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An Unusual Case of Hemosiderotic Fibrohistiocytic Lipomatous Lesion: Correlation of MRI and Pathologic Findings  

PubMed Central

The spectrum of lipomatous lesions ranges from benign to highly malignant disease. Differentiation between these lesions is important to indicate prognosis and choose the most appropriate treatment. Hemosiderotic fibrohistiocytic lipomatous lesion (HFLL) is a rare subtype of lipomatous tumor. The diagnosis is usually based on clinical, histological, and immunohistochemical information. Where magnetic resonance (MR) imaging is a suitable modality to assess fatty tumors, no data is reported on MR imaging of HFLL. Here, the MR characteristics are described in correlation with pathologic findings in a case of HFLL in the left thigh, an unusual location.

de Vreeze, Ronald S. A.; Koops, Wim; Haas, Rick L.; van Coevorden, Frits



Intracranial lipomatous hamartomas (Intracranial “lipomas”)  

Microsoft Academic Search

13 cases of intracranial lipomatous hamartomas are presented, 12 of which were incidentally found at autopsy. Only one case, verified by biopsy, showed progressive focal symptoms and lipomatous infiltration of the acoustic nerve. 5 lipomatous hamartomas were located in the cisterna ambiens region, 3 in the cerebellopontine angle, 2 in the hypothalamic and 1 in the callosal regions. 2 cases

Herbert Budka



Targeted hemosiderotic hemangioma: a case report of multiple asymptomatic lesions.  


Targetoid hemosiderotic hemangioma (THH) is a benign vascular tumor characterized by a central violaceous papule with a clear periphery bordered by an ecchymotic ring. Originally coined by its characteristic halo appearance with hemosiderin deposits, not all THHs have this classic halo or hemosiderin composition. We report a unique case of THH in which the patient presented with multiple lesions with no prior trauma. Multiple THH lesions have been linked to minor trauma; however, the presence of 4 concurrent lesions with the absence of trauma makes this THH presentation atypical and unique. PMID:24087783

Cleaver, Jonathan L; Cleaver, Nathan J; Cleaver, Lloyd J



p53 protein immunoreactivity in fibrohistiocytic tumors of the skin.  


Abnormal expression of the 53 kDa nuclear phosphoprotein produced by the p53 gene is observed in many human cancers. p53 nuclear immunoreactivity is found commonly in tumor cells. Immunohistochemistry was performed using a monoclonal antibody, DO-7 (DAKO, Denmark; cat. no. M7001; 1:100 dilution), to investigate p53 protein immunoreactivity in a group of cutaneous fibrohistiocytic tumors that are known to be locally aggressive. The study group consisted of dermatofibrosarcoma protuberans (DFSP) (n = 14) and atypical fibroxanthoma (AFX) (n = 7). Cases of dermatofibroma (DF) (n = 16) formed the benign control group. Intense nuclear immunostaining for p53 protein was observed in 71% of DFSP and 86% of AFX. None of the dermatofibromas showed strong p53 nuclear immunostaining. Statistical analyses revealed significant differences in p53 immunoreactivity between DFSP and DF (P = 0.0001, chi 2 test) and between AFX and DF (P = 0.0001, chi 2 test). In conclusion, increased p53 protein immunoreactivity is found in DFSP and AFX but not in DF. These differences in p53 immunoreactivity suggest that increased expression of the protein may be important in the pathogenesis of the more aggressive group of fibrohistiocytic tumors. PMID:9770192

Lee, C S; Chou, S T



An atypical case of atypical lipomatous tumor.  


A 56-year-old woman was referred to us with an asymptomatic nodule on the thigh. The nodule was about 11x7 cm in diameter with a well-demarcated, smooth, surface. Magnetic resonance imaging showed the intensity of the nodule to be entirely monotonous or partially heterogeneous. Histological examination revealed a proliferation of adipocytes varying considerably in size and including many mono- or multivacuolated lipoblasts. Immunohistochemically, some of the tumor cells were positive for p53 and MDM2. Histologically, the atypical lipomatous tumor, lipoblastoma, spindle cell lipoma, pleomorphic liposarcoma, and hibernoma comprised the list of possible, differential diagnoses. We considered our case to be a variant of atypical lipomatous tumor, although it did not correspond to any of the 4 atypical lipomatous tumor subgroups. There has been no recurrence to date. PMID:19033936

Miyakura, Takashi; Irisawa, Ryokichi; Miyamoto, Masamitsu; Iwaya, Keiichi; Yamamoto, Toshiyuki; Tsuboi, Ryoji



Inflammatory pseudotumor of the thyroid gland showing prominent fibrohistiocytic proliferation. A case report.  


Inflammatory pseudotumor of the thyroid gland (IPT) appears to be exceedingly rare. Histologically, 14 previously reported cases demonstrated plasma cell granuloma variant. We report here an IPT showing a predominantly fibrohistiocytic proliferation that occurred in a 75-year-old Japanese woman. Histologically, the lesion was characterized by haphazardly arranged spindle cells, histiocytes having foamy cytoplasm containing intracytoplasmic brown pigments, and small lymphocytes. Immunohistochemical study demonstrated that the spindle cells were vimentin+, desmin-, muscle-specific actin+, cytokeratin-, endomysial antibody-, anaplastic lymphoma kinase-, CD34-- CD68+/-, CD99-, cyclin D1-, bcl-2-, and antifollicular dendritic cell antibody-. IPT showing a predominant fibrohistiocytic proliferation should be differentiated from various nonneoplastic or neoplastic disorders showing spindle cell proliferation and/or exuberant fibrosis. They include Riedel's thyroiditis, fibrous variant of chronic thyroiditis, papillary carcinoma with exuberant nodular fasciitis-like stroma, paucicellular variant of anaplastic thyroid carcinoma, and solitary fibrous tumor. PMID:19444653

Kojima, Masaru; Suzuki, Masami; Shimizu, Kazuhiko; Masawa, Nobuhide



Lipomatous interatrial septal hypertrophy: an unusual cause of intracardiac mass  

Microsoft Academic Search

Lipomatous hypertrophy of the interatrial septum is an uncommon entity that usually occurs in elderly patients. We report a patient who presented with atrial fibrillation, congestive cardiac failure and a large intracavitary mass in the right atrium on echocardiography. He underwent successful resection of the mass with septal reconstruction. Pathology was consistent with lipomatous hypertrophy of the interatrial septum.

Khaja Moinuddeen; Silviu Marica; Robert L Clausi; Nche Zama



[Lipomatous hypertrophy of the cardiac interatrial septum].  


Lipomatous hypertrophy of the interatrial septum (LHIS) is a rare lesion of unknown origin usually diagnosed as an incidental finding during autopsy. It can be associated with supraventricular arrhythmias, venous return obstruction and sudden cardiac death. Five necropsy cases (4 females, 1 male) of LHIS were encountered during the last 31 years; only one case from this series was diagnosed ante mortem. The patients' mean age was 68 years; their mean BMI was 28.4. The mean size of the lesion was 31 mm. In three patients the LHIS was asymptomatic, two patients experienced relapsing multifocal atrial tachycardia and sick sinus syndrome, respectively. Histologically, all cases consisted of a mixture of mature and brown adipose tissue with foci of cardiomyocytes. For a pathologist the knowledge of LHIS is important because of an increasing possibility of its ante mortem diagnosis by imaging methods with a following endomyocardial biopsy, and also because it may appear as a cause of sudden cardiac death. PMID:17171973

Laco, J; Steiner, L; Tomsová, M



Targetoid hemosiderotic hemangioma— a dynamic vascular tumor: Report of 3 cases with episodic and cyclic changes and comparison with solitary angiokeratomas  

Microsoft Academic Search

Background: Both targetoid hemosiderotic hemangiomas (THH) and solitary angiokeratomas (SAK) are acquired vascular malformations formed by superficial vascular ectasias possibly caused by trauma. Objective: We compare the clinicopathologic findings of THHs with those of SAKs and report the clinicopathologic findings of 3 singular cases of THH affected by cyclic or episodic morphologic changes. Methods: We performed a clinicopathologic study on

J. Andrew Carlson; Soume Daulat; Herbert P. Goodheart



Lipomatous muscular 'dystrophy' of Piedmontese cattle.  


Lipomatous myopathy is a degenerative muscle pathology characterized by the substitution of muscle cells with adipose tissue, sporadically reported in cattle, pigs, and rarely in sheep, horses and dogs. This study investigated the pathology of this myopathy in 40 muscle samples collected from regularly slaughtered Piedmontese cattle living in Piedmont region (Italy). None of the animals showed clinical signs of muscular disease. Muscle specimens were submitted to histological and enzymatic investigations. Gross pathology revealed a different grade of infiltration of adipose tissue, involving multiple or single muscles. The most affected regions were the ventral abdomen and the shoulders, especially the cutaneous muscles and the muscles of the thoracic group. Morphological staining revealed an infiltration of adipose tissue varying in distribution and severity, changes in muscle fibre size and increased number of fibres with centrally located nuclei, suggesting muscle degeneration-regeneration. Necrosis and non-suppurative inflammatory cells were also seen. Furthermore, proliferation of connective tissue and non-specific myopathic changes were present. Chemical and physical characteristics of the affected tissue were also evaluated. The authors discuss about the aetiopathogenesis and classification of this muscle disorder whose histological lesions were similar to those reported in human dystrophies. PMID:22717052

Biasibetti, E; Amedeo, S; Brugiapaglia, A; Destefanis, G; Di Stasio, L; Valenza, F; Capucchio, M T



Features of plexiform fibrohistiocytic tumor in skin punch biopsies: a retrospective study of 6 cases.  


Plexiform fibrohistiocytic tumor (PFHT) is a mesenchymal neoplasm of intermediate malignant potential, which typically presents as a dermal or subcutaneous nodule, and is therefore often sampled by skin punch biopsy where diagnostic features may be subtle or absent. We retrospectively analyzed a series of 6 cases of PFHT to highlight for dermatopathologists the features of PFHTs useful to distinguish it from the other entities in the differential diagnosis. On the basis of the proportion of spindled fibroblastic cells to histiocytoid nodules in the biopsy specimen, we divided PFHT into 3 histologic variants: cellular, fibrous, and mixed. The biopsies also were compared with the final resection specimens, in an attempt to determine which histologic features in the original biopsies were most helpful in establishing a diagnosis. Clinical follow-up and immunohistochemistry were performed on all cases. The cellular and mixed variants were a lesser diagnostic challenge inasmuch as the distinctive features were more easily identifiable in small punch biopsy specimens. The fibrous variant proved more difficult to diagnose. Features most helpful in the diagnosis of PFHT were biphasic appearance with small, cellular, histiocytoid aggregates and accompanying plump spindled cells in the deep dermis and subcutis. Negative staining for CD34, NK1/C3, factor XIIIa, and beta-catenin by immunohistochemistry proved useful in excluding some of its mimics. PMID:21697703

Jacobson-Dunlop, Erick; White, Clifton R; Mansoor, Atiya



MRI features of lipoblastoma: differentiating from other palpable lipomatous tumor in pediatric patients.  


The purpose of this study was to describe the magnetic resonance imaging (MRI) features of lipoblastomas in pediatric patients and to differentiate them from other palpable benign lipomatous tumors. The relatively specific MRI features of nonenhancing cystic change and enhancing soft tissue nodules seen in lipoblastoma may help to differentiate it from other types of lipomatous tumor in pediatric patients. PMID:21092875

Chen, Chun-Wen; Chang, Wei-Chou; Lee, Herng-Sheng; Ko, Kai-Hsiung; Chang, Chen-Chu; Huang, Guo-Shu


Endobronchial lipomatous tumors: clinicopathologic analysis of 12 cases with molecular cytogenetic evidence supporting classification as "lipoma".  


