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1

Targetoid hemosiderotic hemangioma - Case report*  

PubMed Central

Targetoid Hemosiderotic Hemangioma, also known as Hobnail Hemangioma, is a lesion of vascular origin, probably lymphatic. The most common clinical feature is a solitary violaceous papule surrounded by a pale, thin area and a peripheral ecchymotic ring, simulating a target. Histopathologically, there is a biphasic pattern, with dilated vessels in the superficial dermis and pseudoangiosarcomatous pattern in the deep dermis, and endothelial cells with hobnail morphology. A simple excision is curative. We report a rare case of Targetoid Hemosiderotic Hemangioma. PMID:25387500

Kakizaki, Priscila; Valente, Neusa Yuriko Sakai; Paiva, Daniele Loureiro Mangueira; Dantas, Fernando Luiz Teixeira; Goncalves, Sheila Viana Castelo Branco

2014-01-01

2

Fibrous and fibrohistiocytic neoplasms: an update.  

PubMed

Important advances in fibroblastic and fibrohistiocytic tumors relevant to dermatologists and dermatopathologists include (1) recognition that myxofibrosarcoma is a distinct entity that frequently arises in skin; (2) CD10 is sensitive but not specific atypical fibroxanthoma; (3) neurothekeomas lacking S100 expression are probably fibrohistiocytic/fibroblastic tumors, whereas S100+ myxoid variants are better classified as nerve sheath myxomas; (4) the recognition of a primary cutaneous variant of solitary fibrous tumor; (5) thelimitations of b-catenin immunohistochemistry in desmoid tumors; and (6) the prognostic utility of clinical and histopathologic variables in dermatofibrosarcoma protuberans, and the effects of imatinib mesylate therapy. PMID:23021051

Clarke, Loren E

2012-10-01

3

Mitral valve lipomatous hamartoma: a rare entity.  

PubMed

Lipomatous hamartoma of cardiac valves is a very rare entity, with only three reported cases in children. We describe the case of a 9-year-old girl with a mass in the mitral valve, which was detected in an echocardiogram performed for heart murmur investigation. At surgery, a white round-shaped tumour was removed and histopathological examination revealed a lipomatous hamartoma. PMID:24044592

Francisco, Andreia; Gouveia, Rosa; Anjos, Rui

2014-10-01

4

Cystic fibrohistiocytic tumor of the lung presenting as a solitary lesion  

PubMed Central

Cystic fibrohistiocytic tumor of the lung is a rare neoplasm. In many cases it represents a metastasis from a benign or low-grade fibrohistiocytic tumor of the skin, but occasionally it may be primary. Radiologically it usually occurs as a cystic change of multiple pulmonary nodules, and pneumothorax is the most frequent presenting symptom. We present here a 16-year-old man with recurrent right pneumothorax. The patient had no history of cutaneous fibrohistiocytic lesions. He underwent videothoracoscopic right apical segmentectomy, right lower lobe nodulectomy, and pleuroabrasion. Microscopy of the apical segmentectomy showed a cystic fibrohistiocytic tumor, whereas the nodule of the lower lobe was an intraparenchymal lymph node. The patient is alive with no tumor recurrence. The differential diagnosis includes Langerhans cell histiocytosis, lymphangioleiomyomatosis, pleuropulmonary blastoma, and metastatic endometrial stromal sarcoma. This disease usually occurs with multiple pulmonary cysts and cavitation. This case is the first reported presenting as a single lesion. PMID:21139943

Paci, Massimiliano; Cavazza, Alberto; Annessi, Valerio; Ricchetti, Tommaso; Rapicetta, Cristian; Sgarbi, Giorgio

2010-01-01

5

Endobronchial Lipomatous Polyp: A Rare Benign Tumor of the Lung  

PubMed Central

Endobronchial lipomatous polyp is a rare nonmalignant tumor of the lung. It comprises 5% of the benign lung tumor, with the majority of benign tumors being hamartoma. Lipomatous polyp often leads to endobronchial lesion, associated with postobstructive pneumonia, hemoptysis, and atelectasis. We hereby present a case and discussion of an elderly man with endobronchial lipomatous polyp, presenting as recurrent pneumonia. PMID:24971188

Surani, Saherish; Varon, Joseph

2014-01-01

6

Lipomatous hypertrophy of the interatrial septum: indication for surgery?  

Microsoft Academic Search

A fortuitous finding during open heart surgery of lipomatous hypertrophy of the interatrial septum is described in a 65-year old man with ischaemic heart complaints due to coronary artery disease and with premature ventricular contractions. An incision biopsy confirmed the diagnosis. The choice of treatment of lipomatous hypertrophy of the interatrial septum is controversial. Indications for surgery and surgical techniques

C. J. A. M. Zeebregts; A. G. Hensens; J. Timmermans; M. S. Pruszczynski; L. K. Lacquet

1997-01-01

7

Giant lipomatous tumours of the hand and forearm.  

PubMed

This study examines the presentation, management and outcomes of a series of 10 patients with giant lipomatous tumours (defined as greater than 5 cm diameter) of the hand and forearm who presented to our orthopaedic oncology service. All patients underwent local staging and were discussed at our multidisciplinary tumour meeting prior to definitive surgery. In all cases, neurovascular structures required mobilization in order to excise the tumour. Seven of the tumours were benign lipomas and one was a neural fibrolipoma. The other two were well differentiated lipoma-like liposarcomas/atypical lipomatous tumours. Giant lipomas and well differentiated lipoma-like liposarcomas/atypical lipomatous tumours of the hand and forearm present infrequently and a multidisciplinary approach is recommended in the investigation and surgical management of these patients. PMID:15992974

Cribb, G L; Cool, W P; Ford, D J; Mangham, D C

2005-10-01

8

Anterior Approach in a Huge Lipomatous Tumor of the Thigh  

PubMed Central

Confronted with a huge lipomatous anterior thigh tumor, the surgical approach had to be assessed. Those described in the literature did not seem appropriate for our case so some modifications were made. We present the case of a 77-year-old woman who presented with a huge anterior thigh compartment tumor with one-year evolution. Magnetic resonance imaging informed the presence of a lipomatous tumor with a possible vascular contact. Based on Thomson's anterior approach, but modifying the skin incision, the medial distal femur was reached until the neurovascular bundle and, proximally, the lesser trochanter. The tumor was totally resected due to a good visualization using this approach. PMID:25349617

2014-01-01

9

Surgical Treatment of Lipomatous Hypertrophy of the Interatrial Septum  

Microsoft Academic Search

We report a case of lipomatous hypertrophy of the interatrial septum in a patient with a recent syncopal episode and shortness of breath. Preoperative transesophageal echocardiography demonstrated a large tumor protruding from the interatrial septum. In addition, the patient was found to have significant coronary artery disease and a right internal carotid artery stenosis. The patient underwent successful resection of

Julian J Alcocer; William E Katz; Brack G Hattler

1998-01-01

10

Giant lipomatous tumours of the hand and forearm  

Microsoft Academic Search

This study examines the presentation, management and outcomes of a series of 10 patients with giant lipomatous tumours (defined as greater than 5 cm diameter) of the hand and forearm who presented to our orthopaedic oncology service. All patients underwent local staging and were discussed at our multidisciplinary tumour meeting prior to definitive surgery. In all cases, neurovascular structures required

G. L. Cribb; W. P. Cool; D. J. Ford; D. C. Mangham

2005-01-01

11

Myxoinflammatory fibroblastic sarcoma: morphologic and genetic updates.  

PubMed

Myxoinflammatory fibroblastic sarcoma (MIFS) is a malignant mesenchymal neoplasm most frequently arising in the distal extremities of adults, which usually behaves in a low-grade manner but is capable of metastasizing to local and distant sites, rarely leading to death. It is a rare tumor whose unusual morphology can lead to erroneous histologic diagnosis, either as a nonneoplastic (infectious or inflammatory) process or as a variety of neoplastic diseases. While its exact origin is uncertain, ultrastructural studies have shown at least some of the constituent cells to be modified fibroblasts. Distinct and reproducible genetic abnormalities identified in MIFS are translocation t(1;10)(p22:q24), with rearrangements of the TGFBR3 and MGEA5 genes associated with increased levels of FGF8, and formation of marker/ring chromosome 3, with amplification of the VGLL3 locus. Because these genetic abnormalities are shared by both MIFS and hemosiderotic fibrohistiocytic lipomatous tumor, it is thought that these 2 morphologically distinct neoplasms may comprise a spectrum of disease defined by these genetics. We review the literature on MIFS and discuss morphology (including that of MIFS/hemosiderotic fibrohistiocytic lipomatous tumor hybrid lesions), immunohistochemistry, the differential diagnosis, and recent molecular genetic developments. PMID:25268202

Ieremia, Eleni; Thway, Khin

2014-10-01

12

Hobnail hemangioma: a pseudomalignant vascular lesion with a reappraisal of targetoid hemosiderotic hemangioma.  

PubMed

The clinicopathologic features of 15 cutanous hemangiomas having a distinctive and frequently pseudomalignant morphologic appearance are presented. There were 5 male and 9 female patients, whose ages at diagnosis ranged from 11 to 58 years (median 30.5). An angiomatous/pigmented, nontargetoid, flat, or exophytic lesion of variable duration was the main presenting sign. The tumor sizes ranged from 0.4 cm to 2 cm (median 1 cm). The locations included the lower limb, particularly the thigh (8); the trunk, including the shoulder area (4); the head (1); the gingiva (1); and the tongue (1). One patient had two lesions; none had a concomitant vascular anomaly or was suspected to have HIV infection. Treatment consisted of excisional biopsy in all cases. Follow-up information on 10 patients (range 4-66 months; median 13 months) showed no recurrence. On microscopic examination, the lesion showed a biphasic pattern characterized by the presence of well-formed, dilated, vascular channels in superficial dermis and a collagen-dissecting, pseudoangiosarcomatous pattern as the lesion infiltrated deeper into the dermis. The lining endothelium consistently showed distinctive hobnail cytomorphology; although there were endoluminal stromal papillae, there was no endothelial multilayering or tufting. Cytologic atypia was minimal or absent, and there were no mitoses. In 3 cases, the morphologic features were reminiscent of retiform hemangioendothelioma. Immunohistochemistry performed in 8 cases showed variable reactivity of endothelial cells with CD31, CD34, Factor VIII-related antigen, and Ulex europaeus agglutinin-1 in all cases; smooth muscle actin-positive pericytes were observed focally around some of the abnormal vascular spaces. The above-described hemangiomatous lesions share many features with so-called targetoid hemosiderotic hemangioma (a clinically descriptive term), but show a variable, often minimal, amount of hemosiderin deposition. The histologically descriptive term hobnail hemangioma is proposed to designate these lesions. Hobnail hemangioma should be distinguished from well-differentiated angiosarcoma, patch-stage Kaposi's sarcoma, and retiform hemangioendothelioma, with which it may be confused. PMID:9888709

Guillou, L; Calonje, E; Speight, P; Rosai, J; Fletcher, C D

1999-01-01

13

Fluorescence in situ hybridization for MDM2 gene amplification as a diagnostic tool in lipomatous neoplasms  

Microsoft Academic Search

Well-differentiated liposarcoma\\/atypical lipomatous tumor and dedifferentiated liposarcoma can be difficult to distinguish from benign lipomatous neoplasms and other high-grade sarcomas, respectively. Cytogenetics in these tumors has identified ring and giant chromosomes composed of 12q13-15 amplicons including the MDM2 gene. Identifying MDM2 amplification by fluorescence in situ hybridization may prove an adjunctive tool in the diagnosis of lipomatous neoplasms. Dual color

Joshua Weaver; Erinn Downs-Kelly; John R Goldblum; Sondra Turner; Sucheta Kulkarni; Raymond R Tubbs; Brian P Rubin; Marek Skacel

2008-01-01

14

Diagnostic challenge of lipomatous uterine tumors in three patients  

PubMed Central

Lipomatous uterine tumors are uncommon benign neoplasms, with incidence ranging from 0.03% to 0.2%. They can generally be subdivided into two types: pure or mixed lipomas. A third group of malignant neoplasm has been proposed, which is liposarcoma; however, this is very rare. In this article, we report three patients having lipomatous uterine tumors, including one uterine lipoma and two uterine lipoleiomyomas. All our patients are postmenopausal women, which is the typical presenting age group. They did not have any symptoms and the tumors were only found incidentally on imaging. However, in some patients, symptoms may uncommonly occur. If symptoms occur, these are similar to those of leiomyoma. We illustrate the imaging features of the tumors in our patients with ultrasound, computed tomography (CT) scan and magnetic resonance imaging (MRI). The tumor typically appears as a well-defined homogenously hyperechoic lesion on ultrasound. It shows fat density on CT scan and signal intensity of fat on MRI. MRI is the modality of choice because of its multiplanar capability and its ability to demonstrate fat component of the lesion, as illustrated in our cases. We also discuss the importance of differentiating lipomatous uterine tumors from other lesions, especially ovarian teratoma which requires surgical intervention. Despite the rarity and the common asymptomatic nature of the tumors, we believe that this series of three cases demonstrates a review of a rare tumor which provides important knowledge for patient management. PMID:22423320

Chu, Chi-Yeung; Tang, Yip-Kan; Chan, Tin-Sang Augustine; Wan, Yu-Hon; Fung, Kai-Hung

2012-01-01

15

Diagnostic challenge of lipomatous uterine tumors in three patients.  

PubMed

Lipomatous uterine tumors are uncommon benign neoplasms, with incidence ranging from 0.03% to 0.2%. They can generally be subdivided into two types: pure or mixed lipomas. A third group of malignant neoplasm has been proposed, which is liposarcoma; however, this is very rare. In this article, we report three patients having lipomatous uterine tumors, including one uterine lipoma and two uterine lipoleiomyomas. All our patients are postmenopausal women, which is the typical presenting age group. They did not have any symptoms and the tumors were only found incidentally on imaging. However, in some patients, symptoms may uncommonly occur. If symptoms occur, these are similar to those of leiomyoma. We illustrate the imaging features of the tumors in our patients with ultrasound, computed tomography (CT) scan and magnetic resonance imaging (MRI). The tumor typically appears as a well-defined homogenously hyperechoic lesion on ultrasound. It shows fat density on CT scan and signal intensity of fat on MRI. MRI is the modality of choice because of its multiplanar capability and its ability to demonstrate fat component of the lesion, as illustrated in our cases. We also discuss the importance of differentiating lipomatous uterine tumors from other lesions, especially ovarian teratoma which requires surgical intervention. Despite the rarity and the common asymptomatic nature of the tumors, we believe that this series of three cases demonstrates a review of a rare tumor which provides important knowledge for patient management. PMID:22423320

Chu, Chi-Yeung; Tang, Yip-Kan; Chan, Tin-Sang Augustine; Wan, Yu-Hon; Fung, Kai-Hung

2012-02-28

16

Rare case of paratesticular solitary fibrous tumour (lipomatous hemangiopericytoma)  

PubMed Central

A 26-year-old male presented with an asymptomatic 6-cm left paratesticular mass. Ultrasound and magnetic resonance imaging confirmed this mass as extratesticular, likely a tumour arising from the left spermatic cord. The mass demonstrated marked avid enhancement on post-contrast images, suggestive of a spermatic cord sarcoma. A left inguinal exploration was performed and gross examination of the mass revealed a well-circumscribed tumour with a discrete capsule separating it from the ipsilateral spermatic cord. The mass was resected without performing an orchiectomy and histology demonstrated a solitary fibrous tumour (lipomatous hemangiopericytoma), with minimal proliferative activity and negative margins. The occurrence of a paratesticular solitary fibrous tumour is exceedingly rare, with only a handful of case reports. We review the literature regarding this rare entity and discuss its diagnosis and management. PMID:22709886

Barazani, Yagil; Tareen, Basir

2012-01-01

17

Clinicopathological features of atypical lipomatous tumors of the laryngopharynx  

PubMed Central

Atypical lipomatous tumor (ALT) of the laryngopharynx is rare. Here we report five cases to demonstrate their clinicopathological features. The patients were four males and one female, aged 41 to 69 years (median 53.6 years). All tumors (two in the hypopharynx and three in the larynx) presented as a slowly growing, painless mass. Symptoms included dysphagia (2/5), dysphonia (3/5), and the feeling of a foreign body in the throat (5/5). Tumors were well circumscribed or focally infiltrative, ranging from 2.0 to 5.0 cm (median, 3.4 cm) in size, and microscopically showed the typical features of lipoma-like ALT. Immunohistochemically, tumor cells were stained with S-100, vimentin, murine double minute 2 (MDM-2), and cyclin-dependent kinase 4 (CDK4). Two patients had local tumor recurrences at 6 and 14 months after initial surgery during follow-up. ALT of laryngopharynx is an indolent tumor. Immunohistochemical staining for MDM-2 and CDK4 is helpful in pathological diagnosis. PMID:21121069

Shi, Huai-yin; Wei, Li-xin; Wang, Hong-tian; Sun, Lu

2010-01-01

18

Lipomatous pleomorphic adenoma in the hard palate: Report of a rare case with cyto-histo correlation and review.  

