was 2 per 10 HPF. Branching, medium-sized thin-walled blood vessels in a haemangiopericytomatous growth pattern, some with hyalinised wall were identified. The neoplastic cells were immunoreactive to CD99 and CD34 and were non-immunoreactive to Desmin, Smooth Muscle Actin, S100 protein and EMA. We elucidate the challenges in diagnosing this tumour in this unusual location.

  11. Solitary fibrous tumor of the urinary bladder.

    PubMed

    Kim, Se Hoon; Cha, Kyung Bin; Choi, Young Deuk; Cho, Nam Hoon

    2004-06-30

    Solitary fibrous tumor (SFT) is a distinct spindle cell tumor arising mainly in the pleura, however, SFT of extra-pleural sites had been reported. To our knowledge, five cases of the urinary bladder SFT had been reported. We have recently experienced a case of SFT of the urinary bladder in a 56-year- old man who was admitted to the hospital with a 4-month history of voiding difficulty, frequency, and residual urine sensation. A computerized tomography scan revealed a 12cm sized intravesical mass. The mass was marginally excised under the impression of it being a benign spindle cell tumor by pre-operative needle biopsy. On gross examination, the mass was multinodular, creamy white, and the cut surface was fibrotic with a rubbery consistency. Microscopic examination revealed haphazardly arranged spindle cells with a lace-like deposition of inter- and pericellular collagen. Areas of increased cellularity show nuclear atypia and occasional mitoses (2-3/10HPF). The immunohistochemical staining revealed an intense CD34 and bcl-2 immunoreactivity. Although the exact biologic behavior of these tumors has not been clarified, most of the reported cases have undergone a benign clinical course.

  12. Hierarchically engineered fibrous scaffolds for bone regeneration

    PubMed Central

    Sachot, Nadège; Castaño, Oscar; Mateos-Timoneda, Miguel A.; Engel, Elisabeth; Planell, Josep A.

    2013-01-01

    Surface properties of biomaterials play a major role in the governing of cell functionalities. It is well known that mechanical, chemical and nanotopographic cues, for example, influence cell proliferation and differentiation. Here, we present a novel coating protocol to produce hierarchically engineered fibrous scaffolds with tailorable surface characteristics, which mimic bone extracellular matrix. Based on the sol–gel method and a succession of surface treatments, hollow electrospun polylactic acid fibres were coated with a silicon–calcium–phosphate bioactive organic–inorganic glass. Compared with pure polymeric fibres that showed a completely smooth surface, the coated fibres exhibited a nanostructured topography and greater roughness. They also showed improved hydrophilic properties and a Young's modulus sixfold higher than non-coated ones, while remaining fully flexible and easy to handle. Rat mesenchymal stem cells cultured on these fibres showed great cellular spreading and interactions with the material. This protocol can be transferred to other structures and glasses, allowing the fabrication of various materials with well-defined features. This novel approach represents therefore a valuable improvement in the production of artificial matrices able to direct stem cell fate through physical and chemical interactions. PMID:23985738

  13. Solitary fibrous tumour of the chest wall.

    PubMed

    Mohtarrudin, N; Nor Hanipah, Z; Mohd Dusa, N

    2016-04-01

    Extrapleural solitary fibrous tumours (SFTs) are rare tumours characterized by patternless spindle cells with haemangiopericytoma-like vascular spaces. Previously the tumours have been classified as haemangiopericytoma, an entity that is now considered obsolete. We report a case of extrapleural SFT arising in the soft tissue of the chest wall. The patient was a 31-year-old Malay lady presenting with a mobile swelling of the right chest wall for more than five years. During excision the tumour was noted to be well-circumscribed and yellowish in colour, giving an impression of lipoma. Microscopically, the tumour had patternless architecture, characterized by hypocellular and hypercellular areas. It was composed of uniform, spindle-shaped cells displaying oval nuclei, inconspicuous nucleoli, pale cytoplasm and indistinct cell borders. The mitotic count was 2 per 10 HPF. Branching, medium-sized thin-walled blood vessels in a haemangiopericytomatous growth pattern, some with hyalinised wall were identified. The neoplastic cells were immunoreactive to CD99 and CD34 and were non-immunoreactive to Desmin, Smooth Muscle Actin, S100 protein and EMA. We elucidate the challenges in diagnosing this tumour in this unusual location. PMID:27126667

  14. Preconcentration of phenols by fibrous sorbents

    SciTech Connect

    Andreeva, I.Yu.; Kuvaldina, L.L.

    1995-01-01

    Phenols are among the most toxic contaminants of natural and waste waters. There are standard procedures for determining them in low concentrations. However, the samples cannot be preserved at phenol concentrations of 50 {mu}g/L or lower, and the determination of phenols must be performed no later than 2 h after sampling. This is not always possible. Because of this, the preconcentration of phenols at the site of sampling, followed by analysis of the concentrate in a stationary chemical laboratory after a time, is of interest. The technique of phenol preconcentration with active carbon, recommended in the standard procedure, is unsuitable for these purposes because the adsorption and desorption of phenols are too prolonged. At the same time, a fibrous carbon sorbent provides for a high rate of adsorption and desorption of some organic substances (humic acids, fulvic acids, and surfactants) it can be easily regenerated and repeatedly used. In this work, the authors investigated the possibility of using two fibers-namely, a carbon fiber and a polyethylene-polyamine-modified polyacrylonitrile-based fiber (PAN-PEA) containing amino groups with different numbers of substituents-for the preconcentration of phenols.

  15. Treating Fibrous Insulation to Reduce Thermal Conductivity

    NASA Technical Reports Server (NTRS)

    Zinn, Alfred; Tarkanian, Ryan

    2009-01-01

    A chemical treatment reduces the convective and radiative contributions to the effective thermal conductivity of porous fibrous thermal-insulation tile. The net effect of the treatment is to coat the surfaces of fibers with a mixture of transition-metal oxides (TMOs) without filling the pores. The TMO coats reduce the cross-sectional areas available for convection while absorbing and scattering thermal radiation in the pores, thereby rendering the tile largely opaque to thermal radiation. The treatment involves a sol-gel process: A solution containing a mixture of transition-metal-oxide-precursor salts plus a gelling agent (e.g., tetraethylorthosilicate) is partially cured, then, before it visibly gels, is used to impregnate the tile. The solution in the tile is gelled, then dried, and then the tile is fired to convert the precursor salts to the desired mixed TMO phases. The amounts of the various TMOs ultimately incorporated into the tile can be tailored via the concentrations of salts in the solution, and the impregnation depth can be tailored via the viscosity of the solution and/or the volume of the solution relative to that of the tile. The amounts of the TMOs determine the absorption and scattering spectra.

  16. Drugs Approved for Malignant Mesothelioma

    MedlinePlus

    ... Professionals Questions to Ask about Your Treatment Research Drugs Approved for Malignant Mesothelioma This page lists cancer ... in malignant mesothelioma that are not listed here. Drugs Approved for Malignant Mesothelioma Alimta (Pemetrexed Disodium) Pemetrexed ...

  17. A call to expand regulation to all carcinogenic fibrous minerals

    NASA Astrophysics Data System (ADS)

    Baumann, F.; Steele, I.; Ambrosi, J.; Carbone, M.

    2013-05-01

    The regulatory term "asbestos" groups only the six fibrous minerals that were commercially used among approximately 400. The carcinogenicity of these six regulated minerals has been largely demonstrated and is related to fiber structure, fiber length/diameter ratio, and bio-persistence. From a public perception, the generic term "asbestos" refers to the fibrous minerals that cause asbestosis, mesothelioma and other cancers. However, other non-regulated fibrous minerals are potentially as dangerous as the regulatory asbestos because they share similar physical and chemical properties, epidemiological studies have demonstrated their relationship with asbestos-related diseases, and both in vitro and in vivo experiments have established the toxicity of these minerals. For example, the non-regulated asbestiform winchite and richterite minerals that contaminated the vermiculite mined from Libby, Montana, (USA) were associated with mesothelioma, lung cancer and asbestosis observed among the area's residents and miners. Many other examples of non-regulated carcinogenic fibrous minerals include, but are not limited to, antigorite, arfvedsonite, balangeroite, carlosturanite, erionite, fluoro-edenite, hornblende, mordenite, palygorskite, and sepiolite. To propose a regulatory definition that would provide protection from all carcinogenic fibers, we have conducted an interdisciplinary literature review to compare the characteristics of "asbestos" and of non-regulated mineral fibers that relate to carcinogenicity. We specifically studied two non-regulated fibrous minerals that are associated with asbestos-related diseases: the serpentine antigorite and the zeolite erionite. Both examples underscore the problem of regulation based on commercial, rather than scientific principles: 1) the occurrence of fibrous antigorite in materials used to pave roads has been correlated with high mesothelioma rates in New Caledonia. Antigorite was also the cause of asbestosis in Poland, and in

  18. Pathophysiology and medical treatment of pain in fibrous dysplasia of bone.

    PubMed

    Chapurlat, Roland D; Gensburger, Deborah; Jimenez-Andrade, Juan M; Ghilardi, Joseph R; Kelly, Marilyn; Mantyh, Patrick

    2012-05-24

    One of the most common complications of fibrous dysplasia of bone (FD) is bone pain. Usual pain killers are often of inadequate efficacy to control this bone pain. The mechanism of bone pain in FD remains uncertain, but by analogy with bone tumors one may consider that ectopic sprouting and formation of neuroma-like structures by sensory and sympathetic nerve fibers also occur in the dysplastic skeleton. Bone pain has been reported in up to 81% of adults and 49% of children. It affects predominantly the lower limbs and the spine. The degree of pain is highly variable and adults reports more pain than children. Bisphosphonates have been shown to reduce bone pain in uncontrolled studies. Their influence on bone strength remains unknown. In a randomized trial testing alendronate, bone pain was not significantly improved. Another trial assessing the effect of risedronate is ongoing. Possible future therapies include tocilizumab, denosumab and drugs targeting nerve growth factor and its receptor TrkA.

  19. Solitary fibrous tumor of the central nervous system: report of an additional 5 cases with comprehensive literature review.

    PubMed

    Bisceglia, Michele; Dimitri, Lucia; Giannatempo, Giuseppe; Carotenuto, Vincenzo; Bianco, Mario; Monte, Vincenzo; D'Angelo, Vincenzo; Magro, Gaetano

    2011-08-01

    Solitary fibrous tumor (SFT) of the central nervous system was first described in 1996. A number of cases have been reported since. The authors present 5 new cases: 4 intracranial and 1 intraspinal. All patients were adults (age range, 47 to 75 years); 4 were male and 1 female; 4 cases were primary tumors; and 1 was a second tumor recurrence. All patients were surgically treated with gross total removal. All cases were histologically examined with immunohistochemical confirmation; 2 tumors exhibited diffuse classic histology, 1 tumor was a cellular variant, 1 tumor was myxoid, and 1 was predominantly classic with focal myxoid features and focally pleomorphic. The postoperative course was uneventful in all. The patient with the cellular variant experienced 2 local recurrences and eventually died of disease 10 years after the initial diagnosis. The patient with the myxoid variant--the tumor studied--which was the second recurrence of a previously misdiagnosed fibrous meningioma surgically treated 15 years earlier, had a recurrence after 2 years for the third time and eventually died of disease. Three patients are alive and well 11.6, 6, and 4 years after surgery. SFT is a rare tumor that needs to be differentiated from some mimickers, mainly fibrous meningioma, hemangiopericytoma, and with regard to the myxoid variant, also adult-onset myxochordoid meningioma and myxoid peripheral nerve sheath tumor. Immunohistochemistry is crucial for the correct diagnosis of SFT. The authors also performed a review of the literature and found a little more than 200 cases on record.

  20. Diagnostic value of capsule-like rim enhancement on magnetic resonance imaging for distinguishing malignant from benign parotid tumours.