Lipomatous lesions rarely involve the bronchial tree, and detailed morphologic and molecular cytogenetic analysis of these tumors is lacking. The clinicopathologic features of 12 endobronchial lipomatous neoplasms were studied, with ancillary fluorescence in situ hybridization performed in subsets of cases for CPM, which is amplified in atypical lipomatous tumors/well-differentiated liposarcomas (ALT/WDL), and HMGA1 and HMGA2, which are often rearranged in lipomas. The cases occurred predominately in older men (91%) (age range 44 to 80 y, mean 65 y). Most patients (80%) had a former or current history of heavy smoking (20 to 100 pack-years). Three patients had concurrent pulmonary squamous cell carcinoma, and 1 had a history of multiple lung cancers. Most lesions were small (<2.5 cm) and discovered incidentally. A subset of tumors showed atypical morphologic features that would be suggestive of ALT/WDL in soft tissue sites, including regions of fibrosis and scattered hyperchromatic stromal cells. However, all cases with atypia were CPM negative and behaved in a clinically benign manner. Seven cases were tested for HMGA1 and HMGA2 rearrangement; 4 showed HMGA2 rearrangement, and 1 showed HMGA1 rearrangement, consistent with lipomas. Two cases were negative for HMGA1/2 rearrangements. We conclude that endobronchial lipomatous neoplasms represent lipomas, even in the presence of morphologic features suggestive of ALT/WDL. Ancillary fluorescence in situ hybridization testing may be very valuable in the analysis of these rare tumors, as true ALT/WDL seem to be very rare or nonexistent at this anatomic site. PMID:24121172

Boland, Jennifer M; Fritchie, Karen J; Erickson-Johnson, Michele R; Oliveira, Andre M; Colby, Thomas V; Folpe, Andrew L



Mammary cystosarcoma with mature adipose stromal differentiation (lipophyllodes tumor) arising in a lipomatous hamartoma.  


This report documents an uncommon variant of benign cystosarcoma phyllodes with mature adipose stromal differentiation, for which the term lipophyllodes tumor is proposed. Origin of this unusual tumor within a lipomatous hamartoma was confirmed by histologic and mammographic observations. The lesion developed in a 40-year-old woman 2 months post partum, and she remained well after treatment by biopsy. Lipophyllodes tumor, which features mature adipose tissue in much of the stromal component of a cystosarcoma, should not be mistaken for a malignant neoplasm. Complete excision is adequate therapy. PMID:8285842

Rosen, P P; Romain, K; Liberman, L



Giant lipomatous tumors of the upper extremities--a series of 8 consecutive cases.  


We present a series of eight consecutive patients evaluated in our department from 2002 to 2007 with giant lipomatous tumors (as defined in medical studies as greater than 5 cm) of upper extremities. There were three male and five female, ranged in age from 17 to 77 years (mean age of 55). The tumor's size ranged from 5 to 34 cm. All patients underwent total excision of the tumors with free margins. All specimens were sent to pathology: seven patients had benign tumors and one patient had liposarcoma. There were no recurrences of the tumors. Appropriate preoperative evaluation and complete surgical excision are mandatory for successful treatment of these tumors. PMID:21682189

Costea, R; Vasiliu, Eugenia; Zarnescu, N O; Neagu, S


Sudden death and lipomatous infiltration of the heart involved by fat necrosis resulting from acute pancreatitis.  


The possible causal link between damage to the heart and acute pancreatitis and other pancreatic diseases has been considered in both adults and children, particularly in cases of sudden, unexpected death. However, the cardiac pathological findings so far reported in the literature are neither specific enough, nor of a kind to prove a direct pancreatic pathogenesis. We describe the occurrence of steatonecrosis developed in areas of lipomatous infiltration of the heart following acute exacerbation of latent chronic pancreatitis. The presence of mature adipocytes in the myocardium is an adequate substrate for the pancreatic lipase to give rise to the steatonecrosis, which is a well-known marker of acute pancreatitis. As far as we are aware, this is the first reported case of heart steatonecrosis in the literature. PMID:22079999

Roncati, Luca; Gualandri, Giorgio; Fortuni, Giuseppe; Barbolini, Giuseppe



Lipomatous hypertrophy of the interatrial septum in ECG-gated multislice computed tomography of the heart  

PubMed Central

Summary Background Lipomatous hypertrophy of the interatrial septum (LHIS) is a benign disorder characterized by fat accumulation in the interatrial septum (IAS). The purpose of the study was to analyze the incidental detection of LHIS in patients with various clinical conditions, referred to ECG-gated multislice computed tomography (ECG-MSCT) examinations of the heart. Material/Methods The ECG-MSCT examinations of 5786 patients (2839 women; 2947 men), were analyzed. The examinations were performed using 8-row (1015 patients) and 64-row (4771 patients) MSCT, in pre- and postcontrast scanning. We analyzed the shape of the IAS, density and maximal thickness of IAS, the thickness of the epicardial adipose tissue, and the degree of contact of IAS with the ascending aorta and superior vena cava. We also determined body mass index (BMI) in patients with LHIS. Results LHIS was detected in 56 (0.96%) patients, with an average age of 61.5±9.8 years. The mean BMI in the analyzed group was 30.1±4.86. During the end-diastolic phase the thickness of IAS was significantly higher (p<0.0001), and on average equaled 18.3mm. The mean optical density of the IAS was conspicuously higher (p<0.0001) in post-contrast phase than in pre-contrast phase. The thickness of the epicardial adipose tissue in the region of the left atrioventricular groove was on average 15 mm. In all cases the dumbbell shape of IAS was observed. Conclusions The incidental frequency of LHIS occurrence in patients diagnosed with the ECG-MSCT examinations is about 1%. In most subjects it is linked with a higher BMI and increased thickness of the epicardial adipose tissue.

Czekajska-Chehab, Elzbieta; Tomaszewska, Monika; Olchowik, Grazyna; Tomaszewski, Marek; Adamczyk, Piotr; Drop, Andrzej



Atypical lipomatous tumor mimicking giant fibrovascular polyp of the esophagus: report of a case and a critical review of literature.  


An 81-year-old man presented to the emergency department with a mass protruding from his mouth after an episode of emesis. A computed tomography scan showed a hypodense, polypoid structure with a fatty component. The long polyp was attached by a narrow stalk to the cervical esophagus. A 14.0-cm slender mass resembling a giant fibrovascular polyp (GFP) of the esophagus was resected. The microscopic, immunohistochemical, and molecular findings were, however, those of an atypical lipomatous tumor (ALT). Atypical lipomatous tumor of the esophagus is a rare, low-grade malignant neoplasm, with a potential for local recurrence and, in most instances, presents as a large intraluminal polyp mimicking a GFP. Cases reported in the literature as primary myxoid liposarcomas of the esophagus are in all likelihood examples of ALT with myxoid change. A recent case reported as a GFP with karyotypic abnormalities on comparative genomic hybridization is also most likely to be an ALT mimicking a GFP. Pathologists need to be aware of the pitfalls in the diagnosis of ALT of the esophagus and should carefully evaluate the adipocytic component of these lesions. PMID:23352209

Boni, Andrea; Lisovsky, Mikhail; Dal Cin, Paola; Rosenberg, Andrew E; Srivastava, Amitabh



p16 immunohistochemistry as an alternative marker to distinguish atypical lipomatous tumor from deep-seated lipoma.  


Atypical lipomatous tumor (ALT)/well-differentiated liposarcoma (WDLPS) is a locally aggressive malignant mesenchymal neoplasm, resembling ordinary lipoma in many clinical aspects. This study investigates the value of expression of p16, an important cell cycle regulator, alone or in combination with MDM2, to distinguish the 2 entities. Fifty cases of lipomatous neoplasms, with cytogenetic results, from 45 patients were collected from the archives in Department of Pathology, University of Medicine and Dentistry of New Jersey/New Jersey Medical School during 1998 to 2006. These include 18 cases of deep-seated lipoma, 1 hibernoma, 1 lipoblastoma, and 30 cases of ALT/WDLPS. p16 was detected in 25/30 (83.3%) of ALT/WDLPS, and none (0/18) of the deep-seated lipomas (P<0.0000001, Fisher exact test). MDM2 was detected in 18/30 (60%) of ALT/WDLPS, and was negative in 0/18 of the deep-seated lipomas (P<0.0001, Fisher exact test). Combined together, 27/30 (90%) of ALT/WDLPS showed positive staining of either p16, MDM2, or both, whereas no staining was observed in all the deep-seated lipomas (P<0.0000001, Fisher exact test). The single case of hibernoma and lipoblastoma revealed p16+MDM2- phenotype. These results indicated that p16 is yet another marker which seems to be a valuable marker to differentiate ALT/WDLPS from deep-seated lipomas. PMID:18779733

He, Mai; Aisner, Seena; Benevenia, Joseph; Patterson, Francis; Aviv, Hana; Hameed, Meera



Correlation between clinicopathological features and karyotype in lipomatous tumors. A report of 178 cases from the Chromosomes and Morphology (CHAMP) Collaborative Study Group.  

PubMed Central

Soft tissue tumors commonly show cytogenetic abnormalities, some of which are tumor specific. Lipomatous tumors represent the largest category of soft tissue neoplasms, and numerous karyotypic aberrations have been identified. However, clear-cut correlation between morphology and karyotype has not been undertaken on a systematic basis in a double-blind setting. The morphological features and histological diagnosis of 178 lipomatous neoplasms were reviewed independently without knowledge of the clinical data. The consensus diagnoses were then correlated with the clinical findings and compared with the tumors' karyotypes, using G-banded preparations from short-term cultures. The data were collated by a multicenter collaborative group of pathologists, geneticists, and surgeons. Clonal chromosomal abnormalities were identified in 149 cases studied (84%) and, to a large extent, the karyotype correlated with the morphological diagnosis. Specifically, 26 (96%) of 27 myxoid liposarcomas and its poorly differentiated variants showed a t(12;16); 29 (78%) of 37 atypical lipomatous tumors (including 5 dedifferentiated cases) showed ring chromosomes; 74 (80%) of 93 subcutaneous and intramuscular lipomas had karyotypic aberrations affecting mainly 12q, 6p, and 13q; 7 of 8 spindle cell and pleomorphic lipomas had aberrations of 16q; 3 lipoblastomas showed 8q rearrangements; and 2 hibernomas showed 11q abnormalities. We conclude that cytogenetic abnormalities are common in lipomatous tumors, correlate reliably with morphological sub-type in many cases, and can be of diagnostic value in histologically borderline or difficult cases.

Fletcher, C. D.; Akerman, M.; Dal Cin, P.; de Wever, I.; Mandahl, N.; Mertens, F.; Mitelman, F.; Rosai, J.; Rydholm, A.; Sciot, R.; Tallini, G.; van den Berghe, H.; van de Ven, W.; Vanni, R.; Willen, H.



Lipomatous hemangiopericytoma (adipocytic variant of solitary fibrous tumor) of the parotid gland: A case report and review of the literature  

PubMed Central

The current study presents the first case of a lipomatous hemangiopericytoma (LHPC) developing in the parotid gland in a 33-year-old male. The patient presented with a 4-year history of a progressively growing painless and fixed mass in the left paratid gland region. The patient underwent radical parotidectomy and was followed-up for 12 months without any evidence of metastasis or recurrence. LHPC, a controversial rare HPC variant, is histologically characterized by a varying admixture of hemangiopericytomatous vasculature and the presence of mature adipocytes. To date, 51 cases of LHPC have been documented in the literature. Although the boundary between HPC and solitary fibrous tumors (SFTs) has become increasingly blurred, neither of these variant growth patterns has previously been recognized in the parotid gland.