PubMed

Pleomorphic adenoma is the most common benign tumor of the salivary glands. They are usually composed of epithelial/myoepithelial cells and chondromyxoid stroma. Extensive lipomatous differentiation is very rare. We report a case of lipomatous pleomorphic adenoma (LPA) that presented with a mass in the hard palate of a 32-year-old woman. The fine-needle aspiration cytology material was reported as benign cytology consistent with adenoma with major adipocytic component. Histopathological examination of the excision material displayed that more than 90% of the tumor was adipocytic in texture, containing scant epithelial and myoepithelial cells and chondromyxoid stromal fragments. Preoperative cytodiagnosis of lipomatous pleomorphic adenoma on FNA is based on cytomorphology intimately associated pleomorphic adenomatous and lipomatous tissue elements. LPA should be on the mental list of the (cyto)pathologist in differential diagnosis of lipomatous tumors or non-tumorous lipomatosis or carcinoma invasion in the adipose tissue of the minor salivary gland of the hard palate. PMID:25190982

Musayev, Jamal; Onal, Binnur; Hasanov, Adalat; Farzaliyev, Ismayil

2014-01-01

19

Adrenal lipomatous tumours: a 30 year clinicopathological experience at a single institution  

PubMed Central

Aims—Fatty tumours of the adrenal gland are uncommon and their features have received little attention in the literature. The aim of this study is to analyse the features of adrenal lipomatous tumours. Methods—The histological features of primary adrenal tumours reported over a 30 year period (1970 to 1999) in Queen Mary Hospital, Hong Kong were reviewed and the clinicopathological features of adrenal lipomatous tumours were analysed. Results—Adrenal lipomatous tumours were noted in 20 patients (12 men, eight women), and they accounted for 4.8% of the primary adrenal tumours reported. The adrenal fatty tumours comprised 11 myelolipomas, three lipomas, three teratomas, two angiomyolipomas, and one liposarcoma. Calcification or bone was noted in one third (seven of 20) of the adrenal tumours. In some fatty tumours (myelolipoma and angiomyolipoma), the fatty component may be inconspicuous. This is the first report in the English literature of angiomyolipoma and liposarcoma of the adrenal gland. Conclusions—Different types of fatty tumours were noted in the adrenal gland. A high index of suspicion should be maintained with an aim of surgical treatment for selected patients with large and symptomatic adrenal lipomatous lesions. Histological confirmation is needed for diagnosis. Key Words: myelolipoma • lipoma • angiomyolipoma • liposarcoma • adrenal PMID:11533079

Lam, K; Lo, C

2001-01-01

20

Sensitivity of MDM2 amplification and unexpected multiple faint alphoid 12 (alpha 12 satellite sequences) signals in atypical lipomatous tumor.  

PubMed

This study assessed whether analysis of MDM2 copy number by fluorescence in situ hybridization (FISH) would help distinguish lipomas from atypical lipomatous tumors, otherwise referred to as well-differentiated liposarcomas, using a commercially available MDM2 FISH kit. 227 lipomatous and 201 non-lipomatous tumors were analyzed to assess its sensitivity and specificity. Of 178 mature lipomatous tumors, 86 were classified histologically as lipoma and 92 as atypical lipomatous tumor. Two of the lipomas harboring MDM2 amplification were reclassified as atypical lipomatous tumors. Overall, 13 atypical lipomatous tumors did not reveal MDM2 or CDK4 amplification, although this was reduced to 12 following analysis of multiple slides. Three of these cases revealed very occasional tumor cells harboring high-level MDM2 amplification, two had a dedifferentiated component, and MDM2 amplification was detected when one tumor recurred. The remaining six cases exhibited reactive/inflammatory features and were reclassified as lipomas. The findings indicate that MDM2 amplification is 93.5% sensitive for diagnosing atypical lipomatous tumor. A total of 2 of the 20 dedifferentiated liposarcomas failed to reveal MDM2 amplification. All atypical lipomatous tumors measured >10 cm, two dedifferentiated liposarcoma presented de novo at <10 cm, and ~50% of lipomas measured >10 cm. Spindle cell lipomas, lipoblastomas, hibernomas and pleomorphic liposarcomas did not reveal MDM2 amplification. Of 201 non-lipomatous tumors, eight revealed MDM2 amplification or multiple faint alphoid 12 signals and were reclassified as dedifferentiated liposarcoma. Multiple faint alphoid 12 signals were observed in nine tumors from seven patients, an observation not previously reported on paraffin sections: these included four atypical lipomatous tumors, and three dedifferentiated liposarcomas, one previously diagnosed as a myxofibrosarcoma, all of which also revealed amplification of CDK4, although two lacked MDM2 amplification. MDM2 FISH test is a useful adjunct to histology for distinguishing lipoma from atypical lipomatous tumor. The limitations of molecular genetic tests must be known before introducing them into a clinical service. PMID:22699518

Kashima, Takeshi; Halai, Dina; Ye, Hongtao; Hing, Sandra Nalini; Delaney, David; Pollock, Robin; O'Donnell, Paul; Tirabosco, Roberto; Flanagan, Adrienne Margaret

2012-10-01

21

Lipomatous myofibroblastoma of the breast: case report with diagnostic and histogenetic considerations.  

PubMed

We report rare case of myofibroblastoma (MFB) of the breast comprised predominantly of a mature fatty component, representing approximately 70% of the entire tumour area. This tumour, designated "lipomatous MFB", should be interpreted as the morphological result of an unbalanced bidirectional differentiation of the precursor mammary stromal cell, with the adipocytic component overwhelming the fibroblastic/myofibroblastic one. Lipomatous MFB is a rare variant of mammary MFB, which can mimic malignancy because of the close juxtaposition of fibroblasts/myofibroblasts with mature adipocytes, resulting in a finger-like infiltrative growth pattern of the former towards the latter. Histogenetic considerations and differential diagnostic problems with other bland-looking spindle cell tumours containing infiltrating fat are provided. PMID:25291864

Magro, G; Longo, F R; Salvatorelli, L; Vasquez, E; Vecchio, G M

2014-06-01

22

Benign lipomatous masses of the heart: a comprehensive series of 47 cases with cytogenetic evaluation.  

PubMed

Benign lipomatous lesions of the heart encompass an apparently etiologically diverse group of entities including neoplastic, congenital, and reparative phenomena. Among these, lipomas and lipomatous hypertrophy of the atrial septum (LHAS) represent 2 commonly encountered mass lesions. To date, no study has systematically and comparatively evaluated the morphologic and genetic characteristics of these lesions. Tissue registry archives of Mayo Clinic were queried for cases of cardiac lipoma and LHAS (1994-2011). Clinical, imaging, and pathologic findings were reviewed. Representative cases in each cohort were evaluated by fluorescence in situ hybridization (FISH) for HMGA1 and HMGA2 loci rearrangement and for MDM2/CPM locus amplification. Five cases of cardiac lipoma were identified (mean age, 67 years; range, 48-101; 3 men): 4 right atrial and 1 left ventricular. Forty-two cases of LHAS were identified (mean age, 75.6 years; range 45-95; 20 men), 39 of which were autopsy derived. The median size was 3.4 cm for lipomas and 2.8 cm for LHAS (n = 14). A single case each of cardiac lipoma and LHAS were found to harbor HMGA2 rearrangement, whereas no case showed cytogenetic abnormality of HMGA1 or CPM. This represents the largest series of histopathologically confirmed cardiac lipomas from a single institution. In addition, it is the first to evaluate cardiac lipomas and LHAS for genetic alterations associated with extracardiac lipomatous lesions. The genetic and morphologic similarities found provide evidence in support of the neoplastic classification of cardiac lipomas. A single case of LHAS contained an HMGA2 rearrangement, challenging the currently accepted hypothesis of pathogenesis for this lesion. PMID:24996689

Bois, Melanie C; Bois, John P; Anavekar, Nandan S; Oliveira, Andre M; Maleszewski, Joseph J

2014-09-01

23

Lipomatous tumors of the oral mucosa: histomorphological, histochemical and immunohistochemical features.  

PubMed

We conducted a comprehensive study of all lipomatous tumors of the oral mucosa (1996-2008) accessioned at the Department of Oral Pathology, Tel Aviv University, collected demographic data and analyzed multiple histomorphological features. Furthermore, we examined the immunostaining of aP2 (adipocyte lipid binding protein) and the polarization colors of picrosirius red (PSR)-stained collagen fibers in order to test their potential in differentiating between benign and malignant tumors. All cases were immunohistochemically stained with aP2 antibody; only tumors with considerable collagenous stroma were selected for the PSR staining. A total of 77 tumors were included in the study, 91% benign and 9% malignant. Fibrolipoma (37.7%) and lipoma (36.4%) were the most frequent tumors. Atypical lipomatous tumor (ALT) was the only type of malignancy. The most common location for the benign tumors was the buccal mucosa and for ALT, the tongue. Histomorphological features characteristic of malignant tumors were occasionally present also in the benign entities. Expression of aP2 was similar in all tumors, while the polarization colors of the PSR-stained collagen fibers differed significantly between ALT and benign tumors (p<0.05). Benign and malignant tumors occasionally show overlapping histomorphological features that require a meticulous examination. PSR staining with polarization microscopy could aid in differentiating malignant from benign tumors in equivocal cases. PMID:21376375

Allon, Irit; Aballo, Sara; Dayan, Dan; Vered, Marilena

2011-12-01

24

Lipomatous congenital melanocytic nevus presenting as a neck mass in a young adult.  

PubMed

Congenital melanocytic nevus (CMN) is a melanocytic proliferation that has its onset at birth or shortly thereafter and shows characteristic histopathologic features including symmetric proliferation of benign melanocytes, extension of nevus cells into the deep reticular dermis and subcutis, maturation of melanocytes with descent, tracking of melanocytes around and within adnexal structures, vessels, or nerves and splaying of collagen bundles by nevus cells arranged in single rows or cords. We report the case of a 34 year old previously healthy woman who presented with a progressively enlarging soft tissue mass in the right neck and back adjacent to a medium sized CMN. Magnetic resonance imaging showed multiple lipomatous masses within the soft tissues of the posterior superficial neck. Subsequent excision of the soft tissue mass showed a well circumscribed lipomatous lesion with diffuse infiltration by benign appearing melanocytes within the fat lobules. Excision of the mass was not accompanied by overlying skin and, thus, posed a diagnostic challenge. Sudden increase in the size of a CMN is worrisome for the development of a melanoma, however, this lesion lacked significant cytologic atypia and mitotic figures, and had a low proliferative index by Ki-67 immunohistochemistry. This case serves to illustrate the initial diagnostic dilemma as well as the plasticity of the neural crest cells. PMID:23649716

Patel, Kalyani R; Chernock, Rebecca; Lewis, James S; Raptis, Constantine A; Al Gilani, Maha; Dehner, Louis P

2013-12-01

25

Lipomatous myofibroblastoma: a potential diagnostic pitfall in the spectrum of the spindle cell lesions of the breast  

Microsoft Academic Search

We report on two cases of myofibroblastoma (MFB) of the breast comprised predominantly of a mature fatty component, representing approximately three quarters of the entire tumour area. Both tumours consisted of a well-circumscribed lipomatous tumour mass containing dispersed nodular or irregularly shaped spindled cellular areas. The fatty component was represented exclusively by mature adipocytes, uniform in size and shape, lacking

Gaetano Magro; Michal Michal; Enrico Vasquez; Michele Bisceglia

2000-01-01

26

Intraneural lipomatous tumor of the median nerve: Three case reports with a review of literature  

PubMed Central

INTRODUCTION Intraneural lipoma and fibrolipomatous hamartoma of the nerve are rare soft tissue tumors that most commonly occur in the forearm and the wrist, and particularly within the median nerve. When the lesions are large enough, they may cause progressive compression neuropathy. They are distinct entities each other with different clinical and radiological findings and thereby need different surgical treatments. PRESENTATION OF CASE We report here 3 cases of intraneural lipomatous tumors of the median nerve (1 case of intraneural lipoma and 2 cases of fibrolipomatous hamartoma). DISCUSSION All patients were surgically treated successfully with complete excision for intraneural lipoma and with carpal tunnel releases for the both fibrolipomatous hamartomas. CONCLUSION A careful preoperative planning is necessary for the optimal treatment by distinguishing whether it is a resectable or non-resectable tumor based on the clinical and radiological findings, because they have characteristic findings each other. PMID:22705575

Okubo, Taketo; Saito, Tsuyoshi; Mitomi, Hiroyuki; Takagi, Tatsuya; Torigoe, Tomoaki; Suehara, Yoshiyuki; Katagiri, Hirohisa; Murata, Hideki; Takahashi, Mitsuru; Ito, Ichiro; Yao, Takashi; Kaneko, Kazuo

2012-01-01

27

YKL-40 expression in soft-tissue sarcomas and atypical lipomatous tumors  

PubMed Central

Background and purpose YKL-40 is a glycoprotein that is expressed in many types of cancer cells. In some cancers, there is a correlation between high serum YKL-40 levels on the one hand and more aggressive disease and early death on the other. YKL-40 has never been studied in patients with soft-tissue sarcomas (STSs). We investigated whether YKL-40 is expressed in STS tissue and ascertained that the degree of expression is related to survival and/or the histological grade of the malignancy (FNCLCC). Patients and methods We included archived tissue from 49 patients (40 with STS and 9 with atypical lipomatous tumor, 20 female and 29 male, mean age 58 (4–89) years) who were treated with tumor resection in 2004 or 2005 at the Department of Orthopedics, Rigshospitalet. The minimum length of follow-up with respect to survival was 5–7 years. Immunohistochemical analysis with anti-YKL-40 antibody using tissue microarray was performed on resected tumors, and a semiquantitative measure of the intensity of YKL-40 staining was performed. Results 41 of the 49 tumors were positive for YKL-40, and of these, 36 had moderate to intense staining. 24 of the patients died within the follow-up period, and the intensity of YKL-40 staining was significantly higher in tumors from patients who had died in the follow-up period than in tumors from those who survived (p = 0.01). The staining intensity was different for the 3 grades of malignancy (p = 0.004): it was higher in highly malignant tumors (FNCLCC grade 2 and grade 3) than in low-malignancy tumors (grade 1). Interpretation YKL-40 is expressed in soft-tissue sarcomas. There is a correlation between expression of YKL-40 in STS and both histological grade of the malignancy and survival. Whether or not YKL-40 expression is an independent prognostic variable could not be determined in the present study. PMID:24650028

2014-01-01

28

An update on plexiform fibrohistiocytic tumor and addition of 66 new cases from the Armed Forces Institute of Pathology, in honor of Franz M. Enzinger, MD.  

PubMed

The seminal article of Drs Franz Enzinger and Renyuan Zhang in 1988 defined plexiform fibrohistiocytic tumor (PFHT) as a distinctive entity. They described 65 cases (from 1965 to 1985) in children and young adults, with female and upper extremity predominance. These tumors were morphologically divided into 3 groups: fibroblastic, histiocytic (often with osteoclast-type giant cells), and mixed. Most tumors exhibited a plexiform and infiltrative arrangement of cells at the dermal/subcutaneous junction. Two fibroblastic PFHT had a metaplastic bone formation. Absence of cellular pleomorphism, low mitotic activity, dense hyalinization, hemorrhage, and chronic inflammation were observed. Vascular invasion was present in 1 recurrent, yet nonmetastatic, case. Tumors were negative for S100 protein, desmin, cytokeratin, factor VIIIrag, and lysozyme. Most patients were without disease up to 60 years after excision; 32 (37.5%) cases with follow-up recurred and 2 of those patients had regional lymph node metastasis at 9 and 36 months, respectively, yet there were no systemic metastases. In the interim, there have been additional studies on PFHT. We wanted to update the literature and add 66 new PFHT cases (1986-present) from the Armed Forces Institute of Pathology, since this seminal article, in honor of Dr Franz Enzinger. There were 37 men and 29 women; patient age ranged from 1 to 77 years (median, 20 years; 53% of patients were younger than 20 years). Twenty-eight cases occurred in the upper extremity (mostly forearm), 16 in lower extremity, 11 in trunk, 9 in head and neck, and 2 of unknown site. Although most cases were observed at the dermal/subcutaneous interface, 22 cases were predominantly dermal, and the rest predominantly subcutaneous, with 4 superficially involving skeletal muscle. Except for 12 predominantly dermal cases, most cases had an infiltrative growth pattern. Thirty-four cases were predominantly histiocytic, 16 predominantly fibroblastic, and the remaining 16 mixed. Two fibroblastic cases demonstrated the microfat cells (probably secondary to subcutis infiltration). All cases exhibited a plexiform growth pattern of small- to medium-sized nodules; 41 cases had giant cells, mainly osteoclast type, often the predominantly histiocytic type. The purely fibroblastic often had surrounding inflammation, 2 cases with marked inflammation. Perineural growth was observed in 5 cases, peri-Pacinian corpuscle growth in 2 cases, adnexal trapping in several, and, increased hyalinized collagen in 17 cases. Eight cases demonstrated focal myxoid change. Only 1 case, a histiocytic, had bone formation. Although increased cytologic atypia and mitotic activity were noted in a few cases, an atypical mitosis was only observed in 1 case. No cases demonstrated vascular or lymphatic invasion or necrosis. The tumors were generally positive for CD68 and SMA, occasionally for MSA, and negative for keratin, desmin, HMB45, S100 protein, and CD34. Overall, the findings were very similar to the original observations made by Dr Enzinger and his colleague, with the minor exceptions of roughly equal sex distribution (possibly due to timely referral bias), and additional morphologic features of myxoid change, adnexal sparing, increased inflammation, and microfat similar to recently described lipofibromatosis. The relationship between PFHT and cellular neurothekeoma is also explored. PMID:17870015

Moosavi, Christopher; Jha, Prakash; Fanburg-Smith, Julie C

2007-10-01

29

Atypical lipomatous tumor/well-differentiated liposarcoma of the gingiva: a case report and review of literature.  