    PubMed

    Sakamoto, M; Iikubo, M; Kojima, I; Sasano, T; Mugikura, S; Murata, T; Watanabe, M; Shiga, K; Ogawa, T; Takahashi, S

    2014-09-01

    The purpose of this study was to clarify the diagnostic value of capsule-like rim enhancement (CLRE) on magnetic resonance imaging (MRI) for distinguishing malignant from benign tumours of the parotid gland. We retrospectively evaluated contrast-enhanced T1-weighted images of 100 patients with malignant and benign parotid tumours for the presence, completeness, and irregularity of CLRE and its maximum thickness. We investigated any correlation of imaging and histopathological findings for 51 cases showing CLRE with available histology. The presence and completeness of CLRE did not differ significantly between benign and malignant tumours. Malignant tumours had more irregular CLRE than benign tumours (P<0.05). The mean CLRE thickness was significantly greater for malignant (2.4 mm) than benign tumours (1.4 mm) (P<0.0001). The two types of tumour were most accurately distinguished using a cut-off value of 1.5 mm thickness. Histopathology demonstrated the general correspondence of thick CLRE on MRI in malignant tumours with thick but sparse fibrous tissue and infiltration of tumour cells and lymphocytes, whereas thin CLRE in benign tumours typically represented dense fibrous tissue without infiltration of tumour cells. CLRE was more irregular and thicker in malignant tumours than in benign tumours, which may be of help in differentiating them.

  1. Parotid gland solitary fibrous tumor: a case report and clinicopathologic review of 22 cases from the literature.

    PubMed

    Bauer, Justin L; Miklos, Andrew Z; Thompson, Lester D R

    2012-03-01

    Solitary fibrous tumors (SFTs) are rare tumors in the head and neck, and even more so in the parotid gland. The mass-like clinical presentation and histologic features result in frequent misclassification, resulting in inappropriate clinical management. There are only a few reported cases in the English literature. Twenty-one patients with parotid gland solitary fibrous tumor were compiled from the English literature (Medline 1960-2011) and integrated with this case report. The patients included 11 males and 11 females, aged 11-79 years (mean, 51.2 years), who presented with a parotid gland painless mass gradually increasing in size or with compression symptoms, with a mean duration of symptoms of 24.7 months. The mean tumor size was 4.5 cm. Grossly, all tumors were described as well-circumscribed to encapsulated, firm, homogenous white to tan masses. Seven patients had a preoperative fine needle aspiration performed, with the majority interpreted to represent pleomorphic adenoma or cementifying fibroma. Histologically, the tumors were well circumscribed, although many tumors showed focally entrapped normal salivary gland acini and ducts at the edge. The tumors were cellular, arranged in haphazard short interlacing fascicles of spindled to epithelioid cells. The spindled cells showed tapering cytoplasm with monotonous, round to oval nuclei with coarse nuclear chromatin distribution. Keloid-like to wiry collagen was present between the neoplastic cells. Mitoses were identified in most cases, while necrosis was absent. Isolated, patulous vessels were present, but a well developed "hemangiopericytoma-like" vascular pattern was not seen. Three tumors were classified as malignant, showing marked nuclear pleomorphism and increased mitoses. When immunohistochemistry was performed, all tumors showed strong and diffuse vimentin, with a majority showing CD34, bcl-2 and CD99 immunoreactivity; all cases tested were negative for S100 protein, cytokeratin, EMA, CAM5

  2. Malignant thyroid tumors after iodine-131 therapy

    SciTech Connect

    Holm, L.E.; Dahlqvist, I.; Israelsson, A.; Lundell, G.

    1980-07-24

    We studied the incidence of malignant thyroid tumors after /sup 131/l therapy in 2727 patients with hyperthyroidism and in 273 euthyroid patients with cardiac disease. The patients were all adults, with a mean age of 57 years. The /sup 131/l therapy was given between 1951 and 1965. The mean follow-up period was 13 years for the hyperthyroid patients (15 years for the 85 per cent surviving for more than five years) and six years for the cardiac patients (12 years for the 41 per cent surviving for more than five years). The incidence of malignant thyroid tumors was based on a search of the Swedish Cancer Registry for the occurrence of such tumors in any of the 3000 patients. At present there is no increased incidence of malignant thyroid tumors after /sup 131/l therapy (four cases observed versus 3.2 cases expected).

  3. Diffuse reflectance spectroscopy of fibrous proteins.

    PubMed

    Millington, Keith R

    2012-09-01

    UV-visible diffuse reflectance (DR) spectra of the fibrous proteins wool and feather keratin, silk fibroin and bovine skin collagen are presented. Natural wool contains much higher levels of visible chromophores across the whole visible range (700-400 nm) than the other proteins and only those above 450 nm are effectively removed by bleaching. Both oxidative and reductive bleaching are inefficient for removing yellow chromophores (450-400 nm absorbers) from wool. The DR spectra of the four UV-absorbing amino acids tryptophan, tyrosine, cystine and phenylalanine were recorded as finely ground powders. In contrast to their UV-visible spectra in aqueous solution where tryptophan and tyrosine are the major UV absorbing species, surprisingly the disulphide chromophore of solid cystine has the strongest UV absorbance measured using the DR remission function F(R)(∞). The DR spectra of unpigmented feather and wool keratin appear to be dominated by cystine absorption near 290 nm, whereas silk fibroin appears similar to tyrosine. Because cystine has a flat reflectance spectrum in the visible region from 700 to 400 nm and the powder therefore appears white, cystine absorption does not contribute to the cream colour of wool despite the high concentration of cystine residues near the cuticle surface. The disulphide absorption of solid L: -cystine in the DR spectrum at 290 nm is significantly red shifted by ~40 nm relative to its wavelength in solution, whereas homocystine and lipoic acid showed smaller red shifts of 20 nm. The large red shift observed for cystine and the large difference in intensity of absorption in its UV-visible and DR spectra may be due to differences in the dihedral angle between the crystalline solid and the solvated molecules in solution. PMID:22218994

  4. Diffuse reflectance spectroscopy of fibrous proteins.

    PubMed

    Millington, Keith R

    2012-09-01

    UV-visible diffuse reflectance (DR) spectra of the fibrous proteins wool and feather keratin, silk fibroin and bovine skin collagen are presented. Natural wool contains much higher levels of visible chromophores across the whole visible range (700-400 nm) than the other proteins and only those above 450 nm are effectively removed by bleaching. Both oxidative and reductive bleaching are inefficient for removing yellow chromophores (450-400 nm absorbers) from wool. The DR spectra of the four UV-absorbing amino acids tryptophan, tyrosine, cystine and phenylalanine were recorded as finely ground powders. In contrast to their UV-visible spectra in aqueous solution where tryptophan and tyrosine are the major UV absorbing species, surprisingly the disulphide chromophore of solid cystine has the strongest UV absorbance measured using the DR remission function F(R)(∞). The DR spectra of unpigmented feather and wool keratin appear to be dominated by cystine absorption near 290 nm, whereas silk fibroin appears similar to tyrosine. Because cystine has a flat reflectance spectrum in the visible region from 700 to 400 nm and the powder therefore appears white, cystine absorption does not contribute to the cream colour of wool despite the high concentration of cystine residues near the cuticle surface. The disulphide absorption of solid L: -cystine in the DR spectrum at 290 nm is significantly red shifted by ~40 nm relative to its wavelength in solution, whereas homocystine and lipoic acid showed smaller red shifts of 20 nm. The large red shift observed for cystine and the large difference in intensity of absorption in its UV-visible and DR spectra may be due to differences in the dihedral angle between the crystalline solid and the solvated molecules in solution.

  5. Malignant epitheloid angiomyolipoma of the kidney in a child treated with sunitinib, everolimus and axitinib

    PubMed Central

    Citak, Elvan Caglar; Yilmaz, Eda Bengi; Yaman, Emel; Kaya, Simge; Taskinlar, Hakan; Arpaci, Rabia Bozdogan; Apaydin, Demir

    2015-01-01

    The malignant variant of epithelioid angiomyolipoma (EAML) of the kidney is uncommon, extremely aggressive and behaves like a renal cell carcinoma. We present a case of a 12-year-old male with malignant EAML who was treated according to adult treatment protocols. To our knowledge, axitinib has not been used before in children. We conclude that adult protocols, in this rare case, could be safely used in rare childhood malignancies. PMID:26279736

  6. Biphasic solitary fibrous tumour: a report of two cases with epithelioid features.

    PubMed

    Awasthi, R; O'Neill, J K; Keen, C E; Sarsfield, P T L; Devaraj, V S; Stone, C A; Smith, M E F

    2006-03-01

    We present two cases of solitary fibrous tumour (SFT) showing biphasic morphology with a spectrum of malignant epithelioid components. Slides prepared from formalin-fixed and paraffin-embedded tissue from both cases were stained with haematoxylin and eosin and by immunohistochemistry. Interphase fluorescent in situ hybridisation studies were performed in both cases using paraffin-embedded tissue to look for the t(X;18) translocation, thereby to exclude synovial sarcoma. Both cases showed biphasic morphology with some areas having typical benign spindled SFT morphology (including CD34 expression) and other areas having a malignant epithelioid appearance. In one of the cases, the epithelioid area, which was well circumscribed and showed packeting of cell groups, demonstrated expression of cytokeratin and epithelial cadherin but not of CD34. In the second case, the immunophenotype of the epithelioid component was similar to that of the benign SFT component. These findings suggest that epithelioid change in SFT shows a range of differentiation at one end, similar to that of a standard SFT, and at the other end, possibly acquiring epithelial characteristics.

  7. F-18 FDG PET/CT and Tc-99m sulfur colloid SPECT imaging in the diagnosis and treatment of a case of dual solitary fibrous tumors of the retroperitoneum and pancreas.

    PubMed

    Azadi, Javad; Subhawong, Andrea; Durand, Daniel James

    2012-03-01

    Although FDG PET is increasingly used for the staging of many types of sarcoma, little has been written regarding the FDG PET imaging characteristics of solitary fibrous tumor. We report a patient undergoing FDG PET/CT surveillance for squamous cell carcinoma of the tongue who was incidentally found to have two soft tissue masses in the retroperitoneum and pancreatic tail. Due to their low degree of FDG avidity, they were followed conservatively for approximately one year as they gradually increased in size. Technetium-99m sulfur colloid SPECT helped confirm that the pancreatic tail mass was not a splenule, after which both lesions were surgically resected and found to be extrathoracic solitary fibrous tumors without malignant features. These findings suggest that, as with other low-grade sarcomas, benign extrathoracic solitary fibrous tumors exhibit relatively little glycolytic metabolism in vivo.

  8. Panuveal malignant mesenchymoma.

    PubMed

    Pe'er, J; Neudorfer, M; Ron, N; Anteby, I; Lazar, M; Rosenmann, E

    1995-09-01

    Intraocular malignant mesenchymal tumors are very rare, and only a few case reports of such primary and metastatic tumors have been reported. We report a case of a malignant mesenchymoma involving the entire uveal tract. A 21-year-old woman presented with a tumor on the whole iris of the right eye, which caused intractable glaucoma. Upon enucleation of the eye, a very anaplastic tumor was found to occupy the whole uveal tract; its features were compatible with a tumor of mesenchymal origin, including rhabdomyosarcomatous and liposarcomatous characteristics. Choroidal osteoma was a coincidental finding. The histologic findings of the tumor were of two types of malignant mesenchymal tumors, and therefore the diagnosis of malignant mesenchymoma was made. This is to our knowledge the first tumor of its kind to be reported intraocularly. PMID:7668945

  9. The Malignant Protein Puzzle.

    PubMed

    Walker, Lary C; Jucker, Mathias

    2016-01-01

    When most people hear the words malignant and brain, cancer immediately comes to mind. But our authors argue that proteins can be malignant too, and can spread harmfully through the brain in neurodegenerative diseases that include Alzheimer's, Parkinson's, CTE, and ALS. Studying how proteins such as PrP, amyloid beta, tau, and others aggregate and spread, and kill brain cells, represents a crucial new frontier in neuroscience. PMID:27408676

  10. Oxidative stress inhibition and oxidant activity by fibrous clays.

    PubMed

    Cervini-Silva, Javiera; Nieto-Camacho, Antonio; Gómez-Vidales, Virginia

    2015-09-01

    Fibrous clays (sepiolite, palygorskite) are produced at 1.2m tonnes per year and have a wide range of industrial applications needing to replace long-fibre length asbestos. However, information on the beneficial effects of fibrous clays on health remains scarce. This paper reports on the effect of sepiolite (Vallecas, Spain) and palygorskite (Torrejón El Rubio, Spain) on cell damage via oxidative stress (determined as the progress of lipid peroxidation, LP). The extent of LP was assessed using the Thiobarbituric Acid Reactive Substances assay. The oxidant activity by fibrous clays was quantified using Electron-Paramagnetic Resonance. Sepiolite and palygorskite inhibited LP, whereby corresponding IC50 values were 6557±1024 and 4250±289μgmL(-1). As evidenced by dose-response experiments LP inhibition by palygorskite was surface-controlled. Fibrous clay surfaces did not stabilize HO species, except for suspensions containing 5000μgmL(-1). A strong oxidant (or weak anti-oxidant) activity favours the inhibition of LP by fibrous clays.