Myxoid adrenocortical carcinoma: a clinicopathologic and immunohistochemical study of 7 cases, including 1 case with lipomatous metaplasia.  


Adrenocortical carcinomas (ACCs) with myxoid features are rare neoplasms. We identified 7 cases of myxoid ACC and studied the clinicopathologic and immunohistochemical features of these neoplasms. The patients were 5 men and 2 women with a mean age of 45 years. Histologically, the tumors contained alcian blue-positive myxoid areas ranging from 10% to 50% of the tissue examined. One case showed lipomatous metaplasia. Areas of conventional ACC were present in all cases. Immunohistochemically, the tumors were positive for steroid receptor cofactor 1, inhibin, melan A, calretinin, and synaptophysin but negative for high-molecular-weight cytokeratin, CAM5.2, and Pax8. Clinical follow-up information for 4 patients demonstrated that all patients had died of their disease 11 to 69 months after diagnosis. Myxoid ACCs are rare tumors that expand the differential diagnosis of myxoid neoplasms involving the retroperitoneum. Contrary to previous reports proposing that the biologic behavior is similar to conventional ACC, our series seems to indicate that myxoid morphology is associated with more aggressive behavior. PMID:23690121

Weissferdt, Annikka; Phan, Alexandria; Suster, Saul; Moran, Cesar A



A comparison between histopathology and findings on magnetic resonance imaging of subcutaneous lipomatous soft-tissue tumors.  


OBJECTIVE: To retrospectively analyse the magnetic resonance (MR) findings of subcutaneous lipomatous soft-tissue tumours (SLSTT) in order to define a diagnostic and therapeutic strategy. METHODS: The MR findings of 46 SLSTT were registered for the following data: area, location within the subcutaneous compartment, dimensions, shape and delineation, signal intensity (SI) on different pulse sequences, signal homogeneity, degree and pattern of contrast enhancement. The MR findings, together with patients' age and gender, were compared with histopathological findings. RESULTS: Sixty-eight percent of the lipomas had a homogeneous T1-SI identical to the SI of subcutaneous fat. Heterogeneity in other lipomas was due to fat necrosis and small septa. Other lesions with homogeneous T1-SI identical to the SI of subcutaneous fat included lipoblastoma, lipomatosis and lipoma-like hibernoma. None of the liposarcomas were homogeneous and isointense to SI of subcutaneous fat on T1-weighted imaging (WI). Lipoma variants and liposarcomas showed overlapping MR characteristics. CONCLUSIONS: SLSTT with homogeneous T1-SI identical to the SI of subcutaneous fat are benign. If a SLSTT has other MR characteristics, differentiation based on MRI cannot be made and biopsy is needed. We used this finding in defining a diagnostic and therapeutic strategy for subcutaneous soft tissue tumours that are hyperintense on T1-WI. PMID:22347979

Toirkens, Johannes; De Schepper, Arthur M; Vanhoenacker, Filip; Van Dyck, Pieter; Gielen, Jan; Creytens, David; Wouters, Kristien; Eiber, Matthias; Wörtler, Klaus; Parizel, Paul M



The combination of a decision tree technique with the computer-assisted microscope analysis of Feulgen-stained nuclei to assess aggressiveness in lipomatous and smooth muscle tumors.  


The present study describes a computer-assisted methodology whose purpose is to reduce the degree of subjectivity in the diagnosis of soft tissue tumors. This methodology associates three complementary techniques, namely digital cell image analysis, the discretisation of numerical data and a Decision Tree technique (DT). The first technique relies on the use of the digital cell image analysis of Feulgen-stained nuclei, a technique which makes possible a quantitative and thus objective description of nuclei with the help of 24 numerical parameters (15 morphonuclear and 9 DNA content- (ploidy level and proliferation activity) related). The second technique transforms each numerical parameter into an ordinal one with a small number of values (2 to 4) so that only the relevant physical significance of the parameters is retained. The Decision Tree technique generates classification rules on the basis of the discretised parameters quoted above. This methodology was applied to 53 human soft tissue tumors which included 26 lipomatous tumors (13 malignant liposarcomas and 13 benign lipomas) and 27 smooth muscle tumors (11 malignant leiomyosarcomas and 16 benign leiomyomas). The results show that a distinction between benign (lipoma) and malignant (liposarcoma) lipomatous tumors can easily be made by means of simple logical rules depending on only four discretised cytological parameters (two ploidy- and two morphonuclear-related). In contrast, no stable or predictive characterisation can be obtained with respect to the difference between leiomyosarcomas and the leiomyomas. Hence, while lipomas and liposarcomas appeared to be two completely distinct biological entities, leiomyomas and leiomyosarcomas seem to involve a continuous biological process. PMID:7654014

Decaestecker, C; Remmelink, M; Camby, I; Salmon, I; Goldschmidtf, D; Van Ham, P; Pasteels, J L; Kiss, R



PubMed Central

The authors report on a case of a 65-year-old man with pigmented clear-cell acanthoma located on the right thigh. Dermoscopy disclosed a peculiar picture consisting of diffuse black pigmentation with a superficial greyish veil in the central portion, dotted-to-globular dark red-black structures mainly located at the periphery with a homogenous regular reticular arrangement; peripheral translucid desquamation. Dermoscopic features are correlated with the histology, where hemosiderin deposits present in a sheet-like arrangement in the perivascular papillary dermis and in a band-like disposition in the reticular dermis at the base of the lesion can account for the pigmented picture. The lesion arose on a trauma-prone skin site; thus the authors believe that traumatic irritation may be responsible for the clinical and dermoscopic pictures, giving rise to a reaction similar in a way to the Auspitz's sign provocated by trauma for psoriasis. Red blood cells extravasation from extremely superficialized capillaries may have led to hemosiderin deposition in the papillary and the reticular dermis.

Bugatti, Leonardo; Filosa, Giorgio



Massive Lipomatous Hypertrophy of the Right Atria  

PubMed Central

A case of a 70-year-old female with a history of hypertension, atrial fibrillation, pacer implantation for symptomatic bradycardia, and a prior cerebrovascular accident, and had developed persistent methicillin-sensitive Staphylococcus aureus bacteremia is reported here. As part of her evaluation, a transesophageal echocardiogram was performed, and even though no vegetations were seen on either pacer wires or cardiac valves, a massive homogeneous thickening of the superior portion of the interatrial septum extending to the posterior and roof portions of the right atrial wall as well as to the superior vena cava causing proximal compression of this vessel was noted. Computed tomographic examination of the chest helped to determine that this mass density was not a tumor but in fact intrapericardial fat. Imaging findings and existing literature on this topic are reviewed.

Lopez-Candales, Angel



Inflammatory pseudotumor of lymph nodes. Immunohistochemical evidence for its fibrohistiocytic nature.  

PubMed Central

Five cases of inflammatory pseudotumor (IPT) of the lymph node were investigated by means of light microscopy and immunohistochemistry to elucidate its cellular composition. The IPT is composed of a proliferation of spindle cells, inflammatory cells, and small vessels, forming high and poor cellular areas. Many spindle cells correspond to activated histiocytes as they coexpress vimentin and macrophage-associated markers; they are intermingled with vimentin-positive fibroblasts and variable numbers of vimentin- and actin-positive myofibroblasts. This mixed-cell proliferation invades and/or destroys medium- and large-sized vessels in all cases. This study indicates that the spindle cell proliferation, identified as histiocytic and fibroblastic in nature, represents the main component of the nodal IPT. We speculate that release of cytokines by the activated histiocytes may result in the development of the complex histopathologic aspects of this inflammatory process and, if inappropriate, may represent the underlying pathogenic mechanism. Images Figure 1 Figure 2 Figure 3 Figure 4 Figure 5

Facchetti, F.; De Wolf Peeters, C.; De Wever, I.; Frizzera, G.



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


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



Endobronchial Lipomatous Hamartoma: An Incidental Finding in a Patient with Atrial Fibrillation--A Case Report  

PubMed Central

Introduction. Lung hamartomas are the most common benign tumors of the lung. Typically, they are located in the peripheral lung, while an endobronchial localisation is rare. Case Presentation. We present a case with the rare diagnosis of an endobronchial hamartoma as incidental finding in a 69-year-old male, caucasian patient with atrial fibrillation. At first admission, the patient's exertional dyspnea was caused by atrial fibrillation. Relapse of exertional dyspnea in the absence of arrhythmia was due to postobstructive pneumonia caused by an endobronchial hamartoma. Conclusion. Endobronchial tumors such as endobronchial lipoma or hamartoma should be considered as potential causes of exertional dyspnea and thus as differential diagnosis of atrial fibrillation. Although endobronchial hamartomas are benign, resection is recommended to prevent postobstructive lung damage.

Schneider, Friederike; Winter, Hauke; Schwarz, Florian; Niederhagen, Manuel; Arias-Herrera, Vivian; Martens, Eimo; Kaab, Stefan; Theiss, Hans



Significance of LRP and PPAR-? Expression in Lipomatous Soft Tissue Tumors  

PubMed Central

Background: Molecular mechanism of differentiation in lipogenic tumor is still unknown in detail. Low-density lipoprotein receptor-related protein (LRP) and peroxisome proliferator-activated receptor gamma (PPAR-?), representative regulatory molecules of lipogenic differentiation, have been reported today as multi-functional molecules and to modulate tumorigenesis in various kind of cancer. To date, diagnostic and therapeutic significance of the expression of these molecules in lipogenic tumors are not defined. Methods: The immunohistochemical expression status of LRP and PPAR-? in various grades of 54 lipogenic tumors was analyzed. Correlation between the expression levels and the differentiation of the tumors was confirmed. For statistical analyses, the Kruskal-Wallis test, the Steel-Dwass test and the Mann–Whitney U test were used. Results: LRP and PPAR-? expression was detected in 50 (92.6%) and 44 (81.5%) cases, respectively. The expression level in LRP was significantly higher in cases with well differentiated liposarcoma, pleomorphic liposarcoma and dedifferentiated liposarcoma than in lipoma. Compared with lipoma or well differentiated liposarcoma, significant elevation in expression level of PPAR-? was confirmed in myxoid liposarcoma, pleomorphic liposarcoma, dedifferentiated liposarcoma and the differentiated area of dedifferentiated liposarcoma. Conclusion: The up-regulation of LRP and PPAR-? in higher grade cases, i.e. less differentiated tumors than in low grade cases was shown, suggesting the candidate role of these molecules as tumor progression modulators rather than regulatory molecules of differentiation in lipogenic tumors.

Tajima, Takashi; Morii, Takeshi; Kikuchi, Fumihito; Matsumine, Akihiko; Murata, Hiroaki; Nobuto, Hiroo; Mochizuki, Kazuo



Lipomatous lesions around the shoulder: Recent experience in a Nigerian hospital  

PubMed Central

We present four cases of shoulder lipomas in two females and two males in their fourth to fifth decades of life. All four lipomas were big. Three were subcutaneous and one was intermuscular. None of them were associated with any functional limitation of the affected shoulder. Subcutaneous or intermuscular lipomas around the shoulder do not appear to affect shoulder functions. Complete surgical excision is rewarding and was achieved under local anesthesia in all our patients with no incidence of recurrence.

Rahman, Ganiyu A.; Abdulkadir, Adekunle Y.; Yusuf, I. F.