PubMed

Liposarcoma, first described by Virchow in 1857, is a common mesenchymal malignant tumor arising from the adipose tissue. The most common of all soft tissue sarcomas, liposarcomas account for approximately 20% of all soft tissue sarcomas. However, it is rare in the head and neck, particularly in the oral cavity. Oral liposarcomas have been reported to occur mainly on the buccal mucosa, with other sites including the floor of the mouth, tongue, palate, and mandible. However, almost no cases of a liposarcoma located on the gingiva have been reported. To our knowledge, only 5 cases of liposarcoma of the gingiva have been previously reported in English language studies. We present a rare case of an atypical lipomatous tumor/well-differentiated liposarcoma of the gingiva of the anterior mandible that occurred in a 77-year-old male patient. Our patient underwent surgical excision and alveolar decortication. We also present a review of the current published data. At 18 months of follow-up, the patient remained free of disease. PMID:23992781

Kim, Young Bin; Leem, Dae Ho; Baek, Jin A; Ko, Seung O

2014-02-01

30

Pleomorphic hyalinizing angiectatic tumor arising in the thigh: A case report  

PubMed Central

Pleomorphic hyalinizing angiectatic tumors (PHATs) are rare non-metastasizing tumors of uncertain lineage. The current study presents a case of PHAT arising in the thigh of a 68-year-old female and examines the clinicopathological characteristics of the tumor. Magnetic resonance imaging (MRI) revealed an intramuscular mass located in the adductor longus. The tumor was surrounded by lipomatous tumor. Wide resectioning was performed for the internal tumor, whereas intralesional resectioning was performed for the external tumor. Histopathologically, the internal lesion was diagnosed as a PHAT and the external lesion was diagnosed as an hemosiderotic fibrolipomatous lesion (HFLL). No recurrence or metastases were identified during the 6-year follow-up period. As the adipose tissue surrounding the PHAT resembled a HFLL, therefore, the association between ‘early PHAT’ and HFLL is discussed. Although PHATs may represent low-grade sarcomas, HFLLs may be benign tumors. PMID:24944701

SUZUKI, KAYO; YASUDA, TAKETOSHI; HORI, TAKESHI; OYA, TAKESHI; WATANABE, KENTA; KANAMORI, MASAHIKO; KIMURA, TOMOATSU

2014-01-01

31

An unexpected cause of hemoptysis: endobronchial lipomatous hamartoma.  

PubMed

Hamartomas are the most common benign tumors of the lung. Endobnronchial hamartomas are even rarer and infrequently causes hemoptysis. We report a case of endobronchial hamartoma that was originating from a segment bronchus and invisible in chest X-ray. A 63-year-old man was admitted to hospital with hemoptysis. A CT scan revealed endobronchial mass obstructing anterior bronchus of the right lower lob of the right lung. It wasn't radiographically presented. Flexible bronchoscopy detected a polypoid mass (1.5 x 1.0 cm) that arising from the posterior wall of the anterior segment of right lower lob. Histopathologic examination revealed lipoumatous hamartoma. It was resected with an electro-surgical snare. Cryotherapy was applied to residual lesion on surface of the bronchus. The patient was successfully recovered. In conclusion, lipoumatous hamartoma may presented as rare cause of hemoptysis. Endoscopic treatment is safe and currently modality used for select cases. PMID:24783918

Sarioglu, Nurhan; Susur, Alev; Goksel, Tuncay; Paksoy, Serpil; Erel, Fuat

2014-01-01

32

Lipomatous hamartoma of the tricuspid valve: echocardiographic-pathologic correlations.  

PubMed

We report the eighth example of a cardiac valvular hamartoma (five mitral, three tricuspid). An intracavitary right atrial mass was present in a 76-year-old man and was characterized echocardiographically by a mobile cystic structure involving the septal tricuspid leaflet. Microscopically, the affected leaflet was comprised predominantly of mature adipose tissue. Formation of a valvular windsock in this case was attributed to the chronic effect of right ventricular pressure on tissue that was weaker than a normal leaflet. Valvular hamartomas should be included in the differential diagnosis of lesions that produce prolapsing, cystic, or windsock malformations of atrioventricular valves. PMID:2013183

Crotty, T B; Edwards, W D; Oh, J K; Rodeheffer, R J

1991-03-01

33

Dedifferentiated liposarcoma of the spermatic cord with a hemangioendothelioma-like component: a case report and review of the literature.  

PubMed

Atypical lipomatous tumor or well-differentiated liposarcoma/dedifferentiated liposarcoma (DDLPS) is the most frequent subtype of malignant adipocytic tumor. This tumor typically presents in late adult life, most commonly in the retroperitoneum, extremities, or spermatic cord. It has been reported that the dedifferentiated component consists mainly of high-grade sarcoma, including undifferentiated pleomorphic sarcoma, fibrosarcoma, and myxofibrosarcoma, and it has been recently reported that the dedifferentiated component can be also made up of a low-grade sarcomatous component. Therefore, the dedifferentiated areas exhibit a wide morphological spectrum that commonly includes fibroblastic/myofibroblastic and fibrohistiocytic tumors but very rarely includes vascular tumors. We present here the first reported case of DDLPS with a hemangioendothelioma-like component in the spermatic cord. PMID:23910173

Okano, Shinji; Yamamoto, Hidetaka; Kono, Shinji; Fujii, Hiroshi; Shirabe, Ken; Maehara, Yoshihiko; Oda, Yoshinao

2013-09-01

34

Rare Lipomatous Tumors with Osseous and/or Chondroid Differentiation in the Oral Cavity Report of Two Cases and Review of the Literature.  

PubMed

The purpose of this study was to determine the clinicopathological and immunohistochemical features of lipoma/fibrolipoma with rare occasions as osseous and/or chondroid differentiation in the oral cavity. Two cases of the tumors, who presented with a painless, relatively hard mass on the oral mucosa, were studied. These were consisted of a well-circumscribed mass of fatty tissue with chondroid and significant fibrous component intermixed with the lobules of fat cells with chondroid and woven bone component, respectively. Immunohistochemical study revealed that peripheral spindle cells around chondroid tissue stained diffusely for S-100 alpha & beta and Sox-9, though peripheral spindle cells around osteoid tissue only stained for RUNX-2. According to review of the literature, lipoma/fibrolipoma with osseous and/or chondroid differentiation was 18 cases. Also fibrolipoma with osseous and chondroid differentiation is the first to be reported here. These results indicated that the cartilage/bone is produced by differentiation of undifferentiated mesenchymal cells of stroma. PMID:20309407

Kuyama, Kayo; Fifita, Sisilia Fusi; Komiya, Masamichi; Sun, Yan; Akimoto, Yoshiaki; Yamamoto, Hirotsugu

2009-01-01

35

Spontaneous fibrous histiocytic neoplasms in rats.  

PubMed Central

A total of 85 spontaneous rat fibrohistiocytic tumours were evaluated histologically and assessed for the presence or absence of metastases. The overall incidence in controls from 2-year carcinogenicity studies was 2.7%. The tumours occurred principally in the subcutaneous and deep soft tissues, and generally appeared after 18 months of age. Four histological types were recognized: histiocytic (17%), pleomorphic (33%), cellular (17%) and very fibrous (33%). Histiocytic tumours were highly malignant, and most produced metastases. Pleomorphic and cellular neoplasms occasionally produced metastases and must be regarded as potentially malignant. Very fibrous lesions were essentially benign. The close resemblance, both histologically and biologically, between rat and human fibrohistiocytic neoplasms supports the use of the fibrohistiocytic concept in laboratory-animal pathology. Study of these rat tumours may provide insight into the development of human fibrohistiocytic neoplasms. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 6 Fig. 7 Fig. 8 Fig. 9 Fig. 10 PMID:6261779

Greaves, P.; Faccini, J. M.

1981-01-01

36

Detection of MDM2 gene amplification or protein expression distinguishes sclerosing mesenteritis and retroperitoneal fibrosis from inflammatory well-differentiated liposarcoma  

Microsoft Academic Search

Inflammatory liposarcoma is a variant of well-differentiated liposarcoma\\/atypical lipomatous tumor that consists of a mixture of lymphocytes, histiocytes, scattered atypical stromal cells, mature adipocytes, and rarely lipoblasts. When the inflammatory infiltrate predominates, the morphological features overlap with various fibroinflammatory disorders including sclerosing mesenteritis and retroperitoneal fibrosis, making the diagnosis difficult. Well-differentiated liposarcoma\\/atypical lipomatous tumor and dedifferentiated liposarcoma have characteristic molecular

Joshua Weaver; John R Goldblum; Sondra Turner; Raymond R Tubbs; Wei-Lein Wang; Alexander JF Lazar; Brian P Rubin

2009-01-01

37

Imaging and diagnostic strategy of soft tissue tumors in children  

Microsoft Academic Search

The diagnosis of a soft tissue mass in children is a common clinical situation. Most of the lesions are benign and can be\\u000a treated conservatively or by non-mutilating surgery. Nevertheless, the possibility of a malignant soft tissue tumor must be\\u000a systematically considered. The most frequent benign soft tissue lesions in children are vascular lesions, fibrous and fibrohistiocytic\\u000a tumors and pseudotumors,

Hervé Brisse; Daniel Orbach; Jerzy Klijanienko; Paul Fréneaux; Sylvia Neuenschwander

2006-01-01

38

Atrophic dermatofibroma*  

PubMed Central

Dermatofibroma is a benign fibrohistiocytic tumor, common and easily diagnosed when classical clinicopathologic features are present. The atrophic variant of dermatofibroma is of uncertain origin. This lesion is characterized clinically by a flat or atrophic and depressible surface. Histopathological features show reduction of the thickness of the dermis and elastic fibers. We report a typical case of this uncommon and probably underdiagnosed variant. PMID:24173186

Mota, Amanda Nascimento C. de Macedo; Tortelly, Violeta Duarte; Obadia, Daniel Lago; da Silva, Roberto Souto

2013-01-01

39

CLOVES syndrome.  

PubMed

A cohort of patients with overgrowth syndromes has been identified with congenital lipomatous overgrowth, dysregulated fat deposits, and mixed vascular malformations. The acronym CLOVES was given on a heuristic basis to stand for congenital lipomatous overgrowth (CLO), vascular malformation (V), epidermal nevi (E), and scoliosis and spinal deformities (S). These patients have upper limb anomalies with variable phenotypes. Although hand anomalies alone cannot make the diagnosis, the foot, truncal, cutaneous and spinal anomalies are particularly diagnostic. CLOVES syndrome has emerged as a distinct clinical entity diagnosed by clinical and radiographic examinations. The overgrowth pattern is now easily distinguished from other overgrowth syndromes. PMID:24161472

Bloom, Jacob; Upton, Joseph

2013-12-01

40

Giant fibrolipoma in the mediastinum: an unusual case.  

PubMed

A 50-year-old man presented with a middle and posterior mediastinal mass on chest radiograph and computed tomography. Surgical exploration revealed a large dumbbell-shaped lipomatous lesion. Histologic examination confirmed this to be a fibrolipoma. This is the first reported case of fibrolipoma in the mediastinum but outside of the esophagus. PMID:16181831

Hsu, Jui-Sheng; Kang, Wan-Yi; Liu, Gin-Chung; Kao, Eing-Long; Chuang, Ming-Tsung; Chou, Shah-Hwa

2005-10-01

41

Atypical spindle cell lipoma: a clinicopathologic, immunohistochemical, and molecular study emphasizing its relationship to classical spindle cell lipoma.  

PubMed

We studied a series of spindle cell lipomas arising in atypical sites and showing unusual morphologic features (which we called atypical spindle cell lipoma) to assess if these lesions have the same chromosomal alterations as classical spindle cell lipoma but different from those found in atypical lipomatous tumor/well-differentiated liposarcoma. We investigated alterations of different genes in the 13q14 region and the amplification status of the MDM2 and CDK4 genes at 12q14-15 by multiplex ligation-dependent probe amplification (MLPA) and fluorescence in situ hybridization (FISH) analysis. In the atypical spindle cell lipomas, MLPA revealed deletions in the two nearest flanking genes of RB1 (ITM2B and RCBTB2) and in multiple important exons of RB1. In contrast, in classical spindle cell lipomas, a less complex loss of RB1 exons was found but no deletion of ITM2B and RCBTB2. Moreover, MLPA identified a deletion of the DLEU1 gene, a finding which has not been reported earlier. We propose an immunohistochemical panel for lipomatous tumors which comprises of MDM2, CDK4, p16, Rb, which we have found useful in discriminating between atypical or classical spindle cell lipomas and other adipocytic neoplasms, especially atypical lipomatous tumor/well-differentiated liposarcoma. Our findings strengthen the link between atypical spindle cell lipoma and classical spindle cell lipoma, and differentiate them from atypical lipomatous tumor/well-differentiated liposarcoma. PMID:24659226

Creytens, David; van Gorp, Joost; Savola, Suvi; Ferdinande, Liesbeth; Mentzel, Thomas; Libbrecht, Louis

2014-07-01

42

Neural fibrolipoma: an unusual case.  

PubMed

A 45-year-old gentleman presented with a diffuse left neck mass. Surgical exploration revealed a large lipomatous lesion. Histological examination identified this to be a neural fibrolipoma. This is the first reported case of this lesion in the neck. PMID:10829120

Thornton, M A; O'Leary, G; Fitzgibbon, J

2000-03-01

43

Rectal angiolipoma: A case report and review of literature  

PubMed Central

Angiolipoma is a rare vascular variant of the benign lipomatous tumors and is generally seen in subcutaneous tissues. We report a 70-year-old female with abdominal distension not related to rectal small polypoid mass with peduncule described as angiolipoma by histologically, and review the literature. PMID:17457984

Kacar, Sabite; Kuran, Sedef; Temucin, Tulay; Odemis, Bulent; Karadeniz, Nilufer; Sasmaz, Nurgul

2007-01-01

44

Pathology Case Study: Metastasizing Tumor  

NSDL National Science Digital Library

This is a case study presented by the University of Pittsburgh Department of Pathology in which a woman presented with a low-grade sarcoma with features of plexiform fibrohistiocytic tumor in the subcutaneous soft tissue of left posterior thigh. Visitors can view both gross and microscopic descriptions, including images, and have the opportunity to diagnose the patient. This is an excellent resource for students in the health sciences to familiarize themselves with using patient history and laboratory results to diagnose disease. It is also a helpful site for educators to introduce or test students of soft tissue pathology.

Rao, Uma N.; Rostami, Sassan

2009-02-17

45

Plantar angiomyxolipoma in a child  

PubMed Central

Angiomyxolipoma, a lipoma variant with myxoid areas and vascular proliferation was originally described in 1996 and till date has only 12 cases in published literature. Only two cases have been reported in children involving buccal mucosa and knee, respectively. The authors report a case of angiomyxolipoma, on the plantar surface of the left foot, in a 4-year-old male child who presented to our institution in Abha city (Kingdom of Saudi Arabia). The significant differential diagnosis of this neoplasm from other similar lipomatous tumours occurring in adult and paediatric population is discussed. The importance of recognising these tumours lies in their recognition as separate entity and the present case may add to the knowledge, clinical behaviour and prognosis of these less reported lipomatous neoplasms. PMID:22674110

Shraim, Mubarak Al; Hasan, Mahboob; Hawan, Ali; Radad, Khaled; Eid, Refaat

2011-01-01

46

Pure uterine lipoma.  