  11. Oxidative stress inhibition and oxidant activity by fibrous clays.

    PubMed

    Cervini-Silva, Javiera; Nieto-Camacho, Antonio; Gómez-Vidales, Virginia

    2015-09-01

    Fibrous clays (sepiolite, palygorskite) are produced at 1.2m tonnes per year and have a wide range of industrial applications needing to replace long-fibre length asbestos. However, information on the beneficial effects of fibrous clays on health remains scarce. This paper reports on the effect of sepiolite (Vallecas, Spain) and palygorskite (Torrejón El Rubio, Spain) on cell damage via oxidative stress (determined as the progress of lipid peroxidation, LP). The extent of LP was assessed using the Thiobarbituric Acid Reactive Substances assay. The oxidant activity by fibrous clays was quantified using Electron-Paramagnetic Resonance. Sepiolite and palygorskite inhibited LP, whereby corresponding IC50 values were 6557±1024 and 4250±289μgmL(-1). As evidenced by dose-response experiments LP inhibition by palygorskite was surface-controlled. Fibrous clay surfaces did not stabilize HO species, except for suspensions containing 5000μgmL(-1). A strong oxidant (or weak anti-oxidant) activity favours the inhibition of LP by fibrous clays. PMID:26071933

  12. Stress transfer through fibrous materials in wicking experiments

    NASA Astrophysics Data System (ADS)

    Monaenkova, Daria; Andrukh, Taras; Kornev, Konstantin

    2009-11-01

    Due to the recent progress in preparation of fibers and nanofibers with different properties, the idea of smart textiles attracts much attention. In many situations the probes and sensors are designed for bio fluid detection. The liquid penetration in fibrous materials causes their deformations including stretching, twisting, wrinkling, buckling etc. The most of researches on wicking properties of textiles are focused on determination of media permeability and ignore the specific features of fibrous materials. On the other hand the theoretical works on quantitative analysis of the deformation effects in porous materials filled with liquids are mostly focused on deformation of fully saturated samples. The fundamental understanding of the stress transfer through the fiber network is crucial for sensors development, but to the best of our knowledge, the stress analysis in the fibrous materials absorbing liquids has never been discussed in the literature. This paper sets a physical basis for analysis of absorption processes in nanotubular and nanofibrous materials. We study absorption of droplets by yarns and webs made of fibers, develop a theory which explains the stress distribution in fibrous materials and checked this theory on wicking experiments. The reported theory and experiments propose a new area of research on absorption-induced deformations of fibrous materials.

  13. Electrospun nanocomposite fibrous polymer electrolyte for secondary lithium battery applications

    SciTech Connect

    Padmaraj, O.; Rao, B. Nageswara; Jena, Paramananda; Satyanarayana, N.; Venkateswarlu, M.

    2014-04-24

    Hybrid nanocomposite [poly(vinylidene fluoride -co- hexafluoropropylene) (PVdF-co-HFP)/magnesium aluminate (MgAl{sub 2}O{sub 4})] fibrous polymer membranes were prepared by electrospinning method. The prepared pure and nanocomposite fibrous polymer electrolyte membranes were soaked into the liquid electrolyte 1M LiPF{sub 6} in EC: DEC (1:1,v/v). XRD and SEM are used to study the structural and morphological studies of nanocomposite electrospun fibrous polymer membranes. The nanocomposite fibrous polymer electrolyte membrane with 5 wt.% of MgAl{sub 2}O{sub 4} exhibits high ionic conductivity of 2.80 × 10{sup −3} S/cm at room temperature. The charge-discharge capacity of Li/LiCoO{sub 2} coin cells composed of the newly prepared nanocomposite [(16 wt.%) PVdF-co-HFP+(5 wt.%) MgAl{sub 2}O{sub 4}] fibrous polymer electrolyte membrane was also studied and compared with commercial Celgard separator.

  14. Clonal genomic alterations in glioma malignancy stages.

    PubMed

    James, C D; Carlbom, E; Dumanski, J P; Hansen, M; Nordenskjold, M; Collins, V P; Cavenee, W K

    1988-10-01

    Comparison of constitutional and tumor genotypes at chromosomal loci defined by restriction fragment length alleles has proven useful in determining the genomic position and tissue specificity of recessive mutations that predispose to cancer (Hansen, M.F., and Cavenee, W.K. Cancer Res., 47:5518-5527, 1987). Here we have applied this approach to 53 unrelated patients with glial tumors of varying histological malignancy grade. Loss of constitutional heterozygosity for loci on chromosome 10 was observed in 28 of 29 tumors histologically classified as glioblastoma (malignancy grade IV) whereas no similar losses were observed in any of 22 gliomas of lower malignancy grade. Examination of restriction fragment length alleles on other chromosomes revealed that loss of sequences on chromosomes 13, 17, or 22 had occurred at nonrandom frequencies and in at least one instance of each malignancy grade of adult glioma. The tumors in which loss of constitutional heterozygosity was observed were composed of one or a mixture of glial cell subtypes displaying astrocytic, oligodendrocytic, and/or ependymal differentiation. These results demonstrate a close association of the loss of chromosome 10 sequences with the most malignant histological stage of glioma and that glioblastoma arises as the clonal expansion of an earlier staged precursor. Furthermore they suggest that glioblastoma is a common phenotypic and malignancy terminus for glial tumors of various cellular subtypes which is reached through a common molecular pathway. This approach which involves the identification of malignancy stage specific somatic losses of heterozygosity provides a genotypic, rather than phenotypic, analysis of tumor progression.

  15. Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma

    ClinicalTrials.gov

    2016-09-12

    Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  16. LAMTOR1-PRKCD and NUMA1-SFMBT1 fusion genes identified by RNA sequencing in aneurysmal benign fibrous histiocytoma with t(3;11)(p21;q13).

    PubMed

    Panagopoulos, Ioannis; Gorunova, Ludmila; Bjerkehagen, Bodil; Lobmaier, Ingvild; Heim, Sverre

    2015-11-01

    RNA sequencing of an aneurysmal benign fibrous histiocytoma with the karyotype 46,XY,t(3;11)(p21;q13),del(6)(p23)[17]/46,XY[2] showed that the t(3;11) generated two fusion genes: LAMTOR1-PRKCD and NUMA1-SFMBT1. RT-PCR together with Sanger sequencing verified the presence of fusion transcripts from both fusion genes. In the LAMTOR1-PRKCD fusion, the part of the PRKCD gene coding for the catalytic domain of the serine/threonine kinase is under control of the LAMTOR1 promoter. In the NUMA1-SFMBT1 fusion, the part of the SFMBT1 gene coding for two of four malignant brain tumor domains and the sterile alpha motif domain is controlled by the NUMA1 promoter. The data support a neoplastic genesis of aneurysmal benign fibrous histiocytoma and indicate a pathogenetic role for LAMTOR1-PRKCD and NUMA1-SFMBT1.

  17. CD-34 and keratin expression distinguishes solitary fibrous tumor (fibrous mesothelioma) of pleura from desmoplastic mesothelioma.

    PubMed

    Flint, A; Weiss, S W

    1995-04-01

    Solitary fibrous tumors (SFTs) often involve the pleura and also may encompass the peritoneum and nonserosal sites. On occasion SFTs mimics other neoplasms, including desmoplastic mesothelioma. CD-34, initially characterized as a hematopoietic progenitor cell antigen, recently has been identified in a small number of SFTs. Based on this observation, we compared the keratin, vimentin, and CD-34 expression of 19 SFTs and eight desmoplastic mesotheliomas. Fifteen of 19 SFTs (78.9%) expressed CD-34, whereas keratin expression was absent in all SFTs. In contrast, none of the desmoplastic mesotheliomas expressed CD-34 and keratin expression was found in seven of eight (87.5%). Vimentin expression was noted in 18 of 19 SFTs and in seven of eight desmoplastic mesotheliomas. We conclude that CD-34 expression distinguishes SFT from desmoplastic mesothelioma. Additionally, the results of our study support the idea that SFT is not derived from or related to conventional mesothelium.

  18. Water-related absorption in fibrous diamonds

    NASA Astrophysics Data System (ADS)

    Zedgenizov, D. A.; Shiryaev, A. A.; Kagi, H.; Navon, O.

    2003-04-01

    Cubic and coated diamonds from several localities (Brasil, Canada, Yakutia) were investigated using spectroscopic techniques. Special emphasis was put on investigation of water-related features of transmission Infra-red and Raman spectra. Presence of molecular water is inferred from broad absorption bands in IR at 3420 and 1640 cm-1. These bands were observed in many of the investigated samples. It is likely that molecular water is present in microinclusions in liquid state, since no clear indications of solid H_2O (ice VI-VII, Kagi et al., 2000) were found. Comparison of absorption by HOH and OH vibrations shows that diamonds can be separated into two principal groups: those containing liquid water (direct proportionality of OH and HOH absorption) and those with stronger absorption by OH group. Fraction of diamonds in every group depends on their provenance. There might be positive correlation between internal pressure in microinclusions (determined using quartz barometer, Navon et al., 1988) and affiliation with diamonds containing liquid water. In many cases absorption by HOH vibration is considerably lower than absorption by hydroxyl (OH) group. This may be explained if OH groups are partially present in mineral and/or melt inclusions. This hypothesis is supported by following fact: in diamonds with strong absorption by silicates and other minerals shape and position of the OH band differs from that in diamonds with low absorption by minerals. Moreover, in Raman spectra of individual inclusions sometimes the broad band at 3100 cm-1 is observed. This band is OH-related. In some samples water distribution is not homogeneous. Central part of the diamond usually contains more water than outer parts, but this is not a general rule for all the samples. Water absorption usually correlated with absorption of other components (carbonates, silicates and others). At that fibrous diamonds with relatively high content of silicates are characterized by molecular water. OH

  19. Structural characteristics, dispersion, and modification of fibrous brucite

    NASA Astrophysics Data System (ADS)

    Cao, Xi; Chuan, Xiu-yun

    2014-01-01

    Fibrous brucite has very unique structure and physical properties. Brucite fibers were exfoliated into single nanofibers by using dioctyl sodium sulfosuccinate (AOT) as a dispersant through mechanical agitation and ultrasonic dispersion; and then, the nanofibers were modified by stearic acid and (3-aminopropyl)triethoxysilane (γ-APS) compound modification agent. The nanofibers were characterized by using X-ray diffraction (XRD), scanning electron microscopy (SEM), transmission electron microscopy (TEM), Fourier transform infrared spectroscopy (FTIR), and thermal gravimetric analysis. It is found that AOT has good effect on the dispersion. The single fiber has a consistent morphology, and fibrous brucite is dispersed and modified without destroying the crystal structure. Infrared and thermal analysis shows that the surface modification of fibrous brucite is achieved by forming chemical bonds between the coupling agent and magnesium hydroxide.

  20. Magnetoelectric effects in multiferroic fibrous composite with imperfect interface

    SciTech Connect

    Wang, X.; Pan, E.

    2007-12-01

    This paper investigates the magnetoelectroelastic responses of multiferroic fibrous composites with imperfectly bonded interface under longitudinal shear. The proposed imperfect interface model is a natural generalization of the shear lag (or the spring layer) model. By virtue of the complex variable method, we first consider the case where an isolated circular multiferroic fiber is imperfectly bonded to an infinite multiferroic matrix. Very concise expressions for the complex field potentials characterizing the magnetoelectroelastic fields inside and outside the circular fiber are obtained when the matrix is subjected to the remote uniform magnetoelectroelastic loading. The Mori-Tanaka mean-field method is then employed to derive the effective moduli of the multiferroic fibrous composite made of randomly distributed fibers reinforced to the matrix. Particularly we demonstrate that the interfacial imperfection in elasticity, electricity, and magnetism will always cause a significant reduction in the magnetoelectric coefficient of the BaTiO{sub 3}-CoFe{sub 2}O{sub 4} fibrous composite.

  1. 4D fibrous materials: characterising the deployment of paper architectures

    NASA Astrophysics Data System (ADS)

    Mulakkal, Manu C.; Seddon, Annela M.; Whittell, George; Manners, Ian; Trask, Richard S.