Deep-seated huge hibernoma of soft tissue: a rare differential diagnosis of atypical lipomatous tumor/well differentiated liposarcoma  

PubMed Central

Background: Hibernoma is a rare benign fat-forming soft tissue tumor that differentiates similar to brown fat, hence an origin from remnants of fetal brown adipose tissue has been proposed. Mainly young adults are affected, usually without significant clinical symptoms. Material and methods: We report on four patients with hibernomas, who were treated at our hospital during the last 10 years. The clinicopathologic and immunohistochemical features are presented and treatment and follow-up data discussed. Results: Patients were 2 women and 2 men aged 21-67 years (mean: 45 yrs) who presented with a slowly growing, painless mass. The anatomic location was the thigh, upper arm, lateral thoracic wall and paravertebral soft tissue. Two of them were diagnosed preoperatively through a percutaneous core needle biopsy and the other two underwent surgery because of high clinical and radiological suspicion of liposarcoma. The tumor’s size ranged from 7 cm to 15.5 cm (mean: 11 cm). All were deep-seated subfascial intramuscular masses. Histologically, all four tumors were of the typical variant. All patients underwent a R0-surgical resection of the tumor and they were recurrence-free at last follow-up (mean: 47 months; range: 25-87). Conclusion: Hibernoma may present as huge deep intramuscular soft tissue mass in adults, closely mimicking well differentiated liposarcoma and should be considered in the differential diagnosis of fatty soft tissue tumors in any location. Surgical excision is the treatment of choice. The tumor has no malignant or recurrence potential.

Vassos, Nikolaos; Lell, Michael; Hohenberger, Werner; Croner, Roland S; Agaimy, Abbas



Unusual Thymic Hyperplasia Mimicking Lipomatous Tumor in an Eight-Year-Old Boy with Concomitant Pericardial Lipomatosis and Right Facial Hemihypertrophy  

PubMed Central

We report a case of thymic hyperplasia accompanied by pericardial lipomatosis and right facial hemihypertrophy in an 8-year-old boy. On imaging studies, the hyperplastic thymus had prominent curvilinear and nodular fatty areas simulating a fat-containing anterior mediastinal mass, which is an unusual finding in children. To our knowledge, this is the first report on a child with a combination of thymic hyperplasia, pericardial lipomatosis, and right facial hemihypertrophy. The radiologic findings are presented with a brief discussion.

Kim, Yoo Jin; Cheon, Jung-Eun; Lim, Yun-Jung; Kim, In-One; Yeon, Kyung Mo; Jung, Kyeong Cheon; Byun, Sun-Ju



Action of chelators in iron-loaded cardiac cells: accessibility to intracellular labile iron and functional consequences  

Microsoft Academic Search

Labile iron in hemosiderotic plasma and tissue are sources of iron toxicity. We compared the iron chelators deferox- amine, deferiprone, and deferasirox as scavengers of labile iron in plasma and cardiomyocytes at therapeutic concentra- tions. This comprised chelation of labile plasma iron (LPI) in samples from thalas- semia patients; extraction of total cellular iron; accessing labile iron accumulated in organelles

Hava Glickstein; Rinat Ben El; Gabi Link; William Breuer; Abraham M. Konijn; Chaim Hershko; Hanspeter Nick; Z. Ioav Cabantchik; Charles E. Smith



Angiomatoid “malignant” fibrous histiocytoma: A clinicopathologic study of 158 cases and further exploration of the myoid phenotype  

Microsoft Academic Search

Angiomatoid “malignant” fibrous histiocytoma (AMFH) has been considered to be a low-grade sarcoma of childhood, and, with its fibrous pseudocapsule, angiomatoid change, dense lymphoplasmacytic response, and proliferation of spindled or round cells, has been classified as a fibrohistiocytic neoplasm. We wanted to study the clinicopathologic and immunophenotypic features of a large number of these tumors and to especially further explore

J. C Fanburg-Smith; M Miettinen



Lipomatosis: a diverse form of hemifacial hyperplasia  

PubMed Central

A case of hemifacial hyperplasia that presented with muscular, skeletal, and dental hyperplasia along with lipomatous infiltration was described. Advanced imaging was useful in identifying the lipomatous infiltration present in the lesion, which raises the possibility of lipomatosis having a diverse presentation in hemifacial hyperplasia. As there was a scarcity of related literature in the field of dentomaxillofacial radiology, this report would make us familiar with its computed tomographic and magnetic resonance image findings.

Umarji, Hemant R.; Arora, Aman; Ramaswami, Easwaran



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



Sarcomas other than Kaposi sarcoma occurring in immunodeficiency: interpretations from a systematic literature review  

PubMed Central

Purpose of review In immunodeficiency, an increased sarcoma risk is confirmed for Kaposi’s sarcoma. Whether rates of other sarcoma subtypes are elevated in the setting of immunodeficiency is not known. We therefore reviewed published case reports on HIV/AIDS patients and organ transplant recipients with sarcomas. For comparison, we assessed sarcomas in the U.S. general population using Surveillance Epidemiology End Results (SEER) data. Findings One hundred seventy-six non-KS sarcomas were identified, 75 in people with HIV/AIDS and 101 in transplant recipients. Leiomyosarcomas (n=101) were the most frequently reported sarcomas, followed by angiosarcomas (n=23) and fibrohistiocytic tumors (n=17). Leiomyosarcomas were reported with two age peaks, in children and young adults. Epstein-Barr virus (EBV) was detected in the tumor cells in 85% and 88% of leiomyosarcomas in HIV-infected people and transplant recipients, respectively. Angiosaromas and fibrohistiocytic tumors were most frequently reported in males. Among kidney transplant recipients, 20% of sarcomas arose at the site of an arteriovenous fistula. In comparison, leiomyoscarcomas, angiosarcomas, and fibrohistiocytic tumors comprised 16.9%, 3.8%, and 18.7% of sarcomas in the U.S. general population. Summary Leiomyosarcoma and angiosarcoma may occur disproportionately in immunodeficiency. Leiomyosarcomas appear etiologically linked to EBV while angiosarcomas might be correlated with an arteriovenous fistula. Additional studies are necessary to understand the contribution of immunodeficiency to the etiology of these sarcomas.

Bhatia, Kishor; Shiels, Meredith. S.; Berg, Alexandra; Engels, Eric. A.




Microsoft Academic Search

uncommon tumors, but there are the most common type of non-epithelial tumors of the colon. We present 5 cases of colonic lipomas, 2 in females and 3 in male, with mean age of 57 years. The benign lipomatous tumor was in all cases unique, located in the right segment of the colon, ileo-cecal valve, the left colon, the recto- sigmoid

E. Târcoveanu; Elena Cotea; Simona Nicolescu; A. Vasilescu; R. Moldovanu; Niculina Florea; G. Dimofte


Giant lipoblastoma of the thigh: a rare soft tissue tumor in an infant.  


Lipoblastoma is a rare lipomatous tumor encountered almost exclusively in infants and young children. It arises from embryonic white fat. The common site of involvement is the extremities. In spite of their potential for local invasion, they are benign tumors. We report a case of a lipoblastoma in an infant and review the literature pertaining to clinical management of these tumors. PMID:22531541

Akhtar, Tanveer; Alladi, Anand; Ahmed, Siddiq M; Siddappa, Odda S


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.

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



Computed tomographic evaluation of fatty neoplasms of the extremities. A clinical, radiographic, and histologic review of cases.  


Eleven lipomatous tumors of the extremities (7 liposarcomas, 4 lipomas) were evaluated radiographically by several modalities, including computed tomography (CT) in all lesions. Lipomas were seen on CT scan as well delineated, homogeneous masses with negative attenuation coefficients ranging from -95 to -160. Liposarcomas were inhomogeneous, multilobulated, poorly delineated lesions located exclusively in the subfascial or intramuscular planes, with an intermediate range of attenuation coefficients between those of fat and those of muscle. In six of seven liposarcomas, definite low density areas were identified by attenuation coefficient as fatty tissue. Magnetic resonance imaging (MRI) was obtained in two patients and was helpful in defining the extent of the tumors and their relationship to vital structures, but MRI could not differentiate benign from malignant tissue. Computed tomography is recommended as the cornerstone in the initial radiographic evaluation of growing or painful lipomatous soft tissue masses of the extremities. PMID:2798244

Wolfe, S W; Bansal, M; Healey, J H; Ghelman, B



[Lipoma of the filum terminale: a prospective tomodensitometry study].  


A lipomatous aspect of the filum terminale is sometimes recognized in routine lumbosacral CT studies as a small (1 to 2 mm) tubular fatty hypodensity that lies posteriorly in the dural sac. To study the epidemiologic frequency and pathological significance, the authors prospectively reviewed the standard lumbosacral CT studies of a large series of 1033 adult patients suffering from mechanical problems. The lipomatous aspect was encountered in 39 of them (3.77%). The average size was about 1 mm and never exceeded 2 mm (92%) except for 3 cases. The commonest site was L4-L5 but the anomaly was more commonly seen at two levels. Despite the lack of histological proof, the authors consider the lesion benign and trite with two restrictions: the pediatric cases (not studied in their series) and the patients presenting a lipoma greater than 3 mm; for these two groups further investigations are recommended to exclude a tethered cord syndrome. PMID:7928938

Coulier, B; Mailleux, P



Spindle cell lipoma of the tongue: A case report of unusual occurrence  

PubMed Central

Spindle cell lipoma (SCL) is a benign lipomatous tumor predominantly occurring at the posterior neck and shoulder area. Face, forehead, scalp, cheek, perioral area, and upper arm are less common sites. In oral cavity, it is a relatively uncommon neoplasm, particularly in tongue, which is relatively devoid of fat cells. We present a case report of SCL located on the left lateral border of the tongue in a 64-year-old Caucasian female patient with diabetes mellitus type 2 and arterial hypertension.

Junior, Orlando Cavezzi; de Aguiar, Eduardo Castro Guerra; Sartori, Jose Henrique Franca; Lima, Flavio de Oliveira



[A case of human tail].  


A human tail is a rare anatomical curiosity. A case of a human tail associated with lipomyelomeningocele is reported. The made subject was born, by breech delivery, at the 39th-week with a 3,008 g body weight. He was admitted to our hospital because of the presence of a human tail and subcutaneous mass in the midline lumbosacral region. The tail was about 7.5 cm in length and 2 cm in diameter. It was elastic and covered by normal skin. No systemic anomaly was found. Spina bifida below L5 was revealed, and no bony shadow was found on the plain X-ray film. CT scan showed a low density area in the spinal canal between L3 and lower sacral region that extended into the tail through the spina bifida. MRI also revealed intraspinal long T2 mass which was attached to the spinal cord and extended into the tail. Myelogram indicated intradural extramedullary mass below the L3 level. Surgical treatment was performed on the 3rd month of life with a diagnosis of a human tail with lipomyelomeningocele. At surgery, the tail was found to consist mainly of lipomatous tissue which extended subcutaneously and entered the spinal canal through the spina bifida. The tail and subcutaneous lipomatous tissue were totally excised. The capsule of subcutaneous lipomatous tissue was followed circumferentially down into the spinal canal, and found to be transformed to arachnoid membrane. Intradural lipomatous tissue was excised piece by piece, leaving only a small remnant attached to the conus medullaris to preserve sacral nerve root function.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:2000165

Yamatani, K; Saitoh, T; Oi, M; Oka, N; Endoh, T; Takaku, A



Acute respiratory distress caused by massive thymolipoma.  


The authors present the case of a 40-year-old female with a massive anterior mediastinal tumor who presented with acute respiratory distress. She required emergent intubation and ventilatory support for respiratory compromise caused by lung compression. Preoperative computed tomographic scan results suggested a mediastinal lipomatous mass. Resection of the tumor resulted in immediate improvement in her pulmonary status. Because thymolipomas can attain enormous dimensions and compress adjacent structures, they should be resected at the time of diagnosis. PMID:15586609

Halkos, Michael E; Symbas, John D; Symbas, Panagiotis N



Respiratory failure caused by giant thymolipoma.  