PubMed

Lipomatous tumors of the uterus are unusual, benign neoplasms seen in postmenopausal women. Although many of the mixed-type cases such as lipoleiomyoma and fibrolipoma have been reported, pure uterine lipomas are extremely rare. In the literature, a few cases with pure uterine lipoma have been reported. We first present the advanced magnetic resonance findings of pure uterine lipoma, followed by those of ultrasonography (US) and computed tomography (CT). We markedly detected lipid peaks on the magnetic resonance spectroscopy (MRS) and the apparent diffusion coefficient value to be 0.00 due to chemical-shift effects with diffusion-weighted imaging (DWI). Although pelvic lipomatous tumors can be diagnosed with US and CT, in some cases, further workup may be required to localize the lesion. MRI may yield more valuable data for differential diagnosis. MRS and DWI findings provide additional clues on the nature of the lesion. PMID:17905250

Erdem, Gulnur; Celik, Onder; Karakas, Hakki Muammer; Alkan, Alpay; Hascalik, Seyma

2007-10-01

47

A Neonate with CLOVES Syndrome  

PubMed Central

Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome is a recently delineated disorder that comprises vascular malformations (typically truncal), dysregulated adipose tissue, scoliosis, enlarged bony structures (typically of the legs) without progression, or distorting bony overgrowth. The name CLOVE was subsequently extended to CLOVES to emphasize the association with scoliosis/skeletal and spinal anomalies and seizures/central nervous system malformations. We herein report a very rare case of CLOVES syndrome with the findings of lipomatous overgrowth in the cheek (facial asymmetry), vascular malformation (hemangiomas), epidermal nevi (large port wine stains), and skeletal abnormalities (widened first interdigital space, dystrophia in the nail of the first digit of the right foot, and bilateral hypertrophy of the first digits of the feet).

Akin, Mustafa Ali; Kurtoglu, Selim; Tubas, Filiz; Sarici, Serdar Umit

2014-01-01

48

Oncocytic lipoadenoma of the parotid gland: a report of a new case and review of the literature  

PubMed Central

Oncocytic lipoadenoma is a rare salivary gland tumour composed of adipose tissue and oncocytic epithelial cells in varied proportions. This tumour is still not included in the current WHO classification of salivary gland neoplasms. We herein report a further case of oncocytic lipoadenoma originating in the parotid gland of a 55-year-old woman. The tumour presented as a slowly growing asymptomatic left-sided parotid gland mass. The resected tumour measured 2.7 cm in maximum diameter and was composed of oncocytoma-like epithelial component admixed with mature adipocytes that made up 10% of the whole mass. Foci of sebaceous differentiation were seen. This rare variant of lipomatous salivary gland tumours is in need of more recognition and should be distinguished from other fat-containing salivary gland lesions, particularly lipomatous pleomorphic adenoma and myoepithelioma. PMID:23119120

Mitsimponas, Konstantinos T; Agaimy, Abbas; Schlittenbauer, Tilo; Nkenke, Emeka; Neukam, Friedrich-Wilhelm

2012-01-01

49

Neural fibrolipoma of a digital nerve of the index finger without macrodactyly.  

PubMed

We present a case of neural fibrolipoma arising from the digital nerve in the index finger of the right hand. A 31-year-old man was referred with a soft tissue mass in the ulnar aspect of the index finger of his right hand, which had gradually enlarged during the past seven years. Histological examination of an excisional biopsy specimen identified a neural fibrolipoma, which is a differential diagnosis of a lipomatous lesion of the digits. PMID:20158413

Avci, Gülden; Akan, Mithat; Taylan, Gaye; Akoz, Tayfun

2010-11-01

50

Macrodactyly-Lipofibromatous Hamartoma of Nerves  

Microsoft Academic Search

\\u000a Lipofibromatous hamartoma of nerve (LFHN) is a very uncommon benign lipomatous tumor with specific clinicopathological characteristics which may present with\\u000a or without macrodactyly. This tumor-like lesion is composed of fibrous and fatty tissues arising from the epi- and perineurium\\u000a that surrounds and infiltrates the major nerves and their branches in the body (Enzinger and Weiss 1994). It is believed to

Carola Duràn-Mckinster; Luz Orozco-Covarrubias; Marimar Saez-De-Ocariz; Ramòn Ruiz-Maldonado

51

Fat-containing salivary gland tumors: a review.  

PubMed

Fat-containing tumors of the salivary glands are uncommon. Their wide histological spectrum varies from pure lipomatous neoplasms similar to their cutaneous and soft tissue counterparts to mixed lipoepithelial lesions specific to the salivary glands. With few exceptions, these uncommon lesions affect mainly the elderly, with a mean age at presentation of ? 50 years and show a predilection for males. A few cases occur in childhood; some of them represent congenital lesions. In decreasing order of frequency, ordinary (soft-tissue type) lipoma, oncocytic lipoadenoma, non-oncocytic sialolipoma, and pleomorphic adenoma/myoepithelioma with extensive lipometaplasia are the main variants of fat containing tumors encountered in the salivary glands. While pleomorphic adenoma/myoepithelioma with lipometaplasia behave in the same way as their non-fat-containing counterparts, other lipomatous salivary gland tumors listed above are cured with simple excision and do not carry a risk of recurrence. Other lipoma variants (spindle cell lipoma, osteolipoma, fibrolipoma, angiolipoma, pleomorphic lipoma, lipoblastoma and hibernoma) are exceptionally rare in the salivary gland. Atypical lipomatous tumors/liposarcoma have been only rarely reported in the salivary gland and they behave in a similar fashion to their soft-tissue counterparts. Diffuse lipomatosis and lobular fatty atrophy are the two tumor-like lesions that might closely mimic sialolipoma, particularly in limited biopsy material without knowledge of the gross findings. This review summarizes the clinicopathological features of the main types of salivary fat-containing lesions and discusses their differential diagnoses. PMID:23821211

Agaimy, Abbas

2013-07-01

52

Laparoscopic resection of a retroperitoneal myolipoma presenting in a right inguinal hernia?  

PubMed Central

INTRODUCTION Myolipoma of soft tissue is an extremely rare benign lipomatous lesion. The lesions are most commonly located in the abdominal cavity, retroperitoneum, and inguinal areas. Despite their large size, myolipomas are cured by surgical resection. PRESENTATION OF CASE We present the case of a 79 year-old man who presented with bilateral reducible inguinal hernias (right larger than left). After reducing the right inguinal hernia (RIH), the sensation of a palpable mass was noted in the right iliac fossa. CT scan suggested the content of the right inguinal hernia (RIH) to be small bowel mesentery and no other mass was noted in the right iliac fossa (possibly missed on CT scan). DISCUSSION A very large 1.8 kg retroperitoneal lipomatous lesion, measuring 22 cm × 16 cm × 8 cm, attached to the right spermatic cord was found and excised laparoscopically during a trans-abdominal pre-peritoneal (TAPP) approach to repair the hernias. The lesion was pathologically defined as a myolipoma. CONCLUSION The laparoscopic TAPP approach to repair inguinal hernias allows the surgeon to inspect the peritoneal cavity, and in this case it was possible to safely dissect and remove a large, lipomatous, retroperitoneal lesion laparoscopically. To the best of our knowledge, there are no reports of local recurrence, metastatic disease, or malignant transformation of myolipomas, and the laparoscopic approach to resect such a lesion has not been reported. PMID:23995475

Dan, Dilip; Bascombe, Nigel; Harnanan, Dave; Naraynsingh, Vijay

2013-01-01

53

Composite tumor consisting of dermatofibrosarcoma protuberans and giant cell fibroblastoma associated with intratumoral endometriosis. Report of a case.  

PubMed

We present a unique case of composite skin tumor of the vulva consisting of dermatofibrosarcoma protuberans (DFSP) and giant cell fibroblastoma (GCF) with an intratumoral focus of endometriosis. A 31-year-old female with a 10-year-history of a recurring subcutaneous tumor in the vulvar area underwent excision of the seventh recurrence of the tumor. Microscopic examination revealed a composite fibrohistiocytic tumor consisting of DFSP and GCF. Additionally, a focus of endometriosis within the tumor tissue was found. Malignant transformation of extragonadal endometriosis has already been described; we present, however, the occurrence of a focus of endometriosis within the tissue of a hormonally independent soft tissue tumor. There is a possible link to the occurrence of cutaneous endometriosis at previous surgery sites and in the scars. The presence of endometriosis within the soft tissue tumor represents, to the best of our knowledge, a previously undescribed collision phenomenon. PMID:11358013

Kholová, I; Ryska, A; Dedic, K

2001-01-01

54

Dermatofibrosarcoma protuberans in childhood.  

PubMed

Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy that is very rare in childhood. Only 6% of these tumors present in children. Clinical diagnosis is very difficult in the early stages of disease, but to ensure appropriate treatment it is important to identify DFSP as early as possible and rule out benign conditions that are more common at this age. The clinical presentation and histopathologic and molecular characteristics of DFSP are similar in children and adults. Clinical diagnosis is, however, more difficult in children and requires a high degree of suspicion. The absence of characteristic features and the rarity of this tumor explain why diagnosis is often delayed. Complete surgical excision of the tumor is very important to reduce the risk of recurrence. This article presents a review of current knowledge about the management of DFSP in children and examines the latest treatment options. PMID:23154247

Valdivielso-Ramos, M; Hernanz, J M

2012-12-01

55

Dermatofibrosarcoma Protuberans in Childhood.  

PubMed

Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy that is very rare in childhood. Only 6% of these tumors present in children. Clinical diagnosis is very difficult in the early stages of disease, but to ensure appropriate treatment it is important to identify DFSP as early as possible and rule out benign conditions that are more common at this age. The clinical presentation and histopathologic and molecular characteristics of DFSP are similar in children and adults. Clinical diagnosis is, however, more difficult in children and requires a high degree of suspicion. The absence of characteristic features and the rarity of this tumor explain why diagnosis is often delayed. Complete surgical excision of the tumor is very important to reduce the risk of recurrence. This article presents a review of current knowledge about the management of DFSP in children and examines the latest treatment options. PMID:22482741

Valdivielso-Ramos, M; Hernanz, J M

2012-12-01

56

The significance of double phenotypic patterns and markers in human sarcomas. A new model of mesenchymal differentiation.  

PubMed Central

Six soft-tissue sarcomas with two separate and juxtaposed histologic patterns were selected for immunohistochemical analysis. The first pattern was represented by five phenotypes (schwannian-skeletal muscle [Triton], cartilagenous, synovial, adipocytic, and smooth muscle). In each case the second histologic pattern resembled the fibrohistiocytic phenotype, ie, malignant fibrous histiocytoma (MFH). No other histologic patterns were identified. Appropriate cell markers were demonstrated in each of the first patterns; these were not detected in the second patterns. In contrast, the second pattern in all cases expressed alpha 1-antichymotrypsin, a marker commonly found in fibrohistiocytic lesions; this was not identified in any of the first patterns. This loss of one cell-specific marker and gain of another is termed the "antigenic shift" phenomenon and appeared to foretell the emergence of a true second phenotype (the same in each of these cases, which could be termed "dedifferentiated" sarcomas). Therefore, it is hypothesized that MFH is a final common pathway for some types of sarcomas and is the result of tumor progression or "dedifferentiation." The practical implications of this hypothesis concern the approach to sarcoma differential diagnosis and the meaning of an MFH pattern in both metastatic and primary sites. On a theoretic level, this hypothesis and the antigenic shift phenomenon force a reconsideration of the pathways of soft-tissue differentiation. A new model of mesenchymal differentiation incorporating these concepts is described and supported. It provides an explanation for a number of facts in soft-tissue pathology, and its predictions can be tested. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 PMID:3490790

Brooks, J. J.

1986-01-01

57

Utility of fluorescence in situ hybridization to detect MDM2 amplification in liposarcomas and their morphological mimics  

PubMed Central

The atypical lipomatous tumor (ALT)/well-differentiated liposarcoma (WDLS) and the de-differentiated liposarcoma (DDLS) represent the most common category of liposarcomas. ALT/WDLSs and DDLSs are often difficult to distinguish from other tumors with similar morphological characteristics. In this study, we investigated whether the detection of amplified or overexpressed murine double-minute 2 (MDM2) can be a useful diagnostic ancillary aid. We used fluorescent in situ hybridization (FISH) and immunohistochemistry (IHC) to detect MDM2 amplification and protein overexpression, respectively, in 49 WDLSs, 5 DDLSs, 23 myxoid liposarcomas, 25 benign lipomatous tumors, and 75 spindle and pleomorphic sarcomas. MDM2 amplification was detected in 48 of 49 WDLSs, 5 of 5 DDLSs, 2 of 9 malignant peripheral nerve sheath tumors, and 2 of 10 myxofibrosarcomas. We did not detect MDM2 amplification in any of the benign lipomatous tumors. FISH-mediated detection of MDM2 amplification was the most valuable diagnostic aid for ALT/WDLS, as determined by using the Fisher exact test to compare two different diagnoses of 19 biopsies. On the contrary, unequivocal nuclear overexpression of MDM2 was found in only 10 of 50 ALT/WDLSs. The sensitivity and specificity of MDM2 amplification in distinguishing a DDLS from spindle and pleomorphic sarcomas were 100% and 95%, respectively, while those of MDM2 overexpression were 100% and 87%, respectively. In conclusion, our results indicate that FISH-mediated detection of MDM2 amplification is the most useful adjunct in the diagnosis of both ALT/WDLS and DDLS. However, IHC-mediated detection of MDM2 protein is useful only for the diagnosis of DDLS. PMID:23826411

Kimura, Hiroaki; Dobashi, Yoh; Nojima, Takayuki; Nakamura, Hiroyuki; Yamamoto, Norio; Tsuchiya, Hiroyuki; Ikeda, Hiroko; Sawada-Kitamura, Seiko; Oyama, Takeru; Ooi, Akishi

2013-01-01

58

Identification of COL3A1 and RAB2A as novel translocation partner genes of PLAG1 in lipoblastoma.  

PubMed

Lipoblastoma is a rapidly growing, benign neoplasm in children. Surgical excision is usually curative, with a recurrence rate of about 20%. Because the histology of lipoblastoma is heterogeneous and overlaps with other lipomatous tumors, some lipoblastoma cases have been difficult to diagnose. The detection of PLAG1 gene rearrangement is useful for the diagnosis of lipoblastoma. Three fusion partner genes are known in relation to PLAG1 in lipoblastoma HAS2 at 8q24.1, COL1A2 at 7q22, and RAD51L1 at 14q24. Herein, we describe another two novel fusion genes in lipoblastoma tumor specimens. We checked six tumors for the presence of two known fusion genes, HAS2-PLAG1 and COL1A2-PLAG1. Only HAS2-PLAG1 was found in one of the cases. Next, we attempted to identify potential PLAG1 fusion partners using 5'RACE. Sequence analysis revealed two novel fusion genes, COL3A1-PLAG1 in three cases and RAB2A-PLAG1 in one case, respectively. As a result of the translocations, the constitutively active promoter of the partner gene drives the ectopic expression of PLAG1. We also evaluated whether a high level of PLAG1 expression can be used to help differentiate lipomatous tumors. PLAG1 expression was evaluated by real-time PCR in five lipoblastoma tumor specimens. The expressions were 70-150 times higher in lipoblastomas than in human adipocytes. However, PLAG1 expression was low in one case of lipoma. These results demonstrate that PLAG1 overexpression is a potential marker of lipoblastoma. Our findings, in agreement with previous studies, show that lipoblastoma is a group of lipomatous tumors with PLAG1 rearrangement and overexpression. © 2014 Wiley Periodicals, Inc. PMID:24700772

Yoshida, Hideki; Miyachi, Mitsuru; Ouchi, Kazutaka; Kuwahara, Yasumichi; Tsuchiya, Kunihiko; Iehara, Tomoko; Konishi, Eiichi; Yanagisawa, Akio; Hosoi, Hajime

2014-07-01

59

Myofibroblastoma of the Female Breast with Admixed but Distinct Foci of Spindle Cell Lipoma: A Case Report  

PubMed Central

Mammary myofibroblastoma (MFB) is a rare benign spindle neoplasm that affects both sexes with a male predominance. It can exhibit a wide range of histological patterns. We report a case of epithelioid/spindle MFB of the female breast with admixed, but distinct, foci of spindle cell lipoma. Whilst all the spindle cells within the tumour expressed CD34, AR, ER, BCL2, and CD10, only those within the myofibroblastoma expressed desmin and only those within the lipomatous areas expressed S100. This finding, to our knowledge, is a novel one that has not been reported before. PMID:24459597

Ibrahim, Hazem A. H.

2013-01-01

60

Lipofibromatosis: report of a rare paediatric soft tissue tumour.  