    2016-09-01

    Deployment of folded paper architecture using a fluid medium as the morphing stimulus presents a simple and inexpensive methodology capable of self-actuation; where the underlying principles can be translated to develop smart fibrous materials capable of programmable actuations. In this study we characterise different paper architectures and their stimuli mechanisms for folded deployment; including the influence of porosity, moisture, surfactant concentration, temperature, and hornification. We observe that actuation time decreases with paper grammage; through the addition of surfactants, and when the temperature is increased at the fluid–vapour interface. There is a clear effect of hydration, water transport and the interaction of hydrogen bonds within the fibrous architecture which drives the deployment of the folded regions. The importance of fibre volume fraction and functional fillers in shape recovery was also observed, as well as the effect of a multilayer composite paper system. The design guidelines shown here will inform the development of synthetic fibrous actuators for repeated deployment.

  2. Frontorbital Fibrous Dysplasia Resection and Reconstruction With Custom Polyetherlatone Alloplast.

    PubMed

    Nahumi, Nadav; Shohet, Michael R; Bederson, Joshua B; Elahi, Ebrahim

    2015-11-01

    Fibrous dysplasia (FD) is a benign, pathological development of bone. Craniofacial bones are the most commonly involved and can potentially cause visual disturbance, proptosis, orbital dystopia, and facial deformity. This case involves a 13-year-old girl with significant proptosis (20 mm left, 17.5 mm right) and downward displacement of the left globe (1.5 mm) due to fibrous dysplasia. Reconstruction was performed with computed tomography-derived and 3D printed custom polyetheretherketone (PEEK) implantation. PEEK is a nonabsorbable, nonporous thermoplastic polymer notable for its ability to be modified intraoperatively and ideal imaging properties postoperatively. Never, to our knowledge, has PEEK been used for primary reconstruction of the frontal orbital region in fibrous dysplasia in a child. The lesion was successfully repaired with excellent aesthetic and no apparent damage to neurovascular or ocular structures. PMID:26594985

  3. Fibrous minerals from Somma-Vesuvius volcanic complex

    NASA Astrophysics Data System (ADS)

    Rossi, Manuela; Nestola, Fabrizio; Ghiara, Maria R.; Capitelli, Francesco

    2016-08-01

    A survey on fibrous minerals coming from the densely populated area of Campania around the Somma-Vesuvius volcanic complex (Italy) was performed by means of a multi-methodological approach, based on morphological analyses, EMPA/WDS and SEM/EDS applications, and unit-cell determination through X-ray diffraction data. Such mineralogical investigation aims to provide suitable tools to the identification of fibrous natural phases, to improve the knowledge of both geochemical, petrogenetic and regional mineralogy of Somma-Vesuvius area, and to emphasize the presence of minerals with fibrous habit in all volcanic environments. The survey also fits well in the calls of health and environment of Horizon 2020 program of the European Commission (Climate Action, Environment, Resource Efficiency and Raw Materials).

  4. 4D fibrous materials: characterising the deployment of paper architectures

    NASA Astrophysics Data System (ADS)

    Mulakkal, Manu C.; Seddon, Annela M.; Whittell, George; Manners, Ian; Trask, Richard S.

    2016-09-01

    Deployment of folded paper architecture using a fluid medium as the morphing stimulus presents a simple and inexpensive methodology capable of self-actuation; where the underlying principles can be translated to develop smart fibrous materials capable of programmable actuations. In this study we characterise different paper architectures and their stimuli mechanisms for folded deployment; including the influence of porosity, moisture, surfactant concentration, temperature, and hornification. We observe that actuation time decreases with paper grammage; through the addition of surfactants, and when the temperature is increased at the fluid-vapour interface. There is a clear effect of hydration, water transport and the interaction of hydrogen bonds within the fibrous architecture which drives the deployment of the folded regions. The importance of fibre volume fraction and functional fillers in shape recovery was also observed, as well as the effect of a multilayer composite paper system. The design guidelines shown here will inform the development of synthetic fibrous actuators for repeated deployment.

  5. Heat Transfer in High-Temperature Fibrous Insulation

    NASA Technical Reports Server (NTRS)

    Daryabeigi, Kamran

    2002-01-01

    The combined radiation/conduction heat transfer in high-porosity, high-temperature fibrous insulations was investigated experimentally and numerically. The effective thermal conductivity of fibrous insulation samples was measured over the temperature range of 300-1300 K and environmental pressure range of 1.33 x 10(exp -5)-101.32 kPa. The fibrous insulation samples tested had nominal densities of 24, 48, and 72 kilograms per cubic meter and thicknesses of 13.3, 26.6 and 39.9 millimeters. Seven samples were tested such that the applied heat flux vector was aligned with local gravity vector to eliminate natural convection as a mode of heat transfer. Two samples were tested with reverse orientation to investigate natural convection effects. It was determined that for the fibrous insulation densities and thicknesses investigated no heat transfer takes place through natural convection. A finite volume numerical model was developed to solve the governing combined radiation and conduction heat transfer equations. Various methods of modeling the gas/solid conduction interaction in fibrous insulations were investigated. The radiation heat transfer was modeled using the modified two-flux approximation assuming anisotropic scattering and gray medium. A genetic-algorithm based parameter estimation technique was utilized with this model to determine the relevant radiative properties of the fibrous insulation over the temperature range of 300-1300 K. The parameter estimation was performed by least square minimization of the difference between measured and predicted values of effective thermal conductivity at a density of 24 kilograms per cubic meters and at nominal pressures of 1.33 x 10(exp -4) and 99.98 kPa. The numerical model was validated by comparison with steady-state effective thermal conductivity measurements at other densities and pressures. The numerical model was also validated by comparison with a transient thermal test simulating reentry aerodynamic heating

  6. [Fibrous dysplasia of bone in a 12-year old girl].

    PubMed

    Bieniasz, Jolanta; Maj, Anna; Noczyńska, Anna

    2006-01-01

    Fibrous dysplasia of bone is a rare congenital bone disease, usually coming out before the age of 30. It is 2.5% of all bone tumours and 7.5 % of benign tumours of bones. The authors present a case of a 12-years old girl with fibrous dysplasia of bone admitted to the Department with hyperparathyroidism suspicion. Pathological changes were localized in the orbital cavity and sinuses. The diagnosis was established on the basis of the clinical course, imaging and histopathological examinations. The therapy with pamidronian acid was applied.

  7. Solitary Fibrous Tumor in a Female Urinary Bladder.

    PubMed

    Mustafa, Hiba J; Menon, Sharifa

    2016-07-01

    Solitary fibrous tumors of the bladder are extremely rare especially in the females. This is the third case of SFT in a female urinary bladder. We are presenting a case of 36-years-old female who underwent a procedure for what thought to be cervical myoma. Cystectomy and bilateral ureteral re-implantation were performed. Mass showed to be solitary fibrous tumor of the urinary bladder. These tumors are rarely occur in extrapleural spaces. Only few cases had been reported in the urinary bladder including 2 in the female and 10 in the male bladder.

  8. Solitary Fibrous Tumor in a Female Urinary Bladder.

    PubMed

    Mustafa, Hiba J; Menon, Sharifa

    2016-07-01

    Solitary fibrous tumors of the bladder are extremely rare especially in the females. This is the third case of SFT in a female urinary bladder. We are presenting a case of 36-years-old female who underwent a procedure for what thought to be cervical myoma. Cystectomy and bilateral ureteral re-implantation were performed. Mass showed to be solitary fibrous tumor of the urinary bladder. These tumors are rarely occur in extrapleural spaces. Only few cases had been reported in the urinary bladder including 2 in the female and 10 in the male bladder. PMID:27335777

  9. Solitary fibrous tumor of maxilla: A rare entity

    PubMed Central

    Heera, R; Chandran, M Renu; Padmakumar, S K; Rajeev, R

    2016-01-01

    Solitary fibrous tumor (SFT) is a ubiquitous rare mesenchymal neoplasm. Pleura is the favored site of origin and is rare in the oral cavity. SFT occurs across a wide histopathologic spectrum. Fibrous form characterized by hyalinized, thick-walled vessels with opened lumina and strong CD34 reactivity constitute one end and on the other end, a cellular form representing the conventional hemangiopericytoma, with branched, thin-walled vessels and focal or negative CD34 reactivity characterize the spectrum. A case of oral SFT in a 30-year-old female patient with its clinical, histopathological and immunohistochemical features is being presented here. PMID:27721623

  10. Interfacial Micromechanics in Fibrous Composites: Design, Evaluation, and Models

    PubMed Central

    Lei, Zhenkun; Li, Xuan; Qin, Fuyong; Qiu, Wei

    2014-01-01

    Recent advances of interfacial micromechanics in fiber reinforced composites using micro-Raman spectroscopy are given. The faced mechanical problems for interface design in fibrous composites are elaborated from three optimization ways: material, interface, and computation. Some reasons are depicted that the interfacial evaluation methods are difficult to guarantee the integrity, repeatability, and consistency. Micro-Raman study on the fiber interface failure behavior and the main interface mechanical problems in fibrous composites are summarized, including interfacial stress transfer, strength criterion of interface debonding and failure, fiber bridging, frictional slip, slip transition, and friction reloading. The theoretical models of above interface mechanical problems are given. PMID:24977189

  11. Kinetics of adsorption with granular, powdered, and fibrous activated carbon

    SciTech Connect

    Shmidt, J.L.; Pimenov, A.V.; Lieberman, A.I.; Cheh, H.Y.

    1997-08-01

    The properties of three different types of activated carbon, fibrous, powdered, and granular, were investigated theoretically and experimentally. The adsorption rate of the activated carbon fiber was found to be two orders of magnitude higher than that of the granular activated carbon, and one order of magnitude higher than that of the powdered activated carbon. Diffusion coefficients of methylene blue in the fibrous, powdered, and granular activated carbons were determined experimentally. A new method for estimating the meso- and macropore surface areas in these carbons was proposed.

  12. Interfacial micromechanics in fibrous composites: design, evaluation, and models.

    PubMed

    Lei, Zhenkun; Li, Xuan; Qin, Fuyong; Qiu, Wei

    2014-01-01

    Recent advances of interfacial micromechanics in fiber reinforced composites using micro-Raman spectroscopy are given. The faced mechanical problems for interface design in fibrous composites are elaborated from three optimization ways: material, interface, and computation. Some reasons are depicted that the interfacial evaluation methods are difficult to guarantee the integrity, repeatability, and consistency. Micro-Raman study on the fiber interface failure behavior and the main interface mechanical problems in fibrous composites are summarized, including interfacial stress transfer, strength criterion of interface debonding and failure, fiber bridging, frictional slip, slip transition, and friction reloading. The theoretical models of above interface mechanical problems are given. PMID:24977189

  13. Multifocal peritoneal calcifying fibrous tumour: incidental finding at cholecystectomy

    PubMed Central

    Gatt, Noel; Falzon, Sharon; Ratynska, Marzena

    2011-01-01

    Calcifying fibrous tumour (CFT) is a benign tumour of elusive aetiology and a potential for local recurrence. Despite its peculiar histological characteristics it can still be confused with interrelated differential diagnosis like inflammatory myofibroblastic tumour (IMT) or solitary fibrous tumours. The clinical differential diagnosis is however much wider. To date seven cases of multiple peritoneal CFTs are on record. The authors present a case discovered incidentally during laparoscopic cholecystectomy, with no previous history and no radiological diagnosis achieved despite having undergone magnetic resonance cholangiopancreatography (MRCP) and normal routine perioperative investigation. The patient is disease-free 12 months after diagnosis. The case report is followed by a detailed literature review. PMID:22689663

  14. Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics.

    PubMed

    Doyle, Leona A; Vivero, Marina; Fletcher, Christopher Dm; Mertens, Fredrik; Hornick, Jason L

    2014-03-01

    Solitary fibrous tumor (SFT) is composed of spindled to ovoid cells in a patternless architecture with prominent stromal collagen and hemangiopericytoma-like vessels. Some tumors show hypercellularity, nuclear atypia, and significant mitotic activity; the latter feature in particular often portends an aggressive clinical course. SFT can sometimes be difficult to distinguish from other benign mesenchymal tumors and sarcomas. The most characteristic (albeit nonspecific) immunohistochemical finding in SFT is CD34 expression. A NAB2-STAT6 gene fusion, resulting in a chimeric protein in which a repressor domain of NGFI-A binding protein 2 (EGR1 binding protein 2) (NAB2) is replaced with a carboxy-terminal transactivation domain from signal transducer and activator of transcription 6, interleukin-4 induced (STAT6), was recently identified as a consistent finding in SFT. However, as these genes are located in close proximity on 12q13, this fusion can only rarely be detected by conventional chromosomal banding or fluorescence in situ hybridization analysis. Nuclear expression of the carboxy terminal part of STAT6 is a consistent finding in SFT of the meninges (so-called 'meningeal hemangiopericytoma'). We investigated STAT6 expression by immunohistochemistry in SFTs and other soft tissue tumors arising outside the central nervous system to validate the diagnostic utility of this novel marker. Whole-tissue sections of 231 tumors were evaluated, including 60 cases of SFT as well as other benign and malignant mesenchymal neoplasms and sarcomatoid mesotheliomas. Fifty-nine of 60 SFT cases (98%) showed nuclear expression of STAT6, which was usually diffuse and intense. All other tumor types were negative for STAT6, except for three dedifferentiated liposarcomas and one deep fibrous histiocytoma, which showed weak staining. In conclusion, STAT6 is a highly sensitive and almost perfectly specific immunohistochemical marker for SFT and can be helpful to distinguish this tumor type

  15. Primary Malignant Tumours of Bone Following Previous Malignancy

    PubMed Central

    Patton, J. T.; Sommerville, S. M. M.; Grimer, R. J.