The authors present the case of a 31-year-old woman with a massive anterior mediastinal tumor who presented with respiratory failure. A thoracic computed tomographic scan suggested a mediastinal lipomatous mass, and an operation was performed. Resection of the tumor resulted in immediate improvement in the patient's pulmonary status, and the histopathologic examination revealed thymolipoma. Because thymolipoma can attain enormous dimensions and compress adjacent structures, it should be immediately resected. PMID:18640359

Ceran, Sami; Tulek, Baykal; Sunam, Guven; Suerdem, Mecit



Retroperitoneal lipoblastoma: a discussion of current management.  


Retroperitoneal lipoblastomas are rare tumors found in young pediatric patients. Despite their large size at presentation, complete resection is usually achieved with minimal complications. Diagnosis is typically made after pathologic examination of the operative specimen, and cytogenetic analysis may be a key component in differentiating lipoblastoma from other lipomatous tumors. We present one such case of a large, retroperitoneal lipoblastoma and discuss the management of this uncommon entity. PMID:23084233

Burchhardt, Daniela; Fallon, Sara C; Lopez, Monica E; Kim, Eugene S; Hicks, John; Brandt, Mary L



Syndromes with focal overgrowth in infancy: diagnostic approach and surgical treatment.  


Syndromes with focal overgrowth are sporadic diseases and comprise Proteus syndrome and congenital lipomatous overgrowth, vascular malformations, and epidermal naevi (CLOVE) syndrome, and isolated hemihyperplasia. We describe 3 children classified according to standard criteria with dysregulated growth of various tissues that was excised, together with excess toes, and tumours. Correct classification facilitates diagnosis and operations. Interdisciplinary treatment and follow-up are recommended to prevent disfiguration. PMID:21446802

Mirastschijski, Ursula; Altmann, Silke; Lenz-Scharf, Olivia; Muschke, Petra; Schneider, Wolfgang



Subdeltoid lipoma arborescens combined with rotator cuff tears.  


Lipoma arborescens, a rare benign intra-articular lesion, is characterized by lipomatous proliferation of the synovium in which the subsynovial tissue is replaced by mature adipocytes. Subdeltoid bursa is a rare location for lipoma arborescens, and only a few cases have been reported in the literature. This article reports 2 cases of subdeltoid lipoma arborescens combined with rotator cuff tears, and the possible relationships between subdeltoid lipoma arborescens and rotator cuff tears are discussed. The intra-articular villous proliferations on fat-suppressed T2-weighted magnetic resonance imaging appeared as yellowish-white lipomatous villous proliferations on arthroscopy, and finger-like lipomatous proliferation of the synovium, where the subsynovial connective tissue is replaced by mature adipocytes, on histology. Although further evidence would be necessary, the bony proliferations, in addition to bone-to-bone abrasion and inflammatory processes, may contribute to the relationship between subdeltoid lipoma arborescens and rotator cuff tears. Because this is a rare disease in a rare location, no established treatment guidelines are available for lipoma arborescens in subdeltoid bursa. For the current patients, arthroscopic excision of the lipoma arborescens and concomitant rotator cuff repair were prescribed after more than 6 months of conservative management. All patients had symptom relief and were satisfied with their results. Paying special attention to the radiologic and arthroscopic characteristics of the lipoma arborescens will help physicians and surgeons to achieve a more accurate diagnosis and effective treatment strategy, especially in patients with concomitant rotator cuff tears. PMID:23937762

Kim, Min-Hyung; Chung, Seok Won; Yoon, Jong Pil; Kim, Sae Hoon; Oh, Joo Han



Unclassified glioneuronal tumor with advanced lipidization.  


Lipidization is observed only occasionally in primary neuroectodermal tumors of the central nervous system. It may reflect lipomatous transformation of tumor cells into xanthomatous and/or adipocyte-like cells. We report a unique case of mixed glioneuronal tumor with marked lipomatous changes in a young patient with intractable epilepsy. MRI revealed a well-circumscribed lesion in the right temporal lobe. Histopathological findings showed the pleomorphic tumor with numerous cells containing large lipid droplets, resembling mature adipocytes, that were arranged in clusters or scattered within the neoplastic tissue. The tumor was composed of both glial and neuronal elements. Some tumor cells displayed features intermediate between glial and neuronal cells. The reticulin fibers were limited to blood vessels. Mitotic figures, vascular proliferation, and necrosis were absent, and MIB-1 labeling index was less than 1%. Diffuse immunoreactivity for GFAP and S100-protein was observed. In some heavily lipidized cells, the lipid droplets were surrounded by a cytoplasmic rim of GFAP immunoreactivity. Numerous cells exhibited immunostaining for NSE and synaptophysin. This is the first documented case of glioneuronal tumor with extensive lipomatous transformation, which might be considered as a heavily lipidized unclassified pleomorphic glioneuronal tumor or a variant of lipoganglioglioma with marked pleomorphism and severe lipidization. PMID:21562835

Matyja, Ewa; Grajkowska, Wies?awa; Kunert, Przemyslaw; Rysz, Andrzej; Marchel, Andrzej



Fat-containing salivary gland tumors: a review.  


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



Malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma of the maxillary sinus : report of a case and review of the literature.  


Malignant fibrous histiocytoma (MFH) also known as undifferentiated high-grade pleomorphic sarcoma (UHPS) is a soft tissue sarcoma, composed of undifferentiated mesenchymal tumors possessed fibrohistiocytic morphology without definite true histiocytic differentiation. Head and neck localization is very rare, showing an incidence ranging from 4 % to 10 % in different series of investigations. The most frequent involved sites in UHPS are the neck and parotid, followed by the scalp, face, anterior skull base and orbit. Upper aerodigestive tract, lateral skull base and ear are rare locations. The incidence of the lymphatic metastases is also rare. The aim of this article is to report a case of UHPS in the maxillary sinus with palatal, orbital and ethmoidal involvement, with lymphatic metastasis and its surgical treatment. In addition, we review the literature of similar cases of the past 12 years. PMID:23812657

Vuity, Drazsen; Bogdan, Sandor; Csurgay, Katalin; Sapi, Zoltan; Nemeth, Zsolt



Calcinosis cutis following contact with calcium chloride solution.  


Calcinosis cutis is the deposition of insoluble calcium in the cutaneous tissue. Calcinosis cutis can be classified as metastatic, dystrophic, idiopathic or exogenous. We report a 48-year-old white man who was dismantling a portable ice skating rink when calcium chloride solution from the pipes spilt onto his clothing. Several days later, he started to develop mildly pruritic erythematous papules, some studded with white deposits and some with umbilication over the exposed areas corresponding to the spillage of the calcium chloride solution. Histological features revealed interstitial fibrohistiocytic reaction with calcium-encrusted degenerated collagen bundles in the dermis which was further confirmed by von Kossa stain. He was commenced on topical corticosteroid cream twice daily and the lesions cleared completely between 6 to 10?weeks. PMID:23157788

Lim, Penny P L; Kossard, Steven; Stapleton, Karen



Atypical Fibroxanthoma  

PubMed Central

Atypical fibroxanthoma (AFX) is a nodular dermal ulcerative lesion with a favorable prognosis. AFX most commonly occurs on sun-exposed skin in elderly individuals. AFX is characterized by its association with ultraviolet radiation, not only from a clinical aspect, but also from a molecular aspect. Making a diagnosis of AFX is challenging, and it is important to differentiate it from squamous cell carcinoma and malignant melanoma. Histological features and combined immunohistochemical markers are necessary for a definitive diagnosis (i.e., an absence of immunostaining for cytokeratins, S100 and HMB45 in AFX is helpful for excluding both squamous cell carcinoma and malignant melanoma). AFX, as well as MFH (malignant fibrous histiocytoma), is a fibrohistiocytic lesion with myofibroblastic differentiation. AFX is considered to be a different lesion from MFH. AFX and MFH might share the same pathway which determines their morphology. However, they may have different pathways in development which determine their biological behavior.

Sakamoto, Akio



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

Brooks, J. J.



Mast cells in cutaneous tumors: innocent bystander or maestro conductor?  


BACKGROUND: Evidence favoring a critical role for mast cells (MC) in cutaneous malignancies is conflicting. METHODS: Using the immunohistochemical stain tryptase, MC counts were performed in the following tumor categories: epithelial (basal cell carcinoma [BCC]: nodular [N], n = 10, infiltrative [I], n = 10; squamous cell carcinoma [SCC]: well differentiated [W], n = 9, moderate/poorly differentiated [MP], n = 15); melanocytic (intradermal nevus, n = 10, malignant melanoma in situ [MMIS], n = 8, invasive melanoma, n = 15); vascular (hemangioma [HEM], n = 11, Kaposi's sarcoma [KS], n = 14, angiosarcoma [AS] n = 8); and fibrohistiocytic (dermatofibroma [DF], n = 7, atypical fibroxanthoma [AFX], n = 5, dermatofibrosarcoma protuberans [DFSP], n = 5). MC (intra- and peritumoral) were expressed as cells per 10 high-power fields. RESULTS: Mean MC counts were: BCCN 166.30; BCCI 130; SCCW 167.22; SCCMP 133.80; nevus 156.40; MMIS 93; MM radial growth phase 73.86; MM vertical growth phase 82.13; HEM 165.18; KS 120.57; AS 168.13; DF 247.86; AFX 280.20; and DFSP 83.60. Using a one-way analysis of variance, statistically significant differences were observed in the following pairs: AFX and DF vs. DFSP, nevus vs. melanoma, AS and HEM vs. KS. CONCLUSIONS: Our findings appear to point towards a dichotomous role for mast cells in fibrohistiocytic and vascular neoplasms and argue against their preferential recruitment in epithelial malignancies and malignant melanoma. The value of mast cell counts as a prognostic index appears to be limited in most cutaneous malignancies. PMID:23621615

Biswas, Asok; Richards, Joanna E; Massaro, Joseph; Mahalingam, Meera



Respiratory toxicity of diacetyl in C57BL/6 mice.  


Diacetyl, a component of artificial butter flavoring, is a potential etiological agent of obliterative bronchiolitis (OB); however, the toxic dose and mechanisms of toxicity remain controversial. We evaluated the respiratory toxicity of diacetyl in a murine model using several exposure profiles relevant to workplace conditions at microwave popcorn packaging plants. Male C57Bl/6 mice were exposed to inhaled diacetyl across several concentrations and duration profiles, or by direct oropharyngeal aspiration. Effects of diacetyl on the respiratory tract were evaluated by histopathology and BALF analyses. Subacute exposure to 200 or 400 ppm diacetyl for 5 days caused deaths, necrotizing rhinitis, necrotizing laryngitis and bronchitis. Reducing the exposure to 1 h/day (100, 200, 400 ppm) for 4 weeks resulted in less nasal and laryngeal toxicity, but led to peribronchial and peribronchiolar lymphocytic inflammation. A similar pattern was observed with intermittent high-dose exposures at 1200 ppm (15 min, twice a day, 4 weeks). Subchronic exposures to 100 ppm (6 h/day, 12 weeks) caused moderate nasal injury, and peribronchial lymphocytic inflammation accompanied by epithelial atrophy, denudation, and regeneration. Treatment with 400 mg/kg by oropharyngeal aspiration to bypass the nose caused foci of fibrohistiocytic proliferation with little or no inflammation at the junction of the terminal bronchiole and alveolar duct. Depending on the route and duration of exposure, diacetyl causes significant epithelial injury, peribronchial lymphocytic inflammation, or fibrohistiocytic lesions in the terminal bronchioles. Collectively these results indicate that clinically relevant diacetyl exposures result in a pattern of injury that replicates features of human OB. PMID:18227102

Morgan, Daniel L; Flake, Gordon P; Kirby, Patrick J; Palmer, Scott M



Muco-submucosal elongated polyps of the gastrointestinal tract: A case series and a review of the literature  

PubMed Central

We present three cases of gastrointestinal muco-submucosal elongated polyps, two located in the duodenum and one in the descending colon. All three cases had a characteristic, “worm-like” endoscopic appearance and were lined by unremarkable mucosa. The vascular component was located in the submucosa and was composed of a mixture of variably dilated blood vessels (capillaries and veins) and lymphatics. The duodenal polyps displayed lipomatous metaplasia of the submucosal stroma. The dual vascular phenotype of the vascular component was confirmed by immunohistochemistry with D2-40 and CD31.