PubMed

The clinical, radiological and pathological features of a case of lipofibromatosis, a rare paediatric soft tissue neoplasm, are described. The tumour involved the foot of a male infant and was present at birth. Magnetic resonance imaging showed a lipomatous mass, with splaying of muscles of the sole by lobules of fat. Histopathological examination revealed typical findings of an admixture of mature adipose tissue and fibroblastic elements. The radiological and pathological features helpful in differentiating this entity from other fibro-fatty paediatric soft tissue tumours is discussed, and the relevant literature is briefly reviewed. PMID:18278490

Deepti, A N; Madhuri, Vrisha; Walter, Noel Malcolm; Cherian, Rekha Aley

2008-06-01

61

Intrapericardial Giant Lipoma Displacing the Heart  

PubMed Central

Despite their benign character, intrapericardial lipomas can cause life-threatening complications by rapid growth. This paper presents a case of an intrapericardial lipoma in an almost asymptomatic 41-year-old female patient only suffering from mild dyspnoea on exertion. The tumour was found incidentally by chest X-ray. Echocardiographic examination and a CT scan of the thorax revealed a 16 × 14 × 12?cm lipomatous tumour mass highly suspective of a lipoma. Histological examination of excised tumour specimens confirmed the diagnosis of a lipoma. The patient is currently asymptomatic and has not presented with evidence of recurrence at the 6-month followup. PMID:22347636

Steger, C. M.

2011-01-01

62

Spindle cell lipoma of the mandibular mucogingival junction: a case report of unusual oral neoplasm  

PubMed Central

Spindle cell lipoma (SCL) is a benign lipomatous neoplasm typically located in the posterior neck and back of older males. It presents as a well-circumscribed mass in the buccal mucosa, tongue, floor of the mouth or hard palate. There are only two case reports of SCL in the gingiva and alveolar ridge. Here, we report a case of SCL in the mandibular mucogingival junction of a 68-year-old male. Clinical, histopathological and immunohistochemical findings are presented. Although oral SCL is rare, it should be considered in the differential diagnosis of spindle cell neoplasms occurring in the oral cavity. PMID:24556953

Al Sheddi, Manal Abdulaziz; Assari, Ahmad; Mosadomi, Hezekiah

2014-01-01

63

Fibrolipomatous Hamartoma of the Median Nerve in the Elbow: A Case Report  

PubMed Central

A fibrolipomatous hamartoma—also known as a fibrofatty overgrowth, perineural lipoma, intraneural lipoma, and lipomatous hamartoma—is a rare, benign, congenital lesion most commonly found in the median nerve, usually at the level of the wrist or hand. To our knowledge, no published cases report a hamartoma arising from the median nerve at the level of the elbow. We report a case of a fibrolipomatous hamartoma in a 55-year-old woman that necessitated a surgical intervention because of its size and associated neurologic symptoms. PMID:22778681

Ha, Jennifer Fong; Teh, Bing Mei; Abeysuriya, Disna Thushangi Dahanayake; Luo, Daniel Y. W.

2012-01-01

64

Spindle cell lipoma of the mandibular mucogingival junction: a case report of unusual oral neoplasm.  

PubMed

Spindle cell lipoma (SCL) is a benign lipomatous neoplasm typically located in the posterior neck and back of older males. It presents as a well-circumscribed mass in the buccal mucosa, tongue, floor of the mouth or hard palate. There are only two case reports of SCL in the gingiva and alveolar ridge. Here, we report a case of SCL in the mandibular mucogingival junction of a 68-year-old male. Clinical, histopathological and immunohistochemical findings are presented. Although oral SCL is rare, it should be considered in the differential diagnosis of spindle cell neoplasms occurring in the oral cavity. PMID:24556953

Al Sheddi, Manal Abdulaziz; Assari, Ahmad; Mosadomi, Hezekiah

2014-09-01

65

Mimicking liver iron overload using liposomal ferritin preparations.  

PubMed

Close monitoring of liver iron content is necessary to prevent iron overload in transfusion-dependent anemias. Liver biopsy remains the gold standard; however, MRI potentially offers a noninvasive alternative. Iron metabolism and storage is complicated and tissue/disease-specific. This report demonstrates that iron distribution may be more important than iron speciation with respect to MRI signal changes. Simple synthetic analogs of hepatic lysosomes were constructed from noncovalent attachment of horse-spleen ferritin to 0.4 microm diameter phospholipid liposomes suspended in agarose. Graded iron loading was achieved by varying ferritin burden per liposome as well as liposomal volume fraction. T1 and T2 relaxation times were measured on a 60 MHz NMR spectrometer and compared to simple ferritin-gel combinations. Liposomal-ferritin had 6-fold stronger T2 relaxivity than unaggregated ferritin but identical T1 relaxivity. Liposomal-ferritin T2 relaxivity also more closely matched published results from hemosiderotic marmoset liver, suggesting a potential role as an iron-calibration phantom. PMID:15004804

Wood, John C; Fassler, Joe D; Meade, Tom

2004-03-01

66

Chordoma Cutis - A Diagnosis not to be Missed  

PubMed Central

Chordomas are rare midline tumors of the bone usually arising from sacrum, skull bones and spine, close to neuraxis. However an extensive involvement of the soft tissues can simulate a soft tissue subcutaneous tumour of the gluteal region –a presentation called chordoma cutis. Our patient presented with a gluteal mass and a trucut biopsy was done suspecting a soft tissue tumour. The hematoxylin and eosin stained section of the biopsy closely simulated a lipomatous tumour. However, on closer inspection the clear cells were found to have very fine vacuolations.The usual myxoid background and characteristic physaliferous cells seen in chordomas were not seen. Still a differential of chordoma was entertained in view of the site and age of the patient. Immunohistochemistry for cytokeratin and S-100 was performed and both were unambiguously positive. On literature search, we came across a soft tissue tumour called parachordoma which mimics chordoma both morphologically and immunohistochemically and has also been reported in the gluteal region. An MRI was performed which showed the tumour to be arising from the sacrum and secondarily involving the gluteal soft tissues. This case highlights the importance of considering chordoma in the differential diagnosis of gluteal masses with clear cell morphology even in the absence of physaliferous cells and myxoid background before signing them out as lipomatous tumours. PMID:25120995

Mishra, Kiran

2014-01-01

67

Chordoma Cutis - A Diagnosis not to be Missed.  

PubMed

Chordomas are rare midline tumors of the bone usually arising from sacrum, skull bones and spine, close to neuraxis. However an extensive involvement of the soft tissues can simulate a soft tissue subcutaneous tumour of the gluteal region -a presentation called chordoma cutis. Our patient presented with a gluteal mass and a trucut biopsy was done suspecting a soft tissue tumour. The hematoxylin and eosin stained section of the biopsy closely simulated a lipomatous tumour. However, on closer inspection the clear cells were found to have very fine vacuolations.The usual myxoid background and characteristic physaliferous cells seen in chordomas were not seen. Still a differential of chordoma was entertained in view of the site and age of the patient. Immunohistochemistry for cytokeratin and S-100 was performed and both were unambiguously positive. On literature search, we came across a soft tissue tumour called parachordoma which mimics chordoma both morphologically and immunohistochemically and has also been reported in the gluteal region. An MRI was performed which showed the tumour to be arising from the sacrum and secondarily involving the gluteal soft tissues. This case highlights the importance of considering chordoma in the differential diagnosis of gluteal masses with clear cell morphology even in the absence of physaliferous cells and myxoid background before signing them out as lipomatous tumours. PMID:25120995

Tanveer, Nadeem; Mishra, Kiran

2014-06-01

68

Objective parameters aid the prediction of fistulas in pancreatic surgery  

PubMed Central

Insufficiency of pancreatic anastomosis with leakage from the pancreatic stump and the development of fistulas account for the majority of surgical complications following pancreatic resection, which are often life threatening. The cause of pancreatic fistulas of the remnant tissue on a molecular level remains unclear. Thus, the aim of the present study was to investigate risk factors associated with postoperative pancreatic fistula (POPF) formation and to define parameters that may predict the resection outcome. Pancreatic resection margins were selected from 31 patients, including 16 individuals without and 15 patients with POPF, to analyze the degree of fibrosis, lipomatous atrophy, inflammatory activity and infiltration. Wound healing factors were assessed by luminex technology using tissue homogenates, while the distribution in situ was assessed using immunohistochemistry. Increased chronic inflammatory infiltration, a higher degree of fibrosis and a reduction in lipomatous atrophy were observed in the samples without anastomotic fistulas. Multiplex analysis of 38 wound healing factors demonstrated significantly higher levels of interleukin (IL)-6, -8 and -12, glucagon-like peptide-1 and matrix metalloproteinase (MMP)-1, -2, -3 and -12 in the group without fistulas, while lower concentrations of IL-10, IL-17 and gastric inhibitory polypeptide were observed. Therefore, the observations of the present study indicated that increased inflammatory infiltration and inflammatory activity, as well as higher concentrations of proinflammatory cytokines and higher MMP levels at the resection margins, predisposed individuals to a lower fistula incidence rate following pancreatic resection. PMID:25120588

FELIX, KLAUS; SCHUCK, ANNA; GAIDA, MATTHIAS M.; HINZ, ULF; DOVZHANSKIY, DMITRIY; WERNER, JENS

2014-01-01

69

Labile iron in cells and body fluids: physiology, pathology, and pharmacology  

PubMed Central

In living systems iron appears predominantly associated with proteins, but can also be detected in forms referred as labile iron, which denotes the combined redox properties of iron and its amenability to exchange between ligands, including chelators. The labile cell iron (LCI) composition varies with metal concentration and substances with chelating groups but also with pH and the medium redox potential. Although physiologically in the lower ?M range, LCI plays a key role in cell iron economy as cross-roads of metabolic pathways. LCI levels are continually regulated by an iron-responsive machinery that balances iron uptake versus deposition into ferritin. However, LCI rises aberrantly in some cell types due to faulty cell utilization pathways or infiltration by pathological iron forms that are found in hemosiderotic plasma. As LCI attains pathological levels, it can catalyze reactive O species (ROS) formation that, at particular threshold, can surpass cellular anti-oxidant capacities and seriously damage its constituents. While in normal plasma and interstitial fluids, virtually all iron is securely carried by circulating transferrin (Tf; that renders iron essentially non-labile), in systemic iron overload (IO), the total plasma iron binding capacity is often surpassed by a massive iron influx from hyperabsorptive gut or from erythrocyte overburdened spleen and/or liver. As plasma Tf approaches iron saturation, labile plasma iron (LPI) emerges in forms that can infiltrate cells by unregulated routes and raise LCI to toxic levels. Despite the limited knowledge available on LPI speciation in different types and degrees of IO, LPI measurements can be and are in fact used for identifying systemic IO and for initiating/adjusting chelation regimens to attain full-day LPI protection. A recent application of labile iron assay is the detection of labile components in intravenous iron formulations per se as well as in plasma (LPI) following parenteral iron administration. PMID:24659969

Cabantchik, Zvi Ioav

2014-01-01

70

Lymphohistiocytoid mesothelioma: a rare lymphomatoid variant of predominantly sarcomatoid mesothelioma  

SciTech Connect

Of 394 ''definite'' mesotheliomas entered in the Australian Mesothelioma Surveillance Program, three bore a striking resemblance to malignant lymphoma by conventional light microscopy, and each was misinterpreted at some stage as lymphoma. The lymphoma-like morphology was a combined result of intense lymphoplasmacytic infiltration and the histiocytoid appearances of the underlying neoplastic cell population. Immunocytochemical analysis demonstrated cytokeratins coexpressed with vimentin within the tumor cells, whereas immunoreactivity for leukocyte common antigen was confined to the smaller lymphoid cells. Electron microscopy of two cases revealed a polymorphous population of fibrohistiocytic cells resembling those typical of malignant fibrous histiocytoma, admixed with lymphocytes and plasma cells, but sporadic cells expressed mesothelial properties in the form of sinuous villiform processes, intracytoplasmic neolumina lined by microvilli, and intermediate filaments that were aggregated into tonofilament bundles in some cells. The ultrastructural appearances, the localization of the tumors to the pleura, with effusion, and absence of anterior mediastinal mass lesions facilitated exclusion of lymphocyte-rich thymoma. In addition, a history of prior occupational exposure to asbestos was elicited in each instance. There was no apparent response to radiotherapy or chemotherapy, and the patients died at 4, 5, and 8 months after presentation. Our observations suggest that immunocytochemical or ultrastructural evaluation is mandatory for accurate diagnosis of all pleura-based lymphomatoid lesions with a mixed large and small cell pattern. 42 references.

Henderson, D.W.; Attwood, H.D.; Constance, T.J.; Shilkin, K.B.; Steele, R.H.

1988-01-01

71

Expression of aminopeptidase N (CD13) in mesenchymal tumors.  

PubMed

For a long time, CD13 molecules have been considered to be restricted to myeloid cells and related neoplasms. Meanwhile, however, expression of CD13 has also been detected in some hepatocellular, gallbladder, renal, and lung carcinomas, and even in some fibrosarcomas and malignant melanomas. In this study, expression of CD13 antigen was immunohistochemically examined in non-neoplastic mesenchymal cells, along with 33 benign and 83 malignant mesenchymal tumors (MET) using CD13 monoclonal antibodies (MAb) My7, U71, WM-15, and MoU48. In non-neoplastic mesenchymal cells, expression of CD13 was restricted to perivascular fibrocytes/blasts, tissue histiocytes, osteoclasts, and to the perineurium of peripheral nerve trunks. Under neoplastic conditions, CD13 was detectable in some tumors of smooth muscle, fibrous, fibrohistiocytic, synovial, osteogenic, and peripheral nerve sheath origin, and even in some tumors of adipose tissue. Tumors of striated muscle origin, of autonomic ganglia, and of cartilage-forming tissues were CD13-negative throughout. Thus in most but not all tumors studied the pattern of expression of CD13 mirrors the situation found in their cells of origin. These findings enrich the data on expression of leukocyte differentiation antigens in extra-hematopoietic tissues. Expression of CD13, which meanwhile is known to be identical to aminopeptidase N, an important peptide-cleaving enzyme, in only some MET might reflect a special functional state of these neoplasms. PMID:1978569

Mechtersheimer, G; Möller, P

1990-11-01

72

Painful Late Recurrence of Dermatofibrosarcoma Protuberans of Breast in a Centurian Female  

PubMed Central

Dermatofibrosarcoma protuberans (DFSP) of the breast is rare. Late and painful local recurrence of this entity on this site is even more uncommon. We describe such a case in a 102-year-old woman, who at the age of 77 years had been operated for a breast lump for which only a cytopathological diagnosis of fibrohistiocytic tumour was available. Twenty years later, she noticed a small mass in her right breast over the postsurgical scar area, which gradually increased in size over the last five years. She presented to the surgical out-patient clinic with pain and redness over the swelling. Wide local excision of the tumor with generous tissue margin was performed under intercostal block on account of her age and suboptimal cardiac status. The microscopic and immunohistochemical findings established the diagnosis of recurrent DFSP. We report an exceptionally rare case of local recurrence of DFSP in the female breast and discuss in detail the diagnostic and therapeutic implications of this pathology. PMID:24298499

Roy, Asitava Deb; Nishant, Kumar; Joshi, Deepti

2013-01-01

73

Lipofibromatosis: magnetic resonance imaging features and pathological correlation in three cases.  

PubMed

Lipofibromatosis is a rare, benign, but infiltrative, soft tissue tumor seen in children. We present three cases of lipofibromatosis, each with different magnetic resonance imaging features and correlate this with the histological findings. The patients comprised two males and one female who presented in infancy; at birth, 5 months, and 7 months of age. Clinically, the masses were painless and slow-growing. The masses ranged in size from 2 to 6 cm and involved the distal extremities in two cases (one foot, one wrist) and the trunk. Magnetic resonance imaging showed lipomatous lesions with varying amounts of adipose and solid components in each case. There were no capsules at the periphery of the lesions. One case showed a fat-predominant lesion, another an equal mixture of fat and solid tissue, and the third was predominantly solid. This was reflected in the histology, which showed corresponding features. Radiological and histopathological differential diagnoses are reviewed. PMID:24509814

Vogel, Daniela; Righi, Alberto; Kreshak, Jennifer; Dei Tos, Angelo Paolo; Merlino, Biagio; Brunocilla, Eugenio; Vanel, Daniel

2014-05-01

74

Lipofibromatosis of the knee in a 19-month-old child.  

PubMed

Lipofibromatosis is a rare benign fibrofatty tumor of childhood. The typical presentation of this tumor is as a poorly demarcated and slow-growing mass involving the subcutaneous or deep soft tissues. Lipofibromatosis was first described in 2000, and since then, a small number of cases have been reported in the literature. We report a case of a 19-month-old boy who presented with a swelling of the anterior aspect of the right knee since birth, which had increased in size out of proportion with his growth. Magnetic resonance imaging was extremely useful because it showed the lipomatous nature of the mass, narrowing the differential diagnosis to the pediatric fibrofatty soft tissue tumors. The histologic biopsy revealed the specific diagnosis of lipofibromatosis. We describe the radiologic and pathologic features of this entity and discuss the differential diagnosis in a young child with a fat-containing limb mass. PMID:22595596

Costa Dias, Sílvia; McHugh, Kieran; Sebire, Neil J; Bulstrode, Neil; Glover, Mary; Michalski, Antony

2012-05-01

75

Spindle cell lipoma of the parapharyngeal space: first report of a case.  