    2008-01-01

    Destructive bone lesions occurring in patients who have previously had a malignancy are generally assumed to be a metastasis from that malignancy. We reviewed 60 patients with a previous history of malignancy, who presented with a solitary bone lesion that was subsequently found to be a new and different primary sarcoma of bone. These second malignancies occurred in three distinct groups of patients: (1) patients with original tumours well known to be associated with second malignancies (5%); (2) patients whose second malignancies were likely to be due to the previous treatment of their primary malignancy (40%); (3) patients in whom there was no clearly defined association between malignancies (55%). The purpose of this study is to emphasise the necessity for caution in assuming the diagnosis of a metastasis when a solitary bone lesion is identified following a prior malignancy. Inappropriate biopsy and treatment of primary bone sarcomas compromises limb salvage surgery and can affect patient mortality. PMID:18414590

  16. Positive gallium-67 citrate uptake in a patient with polyostotic fibrous dysplasia

    SciTech Connect

    Creagh, M.F.; Nunan, T.O.

    1988-04-01

    Fibrous dysplasia is an uncommon bone condition with characteristic radiologic features. It is well known that there is increased uptake of Tc-99m hydroxymethylene diphosphonate (HMDP) and methylene diphosphonate (MDP) in fibrous dysplasia. There are no reports of uptake of Ga-67 citrate by fibrous dysplasia. A case is reported in which positive Ga-67 uptake was seen in a patient with polyostotic fibrous dysplasia.

  17. Hemostasis and malignancy.

    PubMed

    Francis, J L; Biggerstaff, J; Amirkhosravi, A

    1998-01-01

    There is considerable evidence that the hemostatic system is involved in the growth and spread of malignant disease. There is an increased incidence of thromboembolic disease in patients with cancers and hemostatic abnormalities are extremely common in such patients. Antihemostatic agents have been successfully used to treat a variety of experimental tumors, and several clinical trials in humans have been initiated. Although metastasis is undoubtedly multifactorial, intravascular coagulation activation and peritumor fibrin deposition seem to be important. The mechanisms by which hemostatic activation facilitates the malignant process remain to be completely elucidated. Of central importance may be the presence on malignant cells of tissue factor and urokinase receptor. Recent studies have suggested that these proteins, and others, may be involved at several stages of metastasis, including the key event of neovascularization. Tissue factor, the principal initiator of coagulation, may have additional roles, outside of fibrin formation, that are central to the biology of some solid tumors.

  18. Hemostasis and malignancy.

    PubMed

    Francis, J L; Biggerstaff, J; Amirkhosravi, A

    1998-01-01

    There is considerable evidence that the hemostatic system is involved in the growth and spread of malignant disease. There is an increased incidence of thromboembolic disease in patients with cancers and hemostatic abnormalities are extremely common in such patients. Antihemostatic agents have been successfully used to treat a variety of experimental tumors, and several clinical trials in humans have been initiated. Although metastasis is undoubtedly multifactorial, intravascular coagulation activation and peritumor fibrin deposition seem to be important. The mechanisms by which hemostatic activation facilitates the malignant process remain to be completely elucidated. Of central importance may be the presence on malignant cells of tissue factor and urokinase receptor. Recent studies have suggested that these proteins, and others, may be involved at several stages of metastasis, including the key event of neovascularization. Tissue factor, the principal initiator of coagulation, may have additional roles, outside of fibrin formation, that are central to the biology of some solid tumors. PMID:9579631

  19. Malignancy-Associated Dyslipidemia

    PubMed Central

    Bielecka-Dąbrowa, Agata; Hannam, Simon; Rysz, Jacek; Banach, Maciej

    2011-01-01

    Cholesterol and triglycerides, important lipid constituents of cell, are essential to carry out several vital physiological functions. Lipids might be associated with cancers because they play a key role in the maintenance of cell integrity. The pathway for cholesterol synthesis may also produce various tumorigenic compounds and cholesterol serves as a precursor for the synthesis of many sex hormones linked to increased risk of various cancers. In some malignant diseases, blood cholesterol undergoes early and significant changes. The mechanism for the link between cancer and cholesterol remains controversial. The dates from studies are confusing because both hypolipidemia and hypercholesterolemia might be connected with malignancy. Not only cancers but also antineoplastic therapies have an influence on lipid profile. There are also dates suggesting that antihyperlipemic drugs might nfluenced malignancy. PMID:21660223

  20. Surveillance for gastrointestinal malignancies

    PubMed Central

    Tiwari, Ashish K; Laird-Fick, Heather S; Wali, Ramesh K; Roy, Hemant K

    2012-01-01

    Gastrointestinal (GI) malignancies are notorious for frequently progressing to advanced stages even in the absence of serious symptoms, thus leading to delayed diagnoses and dismal prognoses. Secondary prevention of GI malignancies through early detection and treatment of cancer-precursor/premalignant lesions, therefore, is recognized as an effective cancer prevention strategy. In order to efficiently detect these lesions, systemic application of screening tests (surveillance) is needed. However, most of the currently used non-invasive screening tests for GI malignancies (for example, serum markers such as alpha-fetoprotein for hepatocellular carcinoma, and fecal occult blood test, for colon cancer) are only modestly effective necessitating the use of highly invasive endoscopy-based procedures, such as esophagogastroduodenoscopy and colonoscopy for screening purposes. Even for hepatocellular carcinoma where non-invasive imaging (ultrasonography) has become a standard screening tool, the need for repeated liver biopsies of suspicious liver nodules for histopathological confirmation can’t be avoided. The invasive nature and high-cost associated with these screening tools hinders implementation of GI cancer screening programs. Moreover, only a small fraction of general population is truly predisposed to developing GI malignancies, and indeed needs surveillance. To spare the average-risk individuals from superfluous invasive procedures and achieve an economically viable model of cancer prevention, it’s important to identify cohorts in general population that are at substantially high risk of developing GI malignancies (risk-stratification), and select suitable screening tests for surveillance in these cohorts. We herein provide a brief overview of such high-risk cohorts for different GI malignancies, and the screening strategies that have commonly been employed for surveillance purpose in them. PMID:22969223

  1. Solitary Fibrous Tumor of the Liver: A Rare Tumor in a Rarer Location

    PubMed Central

    Dey, Biswajit; Gochhait, Debasis; Kaushal, Gourav; Barwad, Adarsh; Pottakkat, Biju

    2016-01-01

    Solitary fibrous tumor is an uncommon mesenchymal neoplasm. Liver is a rare location of this tumor. We report a case of hepatic solitary fibrous tumor in a 56-year-old female, who presented with right upper abdominal pain. An extended right hepatectomy was performed. Histopathological and immunohistochemical examination revealed solitary fibrous tumor of the liver. PMID:27746883

  2. Novel immunotherapies in lymphoid malignancies

    PubMed Central

    Batlevi, Connie Lee; Matsuki, Eri; Brentjens, Renier J.; Younes, Anas

    2016-01-01

    The success of the anti-CD20 monoclonal antibody rituximab in the treatment of lymphoid malignancies provided proof-of-principle for exploiting the immune system therapeutically. Since the FDA approval of rituximab in 1997, several novel strategies that harness the ability of T cells to target cancer cells have emerged. Reflecting on the promising clinical efficacy of these novel immunotherapy approaches, the FDA has recently granted ‘breakthrough’ designation to three novel treatments with distinct mechanisms. First, chimeric antigen receptor (CAR)-T-cell therapy is promising for the treatment of adult and paediatric relapsed and/or refractory acute lymphoblastic leukaemia (ALL). Second, blinatumomab, a bispecific T-cell engager (BiTE®) antibody, is now approved for the treatment of adults with Philadelphia-chromosome-negative relapsed and/or refractory B-precursor ALL. Finally, the monoclonal antibody nivolumab, which targets the PD-1 immune-checkpoint receptor with high affinity, is used for the treatment of Hodgkin lymphoma following treatment failure with autologous-stem-cell transplantation and brentuximab vedotin. Herein, we review the background and development of these three distinct immunotherapy platforms, address the scientific advances in understanding the mechanism of action of each therapy, and assess the current clinical knowledge of their efficacy and safety. We also discuss future strategies to improve these immunotherapies through enhanced engineering, biomarker selection, and mechanism-based combination regimens. PMID:26525683

  3. Transarterial Onyx Embolization of an Orbital Solitary Fibrous Tumor.

    PubMed

    Hashemi, Nafiseh; Ling, Jeanie D; Soparkar, Charles; Sami, Mirwat; Ellezam, Benjamin; Klucznik, Richard; Lee, Andrew G; Chévez-Barrios, Patricia

    2015-02-01

    Solitary fibrous tumor (SFT) is an uncommon mesenchymal neoplasm sometimes found in the orbit. We report a case of an aggressive orbital SFT with enlarged feeding vessels that was successfully resected immediately after transarterial embolization with Onyx (ethylene vinyl alcohol copolymer). To our knowledge, this is the first report showing the histopathology of Onyx embolization material in an orbital SFT.

  4. Disseminated solitary fibrous tumour of the lung and pleura.

    PubMed

    Singh, Ranjit Kumar; Thangakunam, Balamugesh; Isaac, Barney Thomas Jesudason; Gupta, Ashumi

    2013-01-01

    Solitary fibrous tumours (SFTs) are a heterogeneous group of rare spindle-cell tumours. Classically they presented as a solitary pleural-based mass. Pulmonary parenchymal SFT is rare and multiple bilateral lesions are extremely rare. We present the clinical, imaging and histological features of SFT which are presented as multiple nodular lesions of the lung and pleura with probable distant metastasis.

  5. [Extra-thoracic solitary fibrous tumor. Report of 2 cases].

    PubMed

    Trabelsi, Amel; Mestiri, Sarra; Mokni, Moncef; Stita, Wided; Ikram, Bellara; Sriha, Badreddine; Korbi, Sadok

    2006-09-01

    The solitary fibrous tumor (SFT), is an unusual entity, first described in the pleura, but can involve other serosal surfaces and viscera. We report two cases of extra-thoracic SFT involving the retro-peritoneum and the upper arm. Extra-thoracic TFS is a rarily wide morphologic and evolutive spectrum.

  6. Thermal protection studies of plastic films and fibrous materials

    NASA Technical Reports Server (NTRS)

    Saad, Michel A.; Altman, Robert L.

    1988-01-01

    The thermal protection properties of various film and woven materials were studied using an experimental method of radiant heating. The materials studied included aluminized and unaluminized synthetic plastic films and fibrous materials like silicon carbide and phenolic novolac. It is shown that a thin metallized coating with good reflectivity significantly enhances the heat blocking capability of a variety of insulative materials.

  7. Use of Zoledronic Acid in Paediatric Craniofacial Fibrous Dysplasia

    PubMed Central

    Rossin, Sara; Divisic, Antuan; De Gregorio, Alesandra; Agosto, Caterina; Catalano, Igor; Mazza, Alessandro; Sartori, Leonardo; Benini, Franca

    2016-01-01

    We describe a case of a paediatric patient affected by mandibular fibrous dysplasia (FD) with severe and chronic pain who was successfully treated with zoledronic acid (ZOL): a third-generation bisphosphonate. Further research is needed to assess its safety and efficacy as a treatment option for FD in the paediatric population. PMID:27747122

  8. Fibrous dysplasia-like tumor of the lacrimal sac.

    PubMed

    Scott, Garrett R; Frueh, Bartley R; Flint, Andrew; Elner, Victor M

    2008-01-01

    A 75-year-old woman developed epiphora and a slowly enlarging right medial canthal mass for 1 year. CT revealed a mass with ground-glass radiodensity and hazy borders in the lacrimal sac. At external dacryocystorhinostomy, a tan, gritty, spherical mass was easily removed from the sac lumen. Histopathologic characteristics were typical of fibrous dysplasia. The postoperative course was uneventful.