Tan, Char Loo; Tan, Sze Hwa; So, Jimmy BY; Petersson, Fredrik



Gigantic thymolipoma.  


A 48-year-old woman with an abnormal shadow in chest radiography during an annual physical examination was found by chest computed tomography to have a large fatty mass lesion found to be diagnosed as a gigantic lipoma. Histopathological diagnosis was found to be benign thymolipoma consisting of mature fatty tissue and hyperplastic thymic tissue structures with Hassall,s corpuscles. Although the diagnosis is supported by imaging studies that demonstrate fat and soft tissue within the tumor, variations occur in computed tomography appearance. We suggest that surgical excision be considered when a gigantic intrathoracic lipomatous mass is in scanning as in this case. PMID:11855099

Hirai, Shinji; Hamanaka, Yoshiharu; Mitsui, Norimasa; Kumagai, Hajime; Kobayashi, Taira



Giant intramuscular lipoma of the tongue: a case report and literature review  

PubMed Central

We herein report a rare case of giant intramuscular lipoma of the tongue. A 75-year-old Italian male presented at our department with a large tumor at the tip of the tongue that had been present for over 30 years. Clinical examination revealed a yellowish lesion, measuring 10 cm in maximum diameter, protruding from lingual surface. Histological examination showed an unencapsulated lipomatous tumor composed of mature adipocytes, uniform in size and shape, diffusely infiltrating striated muscle fibers of the tongue. The patient is well with no local recurrence after a 15-month follow-up period.



Rearrangement of HMGA2 in a case of infantile lipoblastoma without Plag1 alteration.  


Lipoblastoma is a rare benign adipocytic tumor that occurs usually in children. It can be difficult to distinguish a lipoblastoma from other lipogenic tumors. In such cases, the detection of a rearrangement of the PLAG1 gene by fluorescence in situ hybridization analysis is useful for characterizing a lipoblastoma. We present here a novel case of morphological infantile lipoblastoma showing a rearrangement of HMGA2 instead of the classical PLAG1 alteration. HMGA2 is the main target of clonal aberrations encountered in lipomas. This result supports the hypothesis that benign lipomatous tumors harboring PLAG1 or HMGA2 rearrangement could constitute a unique pathogenetic entity. PMID:22223189

Pedeutour, Florence; Deville, Anne; Steyaert, Henri; Ranchere-Vince, Dominique; Ambrosetti, Damien; Sirvent, Nicolas



A case of encephalocraniocutaneous lipomatosis syndrome with epilepsy (Haberland syndrome).  


Encephalocraniocutaneous lipomatosis is a rare congenital neurocutaneous syndrome characterized by scalp, facial, and ocular lesions and multiple intracranial malformations. Approximately 50 cases have been described in the literature. We report a 34-year-old woman with a 6-year history of epilepsy, without mental retardation, with predominantly ipsilateral skin lesions evident at birth, with limbal lipodermoid of the left eye and multiple non-progressive, ipsilateral intracranial structures of soft, cystic components. The described malformations are congenital, mostly unilaterally located and with similar lipomatous structure. PMID:20232658

Zahariev, Zahari Iv; Peycheva, Marieta V; Dobrev, Hristo P


MR imaging of soft tissue masses in children.  


This article discusses the role of MR imaging in the evaluation of musculoskeletal soft tissue masses, reviewing limitations in its ability to establish tissue diagnosis and its critical role in determining lesion extent. It also reviews benign soft tissue masses, such as fibrous lesions, popliteal cysts, lipomatous lesions, and neurofibromatous tumors. Reactive entities, such as abscess, myositis ossificans, and hemangioma, are discussed. Differentiation between high- and low-flow vascular and lymphatic anomalies is reviewed. The article concludes with a discussion of the uncommon malignant musculoskeletal soft tissue tumors, focusing on infantile fibrosarcoma, rhabdomyosarcoma, synovial sarcoma, and granulocytic sarcoma. PMID:19524198

Stein-Wexler, Rebecca



Typical and atypical dermoscopic presentations of dermatofibroma.  


Background? Dermatofibroma is a common skin neoplasm that is usually easy to recognize, but in some cases its differentiation from melanoma and other tumours may be difficult. Objective? To describe the dermoscopic features of dermatofibromas, with special emphasis on the characteristics of atypical patterns, and to calculate pattern frequency according to the patients age and gender, anatomical site and histopathological subtype. Methods? Two groups of patients were consecutively seen, one with dermatofibromas that were surgically excised because of clinically and/or dermoscopically equivocal aspects or following patient request, and another with non-equivocal dermatofibromas. Each lesion was scored for previously reported global dermoscopic patterns and for additional features. Results? A typical pattern was observed in 92 of 130 (70.8%) lesions, whereas an atypical pattern, that we named the 'non Dermatofibroma (DF)-like' pattern, was seen in 38 of 130 (29.2%). Atypical dermatofibromas showed features reminiscent of different conditions, such as melanoma in 21(16.2%) cases, vascular tumour in six (4.6%), basal cell carcinoma in five (3.8%), collision tumour in three (2.3%) and psoriasis in three (2.3%). A significant association was found between the 'melanoma-like' pattern/'vascular tumour-like' pattern and males, whereas a trend was observed between the above-mentioned patterns and hemosiderotic/aneurysmal DFs. 'Peripheral pigment network and central white scar-like patch' pattern was found associated with females and classic histopathological variant of DF. Conclusion? Dermatofibromas may display different morphological faces. The typical dermoscopic patterns allow a confident diagnosis, whereas a full surgical excision is always recommended in all doubtful cases. PMID:23176079

Ferrari, A; Argenziano, G; Buccini, P; Cota, C; Sperduti, I; De Simone, P; Eibenschutz, L; Silipo, V; Zalaudek, I; Catricalà, C



Hyalinising spindle cell tumour with giant rosettes: report of a case with unusual features including original histological and ultrastructural observations.  


Hyalinising spindle cell tumour with giant rosettes (HSCTGR) is an uncommon, recently described low-grade sarcoma which shares many histological features with low-grade fibromyxoid sarcoma (LGFMS). We report a case of HSCTGR occurring in the deep soft tissues of the thigh of a 46-year-old woman, that presented as a slowly growing, painless mass. Microscopically the tumour was composed of spindled stromal cells amongst which were scattered so-called collagen rosettes. The distinctive feature of this case was the previously unreported finding of lymphoid cells of T-cell phenotype admixed with fibrohistioctyic cells in the cellular cuff surrounding the collagenous core of the rosettes. The case was further unusual in that it included focal areas of increased cellularity with a mitotic count of up to three per 10 high-power fields. While the latter feature has been associated with increased recurrences and metastases in LGFMS, it is not known whether the significance is similar in HSCTGR. The spindled stromal cells showed ultrastructural features of poorly differentiated fibroblasts, while those at the edges of the rosettes showed features of altered fibroblasts, some with a fibrohistiocytic appearance. These findings support the interpretation that HSCTGR forms part of the spectrum of sarcomas showing fibroblastic differentiation. PMID:11280598

Scolyer, R A; McCarthy, S W; Wills, E J; Palmer, A A



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.



Vomerine hamartoma in a cleft palate child  

PubMed Central

A case of a female child born at full term after normal vaginal delivery with bilateral secondary complete cleft palate and vomerine hamartoma mimicking intra-oral midline encephalocoele. Radiologically the tumour was confined to the vomer without intra-cranial extension. The lesion was occupying the oral cavity causing feeding problem. Surgical excision of the tumour at the age of six months and two flaps palatoplasty at the age of twelve months were performed. On histopathology the lesion turned out to be a lipomatous hamartoma of a benign nature. The child was followed for 9 years with no evidence of recurrence and a satisfactory speech. To our knowledge this is the first report of cleft palate with vomerine hamartoma in the English literature.

Bang, Rameshwar L.; Burezq, Hisham; Al-Najjadah, Imad



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.



Encephalocraniocutaneous Lipomatosis (Haberl and syndrome): A Case Report and Review of Literature  

PubMed Central

Encephalocraniocutaneous lipomatosis (ECCL) is a rare sporadic neurocutaneous syndrome characterized by presence of central nervous system, ocular and cutaneous anomalies. The exact pathogenesis is still not known. We present the third case from the Indian subcontinent, who is a five year old girl with history of right sided seizures. Dermatological examination showed alopecia on right side of the scalp and ipsilateral limbal dermoid and nodular skin tags over the upper eyelid. The computerized tomography scan of the brain revealed porencephalic cyst, cerebral calcifications and atrophy of right brain. The histopathology of the skin lesions showed lipomatous hamartoma and features of non scarring alopecia. The constellation of these findings and in adherence to the diagnostic criteria of ECCL proposed in 2009, we consider this report as a definite case of ECCL.

Koti, Kalyan; Bhimireddy, Vijayalakshmi; Dandamudi, Srinivas; Gunnamreddy, Ramanareddy



Patient presenting with lipoma of the index finger: a case report  

PubMed Central

Introduction Lipomas can be found anywhere in the body with the majority located in the head and neck region as well as in the shoulder and back. They are not very common in the hand and those involving the fingers are very rare. Although, it is not the only case reported, lipoma of the index finger is very uncommon. Case presentation A 52-year-old Caucasian man presented with a lipoma of the right index finger. He complained of no pain but he had difficulty in manual movements. Treatment was surgical excision of the lipoma. There has been no recurrence for two years. Conclusion Although lipomas of the fingers are rare entities, their awareness is imperative since the differential diagnosis from other soft tissue tumors and from the special lipomatous subtype involved is quite extensive.



Encephalocraniocutaneous Lipomatosis (Haberl and syndrome): A case report and review of literature.  


Encephalocraniocutaneous lipomatosis (ECCL) is a rare sporadic neurocutaneous syndrome characterized by presence of central nervous system, ocular and cutaneous anomalies. The exact pathogenesis is still not known. We present the third case from the Indian subcontinent, who is a five year old girl with history of right sided seizures. Dermatological examination showed alopecia on right side of the scalp and ipsilateral limbal dermoid and nodular skin tags over the upper eyelid. The computerized tomography scan of the brain revealed porencephalic cyst, cerebral calcifications and atrophy of right brain. The histopathology of the skin lesions showed lipomatous hamartoma and features of non scarring alopecia. The constellation of these findings and in adherence to the diagnostic criteria of ECCL proposed in 2009, we consider this report as a definite case of ECCL. PMID:23723477

Koti, Kalyan; Bhimireddy, Vijayalakshmi; Dandamudi, Srinivas; Gunnamreddy, Ramanareddy



Thymolipoma with sebaceous differentiation: a hitherto unreported variant of thymolipoma.  