PubMed

Spindle cell lipomas are usually located in the subcutaneous tissue of the back, shoulders, and neck. To our knowledge, the presence of such a tumor in the parapharyngeal space has not yet been described. We evaluated a 45-year-old man with a tender swelling of the right parotid area that had reached the submandibular area. Clinical examination and magnetic resonance imaging revealed the presence of a tumor that coated the parotid area laterally and extended into the center of the parapharyngeal space, thus causing a dislocation of the pharyngeal muscles and mucosa. We performed a total parotidectomy and submandibulectomy on the right side and extirpated the parapharyngeal tumor. We were able to spare the facial nerve, and no facial paralysis occurred. Histologic examination revealed an atypical lipomatous tumor with a remarkably large portion of spindles. PMID:11338650

Baumann, I; Dammann, F; Horny, H P; Plinkert, P K

2001-04-01

76

Fibrolipoma of the ring finger: MR imaging and histological correlation.  

PubMed

Fibrolipoma is characterized by the presence of prominent bundles of mature fibrous tissue traversing the fatty lobules. We present a case of a pathologically-proven fibrolipoma arising in the right ring finger of a 66-year-old female. Physical examination showed a 2-cm, soft, mobile, nontender mass. Neurovascular examinations including Tinel sign were normal. Plain radiographs revealed a well-defined radiolucent area with no calcification. Magnetic resonance imaging showed a lipomatous tumor with an unusual biphasic pattern. The patient underwent an excisional biopsy. Histologically, the tumor consisted of mature adipocytes with sclerotic fibrous elements as well as myxoid changes. The patient has had no evidence of local recurrence within seven months of follow-up. To the best of our knowledge, this is the first report of fibrolipoma without nerve involvement in the finger. Although nonspecific, clinicians should know the various imaging features of fibrolipoma to avoid an unnecessarily extensive surgery. PMID:23812228

Nishio, Jun; Ideta, Soshi; Aoki, Mikiko; Hamasaki, Makoto; Nabeshima, Kazuki; Iwasaki, Hiroshi; Naito, Masatoshi

2013-01-01

77

Myolipoma Affecting the Erector Spinae: A Case Report in a Child  

PubMed Central

Myolipoma is a rare, benign, lipomatous tumour which most commonly occurs in the retroperitoneum, pelvis, and abdomen. A 4-year-old boy presented with a painless enlarging mass in the left paraspinal region. Magnetic Resonance Imaging (MRI) revealed a soft tissue lesion with high fat content and areas of calcification. Excision and histopathological analysis revealed a tumour composed of lobules of mature adipose tissue and broad septa of well-differentiated smooth muscle tissue. The smooth muscle nature of the nonfatty component was demonstrated by a diffuse and strong immunoreactivity for smooth muscle actin and desmin. The mass was reported as a myolipoma. The patient made an unremarkable recovery from surgery and remains healthy with no signs of recurrence at seven years. This paper represents the youngest patient diagnosed with this rare soft tissue tumour which is normally confined to the adult population. A newly reported site of the tumour is also highlighted. PMID:19902012

Parratt, M. T. R.; Gokaraju, K.; Spiegelberg, B. G. I.; Miles, J.; Cannon, S. R.; Briggs, T. W. R.

2009-01-01

78

Neoplasms involving the heart, their simulators, and adverse consequences of their therapy  

PubMed Central

Primary cardiac tumors involving the heart may be either benign or malignant. Most of the benign tumors are myxomas, which are most commonly located in the left atrium. Primary malignant neoplasms usually involve the myocardium and the interior of the cardiac cavities, whereas neoplasms metastatic to the heart most commonly involve pericardium, and pericardial effusion and constriction are the most common consequences. Computed tomography and magnetic resonance imaging are becoming the most useful instruments of precision for the diagnosis of cardiac tumors. Pericardial cysts, teratomas, lipomatous hypertrophy of the atrial septum, papillary fibroelastomas, thrombi, and sarcoid are frequently mistaken for cardiac neoplasms. There are a number of cardiac consequences of malignancy, including radiation heart disease, cardiac hemorrhages, cardiac infection, cardiac adiposity or the corticosteroidtreated heart, cardiac hemosiderosis, and toxicity due to anthracycline chemotherapy. PMID:16369647

2001-01-01

79

Lumbar spinal angiolipoma: case report and review of the literature.  

PubMed

Spinal angiolipomas are extremely rare benign tumors composed of mature lipomatous and angiomatous elements. Most are symptomatic due to progressive spinal cord or root compression. This article describes the case of a 60-year-old woman who presented with a 6-month history of low back pain radiating to her right leg. The pain was multisegmental. The condition had worsened with time. Lumbar magnetic resonance imaging revealed a dorsal epidural mass at L5 and erosion of the lamina of the L5 vertebra. Laminectomy was performed, and an extradural tumor was totally excised. Neuropathologic examination identified it as a lumbar spinal angiolipoma. There was no evidence of recurrence in follow-up 12 months later. This rare clinical entity must be considered in the differential diagnosis for any spinal epidural lesion. PMID:16172903

Konya, Deniz; Ozgen, Serdar; Kurtkaya, Ozlem; Pamir, Necmettin M

2006-06-01

80

Morphological diversity of trichodiscomas and fibrofolliculomas.  

PubMed

Trichodiscomas (TDs) and fibrofolliculomas (FFs) are hamartomatous tumors, which are usually small facial papules; they are frequently a disease marker for Birt-Hogg-Dubé syndrome, their recognition is important because of this association. Some believe that TD and FF is the same lesion, others have proposed the term "mantleoma" to include the morphological continuum of both of these lesions. Recently, some variants (eg, spindle cell TD, TD with lipomatous metaplasia) have been described. The authors attempt to describe a review of cases with combined features of TF and FF, as well as the recently described variants, and variants not previously described to our knowledge (myxoid/mucin predominant, pseudocartilaginous, sclerotic/fibrotic, prominent apocrine/eccrine, leiomyoma-like, with vascular proliferation). It has been hypothesized that the hair bulge stem cells may be responsible for the broad spectrum of histological variation in these tumors. PMID:24786577

Lopez-Garcia, Dan R; Teague, Daniel; Landis, Erin T; Sangueza, Omar P

2014-09-01

81

Incidental lipoma-like hibernoma arising from the adrenal gland: a well-differentiated liposarcoma mimicker.  

PubMed

Hibernomas are uncommon benign lipomatous tumors which show differentiation toward brown fat. To our knowledge, only one case of adrenal hibernoma has been previously reported. We describe a 55-year-old woman showing an incidental, 1.7 cm-hibernoma associated with a 2.6 cm-cortical adenoma producing primary hyperaldosteronism (Conn's syndrome), both in the left adrenal gland. The hibernoma was composed predominantly of univacuolated mature fat cells admixed with small vessels. Scattered areas composed of large multivacuolated pale cells with central or paracentral nuclei, mimicking lipoblasts, accounting for less than 30% of the tumor, were found. These cells lacked nuclear hyperchromasia or marked atypia, were S100-positive, and showed numerous mitochondria reactive with the anti-mitochondrial antibody. A diagnosis of lipoma-like hibernoma was made. Pathologists should be aware of this variant of hibernoma to avoid misdiagnosis and excessive treatment. PMID:24002005

Val-Bernal, José Fernando; Azueta, Ainara; Ortiz-Rivas, Luis Antonio; Fuentes, Javier; Ballestero, Roberto

2013-12-01

82

Pleomorphic lipoma lacking mature fat component in extensive myxoid stroma: a great diagnostic challenge  

PubMed Central

Pleomorphic lipoma is a relatively uncommon entity, and is considered as a variant of spindle cell lipoma. Histologically, spindle cell lipoma/pleomorphic lipoma consists of varying quantity of mature fat, bland spindle cells and ropey collagen. In addition, pleomorphic lipoma is characterized by multinucleate giant cells, which possess the “floret-like” nuclei and marked pleomorphism. So, in contrast to spindle cell lipoma, pleomorphic lipoma is more easily misdiagnosed as a malignant tumor. Herein, we report a peculiar case of pleomorphic lipoma occurring in axilla with entirely devoid of mature fat in a 71-year-old male. The histopathological findings demonstrated the tumor was made up of bland spindle cells admixed with scattered “floret-like” cells and irregular ropey collagen in an extensive myxoid stroma. Immunostaining showed that the tumor was positive for the Vimentin, Bcl-2 and CD34, and was negative for S-100, desmin, CD68, and ?–SMA. Although no fat component was found in the whole section, the tumor was still diagnosed as a pelomprphic lipoma. To our knowledge, this is the third reported case of pelomprphic lipoma which entirely lacked lipomatous component. Because of the existence of atypical multinucleate giant cells and lack of mature fat, this tumor may be easily misdiagnosed nonlipomatous lesions, such as myxoid fibrosarcoma, giant cell fibroblastoma. So, it is necessary to pay careful attention to the histological spectrum of pleomorphic lipoma, including the tumor with devoid of fat, and it should be kept in mind that pelomprphic lipoma still can be diagnosed even if lacking lipomatous component. Virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1967123180611361 PMID:23148444

2012-01-01

83

TGFBR3 and MGEA5 rearrangements in pleomorphic hyalinizing angiectatic tumors and the spectrum of related neoplasms.  

PubMed

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare, locally aggressive tumor of the distal extremities with a proclivity for local recurrence. PHATs contain characteristic ectatic, thin-walled vessels, lined by fibrin, and are surrounded by groups of variably pleomorphic spindled to epithelioid neoplastic cells. The putative precursor lesion of PHAT, originally termed "early PHAT" shares many clinicopathologic features with hemosiderotic fibrolipomatous tumor (HFLT). HFLT, myxoinflammatory fibroblastic sarcoma (MIFS), and tumors showing hybrid features of HFLT and MIFS often show TGFBR3 and MGEA5 gene rearrangements. To date, only a small number of PHATs has been tested for either rearrangement; all have been negative. We hypothesized that PHATs contain TGFBR3 and/or MGEA5 rearrangements. Cases of PHAT (all containing areas of HFLT) (N=10), HFLT (N=7), MIFS (N=6), hybrid HFLT/MIFS (N=3), and PHAT-like undifferentiated pleomorphic sarcomas (N=7) were retrieved from our institutional and consultation archives and analyzed for TGFBR3 and MGEA5 rearrangements using a break-apart probe strategy for FISH. Six of 10 PHATs harbored TGFBR3 and/or MGEA5 gene rearrangements: 4 cases had both TGFBR3 and MGEA5 rearrangements, and 2 cases contained MGEA5 rearrangements. Two of 7 HFLTs were positive: 1 case had a TGFBR3 rearrangement, and 1 case had an MGEA5 rearrangement. One of 6 MIFSs had an MGEA5 rearrangement. All 3 hybrid HFLT/MIFS cases were positive: 2 cases had both TGFBR3 and MGEA5 rearrangements, and 1 case had a TGFBR3 rearrangement. All PHAT-like undifferentiated pleomorphic sarcomas were negative. We report, for the first time, the presence of TGFBR3 and/or MGEA5 rearrangements in tumors showing mixed features of HFLT and PHAT. The presence of such rearrangements strongly suggests that HFLT is related to both PHAT and MIFS and that the latter 2 tumors may represent morphologic variants of a single, genetically defined entity in which only MIFS has acquired the capacity to metastasize. PMID:24705316

Carter, Jodi M; Sukov, William R; Montgomery, Elizabeth; Goldblum, John R; Billings, Steven D; Fritchie, Karen J; Folpe, Andrew L

2014-09-01

84

Tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue: new entities and new variants of old entities recorded during the last 25 years. Part XII: appendix.  

PubMed

In an eleven part series published in Pathologica, we have presented various tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue (ST), which emerged as new entities or as variants of established entities during the last quarter of a century. Detailed clinicomorphological and differential diagnostic features of approximately sixty entities were chosen on the basis of their clinical significance and morphologic distinctiveness. The series included fibrous and myofibroblastic tumors (e.g. solitary fibrous tumor, high grade classic and pigmented dermatofibrosarcoma protuberans, inflammatory myofibroblastic tumor and myofibrosarcomas), fibromyxoid and fibrohistiocytic neoplasms (e.g., Evans' tumor, phosphaturic mesenchymal tumor, inflammatory myxohyaline tumor), special adipocytic/vascular/and smooth muscle lesions (e.g., chondroid lipoma, Dabska's tumor, ST hemangioblastoma, lipoleiomyosarcoma), epithelioid mesenchymal malignancies of diverse lineages (e.g., epithelioid liposarcoma, proximal-type epithelioid sarcoma, neuroendocrine extraskeletal chondromyxoid sarcoma), ST Ewing's tumor and peripheral nerve sheath tumors (perineuriomas and pigmented and rosetting tumors of the schwannoma/neurofibroma group), extranodal dendritic or histiocytic proliferative processes (follicular dendritic cell sarcoma, Rosai-Dorfman disease, Castleman's disease, and plexiform xanthomatous tumor), and tumors with myoepithelial differentiation. The section devoted to selected pseudotumoral entities considered representatives of the hamartoma group (neural fibrolipomatous hamartoma, ectopic hamartomatous thymoma, rudimentary meningocele), metabolic diseases (amyloid tumor, nephrogenic fibrosing dermopathy, tophaceous pseudogout, pseudoinfiltrative parathyromatosis), stromal tissue reactions to trauma (fibroosseous pseudotumors of digits) and infections (bacillary angiomatosis), and normal organs (glomus coccygeum). To conclude the descriptive phase, supplementary material has now been collected and appended in an attempt to provide a quick digest of essential knowledge both for comparison and differential diagnosis. The data have been tailored to synthesize diverse sources, integrating clinical elements and references to articles that previously appeared in Part I ("Introduction"), Part II ("The List and Review of New Entities") and Parts III to XI ("Excerpta"). At the very least we hope this final part ("Appendix") will provide the reader with a useful tabular organization of ST lesions and a reference resource. PMID:17175794

Bisceglia, M; Spagnolo, D; Galliani, C; Fisher, C; Suster, S; Kazakov, D V; Cooper, K; Michal, M

2006-08-01

85

Contributions of Cytogenetics and Molecular Cytogenetics to the Diagnosis of Adipocytic Tumors  

PubMed Central

Over the last 20 years, a number of tumor-specific chromosomal translocations and associated fusion genes have been identified for mesenchymal neoplasms including adipocytic tumors. The addition of molecular cytogenetic techniques, especially fluorescence in situ hybridization (FISH), has further enhanced the sensitivity and accuracy of detecting nonrandom chromosomal translocations and/or other rearrangements in adipocytic tumors. Indeed, most resent molecular cytogenetic analysis has demonstrated a translocation t(11;16)(q13;p13) that produces a C11orf95-MKL2 fusion gene in chondroid lipoma. Additionally, it is well recognized that supernumerary ring and/or giant rod chromosomes are characteristic for atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma, and amplification of 12q13–15 involving the MDM2, CDK4, and CPM genes is shown by FISH in these tumors. Moreover, myxoid/round cell liposarcoma is characterized by a translocation t(12;16)(q13;p11) that fuses the DDIT3 and FUS genes. This paper provides an overview of the role of conventional cytogenetics and molecular cytogenetics in the diagnosis of adipocytic tumors. PMID:21274402

Nishio, Jun

2011-01-01

86

Wilms' tumor protein (WT1) in mammary myofibroblastoma: an immunohistochemical study.  

PubMed

Wilms' tumor protein (WT1) has been immunohistochemically detected in the cytoplasm of some developing, adult normal and neoplastic human tissues, suggesting its complex regulator activity in transcriptional/translational processes. Among neoplastic tissues, WT1 has been documented in the cytoplasm of benign and malignant vascular tumors and in rhabdomyosarcoma, while there are no available studies about its expression in myofibroblastic tumors. Accordingly, we studied immunohistochemically the potential expression of WT1 in mammary myofibroblastoma (MFB), a prototypical myofibroblastic tumor. A series of 18 cases of mammary MFB, including several morphological variants (classic, fibrotic, myxoid, lipomatous, Schwannian-like, and epithelioid variants), were tested with antibodies against the N-terminal of WT1. The most striking finding was a diffuse and strong WT1 cytoplasmic immunostaining restricted to the "epithelioid cell MFB", a rare and diagnostically challenging variant. Conversely the other variants of MFB, including the classic-type, were negative or only focally positive. The present study shows that mammary epithelioid cell MFB should be added to the list of mesenchymal tumors which express WT1 in the cytoplasm of neoplastic cells. Accordingly, we suggest that the detection of WT1 cytoplasmic immunoreactivity is of complementary diagnostic value to conventional myofibroblastic markers in identifying epithelioid cell myofibroblastoma. PMID:24709316

Magro, Gaetano; Longo, Francesca; Salvatorelli, Lucia; Vecchio, Giada Maria; Parenti, Rosalba

2014-06-01

87

Lipofibromatosis arising in a pediatric forearm--a case report.  