  9. A Solitary Fibrous Tumor (Cellular Form) of the Ankle.

    PubMed

    Lee, Jun Yong; Kim, Dong-Hwi; Seo, Kyung-Jin; Jung, Sung-No

    2016-01-01

    A solitary fibrous tumor (SFT) is a rare type of mesenchymal tumor composed of uniform spindle cells that is classically described as a patternless feature. SFT normally originates from the pleura, with an SFT originating from skin rarely reported. We report what we believe to be the first case of an SFT arising from the ankle. Our case was confirmed histopathologically with immunohistochemical staining.

  10. Torsion of omental fibrous pseudotumour mimicking acute appendicitis

    PubMed Central

    Pennington, Thomas E.; Ozmen, John; Fenton-Lee, Douglas

    2016-01-01

    Unusual pathologies are occasionally found at laparoscopy when appendicitis is suspected. We present a case of strangulated inflammatory fibrous pseudotumour of the omentum presenting in a similar fashion to appendicitis. The infarcted omentum was excised, facilitating prompt resolution of symptoms. PMID:26811304

  11. Physical properties of collagen fibrous networks derived from bovine hides

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The hides and leather industry has been facing a serious challenge in the disposal of solid wastes such as trimmings and lime-splits. One strategy to solve this problem is to convert these wastes into useful fibrous products and green composites. Therefore research is needed to investigate the pre...

  12. Fibrous trabeculae in the liver of alligator (alligator mississippiensis).

    PubMed

    Beresford, W A

    1993-08-01

    In the mature alligator, fibrous trabeculae run from the portal areas and capsule through the hepatic parenchyma. The extent of these trabeculae becomes clear only after staining for collagen with, for example, Fast green or Picrosirius red. The trabeculae are less well developed in young caiman. The alligator's liver might use the trabeculae to withstand thrashing of the body.

  13. Electrospinning of caseinates to create protective fibrous mats

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Electrospinning is a nonthermal process that produces fibers on the micron- or nano-scale from a polymer solution. If produced by electrospinning of biopolymer solutions, fibrous mats may be created for protecting foods and allowing for the preservation and controlled release of bioactives for healt...

  14. Electrospinning of caseinates to create protective fibrous mats

    Technology Transfer Automated Retrieval System (TEKTRAN)

    JUSTIFICATION Electrospinning is a nonthermal process that produces fibers with diameters on the micron- or nano-scales from a polymer solution. If produced by electrospinning of biopolymer solutions, fibrous mats may be created for protecting foods, improving food quality and allowing for the prese...

  15. An improved automotive brake lining using fibrous potassium titanate

    NASA Technical Reports Server (NTRS)

    Mansfield, J. A.; Halberstadt, M. L.; Riccitiello, S. R.; Rhee, S. K.

    1976-01-01

    Simultaneous fade reduction and wear improvement of a commercial automotive brake lining were achieved by adding fibrous potassium titanate. The dependence of friction and wear characteristics on quantitative variations in potassium titanate, asbestos, phenolic binder, and organic and inorganic modifiers was evaluated.

  16. Deposition of fibrous nanostructure by ultrafast laser ablation

    NASA Astrophysics Data System (ADS)

    Tavangar, Amirhossein; Tan, Bo; Venkatakrishnan, K.

    2010-05-01

    This research work demonstrated that laser-induced reverse transfer (LIRT) can be used for controllable site-specific deposition of fibrous nanostructure. The LIRT method makes it possible to generate and deposit the fibrous nanostructure of a wide variety of materials on a transparent acceptor in a single-step process at an ambient condition. The deposition of fibrous nanostructures was conducted using ultrafast laser ablation of silicon and aluminum targets placed behind a glass acceptor. Femtosecond laser pulses pass through the transparent acceptor and hit the bulk donor. Consequently a mass quantity of nanoparticles ablates from the donor and then aggregates and forms a porous fibrous nanostructure on the transparent acceptor. Our experiments demonstrated that the gap between the target and the glass acceptor was critical in the formation and accumulation of nanofibers and it determines the density of the formed nanostructure. The formation mechanism of the nanostructures can be explained by the well-established theory of vapor condensation within the plume induced by ultrafast laser ablation. Experimental results also show that the length of the nanostructure can be controlled by the gap between the target and glass acceptor. Lastly, energy-dispersive x-ray spectroscopy (EDS) analysis shows the oxygen concentration in the nanofibrous structure which is associated with oxidation of ablated material at ambient atmosphere.

  17. Malignant pleural mesothelioma: a clinicopathological study.

    PubMed

    Qua, J C; Rao, U N; Takita, H

    1993-09-01

    In this paper the results of a retrospective review of 58 patients with malignant pleural mesothelioma treated at our Institute are reported. There were 50 males and 8 females; the mean age was 56.3 years (range: 13-77). History of asbestos exposure was ascertained in 25 patients (43%). The most common finding in chest X-ray was pleural effusion which was seen in 47/58 patients on presentation. The cytological examination of pleural effusion was most of the time nondiagnostic. Pleural biopsy was needed for the correct diagnosis. Pathologically, 26 patients (44.8%) had epithelial type, 24 patients (41.4%) had mixed type, and 8 patients (13.8%) had fibrous or sarcomatous type of pleural mesothelioma. Most of the patients on presentation had Stage I disease by Butchart's classification. The overall survival time ranged from 1 month to as long as 17 years with a median of 12.5 months. The mean survival of patients who received nonsurgical therapies was 7-13.4 months. Thirteen patients were treated surgically: three patients survived over 5 years, but the median survival was 15 months. Six patients received no treatment, and the median survival was seven months.

  18. Direct Numerical Simulation of Liquid Transport Through Fibrous Porous Media

    NASA Astrophysics Data System (ADS)

    Palakurthi, Nikhil Kumar

    Fluid flow through fibrous media occurs in many industrial processes, including, but not limited, to fuel cell technology, drug delivery patches, sanitary products, textile reinforcement, filtration, heat exchangers, and performance fabrics. Understanding the physical processes involved in fluid flow through fibrous media is essential for their characterization as well as for the optimization and development of new products. Macroscopic porous-media equations require constitutive relations, which account for the physical processes occurring at the micro-scale, to predict liquid transport at the macro-scale. In this study, micro-scale simulations were conducted using conventional computational fluid dynamics (CFD) technique (finite-volume method) to determine the macroscopic constitutive relations. The first part of this thesis deals with the single-phase flow in fibrous media, following which multi-phase flow through fibrous media was studied. Darcy permeability is an important parameter that characterizes creeping flow through a fibrous porous medium. It has a complex dependence on the medium's properties such as fibers' in-plane and through-plane orientation, diameter, aspect ratio, curvature, and porosity. A suite of 3D virtual fibrous structures with a wide range of geometric properties were constructed, and the permeability values of the structures were calculated by solving the 3D incompressible Navier-Stokes equations. The through-plane permeability was found to be a function of only the fiber diameter, the fibers' through-plane orientation, and the porosity of the medium. The numerical results were used to extend a permeability-porosity relation, developed in literature for 3D isotropic fibrous media, to a wide range of fibers' through-plane orientations. In applications where rate of capillary penetration is important, characterization of porous media usually involves determination of either the effective pore radius from capillary penetration experiments

  19. "Malignant Cutaneous Ulcer".

    PubMed

    Sundriyal, Deepak; Kotwal, Sumedha

    2016-09-01

    Renal cell carcinoma (RCC) is an aggressive malignancy and the rich vascular supply enables it to metastasize early via haematogenous route. Skin lesions are a late manifestation of the disease. Clinicians should be aware of cutaneous presentation of RCC while evaluating a case of unknown primary with skin lesions. PMID:27651705

  20. Immunotherapy for malignant glioma.

    PubMed

    Suryadevara, Carter M; Verla, Terence; Sanchez-Perez, Luis; Reap, Elizabeth A; Choi, Bryan D; Fecci, Peter E; Sampson, John H

    2015-01-01

    Malignant gliomas (MG) are the most common type of primary malignant brain tumor. Most patients diagnosed with glioblastoma (GBM), the most common and malignant glial tumor, die within 12-15 months. Moreover, conventional treatment, which includes surgery followed by radiation and chemotherapy, can be highly toxic by causing nonspecific damage to healthy brain and other tissues. The shortcomings of standard-of-care have thus created a stimulus for the development of novel therapies that can target central nervous system (CNS)-based tumors specifically and efficiently, while minimizing off-target collateral damage to normal brain. Immunotherapy represents an investigational avenue with the promise of meeting this need, already having demonstrated its potential against B-cell malignancy and solid tumors in clinical trials. T-cell engineering with tumor-specific chimeric antigen receptors (CARs) is one proven approach that aims to redirect autologous patient T-cells to sites of tumor. This platform has evolved dramatically over the past two decades to include an improved construct design, and these modern CARs have only recently been translated into the clinic for brain tumors. We review here emerging immunotherapeutic platforms for the treatment of MG, focusing on the development and application of a CAR-based strategy against GBM.

  1. Massive chest wall resection and reconstruction for malignant disease

    PubMed Central

    Foroulis, Christophoros N; Kleontas, Athanassios D; Tagarakis, George; Nana, Chryssoula; Alexiou, Ioannis; Grosomanidis, Vasilis; Tossios, Paschalis; Papadaki, Elena; Kioumis, Ioannis; Baka, Sofia; Zarogoulidis, Paul; Anastasiadis, Kyriakos

    2016-01-01

    Objective Malignant chest wall tumors are rare neoplasms. Resection with wide-free margins is an important prognostic factor, and massive chest wall resection and reconstruction are often necessary. A recent case series of 20 consecutive patients is reported in order to find any possible correlation between tumor histology, extent of resection, type of reconstruction, and adjuvant treatment with short- and long-term outcomes. Methods Twenty patients were submitted to chest wall resection and reconstruction for malignant chest wall neoplasms between 2006 and 2014. The mean age (ten males) was 59±4 years. The size and histology of the tumor, the technique of reconstruction, and the short- and long-term follow-up records were noted. Results The median maximum diameter of tumors was 10 cm (5.4–32 cm). Subtotal sternal resection was performed in nine cases, and the resection of multiple ribs was performed in eleven cases. The median area of chest wall defect was 108 cm2 (60–340 cm2). Histology revealed soft tissue, bone, and cartilage sarcomas in 16 cases (80%), most of them chondrosarcomas. The rest of the tumors was metastatic tumors in two cases and localized malignant pleural mesothelioma and non-Hodgkin lymphoma in one case. The chest wall defect was reconstructed by using the “sandwich technique” (propylene mesh/methyl methacrylate/propylene mesh) in nine cases of large anterior defects or by using a 2 mm polytetrafluoroethylene (e-PTFE) mesh in nine cases of lateral or posterior defects. Support from a plastic surgeon was necessary to cover the full-thickness chest wall defects in seven cases. Adjuvant oncologic treatment was administered in 13 patients. Local recurrences were observed in five cases where surgical reintervention was finally necessary in two cases. Recurrences were associated with larger tumors, histology of malignant fibrous histiocytoma, and initial incomplete resection or misdiagnosis made by nonthoracic surgeons. Three patients died

  2. Endothelial progenitor cells in hematologic malignancies

    PubMed Central

    Saulle, Ernestina; Castelli, Germana; Pelosi, Elvira

    2016-01-01

    Studies carried out in the last years have improved the understanding of the cellular and molecular mechanisms controlling angiogenesis during adult life in normal and pathological conditions. Some of these studies have led to the identification of some progenitor cells that sustain angiogenesis through indirect, paracrine mechanisms (hematopoietic angiogenic cells) and through direct mechanisms, i.e., through their capacity to generate a progeny of phenotypically and functionally competent endothelial cells [endothelial colony forming cells (ECFCs)]. The contribution of these progenitors to angiogenetic processes under physiological and pathological conditions is intensively investigated. Angiogenetic mechanisms are stimulated in various hematological malignancies, including chronic myeloid leukemia (CML), acute myeloid leukemia (AML), myelodysplastic syndromes and multiple myeloma, resulting in an increased angiogenesis that contributes to disease progression. In some of these conditions there is preliminary evidence that some endothelial cells could derive from the malignant clone, thus leading to the speculation that the leukemic cell derives from the malignant transformation of a hemangioblastic progenitor, i.e., of a cell capable of differentiation to the hematopoietic and to the endothelial cell lineages. Our understanding of the mechanisms underlying increased angiogenesis in these malignancies not only contributed to a better knowledge of the mechanisms responsible for tumor progression, but also offered the way for the discovery of new therapeutic targets. PMID:27583252