Thymolipoma is a very rare anterior mediastinal mass of thymic origin, accounting for only 2% to 10% of all thymic neoplasm. Histologically, the tumor is usually composed of adipose tissue interspersed with thymic tissues. We report a case of a 58-year-old man who presented with a few weeks history of shortness of breath, cough, and chest pain, found to have a retrocardiac infiltrate on chest X-ray. Computed tomography scan of the chest showed a large anterior mediastinal mass. Surgical excision revealed an ovoid fatty mass, which on histological examination displayed thymic tissue intermixed with lipomatous tissue consistent with a thymolipoma. Of note was the presence of a microscopic nodule of thymic epithelial proliferation with sebaceous differentiation and focal cylindroma-like architecture. Although sebaceous tissue has been rarely reported in thymic tissue, to the best of our knowledge, this is the first case report of sebaceous differentiation in a thymolipoma. PMID:23358435

Ajaz, Bushra; Tran, Tien Anh Nguyen; Truong, Tuan; Manoucheri, Manoucher



Compression of the Superior Vena Cava by an Interatrial Septal Lipoma: A Case Report  

PubMed Central

Primary cardiac tumours are rare; their prevalence ranges from 0.0017% to 0.28% in various autopsy series. Cardiac lipomas are well-encapsulated benign tumours typically composed of mature fat cells, and their reported size ranges from 1 to 15?cm. They are usually seen in the left ventricle and the right atrium. Lipomas are true neoplasms, as opposed to lipomatous hypertrophy of the interatrial septum, which is a nonencapsulated hyperplastic accumulation of mature and foetal adipose tissue. Cardiac lipomas occur in patients of all ages, and the frequency of occurrence has been found to be equal in both sexes. Patients are usually asymptomatic, although the manifestation of symptoms depends upon both size and location of the tumour. We present the case of a patient with an interatrial septal lipoma, causing obstruction of the superior vena cava.

Grech, R.; Mizzi, A.; Grech, S.



Optic nerve glioma treatment with fractionated stereotactic radiotherapy.  


In the current report, the authors present a case of optic nerve glioma treated with fractionated stereotactic radiotherapy (FSRT). An 11-year-old girl was referred to our clinic with increasing proptosis over a 1-year period. At that time orbital MRI revealed a 20 × 17-mm mass in the right retroorbital lipomatous tissue, and FSRT was delivered to the tumor using the CyberKnife. During the 1.5-year follow-up, ophthalmological examinations did not indicate any treatment-related severe toxicity, and posttreatment MRI demonstrated marked regression of the lesion to 13 × 10 mm. Given the scarcity of reports on this subject, the authors support more extended studies of the CyberKnife for the effective treatment of this relatively common childhood tumor. PMID:23495806

Uslu, Nuri; Karakaya, Ebru; Dizman, Aysen; Yegen, Dincer; Guney, Yildiz



Asymptomatic giant mediastinal mass: a rare case of thymolipoma.  


Thymolipoma is a very rare benign neoplasm of the thymus. We present the case of a 42-year-old male with a massive mediastinal tumor discovered on a chest X-ray after a motorcycle accident. The patient had no complaints, and his physical examination was unremarkable. Chest CT scans revealed a lipomatous mass containing areas of soft tissue density. The patient was submitted to median sternotomy expanded to left anterolateral thoracotomy, resulting in the complete excision of the tumor. The pathological diagnosis was thymolipoma. Thymolipoma usually attains enormous dimensions by the time of diagnosis, and the occasional symptoms are related to compression of adjacent structures. Surgical resection is the treatment of choice and offers the only possibility of cure. The airway deserves special care during anesthesia induction. PMID:19918636

Mourad, Omar Moté Abou; Andrade, Filipe Moreira de; Abrahão, Pedro; Monnerat, Andréa; Judice, Luiz Felippe



Lipoblastoma of the back; a case of a 7-month-old boy.  


Lipoblastomatous tumours are rare, benign tumours of embryonic adipose tissue which primarily occur in children younger than 3 years. Most common, these tumours can be found in the extremities, yet cases involving other locations, such as the trunk, face, neck and mediastinum, have been described and they have an excellent prognosis despite its potential to local invasion and rapid growth. In medical literature two types are known, the focal well circumscribed lipoblastoma, and the more diffusely infiltrating lipoblastomatosis. The authors report a case of a 7-months old Ethiopian boy with a congenital lipoblastoma on the back. Despite surgical intervention, we were confronted with recurrences. In conclusion, we stress the morbidity of possible surgical management and the difficulty in decision-making. Therefore, awareness in young children with a fast growing lipomatous mass for this clinical and histopathological entity is crucial. PMID:19803275

Van Waes, C; Bronckaers, M


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.

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



Phenotypic progression of skeletal anomalies in CLOVES syndrome.  


Overgrowth syndromes, defined as genetic disorders in which there is disproportionate somatic growth, are challenging to diagnose due to their heterogeneous presentations and possible differing genetic etiologies. CLOVES syndrome is characterized by congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal abnormalities (Scoliosis). We describe a developmental follow up of the skeletal changes in CLOVES syndrome and a detailed account of its management. We demonstrate the asymmetric growth rate of toes responsible for the macrodactyly observed, and present additional phenotypic findings, including postnatal onset of abdominal symmetry and hepatomegaly. While the etiology of CLOVES is still a mystery, its similarity to Klippel-Trenaunay syndrome suggests a shared defect in a common signaling pathway, and its asymmetric bone overgrowth supports a mosaic genetic defect as its etiology. PMID:22628280

Klein, Steven; Stroberg, Albert; Ghahremani, Shahnaz; Martinez-Agosto, Julian A



Congenital diffuse infiltrating facial lipomatosis  

PubMed Central

Congenital diffuse infiltrating lipomatosis of the face (CDIL-F) is a rare pathological entity belonging to the subgroup of lipomatous tumors. Till date only a handful of cases has been documented and known to occur exclusively in infancy. On microscopical examination, it is characterized by diffuse infiltration of mature adipose tissue over normal muscle fibers, rapid growth, associated osseous hyperplasia, and a high recurrence rate after surgical intervention. An attempt has been made to identify and characterize all the 49 documented cases of CDIL-F in literature along with describing a report of a male child with CDIL-F. Follow-up of 8 years has been documented. The pathogenesis and spectrum of treatment modality are discussed with identified clinical features.

Balaji, S. M.



Somatic Mosaic Activating Mutations in PIK3CA Cause CLOVES Syndrome  

PubMed Central

Congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies (CLOVES) is a sporadically occurring, nonhereditary disorder characterized by asymmetric somatic hypertrophy and anomalies in multiple organs. We hypothesized that CLOVES syndrome would be caused by a somatic mutation arising during early embryonic development. Therefore, we employed massively parallel sequencing to search for somatic mosaic mutations in fresh, frozen, or fixed archival tissue from six affected individuals. We identified mutations in PIK3CA in all six individuals, and mutant allele frequencies ranged from 3% to 30% in affected tissue from multiple embryonic lineages. Interestingly, these same mutations have been identified in cancer cells, in which they increase phosphoinositide-3-kinase activity. We conclude that CLOVES is caused by postzygotic activating mutations in PIK3CA. The application of similar sequencing strategies will probably identify additional genetic causes for sporadically occurring, nonheritable malformations.

Kurek, Kyle C.; Luks, Valerie L.; Ayturk, Ugur M.; Alomari, Ahmad I.; Fishman, Steven J.; Spencer, Samantha A.; Mulliken, John B.; Bowen, Margot E.; Yamamoto, Guilherme L.; Kozakewich, Harry P.W.; Warman, Matthew L.



Angiolymphoid hyperplasia with eosinophilia--an incidental finding after surgical excision.  


Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign vasoproliferative lesion. Although it is a benign disease, lesions are often persistent and difficult to eradicate. ALHE typically presents clinically as papules or nodules, tan, brown, pink or dull red in colour, located predominantly in the head and neck region, especially around the ears and on the forehead and scalp.All races can be affected and no gender predominance exists. The disease also has nonspecific clinical features, hence it requires in most of the cases biopsy for accurate diagnosis. We present an uncommon clinical presentation of the disease, mimicking clinically a subcutaneous lipomatous mass, which has been treated successfully with surgical excision. PMID:22956004

Tchernev, Georgi; Taneva, Teodora; Ananiev, Julian; Cardoso, José Carlos; Gulubova, Maya; Velev, Valentin; Karashmalakov, Anatoli; Zisova, Liliya; Geilen, Christoph C



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.  


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



Evaluation of cardiac masses: the role of cardiovascular magnetic resonance.  


Evaluation of known or suspected cardiac masses is a frequent and expanding indication for referral to the cardiac magnetic resonance (CMR) laboratory. Most patients will have undergone an initial echocardiogram that raised the suspicion of an abnormality. However, echocardiography suffers from several well-described limitations: restricted field of view; incomplete assessment of an invading cardiac mass due to an unfavourable patient body habitus; and limited ability to perform tissue characterization. The role of CMR in this setting is well established because of its ability to obtain a wide field of view, generate high contrast and spatial resolution, and perform multiplanar imaging, allowing precise demonstration and localization of a mass. In clinical practice, CMR serves several useful purposes. First, it is able to help discriminate between a true cardiac mass and a pseudomass. Second, tissue characterization by CMR can assist in generating a differential diagnosis, and can distinguish a cardiac neoplasm (which generally will require excision) from other conditions, such as intracardiac thrombus, lipomatous hypertrophy or benign lipomas (all of which generally do not require excision). Third, even when the etiology of a mass is known, CMR can provide useful information as to the extent of invasion into cardiac, as well as extracardiac structures, and associated findings. This article provides a general overview as to how CMR may be clinically useful to the practicing cardiovascular specialist. PMID:20834205

Shah, Dipan J


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.

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



The tip of the iceberg: a giant pelvic atypical lipoma presenting as a sciatic hernia  

PubMed Central

Background This case report highlights two unusual surgical phenomena: lipoma-like well-differentiated liposarcomas and sciatic hernias. It illustrates the need to be aware that hernias may not always simply contain intra-abdominal viscera. Case presentation A 36 year old woman presented with an expanding, yet reducible, right gluteal mass, indicative of a sciatic hernia. However, magnetic resonance imaging demonstrated a large intra- and extra-pelvic fatty mass traversing the greater sciatic foramen. The tumour was surgically removed through an abdomino-perineal approach. Subsequent pathological examination revealed an atypical lipomatous tumour (synonym: lipoma-like well-differentiated liposarcoma). The patient remains free from recurrence two years following her surgery. Conclusion The presence of a gluteal mass should always suggest the possibility of a sciatic hernia. However, in this case, the hernia consisted of an atypical lipoma spanning the greater sciatic foramen. Although lipoma-like well-differentiated liposarcomas have only a low potential for recurrence, the variable nature of fatty tumours demands that patients require regular clinical and radiological review.

Skipworth, Richard JE; Smith, Graeme HM; Stewart, Ken J; Anderson, David N



Soft Tissue Tumours of the Retroperitoneum  

PubMed Central

Purpose. This review summarizes the more prevalent soft tissue tumours arising in the retroperitoneum and highlights some recent fundamental and diagnostic developments relevant to mesenchymal tumours. Discussion. The retroperitoneum is an underestimated site for benign and malignant neoplastic disease, and represents the second most common site of origin of primary malignant soft tissue tumours (sarcomas) after the deep tissues of the lower extremity. In contrast to the predominance of benign soft tissue lesions over malignant sarcomas elsewhere, retroperitoneal mesenchymal lesions are far more likely to be malignant. The differential diagnosis is primarily with the more common lymphoproliferative and parenchymatous epithelial lesions arising in this area, and with metastatic disease from known or unknown primary sites elsewhere.The most prevalent mesenchymal tumours at this site are of a lipomatous, myogenic or neural nature.Their generally late clinical presentation and poorly accessible location provides numerous clinical challenges; optimal radiological imaging and a properly performed biopsy are essential cogs in the management route. Histopathological diagnosis may be complicated, but has been aided by developments in the fields of immunohistochemistry and tumour (cyto)genetics. Despite significant advances in oncological management protocols, the prognosis remains generally less favourable than for similar tumours at more accessible sites.