PubMed

Lipofibromatosis is a rare pediatric tumour described by Fetsch et al. in 2000. There have been few reports about this tumour and few descriptions of its imaging features. We report a case of this tumour forming a slowly growing, painless mass arising in the forearm of a ten-month-old boy. This is the first report of lipofibromatosis to present the radiological findings before surgery. Plain radiography and computed tomography demonstrated a tumour located just beneath the fascia. Ultrasonic study showed a hyperechoic tumour of 33 x 30 x 7 mm in size. Magnetic resonance imaging demonstrated a multilobular mass with high signal intensities on both T1/T2 weighted images. Macroscopically, the tumour was poorly marginated. Microscopically, the tumour was composed of abundant adipose tissues transversed by fibroblastic bands. The adipose tissue occupied over 70% of the tumour. Adipocytes and fibroblastic element infiltrated into the normal skeletal muscle tissues. Recognition of this clinical entity is needed, especially in cases of lipomatous tumours arising in pediatric hands and feet. PMID:16454165

Sasaki, Daizo; Hatori, Masahito; Hosaka, Masami; Watanabe, Mika; Kokubun, Shoichi

2005-01-01

88

Dynamic 18F-fluorodeoxyglucose positron emission tomography/CT in hibernoma: Enhanced tracer uptake mimicking liposarcoma  

PubMed Central

We report on two cases of patients with fat-equivalent masses in computed tomography (CT), referred to our department for dynamic positron emission tomography/CT (dPET/CT) with 18F-fluorodeoxyglucose (18F-FDG) in order to investigate their dignity. Both qualitative and quantitative information, as derived from dPET/CTs, couldn’t exclude a high-grade liposarcoma: Visual evaluation, revealed a large hypermetabolic focus of intense 18F-FDG uptake in each patient (average SUVs 8.3 and 11.3). Regression-based parametric imaging demonstrated an enhanced distribution volume, which correlates to perfusion, and a high phosphorylation rate that correlates to cell viability. Kinetic analysis, based on a two-tissue compartment model demonstrated an enhanced FDG transport k1 and an enhanced phosphorylation rate k3. A non-compartmental approach based on fractal dimension revealed also enhanced values. However, final diagnosis was based on biopsy, which revealed hibernoma, a benign brown fat tumor. Brown adipose contains increased numbers of mitochondria and a high-rate of glucose metabolism. Therefore, they have increased FDG uptake. The evaluation of lipomatous lesions on CT, with high FDG uptake, should include the possibility of hibernoma as a differential diagnosis. PMID:24379937

Sachpekidis, Christos; Roumia, Safwan; Schwarzbach, Matthias; Dimitrakopoulou-Strauss, Antonia

2013-01-01

89

Posterior mediastinal ganglioneuroma with peripheral replacement by white and brown adipocytes resulting in diagnostic fallacy from a false-positive 18F-2-fluoro-2-deoxyglucose-positron emission tomography finding: a case report  

PubMed Central

Introduction Ganglioneuroma is a rare tumor in the posterior mediastinum; fat-containing ganglioneuromas are rarely reported. The present case report documents a brown fat-containing, posterior mediastinal ganglioneuroma, which has not been reported previously. Radiological examination, in particular 18F-2-fluoro-2-deoxyglucose-positron emission tomography, suggested that the tumor had low-grade malignant potential. This led to uncertainty at preoperative diagnosis. Case presentation An asymptomatic 66-year-old Japanese woman with no significant past medical history was referred for the evaluation of a posterior mediastinal mass. Although its size had not changed in the past 5 years, a malignant lipomatous tumor could not be excluded due to the presence of intratumoral fat and increased 18F-2-fluoro-2-deoxyglucose uptake observed by positron emission tomography imaging. A computed tomography-guided core-needle biopsy revealed a mixture of mature adipocytes, spindle-shaped cells, and fibrotic stroma. Definite diagnosis was not possible, and surgical resection was performed. Three years after the surgery, she remains disease-free. Conclusions Histological diagnosis of the surgically resected mass confirmed ganglioneuroma with substantial amounts of white and brown adipose tissues in peripheral areas. The existence of both ganglion cells and brown fat tissue intensified the accumulation of 18F-2-fluoro-2-deoxyglucose, resulting in a false-positive result by positron emission tomography. Considering this, ganglioneuroma should not be excluded either clinically or pathologically in fat-containing, posterior mediastinal tumors. PMID:25319096

2014-01-01

90

The hypointense liver lesion on T2-weighted MR images and what it means.  

PubMed

The vast majority of focal liver lesions are hyperintense on T2-weighted magnetic resonance (MR) images. Rarely, however, hepatic nodules may appear totally or partially hypointense on those images. Causes for this uncommon appearance include deposition of iron, calcium, or copper and are related to the presence of blood degradation products, macromolecules, coagulative necrosis, and other conditions. Although rare, low signal intensity relative to surrounding liver on T2-weighted images may be seen in a wide spectrum of lesions. Examples include cases of focal nodular hyperplasia, hepatocellular adenoma, hepatocellular carcinoma, metastases, leiomyoma, siderotic or dysplastic nodules, nodules in Wilson disease, granuloma, and hydatid cyst. On fat-suppressed T2-weighted images, nodules with a lipomatous component, such as lipoma, angiomyolipoma, hepatocellular adenoma, and hepatocellular carcinoma may also appear partially or totally hypointense. The conjunction of other MR imaging findings and their integration in the clinical setting may allow a correct diagnosis in a considerable proportion of cases. The cause for T2-weighted hypointensity may not be, however, always recognized, and only pathologic correlation may provide the answer. The aims of this work are to discuss the causes and mechanisms of hypointensity of liver lesions on T2-weighted images and proposing an algorithm for classification that may be useful as a quick reminder for the interested reader. PMID:19901085

Curvo-Semedo, Luís; Brito, Jorge B; Seco, Miguel F; Costa, João F; Marques, Cristina B; Caseiro-Alves, Filipe

2010-01-01

91

An overview on development and application of an experimental platform for quantitative cardiac imaging research in rabbit models of myocardial infarction  

PubMed Central

To exploit the advantages of using rabbits for cardiac imaging research and to tackle the technical obstacles, efforts have been made under the framework of a doctoral research program. In this overview article, by cross-referencing the current literature, we summarize how we have developed a preclinical cardiac research platform based on modified models of reperfused myocardial infarction (MI) in rabbits; how the in vivo manifestations of cardiac imaging could be closely matched with those ex vivo macro- and microscopic findings; how these imaging outcomes could be quantitatively analyzed, validated and demonstrated; and how we could apply this cardiac imaging platform to provide possible solutions to certain lingering diagnostic and therapeutic problems in experimental cardiology. In particular, tissue components in acute cardiac ischemia have been stratified and characterized, post-infarct lipomatous metaplasia (LM) as a common but hardly illuminated clinical pathology has been identified in rabbit models, and a necrosis avid tracer as well as an anti-ischemic drug have been successfully assessed for their potential utilities in clinical cardiology. These outcomes may interest the researchers in the related fields and help strengthen translational research in cardiovascular diseases. PMID:25392822

Feng, Yuanbo; Bogaert, Jan; Oyen, Raymond

2014-01-01

92

Neural fibrolipoma of the superficial peroneal nerve in the ankle: a case report with immunohistochemical analysis.  

PubMed

This report presents a case of neural fibrolipoma arising from the superficial peroneal nerve in the ankle. A 28-year-old woman was referred with a soft tissue mass in the anterior aspect of the right ankle, which had been gradually enlarging for the past 10 years. Magnetic resonance imaging showed a mass lesion, measuring approximately 8 x 3 x 2 cm, with high to partially low signal intensity on both T1- and T2-weighted images. A band of low signal intensity within the lesion, which is indicative of coexistence with the tumor and the superficial peroneal nerve, could be detected on both T1- and T2-weighted images. The patient underwent an excisional biopsy. The specimen microscopically consisted of nerve bundles and fibro-fatty proliferation with abundant collagen fibers. Immunoreactivity for CD34 antigen antibody was detected in fibrous spindle cells. This is the first report to present an immunohistochemical profile of neural fibrolipoma. Neural fibrolipoma should be considered as a differential diagnosis when a lipomatous lesion is encountered in the foot or ankle as well as in the upper extremities. PMID:12685550

Akisue, Toshihiro; Matsumoto, Keiji; Yamamoto, Tetsuji; Kizaki, Tomohiko; Fujita, Ikuo; Yoshiya, Shinichi; Kurosaka, Masahiro

2002-11-01

93

p63 immunohistochemical staining is limited in soft tissue tumors.  

PubMed

p63 is a p53 homolog that is expressed in various normal epithelial tissues and epithelial malignancies. Its expression in mesenchymal lesions has not been examined in depth; therefore, we studied p63 expression by immunohistochemical analysis in 650 soft tissue tumors. We found that p63 expression is limited in soft tissue tumors. The majority of tumors studied were p63-, including all cases of angiosarcoma, lipomatous neoplasms, dermatofibrosarcoma protuberans, solitary fibrous tumor, schwannoma, neurofibroma, gastrointestinal stromal tumor, and leiomyosarcoma. Nuclear p63 reactivity was found in a subset of soft tissue myoepithelioma and myoepithelial carcinoma of soft tissue, cellular neurothekeoma, soft tissue perineurioma, Ewing sarcoma/peripheral neuroectodermal tumor, diffuse-type giant cell tumor, and giant cell tumor of soft parts. Infrequent, weak, or focal p63-staining patterns were observed in low-grade fibromyxoid sarcoma, malignant peripheral nerve sheath tumor, extraskeletal myxoid chondrosarcoma, myxofibrosarcoma, proximal-type epithelioid sarcoma, synovial sarcoma, embryonal rhabdomyosarcoma, desmoplastic small round cell tumor, atypical fibroxanthoma, and spindle cell melanoma. Absent p63 expression is typical for most soft tissue tumors, including most (but not all) that would be in the differential diagnosis of spindle cell squamous carcinoma. PMID:22031315

Jo, Vickie Y; Fletcher, Christopher D M

2011-11-01

94

Resection of soft tissue tumors extending through the obturator ring.  

PubMed

Pelvic soft tissue sarcomas are rare tumors often presenting larger than other soft tissue sarcomas and can extend into the thigh through various anatomic routes. Surgical resection is the main modality of curative treatment. En bloc tumor excision with wide, negative margins may reduce the risk of local recurrence. Soft tissue sarcomas extending through the obturator foramen create unique challenges to operative management. This case report describes 2 cases of lipomatous lesions that extend through the obturator foramen, presenting as dumbbell-shaped lesions with large intra- and extrapelvis portions. One possible surgical approach performed in both patients is detailed with long-term follow-up. Postoperatively, 1 incidence of infection was reported. Functional outcomes were acceptable, with full restoration of ambulation without assistive devices in both cases and no hernia observed. Oncologic outcomes included locoregional recurrence in 1 patient at 24 months outside the radiation field. The ideal primary treatment for all localized soft tissue sarcomas, including those extending through the obturator foramen, is resection. However, the unique subgroup of obturator ring soft tissue sarcomas has undefined outcomes and complications. The authors' goal was to achieve en bloc resection with wide negative margins while preserving ipsilateral limb function. The surgical approach described in this case study offers a description of feasibility and discussion of theoretical and observed complications. PMID:24025018

Starks, Alexandria; Guo, Lifei; Abraham, John A

2013-09-01

95

Prenatal diagnosis of CLOVES syndrome confirmed by detection of a mosaic PIK3CA mutation in cultured amniocytes.  

PubMed

Congenital lipomatous asymmetric overgrowth of the trunk, lymphatic, capillary, venous, and combined-type vascular malformations, epidermal nevi, skeletal and spinal anomalies (CLOVES) syndrome, a segmental overgrowth syndrome, is caused by post zygotic somatic mutations in PIK3CA, a gene involved in the receptor tyrosine kinase phosphatidylinositol 3-kinase (PI3)-AKT growth-signaling pathway. Prenatal ultrasound findings of lymphovascular malformations, segmental overgrowth and skeletal defects can raise suspicion for CLOVES syndrome, but molecular confirmation of PIK3CA mutations on prenatally obtained samples is challenging because of somatic mosaicism. We detected a mosaic disease-causing mutation in PIK3CA by sequencing of DNA extracted from cultured amniotic cells, but not from DNA directly prepared from an amniotic fluid sample in a fetus with prenatally suspected CLOVES syndrome. The infant was born prematurely and displayed severe lymphovascular malformations and segmental overgrowth consistent with a clinical diagnosis of CLOVES syndrome; he passed away at 29 days of life. We discuss the complexities and limitations of genetic testing for somatic mosaic mutations in the prenatal period and highlight the potential need for multiple approaches to arrive at a molecular diagnosis. © 2014 Wiley Periodicals, Inc. PMID:25044986

Emrick, Lisa T; Murphy, Lauren; Shamshirsaz, Alireza A; Ruano, Rodrigo; Cassady, Christopher I; Liu, Liu; Chang, Fengqi; Sutton, V Reid; Li, Marilyn; Van den Veyver, Ignatia B

2014-10-01

96

The clinical characteristics of benign oral mucosal tumors  

PubMed Central

Objectives: To investigate the clinical characteristics and pre-biopsy provisional diagnoses of benign oral mucosal tumors. Material and Methods: A 10- year retrospective analysis of all benign tumors of the oral mucosa, from a university- affiliated oral and maxillofacial surgery department. Results: 146 benign tumors were included. The mean age was 49.6 years, with an approximately equal gender distribution. The most prevalent tumor types were lipomatous tumors (27.4%), vascular (23.3%), and salivary gland tumors (16.5%). Tongue, labial and buccal mucosa were the most frequently involved sites. The vast majority (98.6%) presented as non-ulcerated masses. Only 2 (1.4%) presented as ulcerated masses. The clinical provisional diagnosis correctly classified lesions as non-malignant in 93.3%. In only 9 (6.7%) suspicion of malignancy was included in the provisional diagnosis. However, benign neoplasia was unsuspected in 42.1% of tumors. These cases were clinically classified as reactive. Conclusions: Benign tumors were most likely to be clinically correctly classified as non-malignant, but even in the setting of experienced oral surgeons, neoplasia was unsuspected in more than 40% of cases. This data strongly supports the need to biopsy every oral mucosal mass, since inaccurate clinical evaluation of the lesion’s biological nature was a frequent event. Key words:Malignant, benign, reactive, ulcerated mass, non-ulcerated mass, clinical diagnosis. PMID:24316705

Kaplan, Ilana; Gal, Gavriel; Chaushu, Gavriel; Allon, Dror M.

2014-01-01

97

IgG4-associated inflammatory pseudotumor of ureter: clinicopathologic and immunohistochemical study of 3 cases.  