  3. Endothelial progenitor cells in hematologic malignancies.

    PubMed

    Testa, Ugo; Saulle, Ernestina; Castelli, Germana; Pelosi, Elvira

    2016-01-01

    Studies carried out in the last years have improved the understanding of the cellular and molecular mechanisms controlling angiogenesis during adult life in normal and pathological conditions. Some of these studies have led to the identification of some progenitor cells that sustain angiogenesis through indirect, paracrine mechanisms (hematopoietic angiogenic cells) and through direct mechanisms, i.e., through their capacity to generate a progeny of phenotypically and functionally competent endothelial cells [endothelial colony forming cells (ECFCs)]. The contribution of these progenitors to angiogenetic processes under physiological and pathological conditions is intensively investigated. Angiogenetic mechanisms are stimulated in various hematological malignancies, including chronic myeloid leukemia (CML), acute myeloid leukemia (AML), myelodysplastic syndromes and multiple myeloma, resulting in an increased angiogenesis that contributes to disease progression. In some of these conditions there is preliminary evidence that some endothelial cells could derive from the malignant clone, thus leading to the speculation that the leukemic cell derives from the malignant transformation of a hemangioblastic progenitor, i.e., of a cell capable of differentiation to the hematopoietic and to the endothelial cell lineages. Our understanding of the mechanisms underlying increased angiogenesis in these malignancies not only contributed to a better knowledge of the mechanisms responsible for tumor progression, but also offered the way for the discovery of new therapeutic targets. PMID:27583252

  4. Malignant Melanoma of the Foot

    MedlinePlus

    ... Javascript in your browser. Malignant Melanoma of the Foot What is Malignant Melanoma? Melanoma is a cancer ... age groups, even the young. Melanoma in the Foot Melanoma that occurs in the foot or ankle ...

  5. Solitary fibrous tumor of the breast: report of a case with emphasis on diagnostic role of STAT6 immunostaining.

    PubMed

    Magro, Gaetano; Angelico, Giuseppe; Leone, Giorgia; Palazzo, Juan

    2016-05-01

    We herein report the clinical, radiological, and pathological findings of a rare case of a solitary fibrous tumor (SFT) occurring in the breast parenchyma of a 62-year-old female. The tumor was incidentally detected at a mammographic screening, and, ultrasonographically, presented as a single, well-circumscribed nodule. On needle core biopsy, the diagnosis of SFT was suggested based on a proliferation of CD34-positive spindly cells set in a fibrous stroma containing medium-sized blood vessels with hyalinization of their walls and branching configuration. The diagnosis was confirmed in the excised specimen, which exhibited a tumor with an immunohistochemical profile consistent with SFT, including diffuse expression of CD34, CD99 and bcl2. As STAT6 nuclear immunoexpression is the result of the inv12(q13q13)-derived NAB2-STAT6 fusion, which characterizes SFT, we analyzed immunohistochemically our case with a commercially available anti-STAT6 antibody. We showed that mammary SFT exhibits a diffuse nuclear STAT6 immunoreactivty, suggesting its potential diagnostic role. The present case emphasizes that the diagnosis of SFT can be confidentially rendered on needle core biopsy. Although SFT is suspected on characteristic morphologic features, immunohistochemistry, revealing immunoreactivity for CD34, bcl-2, CD99 and STAT6, is crucial in the differential diagnosis of potential benign and malignant mimics. PMID:26778386

  6. Solitary fibrous tumor of the breast: report of a case with emphasis on diagnostic role of STAT6 immunostaining.

    PubMed

    Magro, Gaetano; Angelico, Giuseppe; Leone, Giorgia; Palazzo, Juan

    2016-05-01

    We herein report the clinical, radiological, and pathological findings of a rare case of a solitary fibrous tumor (SFT) occurring in the breast parenchyma of a 62-year-old female. The tumor was incidentally detected at a mammographic screening, and, ultrasonographically, presented as a single, well-circumscribed nodule. On needle core biopsy, the diagnosis of SFT was suggested based on a proliferation of CD34-positive spindly cells set in a fibrous stroma containing medium-sized blood vessels with hyalinization of their walls and branching configuration. The diagnosis was confirmed in the excised specimen, which exhibited a tumor with an immunohistochemical profile consistent with SFT, including diffuse expression of CD34, CD99 and bcl2. As STAT6 nuclear immunoexpression is the result of the inv12(q13q13)-derived NAB2-STAT6 fusion, which characterizes SFT, we analyzed immunohistochemically our case with a commercially available anti-STAT6 antibody. We showed that mammary SFT exhibits a diffuse nuclear STAT6 immunoreactivty, suggesting its potential diagnostic role. The present case emphasizes that the diagnosis of SFT can be confidentially rendered on needle core biopsy. Although SFT is suspected on characteristic morphologic features, immunohistochemistry, revealing immunoreactivity for CD34, bcl-2, CD99 and STAT6, is crucial in the differential diagnosis of potential benign and malignant mimics.

  7. Bone sarcomas as second malignant neoplasms following childhood cancer

    SciTech Connect

    Newton, W.A. Jr.; Meadows, A.T.; Shimada, H.; Bunin, G.R.; Vawter, G.F. )

    1991-01-01

    This study explores the relationship between histologic variants of bone sarcomas and previous therapy in patients in whom an unrelated malignant neoplasm had been diagnosed during childhood. Sarcomas of bone were the most common second malignant neoplasm (SMN) reported to the Late Effects Study Group, a 13-institution consortium consisting of pediatric oncology centers from western Europe, Canada, and the United States. The authors attempted to relate the histologic subtypes of the 91 bone tumors to clinical factors such as previous therapy and genetic predisposition because morphologic variants have been shown to have biologic significance in other tumors and may have etiologic import. The literature concerning the subtypes of bone tumors, clinical and experimental, is also reviewed. The authors also investigated the effect of several factors on the time interval from the first diagnosis to the SMN (i.e., the bone sarcoma). Anthracyclines significantly shortened the interval by about 3 years. The primary diagnosis also significantly affected the interval, with leukemia/lymphomas having the shortest interval and retinoblastoma the longest. The authors could not demonstrate any significant relationship between morphologic characteristics of the osteosarcoma and predisposing conditions. However, lesions diagnosed as chondrosarcoma and malignant fibrous histiocytoma occurred almost exclusively in patients who had received radiation therapy to the site in which the SMN developed.

  8. A Rare Case of a Recurrent Giant Solitary Fibrous Tumor of the Ciliary Body of the Orbit.

    PubMed

    Krishnamurthy, Arvind; Singh, Shirley Sundar; Majhi, Urmila; Ramshankar, Vijayalakshmi; Krishnamurthy, Arvind

    2016-07-01

    Solitary fibrous tumors (SFTs) are uncommon spindle-cell tumors of mesenchymal origin initially described in the pleura and subsequently in other extra-pleural sites. These tumors are categorized as tumors of 'intermediate malignancy' under the World Health Organization classification of soft tissue tumors. SFT was virtually non-existent or misdiagnosed until its characteristic features, particularly the strong and diffuse immuno-reactivity to CD 34 were described. Extra-pleural manifestations of SFT, particularly in the head and neck region are rare. Although a number of isolated case reports of orbital SFTs have been described ever since its initial description in 1994, cases of recurrent SFTs have been very few. Recurrences of these tumors following surgery are considered unusual and metastasis exceptional. We describe clinical presentation and the management challenges of recurrent giant orbital SFT in a 15-year old girl along with a brief review of literature.

  9. [Solitary fibrous tumor of the parietal pleura resected by video-assisted thoracic surgery; report of a case].

    PubMed

    Hayama, Makio; Maeda, Hiroya

    2010-06-01

    A 35-year-old male was admitted to our hospital for further examination of abnormal shadow on chest X-ray. Contrast-enhanced chest computed tomography (CT) showed a clear-shaped tumor with homogenous enhancement, measuring 25 x 20 mm in size, which had enlarged, compared with chest CT of 2 years before. The tumor appeared to be based partially on the parietal pleura. Under the diagnosis of solitary fibrous tumor (SFT) originated from the visceral or parietal pleura, video-assisted thoracic surgery (VATS) was performed. Under thoracoscopy, the tumor was pedunculated and arose from the parietal pleura. The tumor was resected completely. The pathologic findings revealed proliferation of spindle cells and collagen fibers without malignant aspects. Immuno-histochemical findings showed positive immunostaining for CD34 and vimentin in the tumor cells. These findings confirmed that the tumor was SFT.

  10. [Solitary fibrous tumor of the visceral pleura being suspected of chest wall origin: report of a case].

    PubMed

    Hayashi, S; Kitada, M; Ozawa, K; Sato, K; Hirata, S; Tokusashi, Y; Miyokawa, N; Sasajima, T

    2009-11-01

    We report a case of a solitary fibrous tumor (SFT) of the pleura which is suspected of chest wall tumor. A 52-year-old female was admitted to our hospital because of epigastralgia and body weight loss. Chest X-ray and computed tomography showed a circumscribed mass of 35 x 22 mm in diameter arising from the parietal pleura. Positron emission tomography showed uptake valve of 1.5. SFT of chest wall origin was suspected and performed video-assisted thoracic surgery. The pedunculated tumor attached to the visceral pleura. The tumor was diagnosed as a benign SFT in intraoperative diagnosis. Long term clinical follow-up is recommended for patients with SFT, because the tumor recurrence and malignant transformation may occur in tumors with benign histological features.

  11. A Rare Case of a Recurrent Giant Solitary Fibrous Tumor of the Ciliary Body of the Orbit.

    PubMed

    Krishnamurthy, Arvind; Singh, Shirley Sundar; Majhi, Urmila; Ramshankar, Vijayalakshmi; Krishnamurthy, Arvind

    2016-07-01

    Solitary fibrous tumors (SFTs) are uncommon spindle-cell tumors of mesenchymal origin initially described in the pleura and subsequently in other extra-pleural sites. These tumors are categorized as tumors of 'intermediate malignancy' under the World Health Organization classification of soft tissue tumors. SFT was virtually non-existent or misdiagnosed until its characteristic features, particularly the strong and diffuse immuno-reactivity to CD 34 were described. Extra-pleural manifestations of SFT, particularly in the head and neck region are rare. Although a number of isolated case reports of orbital SFTs have been described ever since its initial description in 1994, cases of recurrent SFTs have been very few. Recurrences of these tumors following surgery are considered unusual and metastasis exceptional. We describe clinical presentation and the management challenges of recurrent giant orbital SFT in a 15-year old girl along with a brief review of literature. PMID:27408474

  12. [Malignant insulinoma: recommendations for workup and treatment].

    PubMed

    Baudin, Eric; Caron, Philippe; Lombard-Bohas, Catherine; Tabarin, Antoine; Mitry, Emmanuel; Reznick, Yves; Taieb, David; Pattou, François; Goudet, Pierre; Vezzosi, Delphine; Scoazec, Jean-Yves; Cadiot, Guillaume; Borson-Chazot, Françoise; Do Cao, Christine

    2014-06-01

    Insulinoma are malignant in 4 to 14 % of cases. Their rarity and the sparse data available in the literature have limited publication of specific guidelines for their management. The following review aim to provide up-to-date recommendations on initial evaluation including pathologic grading, measures to control hypoglycemia, antitumor strategies and long term follow-up. Will be discussed in detail respective indications of surgery, diazoxide, somatostatin analogs, everolimus, sunitinib, liver directed treatments including arterial embolization, chemotherapy and radiometabolic therapy. A Medline search using terms "insulinoma", "neuroendocrine pancreatic tumors", "islet cell carcinoma", "malignant insulinoma" was performed limiting the selection to English language articles and adult age cases, along with cross referencing.

  13. BMI1: A Biomarker of Hematologic Malignancies

    PubMed Central

    Sahasrabuddhe, Anagh A.

    2016-01-01

    BMI1 oncogene is a catalytic member of epigenetic repressor polycomb group proteins. It plays a critical role in the regulation of gene expression pattern and consequently several cellular processes during development, including cell cycle progression, senescence, aging, apoptosis, angiogenesis, and importantly self-renewal of adult stem cells of several lineages. Preponderance of evidences indicates that deregulated expression of PcG protein BMI1 is associated with several human malignancies, cancer stem cell maintenance, and propagation. Importantly, overexpression of BMI1 correlates with therapy failure in cancer patients and tumor relapse. This review discusses the diverse mode of BMI1 regulation at transcriptional, posttranscriptional, and posttranslational levels as well as at various critical signaling pathways regulated by BMI1 activity. Furthermore, this review highlights the role of BMI1 as a biomarker and therapeutic target for several subtypes of hematologic malignancies and the importance to target this biomarker for therapeutic applications. PMID:27168727

  14. Tumor size-related DNA copy number changes occur in solitary fibrous tumors but not in hemangiopericytomas.

    PubMed

    Miettinen, M M; el-Rifai, W; Sarlomo-Rikala, M; Andersson, L C; Knuutila, S

    1997-12-01

    Solitary fibrous tumor (SFT) presenting in the pleura and other soft tissue sites and hemangiopericytoma (HPC) presenting at various soft tissue sites are mesenchymal tumors that share many histologic and immunohistochemical features. This raises the questions of whether these tumors are related and whether they belong within the spectrum of a single biologic entity. The behavior of both SFTs and HPCs is difficult to predict histologically. The genetic background of both SFTs and HPCs is poorly known, but it could be helpful in the evaluation of malignancy and could give clues to their possible relationship. In this study, we analyzed 15 SFTs and 11 HPCs by comparative genomic hybridization (CGH), a powerful molecular cytogenetic tool that can be applied to DNA extracted from formaldehyde-fixed and paraffin-embedded tissue. All of these tumors were immunohistochemically similar and showed reactivity for CD34-antigen but not for keratins, desmin, or muscle actins. Only 1 SFT smaller than 10 cm showed DNA copy number changes (a single loss in chromosome 13), but 7 of 8 SFTs larger than 10 cm (including all 4 tumors with more than 4 mitoses per 10 high power fields) showed changes, mostly chromosomal gains in 5q 7, 8, 12, and 18. Four cases showed losses, two of them in chromosome 13 and two others in 20q. These findings suggest that CGH might be useful in the evaluation of malignant transformation in SFT. The most common change, gain of the entire chromosome 8, seen in two cases as the only change, suggests trisomy 8 and parallels a similar finding previously described in other fibrous tumors, such as subsets of desmoid fibromatosis and infantile fibrosarcoma. In contrast, HPCs, including large and mitotically active tumors, showed no DNA copy number changes on CGH. This suggests that HPC is genetically different from SFT.

  15. High-grade sarcomatous overgrowth in solitary fibrous tumors: a clinicopathologic study of 10 cases.

    PubMed

    Collini, Paola; Negri, Tiziana; Barisella, Marta; Palassini, Elena; Tarantino, Eva; Pastorino, Ugo; Gronchi, Alessandro; Stacchiotti, Silvia; Pilotti, Silvana

    2012-08-01

    We describe 10 solitary fibrous tumors (SFT) with high-grade malignant overgrowth, all of which showed the presence of a synchronous or previous classic SFT/malignant SFT (MSFT) component. Seven were "dedifferentiated," with an abrupt transition from a classic SFT/MSFT to a high-grade component consisting of a nondistinctive high-grade sarcoma in 4 cases and divergent differentiation in 3. The nondistinctive high-grade component consisted of epithelioid and/or spindle cells often associated with overt pleomorphism or small round cell sarcomas. The divergent differentiation featured a rhabdomyosarcoma in 2 cases and an osteosarcoma in 1. Three cases (tentatively called "evolved") showed a gradual transition from classic SFT/MSFT to a nondistinctive high-grade sarcoma or presented features of high-grade sarcoma at the time of metastasis (assessed by fine-needle aspiration cytology) without any component suggesting a diagnosis of classic SFT/MSFT. The high-grade component showed loss of CD34 expression in half of the dedifferentiated SFTs and all of the dedifferentiated SFTs with divergent differentiation, whereas Ki-67 was markedly increased in all of the evaluable cases and paralleled the tumor grade. In 4 cases, the expression and phosphorylation status of key factors that control transcription and protein synthesis were also investigated. Both S6 and 4E-BP1 showed low activation in the low-grade MSFT and a high level of activation in the high-grade component. Seven of the 10 patients died of their disease during follow-up, with a median overall survival of 73 months (range, 5 to 288 mo). The median time to distant metastasis was 156 months after the initial diagnosis, and median overall survival from the first signs of metastasis was 8 months.

  16. Recurrent intracranial solitary fibrous tumor with cerebrospinal fluid dissemination. Case report.

    PubMed

    Miyashita, Katsuyoshi; Hayashi, Yutaka; Fujisawa, Hironori; Hasegawa, Mitsuhiro; Yamashita, Junkoh

    2004-12-01

    Solitary fibrous tumor (SFT) is a benign and rare neoplasm. To date, only 37 patients with intracranial SFTs have been reported. Although a number of the tumors were recurrent and some later underwent malignant transformation, none of these lesions progressed to cerebrospinal fluid (CSF) dissemination. In this paper the authors report a case of SFT in which the lesion recurred several times and ultimately was disseminated by the CSF. The patient was a 63-year-old woman with multiple intracranial and spinal tumors. Fifteen years before this presentation, at the age of 48 she had been hospitalized for resection of a falcotentorial tumor. During the ensuing 15 years she underwent multiple surgeries and sessions of radiation therapy for recurrent lesions. The exclusive location of her tumors in the subarachnoid space at the end of this 15-year period indicate CSF dissemination of the tumor. The tumor that was resected when the patient was 48 years old and the latest resected lesion were analyzed by performing immunohistological CD34, epithelial membrane antigen, vimentin, S100 protein, and reticulin staining, and determining the MIB-1 labeling index (LI). Most of the results were identical, and both tumors were diagnosed as SFT according to a staining pattern that showed a strong and diffuse positive reaction for CD34. Nevertheless, the authors noted that the MIB-1 LI increased from less than 1% in the original tumor to 13% in the latest tumor. The increased proliferation of MIB-1 indicates that the malignant transformation could have occurred during tumor recurrence with CSF dissemination.

  17. Myxoid solitary fibrous tumor: a study of seven cases with emphasis on differential diagnosis.

    PubMed

    de Saint Aubain Somerhausen, N; Rubin, B P; Fletcher, C D

    1999-05-01

    Focal myxoid change is a well-recognized feature of solitary fibrous tumor (SFT), but to date, predominantly myxoid examples of SFT have not been reported. We describe seven cases of SFT in which stromal myxoid change affected 50% or more of the tumor examined, thus obscuring typical diagnostic features. Patients ranged in age from 35 to 68 years old (median, 45 yr), with an equal sex distribution. Tumor locations included pleura, orbit, and periparotid subcutaneous tissue, as well as four cases in deep soft tissue (two in the abdominal wall and one each in the chest wall and thigh). Myxoid areas were identified grossly in four cases. Histologically, the lesions were composed of bland spindle cells disposed haphazardly or with a lacy or reticulated appearance in a myxoid, richly vascularized stroma These myxoid areas were punctuated by small cellular aggregates in four cases, and areas showing diagnostic features of SFT were present in five of seven primary excision specimens. Atypical features suggestive of malignancy were not present in any of the cases. Immunohistochemically, all of the seven cases stained positively for CD34 and CD99 (013), and all were negative for smooth muscle actin, desmin, S-100 protein, epithelial membrane antigen, and pan-keratin. There were no recurrences or metastases reported in four patients with limited follow-up (median duration, 19 mo). Recognition of this uncommon morphologic subset of SFT is important because of possible confusion, particularly in small biopsy specimens, with a variety of myxoid spindle cell neoplasms with different biologic potential. These include low-grade fibromyxoid sarcoma, myxoid synovial sarcoma, malignant peripheral nerve sheath tumor, low-grade myxofibrosarcoma, myxoid liposarcoma, myxoid spindle cell lipoma, myxoid neurofibroma, and so-called "hemangiopericytoma."

  18. The Current Status of Solitary Fibrous Tumor: Diagnostic Features, Variants, and Genetics.

    PubMed

    Thway, Khin; Ng, Wen; Noujaim, Jonathan; Jones, Robin L; Fisher, Cyril

    2016-06-01

    Solitary fibrous tumor (SFT) is a fibroblastic mesenchymal tumor originally described in the pleura but now shown at almost every anatomic site. Histopathologically, SFT is characteristically a circumscribed neoplasm composed of variably cellular and patternless distributions of bland spindle and ovoid cells within prominent collagenous stroma and shows diffuse expression of CD34, but it has a broad spectrum of both morphology and of biologic behavior. Many different names (particularly hemangiopericytoma) were previously used in the course of our understanding of this neoplasm but are now subsumed under the term "SFT," and the putative cell of origin was debated. However, it is now recognized that SFT is a translocation-associated neoplasm, consistently associated with NAB2-STAT6 gene fusions arising from recurrent intrachromosomal rearrangements on chromosome 12q, and this translocation is a likely major contributor to its pathogenesis. While most SFT with classical morphologic features behave in an indolent manner and those with overtly malignant histologic features tend to be aggressive neoplasms that behave as high-grade sarcomas, the behavior of SFT is unpredictable, and it is important to be aware of the propensity for aggressive behavior in a minority of histologically classical SFT and to ensure adequate clinical follow-up. Surgical excision remains the treatment gold standard; while radiotherapy and conventional chemotherapeutic agents have only shown limited efficacy, further understanding of the molecular events underlying tumorigenesis may allow the development of novel targeted treatments. We review SFT, discussing the morphologic spectrum and variants, including malignant and dedifferentiated subtypes, clinicopathological aspects, recent molecular genetic findings, and the differential diagnosis. PMID:26811389

  19. The Current Status of Solitary Fibrous Tumor: Diagnostic Features, Variants, and Genetics.

    PubMed

    Thway, Khin; Ng, Wen; Noujaim, Jonathan; Jones, Robin L; Fisher, Cyril

    2016-06-01

    Solitary fibrous tumor (SFT) is a fibroblastic mesenchymal tumor originally described in the pleura but now shown at almost every anatomic site. Histopathologically, SFT is characteristically a circumscribed neoplasm composed of variably cellular and patternless distributions of bland spindle and ovoid cells within prominent collagenous stroma and shows diffuse expression of CD34, but it has a broad spectrum of both morphology and of biologic behavior. Many different names (particularly hemangiopericytoma) were previously used in the course of our understanding of this neoplasm but are now subsumed under the term "SFT," and the putative cell of origin was debated. However, it is now recognized that SFT is a translocation-associated neoplasm, consistently associated with NAB2-STAT6 gene fusions arising from recurrent intrachromosomal rearrangements on chromosome 12q, and this translocation is a likely major contributor to its pathogenesis. While most SFT with classical morphologic features behave in an indolent manner and those with overtly malignant histologic features tend to be aggressive neoplasms that behave as high-grade sarcomas, the behavior of SFT is unpredictable, and it is important to be aware of the propensity for aggressive behavior in a minority of histologically classical SFT and to ensure adequate clinical follow-up. Surgical excision remains the treatment gold standard; while radiotherapy and conventional chemotherapeutic agents have only shown limited efficacy, further understanding of the molecular events underlying tumorigenesis may allow the development of novel targeted treatments. We review SFT, discussing the morphologic spectrum and variants, including malignant and dedifferentiated subtypes, clinicopathological aspects, recent molecular genetic findings, and the differential diagnosis.

  20. Hyaluronan in human malignancies

    SciTech Connect

    Sironen, R.K.; Tammi, M.; Tammi, R.; Auvinen, P.K.; Anttila, M.; Kosma, V-M.

    2011-02-15

    Hyaluronan, a major macropolysaccharide in the extracellular matrix of connective tissues, is intimately involved in the biology of cancer. Hyaluronan accumulates into the stroma of various human tumors and modulates intracellular signaling pathways, cell proliferation, motility and invasive properties of malignant cells. Experimental and clinicopathological evidence highlights the importance of hyaluronan in tumor growth and metastasis. A high stromal hyaluronan content is associated with poorly differentiated tumors and aggressive clinical behavior in human adenocarcinomas. Instead, the squamous cell carcinomas and malignant melanomas tend to have a reduced hyaluronan content. In addition to the stroma-cancer cell interaction, hyaluronan can influence stromal cell recruitment, tumor angiogenesis and epithelial-mesenchymal transition. Hyaluronan receptors, hyaluronan synthases and hyaluronan degrading enzymes, hyaluronidases, are involved in the modulation of cancer progression, depending on the tumor type. Furthermore, intracellular signaling and angiogenesis are affected by the degradation products of hyaluronan. Hyaluronan has also therapeutic implications since it is involved in multidrug resistance.