PubMed

Inflammatory pseudotumors are lesions characterized by proliferation of fibroblasts/myofibroblasts with variable chronic inflammatory cell infiltration. Recent studies have suggested that inflammatory pseudotumor with abundant IgG4-positive plasma cells may be a unique entity associated with systemic IgG4-related sclerosing disease and should be distinguished from other similar lesions such as inflammatory myofibroblastic tumor and fibrohistiocytic-type inflammatory pseudotumor. Localized inflammatory pseudotumor has been rarely reported in the ureter, and IgG4-associated inflammatory pseudotumor of ureter has not been described. We describe herein 3 cases of ureteral inflammatory pseudotumor of IgG4-associated lymphoplasmacytic type, focusing on density of IgG4-positive plasma cells; infiltration pattern of eosinophils and histiocytes; presence of obliterative phlebitis; and immunohistochemical profiles of smooth muscle actin, anaplastic lymphoma kinase, and CD68. Three patients, 45- and 47-year-old men and 84-year-old woman, all presented with flank pain and ureteral narrowing by a mass effect. Microscopic examination of the resected ureters showed suburothelial masslike lesions with fibroblasts/myofibroblasts without atypia, abundant plasma cells, and scattered eosinophils and histiocytes. The lesion of the 47-year-old man showed obliterative phlebitis in addition to the above findings. The lesion of the 84-year-old woman was accompanied by urothelial carcinoma in situ in the overlying urothelium. Spindle cells were diffusely or focally positive for smooth muscle actin but negative for anaplastic lymphoma kinase in all 3 cases. For each case, respectively, an average of 154, 112, and 50 plasma cells per high-power fields were immunoreactive for IgG4, a diagnostic feature of IgG4 inflammatory pseudotumor. We described 3 cases of IgG4-associated inflammatory pseudotumor of ureter with pathologic and immunohistochemical features that are compatible for lymphoplasmacytic type of inflammatory pseudotumor. Further study is needed to characterize any relationship between this entity and systemic sclerosing disease and/or urothelial carcinogenesis. PMID:21334715

Kim, Sun A; Lee, Sang-Ryung; Huh, Jooryung; Shen, Steven S; Ro, Jae Y

2011-08-01

98

Expression levels of HMGA2 in adipocytic tumors correlate with morphologic and cytogenetic subgroups  

PubMed Central

Background The HMGA2 gene encodes a protein that alters chromatin structure. Deregulation, typically through chromosomal rearrangements, of HMGA2 has an important role in the development of several mesenchymal neoplasms. These rearrangements result in the expression of a truncated protein lacking the acidic C-terminus, a fusion protein consisting of the AT-hook domains encoded by exons 1–3 and parts from another gene, or a full-length protein; loss of binding sites for regulatory microRNA molecules from the 3' untranslated region (UTR) of HMGA2 has been suggested to be a common denominator. Methods Seventy adipocytic tumors, representing different morphologic and cytogenetic subgroups, were analyzed by qRT-PCR to study the expression status of HMGA2; 18 of these tumors were further examined by PCR to search for mutations or deletions in the 3'UTR. Results Type (full-length or truncated) and level of expression varied with morphology and karyotype, with the highest levels in atypical lipomatous tumors and lipomas with rearrangements of 12q13-15 and the lowest in lipomas with 6p- or 13q-rearrangements, hibernomas, spindle cell lipomas and myxoid liposarcomas. All 18 examined tumors showed reduced or absent expression of the entire, or parts of, the 3'UTR, which was not due to mutations at the DNA level. Conclusion In adipocytic tumors with deregulated HMGA2 expression, the 3'UTR is consistently lost, either due to physical disruption of HMGA2 or a shift to production of shorter 3'UTR. PMID:19508721

Bartuma, Hammurabi; Panagopoulos, Ioannis; Collin, Anna; Trombetta, Domenico; Domanski, Henryk A; Mandahl, Nils; Mertens, Fredrik

2009-01-01

99

Primary oral and maxillofacial liposarcoma: a clinicopathological and immunohistochemical study of eleven cases  

PubMed Central

Introduction The present study was aimed to characterize the clinicopathological, immunohistochemical features and treatment outcomes of primary oral and maxillofacial liposarcomas by presenting the experience over a 16-year period at a tertiary referral Chinese institution for head neck cancer. Material and methods This retrospective clinical study included 11 cases of pathologically confirmed primary liposarcomas treated from January 1993 to September 2009. Detailed information regarding primary site, clinical manifestations, histopathological and immunohistochemical analysis, treatments and prognosis was collected and reported. Results Eight female and 3 male patients aged from 8 to 76 years old. These lesions occurred in buccal (3), parotid (2), temporal (2), tongue (2), palate (1) and oropharyngeal (1) region. They were histopathologically categorized into 4 subtypes based on WHO classification scheme: atypical lipomatous tumor/well-differentiated (4), myxoid (4), mixed-type (2) and pleomorphic (1) liposarcomas. Immunohistochemical staining indicated mostly positive for Vimentin and S-100 but negative for other markers. Most patients presented as slow-growing painless masses and underwent surgery alone or combined with postoperative radiotherapy. Two patients were misdiagnosed and inappropriate treated and developed local relapse before referred to our institute. No distant metastasis and one disease-related death were recorded during the follow-up (ranging: 1-11 years, mean: 4.5 years). Conclusions Oral and maxillofacial liposarcoma is exceedingly rare and has atypical clinical manifestations but characteristic histopathology. Complete excision with negative margins followed by long-term follow-up is recommended as the treatment of choice for these uncommon entities. PMID:22662006

Cheng, Jie; Yu, Hongbo; Wang, Lizhen; Wang, Xudong

2012-01-01

100

Islet vasculature in atrophic pancreas: evidence for coexisting parallel and serial (insuloacinar) angioarchitecture.  

PubMed

Islet vasculature was studied in rats rendered dietarily copper-deficient, a regimen that effects progressive acinar atrophy while leaving islets and ducts intact. Scanning electron microscopy of corrosion casts produced from Mercox injected rats was used to analyze islet angioarchitecture in the atrophied gland. The results indicate that with the onset of acinar atrophy, the vasculature of islets remained intact despite loss of insuloacinar interconnectivity. With atrophy of the dense acinar vascular matrix, three populations of islets could be distinguished according to their size and vascular structures: (a) All small islets (40-150 microns in diameter) were found to have exclusively serial vasculature since no postcapillary collecting venules were found directly continuous with parallel efferent veins. (b) A second population of intermediate size islets (160-250 microns) was found to have serial vessels in addition to postcapillary collecting venules that were directly continuous with larger interlobular veins indicative of a parallel mode of microcirculation. (c) All larger islets (260-700 microns) were also found to have both serial as well as parallel microcirculatory patterns based upon the coexistence of both types of postcapillary venules within the same islet. In all cases, postcapillary venules, which normally link islet capillaries to peri-insular acinar plexuses, were continuous with veins that extended through lipomatous lobules devoid of acini to link with branches of interlobular veins. It is proposed that the functional integrity of islet blood flow remains in the atrophied pancreas because of preservation of both serial and parallel microcirculation that facilitate normal glucose-stimulated insulin secretion in the atrophied pancreas, as demonstrated in an earlier study. Furthermore, it is suggested that analysis of the atrophied state of the gland provides a valid comparative model with which to study islet angioarchitecture and microcirculation in the normal gland. PMID:2654925

Weaver, F C; Sorenson, R L

1989-01-01

101

Clinical delineation and natural history of the PIK3CA-related overgrowth spectrum.  

PubMed

Somatic mutations in the phosphatidylinositol/AKT/mTOR pathway cause segmental overgrowth disorders. Diagnostic descriptors associated with PIK3CA mutations include fibroadipose overgrowth (FAO), Hemihyperplasia multiple Lipomatosis (HHML), Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi, Scoliosis/skeletal and spinal (CLOVES) syndrome, macrodactyly, and the megalencephaly syndrome, Megalencephaly-Capillary malformation (MCAP) syndrome. We set out to refine the understanding of the clinical spectrum and natural history of these phenotypes, and now describe 35 patients with segmental overgrowth and somatic PIK3CA mutations. The phenotypic data show that these previously described disease entities have considerable overlap, and represent a spectrum. While this spectrum overlaps with Proteus syndrome (sporadic, mosaic, and progressive) it can be distinguished by the absence of cerebriform connective tissue nevi and a distinct natural history. Vascular malformations were found in 15/35 (43%) and epidermal nevi in 4/35 (11%) patients, lower than in Proteus syndrome. Unlike Proteus syndrome, 31/35 (89%) patients with PIK3CA mutations had congenital overgrowth, and in 35/35 patients this was asymmetric and disproportionate. Overgrowth was mild with little postnatal progression in most, while in others it was severe and progressive requiring multiple surgeries. Novel findings include: adipose dysregulation present in all patients, unilateral overgrowth that is predominantly left-sided, overgrowth that affects the lower extremities more than the upper extremities and progresses in a distal to proximal pattern, and in the most severely affected patients is associated with marked paucity of adipose tissue in unaffected areas. While the current data are consistent with some genotype-phenotype correlation, this cannot yet be confirmed. PMID:24782230

Keppler-Noreuil, Kim M; Sapp, Julie C; Lindhurst, Marjorie J; Parker, Victoria E R; Blumhorst, Cathy; Darling, Thomas; Tosi, Laura L; Huson, Susan M; Whitehouse, Richard W; Jakkula, Eveliina; Grant, Ian; Balasubramanian, Meena; Chandler, Kate E; Fraser, Jamie L; Gucev, Zoran; Crow, Yanick J; Brennan, Leslie Manace; Clark, Robin; Sellars, Elizabeth A; Pena, Loren D M; Krishnamurty, Vidya; Shuen, Andrew; Braverman, Nancy; Cunningham, Michael L; Sutton, V Reid; Tasic, Velibor; Graham, John M; Geer, Joseph; Henderson, Alex; Semple, Robert K; Biesecker, Leslie G

2014-07-01

102

Hepatic angiomyolipoma: a series of six cases with emphasis on pathological-radiological correlations and unusual variants diagnosed by core needle biopsy  

PubMed Central

Hepatic angiomyolipoma is rare and may pose differential diagnostic difficulty, particularly if encountered in core needle biopsy. We studied 6 cases from 5 males and one female (median age, 48.6 yrs). All presented with non-specific symptoms or an incidentally discovered tumor mass. Two patients had a remote history of chemotherapy for hematological neoplasms (acute lymphoblastic leukemia and Hodgkin lymphoma respectively) and another had clear cell renal cell carcinoma and anaplastic pancreatic carcinoma diagnosed at autopsy without definable syndrome. None of the patients had evidence of the tuberous sclerosis complex or renal or other extra-renal angiomyolipoma. Three tumors were resected completely and three have been only biopsied and followed up. None of the resected cases recurred at a mean follow-up of 35 months. Histologically, tumors were classified as classical triphasic (1), lipomatous (2), epithelioid/oncocytoid (1), epithelioid trabecular (1) and myelolipoma-like (1). The adjacent liver parenchyma was normal in 3 cases, showed pigment cirrhosis in one case and mild fatty change in another case. One case had clinically diagnosed but histologically unverified cirrhosis. The initial diagnostic impression/frozen section was misleading in 5 of the cases and included vascular lesion, focal fatty change, myelolipoma, hepatocellular tumor and oncocytic neoplasm. All tumors expressed HMB45 and variably desmin. One epithelioid lesion expressed HMB45 and TFE3, but lacked desmin expression. In conclusion, hepatic angiomyolipomas are increasingly recognized as incidental findings during surveillance for cirrhosis or investigations for unrelated conditions. Awareness of their diverse morphological spectrum in liver biopsy is necessary to avoid misdiagnosis as hepatocellular carcinoma, metastatic melanoma or other malignant neoplasms. PMID:22949933

Agaimy, Abbas; Vassos, Nikolaos; Croner, Roland S; Strobel, Deike; Lell, Michael

2012-01-01

103

Hepatic angiomyolipoma: a series of six cases with emphasis on pathological-radiological correlations and unusual variants diagnosed by core needle biopsy.  

PubMed

Hepatic angiomyolipoma is rare and may pose differential diagnostic difficulty, particularly if encountered in core needle biopsy. We studied 6 cases from 5 males and one female (median age, 48.6 yrs). All presented with non-specific symptoms or an incidentally discovered tumor mass. Two patients had a remote history of chemotherapy for hematological neoplasms (acute lymphoblastic leukemia and Hodgkin lymphoma respectively) and another had clear cell renal cell carcinoma and anaplastic pancreatic carcinoma diagnosed at autopsy without definable syndrome. None of the patients had evidence of the tuberous sclerosis complex or renal or other extra-renal angiomyolipoma. Three tumors were resected completely and three have been only biopsied and followed up. None of the resected cases recurred at a mean follow-up of 35 months. Histologically, tumors were classified as classical triphasic (1), lipomatous (2), epithelioid/oncocytoid (1), epithelioid trabecular (1) and myelolipoma-like (1). The adjacent liver parenchyma was normal in 3 cases, showed pigment cirrhosis in one case and mild fatty change in another case. One case had clinically diagnosed but histologically unverified cirrhosis. The initial diagnostic impression/frozen section was misleading in 5 of the cases and included vascular lesion, focal fatty change, myelolipoma, hepatocellular tumor and oncocytic neoplasm. All tumors expressed HMB45 and variably desmin. One epithelioid lesion expressed HMB45 and TFE3, but lacked desmin expression. In conclusion, hepatic angiomyolipomas are increasingly recognized as incidental findings during surveillance for cirrhosis or investigations for unrelated conditions. Awareness of their diverse morphological spectrum in liver biopsy is necessary to avoid misdiagnosis as hepatocellular carcinoma, metastatic melanoma or other malignant neoplasms. PMID:22949933

Agaimy, Abbas; Vassos, Nikolaos; Croner, Roland S; Strobel, Deike; Lell, Michael

2012-01-01

104

Real-time polymerase chain reaction analysis of MDM2 and CDK4 expression using total RNA from core-needle biopsies is useful for diagnosing adipocytic tumors  

PubMed Central

Background Diagnosing adipocytic tumors can be challenging because it is often difficult to morphologically distinguish between benign, intermediate and malignant adipocytic tumors, and other sarcomas that are histologically similar. Recently, a number of tumor-specific chromosome translocations and associated fusion genes have been identified in adipocytic tumors and atypical lipomatous tumors/well-differentiated liposarcomas (ALT/WDL), which have a supernumerary ring and/or giant chromosome marker with amplified sequences of the MDM2 and CDK4 genes. The purpose of this study was to investigate whether quantitative real-time polymerase chain reaction (PCR) could be used to amplify MDM2 and CDK4 from total RNA samples obtained from core-needle biopsy sections for the diagnosis of ALT/WDL. Methods A series of lipoma (n?=?124) and ALT/WDL (n?=?44) cases were analyzed for cytogenetic analysis and lipoma fusion genes, as well as for MDM2 and CDK4 expression by real-time PCR. Moreover, the expression of MDM2 and CDK4 in whole tissue sections was compared with that in core-needle biopsy sections of the same tumor in order to determine whether real-time PCR could be used to distinguish ALT/WDL from lipoma at the preoperative stage. Results In whole tissue sections, the medians for MDM2 and CDK4 expression in ALT/WDL were higher than those in the lipomas (P?

2014-01-01

105

In vivo verification of proton beam path by using post-treatment PET/CT imaging  

SciTech Connect

Purpose: The purpose of this study is to establish the in vivo verification of proton beam path by using proton-activated positron emission distributions. Methods: A total of 50 PET/CT imaging studies were performed on ten prostate cancer patients immediately after daily proton therapy treatment through a single lateral portal. The PET/CT and planning CT were registered by matching the pelvic bones, and the beam path of delivered protons was defined in vivo by the positron emission distribution seen only within the pelvic bones, referred to as the PET-defined beam path. Because of the patient position correction at each fraction, the marker-defined beam path, determined by the centroid of implanted markers seen in the post-treatment (post-Tx) CT, is used for the planned beam path. The angular variation and discordance between the PET- and marker-defined paths were derived to investigate the intrafraction prostate motion. For studies with large discordance, the relative location between the centroid and pelvic bones seen in the post-Tx CT was examined. The PET/CT studies are categorized for distinguishing the prostate motion that occurred before or after beam delivery. The post-PET CT was acquired after PET imaging to investigate prostate motion due to physiological changes during the extended PET acquisition. Results: The less than 2 deg. of angular variation indicates that the patient roll was minimal within the immobilization device. Thirty of the 50 studies with small discordance, referred as good cases, show a consistent alignment between the field edges and the positron emission distributions from the entrance to the distal edge. For those good cases, average displacements are 0.6 and 1.3 mm along the anterior-posterior (D{sub AP}) and superior-inferior (D{sub SI}) directions, respectively, with 1.6 mm standard deviations in both directions. For the remaining 20 studies demonstrating a large discordance (more than 6 mm in either D{sub AP} or D{sub SI}), 13 studies, referred as motion-after-Tx cases, also show large misalignment between the field edge and the positron emission distribution in lipomatous tissues around the prostate. These motion-after-Tx cases correspond to patients with large changes in volume of rectal gas between the post-Tx and the post-PET CTs. The standard deviations for D{sub AP} and D{sub SI} are 5.0 and 3.0 mm, respectively, for these motion-after-Tx cases. The final seven studies, referred to as position-error cases, which had a large discordance but no misalignment, were found to have deviations of 4.6 and 3.6 mm in D{sub AP} and D{sub SI}, respectively. The position-error cases correspond to a large discrepancy on the relative location between the centroid and pelvic bones seen in post-Tx CT and recorded x-ray radiographs. Conclusions: Systematic analyses of proton-activated positron emission distributions provide patient-specific information on prostate motion ({sigma}{sub M}) and patient position variability ({Sigma}{sub p}) during daily proton beam delivery. The less than 2 mm of displacement variations in the good cases indicates that population-based values of {Sigma}{sub p} and {sigma}{sub M} used in margin algorithms for treatment planning at the authors' institution are valid for the majority of cases. However, a small fraction of PET/CT studies (approximately 14%) with {approx}4 mm displacement variations may require different margins. Such data are useful in establishing patient-specific planning target volume margins.

Hsi, Wen C.; Indelicato, Daniel J.; Vargas, Carlos; Duvvuri, Srividya; Li Zuofeng; Palta, Jatinder [Proton Therapy Institute, University of Florida, Jacksonville, Florida 32206 (United States); Boca Radiation Oncology Associates, Boca Raton, Florida 33431 (United States); Proton Therapy Institute, University of Florida, Jacksonville, Florida 32206 (United States); Department of Radiation Oncology, University of Florida, Gainesville, Florida 32610 (United States)

2009-09-15