Van Roggen, J. Frans Graadt



[Giant atypical muscle-involving lipoma of the right thigh: a case report and review of the literature].  


The clinical case reported here concerns a giant lipoma (22 x 12 x 10 cm; 2740 g) located in the distal region of the right thigh, in a subfascial zone. The patient was referred to our department for a relapse of thrombophlebitis of the right lower limb (occurring about two years earlier) and presented a large tumefaction of the mid third of thigh at objective examination, the presumed onset of which dated back about ten years after a traumatic accident. Ultrasonography confirmed the presence of the tumefaction but did not allow us to identify its origin with certainty. Musculoskeletal magnetic resonance imaging, however, revealed a gross expansive lesion closely connected to the distal part of the femur, suggesting its probable benign nature. The mass was totally resected without either muscle section or the sacrifice of periosteum. The histological findings indicated an atypical lipomatous tumour. The therapy of giant lipomas is invariably surgical excision. Nevertheless, the high risk of relapse (which is typical of this kind of neoplastic proliferation) despite radical resection, makes a systematic postoperative follow-up necessary. PMID:16400775

Fimmanò, Antonio; Coppola Bottazzi, Enrico; Cirillo, Claudia; Tammaro, Pasquale; Casazza, Dino


A case report of an intermuscular lipoma: presentation, pathophysiology, differential diagnosis  

PubMed Central

Objective The purpose of this report is to present the case of a 54-year-old female patient with a pathologically confirmed diagnosis of soft tissue lipoma. The clinical presentation, pathophysiology, differential diagnosis, and treatment options for lipoma are described. Clinical Features The patient presented to a chiropractic clinic with painful swelling in the right anterior thigh. Physical examination revealed a palpable mobile mass with minimal tenderness. Intervention and Outcome The initial consultation was with the primary care physician, and it resulted in a sonographic examination as the initial imaging modality to rule out deep venous thrombosis. The result of this examination was negative. Following a delay by additional incorrect diagnoses and treatments, a diagnosis of an intermuscular lipoma was established by magnetic resonance imaging and confirmed on biopsy as a soft tissue lipoma. The patient underwent complete excision of the mass. Conclusion Lipomas are common benign soft tissue masses and should be considered in the differential diagnosis of a thigh mass. Magnetic resonance imaging with contrast depicts the lipomatous mass as having a characteristic high-intensity fat signal without enhancement. It is important for clinicians to have an increased awareness in recognizing the clinical and imaging manifestations of a soft tissue lipoma to optimize an initial diagnosis and treatment.

Kaeser, Martha A.; Smith, Linda W.; Kettner, Norman W.



MRI Features of Spinal Epidural Angiolipomas  

PubMed Central

Objective To describe the MRI findings in ten patients of spinal epidural angiolipoma for differentiated diagnosis presurgery. Materials and Methods Ten surgically proved cases of spinal epidural angiolipomas were retrospectively reviewed, and the lesion was classified according to the MR findings. Results Ten tumors were located in the superior (n = 4), middle (n = 2), or inferior (n = 4) thoracic level. The mass, with the spindle shape, was located in the posterior epidural space and extended parallel to the long axis of the spine. All lesions contained a fat and vascular element. The vascular content, correlating with the presence of hypointense regions on T1-weighted imaging (T1WI) and hyperintense signals on T2-weighted imaging, had marked enhancement. However, there were no flow void signs on MR images. All tumors were divided into two types based on the MR features. In type 1 (n = 3), the mass was predominantly composed of lipomatous tissue (> 50%) and contained only a few small angiomatous regions, which had a trabeculated or mottled appear. In type 2 (n = 7), the mass, however, was predominantly composed of vascular components (> 50%), which presented as large foci in the center of the mass. Conclusion Most spinal epidural angiolipomas exhibit hyperintensity on T1WI while the hypointense region on the noncontrast T1WI indicates to be vascular, which manifests an obvious enhancement with gadolinium administration.

Hu, Su; Hu, Xiao-yun; Wang, Xi-ming; Dai, Hui; Fang, Xiang-ming; Cui, Lei



Sudden onset of paraplegia caused by hemorrhagic spinal epidural angiolipoma. A case report  

PubMed Central

Spinal epidural angiolipoma is a rare benign tumor containing vascular and mature adipose elements. A slow progressive clinical course was mostly presented and rarely a fluctuating course during pregnancy. The authors report the original case of spontaneous spinal epidural bleeding resulting from thoracic epidural angiolipoma who presented with hyperacute onset of paraplegia, simulating an extradural hematoma. The patient was admitted with sudden non-traumatic hyperacute paraplegia during a prolonged walk. Neurologic examination showed sensory loss below T6 and bladder disturbances. Spinal MRI revealed a non-enhanced heterogeneous thoracic epidural lesion, extending from T2 to T3. A bilateral T2–T4 laminectomy was performed to achieve resection of a lipomatous tumor containing area of spontaneous hemorrhage. The postoperative course was uneventful with complete neurologic recovery. Histologic examination revealed the tumor as an angiolipoma. Because the prognosis after rapid surgical management of this lesion is favorable, the diagnosis of spinal angiolipoma with bleeding should be considered in the differential diagnosis of hyperacute spinal cord compression.

Albouzidi, Abderrahmane; Elmostarchid, Brahim; Gazzaz, Miloudi; Boucetta, Mohamed



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.

Nishio, Jun



CT characteristics of primary retroperitoneal neoplasms in children.  


Primary retroperitoneal neoplasms are uncommon in children. Retroperitoneal neoplasms are either mesodermal, neurogenic, germ cell ectodermal or lymphatic in origin. In general, primary retroperitoneal neoplasms in children have different spectrum and prevalence compared to those in adults. Neuroblastoma, rhabdomyosarcoma, benign teratoma and lymphoma are the common retroperitoneal neoplasms. In this review, the clinical and CT futures of common retroperitoneal neoplasms in children are described. Coarse, amorphous, and mottled calcification are very common in neuroblastoma. Paraganglioma tends to show marked and early enhancement and may present with clinical symptoms associated with the excess catecholamine. Sarcomas are often very large and have heterogeneous appearance. Imaging cannot be reliably used to identify the type of retroperitoneal sarcomas due to overlapped radiographic features. In children, lipoblastoma is the most common lipomatous tumor in the retroperitoneum. The percentage of visible fat in tumor varies depending on the cellular composition of the lesion. The CT characteristics of teratoma are quite variable, which may be cystic, solid, on a combination of both. Typically teratoma appears as a large complex mass containing fluid, fat, fat-fluid level, and calcifications. Lymphoma is often homogeneous on both enhanced and unenhanced CT scans. Necrosis and calcification are rare on CT. In conclusion, making a final histological diagnosis of retroperitoneal tumor base on CT features is not often possible; however, CT can help to develop a differential diagnosis and determine the size and extent of the retroperitoneal neoplasms. PMID:20591598

Xu, Yufeng; Wang, Jichen; Peng, Yun; Zeng, Jinjin



Desmin-positivity in spindle cells: under-recognized immunophenotype of lipoblastoma.  


Lipoblastoma is a distinct benign fatty tumor composed of adipocytes, lipoblasts, and primitive mesenchymal cells with a myxoid stroma. Lipoblastoma harbors characteristic fusion genes involving the PLAG1, resulting in aberrant expression of PLAG1. However, the nature of the primitive mesenchymal cells remains obscure. In our routine pathology practice, we noticed desmin-positive spindle mesenchymal cells in lipoblastomas, which is a hitherto poorly described phenomenon. Thus, we examined the expression of several myogenic markers including desmin in a variety of 95 mesenchymal tumors with fatty elements. Fourteen of the 15 lipoblastomas examined contained desmin-positive spindle cells, which also showed nuclear expression of PLAG1, whereas ?-smooth muscle actin, muscle specific actin, h-caldesmon, and myogenin were negative. Some spindle cells in subsets of atypical lipomatous tumors/well differentiated liposarcomas (6/20), dedifferentiated liposarcomas (11/31) and pleomorphic liposarcomas (2/10) were positive for actins and/or desmin, supporting focal myofibroblastic or smooth muscle differentiation. The other tumors, including 11 myxoid/round cell liposarcomas, four spindle cell lipomas, and four lipofibromatoses, were negative for all of the myogenic markers assessed. The almost consistent desmin expression in spindle mesenchymal cells suggests a potential diagnostic utility of this marker and myofibroblastic phenotype of fractions in lipoblastoma cells. PMID:23865573

Kubota, Fumie; Matsuyama, Atsuji; Shibuya, Ryo; Nakamoto, Mitsuhiro; Hisaoka, Masanori



Fine-Needle Aspiration Cytology of Soft Tissue Sarcoma: Benefits and Limitations  

PubMed Central

Purpose. Examine the benefits and limitations of fine-needle aspiration cytology (FNA) used as the definitive diagnostic method before treatment. Method. Review of the 25 year experience at a multidisciplinary musculo-skeletal centre where FNA is the primary diagnostic approach to soft tissue sarcoma in the extremities and trunk wall and the experience of various experts in the field. Results. FNA has several benefits compared with coarse needle or open surgical biopsy. The most important are rapid preliminary diagnosis, no need for hospitalization and anaesthesia, negligible complications and fear for tumour cell spread. With the collected experience gained during the years a reliable diagnosis of sarcoma is the rule in general and specific-type diagnoses are possible in many histotypes, especially when the cytologic examination is supplemented with ancillary diagnostics. The most important limitations are inability to hit small deep-seated sarcoma and some diagnostic pitfalls such as the correct diagnosis of spindle cell neoplasms, variants of benign lipomatous tumours and ‘new soft tissue tumour entities’. Discussion. Optimal use of FNA calls for certain requirements such as centralization, experience in soft tissue tumour cytology–histopathology, the FNA technique and close co-operation between the orthopaedic surgeon and cytopathologist.



Left Supraclavicular Spindle Cell Lipoma  

PubMed Central

Background. Spindle cell lipoma (SCL) is a benign lipomatous tumour, typically occurring in the posterior neck, shoulder or upper back of elderly males. They compose of fat, CD34 positive spindle cells, and ropey collagen on a myxoid matrix. This case highlights a rare presentation of SCL and the need for pre-operative diagnosis. Case Report. A 63-year-old gentleman presented with a pre-existing left supraclavicular mass that had recently increased in size. FNA and CT Scans were performed and results discussed in the mutidisciplinary team meeting. Excisional biopsy was recommended. Radiology. CT neck showed a left supraclavicular mass of fatty density with fine internal septations. A low-grade liposarcoma could not be excluded. Histopathology. FNA was indeterminate. Histology of specimen showed bland spindle cells with no evidence of malignancy. Immuno-histochemistry showed SCL with CD34 positivity and negative staining on CDK4 and p16. Management. Excision biopsy of the mass was performed which was technically difficult as the mass invaginated around the brachial plexus. The patient recovered well post-operatively with no neurological deficits. Conclusion. Spindle cell lipoma is a rare benign tumour and a pre-operative diagnosis based on the clinical context, imaging and immuno-histochemistry is crucial to management.

Olaleye, Oladejo; Fu, Bertram; Moorthy, Ram; Lawson, Charles; Black, Myles; Mitchell, David



PLAG1 expression in mesenchymal tumors: an immunohistochemical study with special emphasis on the pathogenetical distinction between soft tissue myoepithelioma and pleomorphic adenoma of the salivary gland.  


PLAG1, a proto-oncogene activated in several types of tumors including pleomorphic adenoma of the salivary gland and lipoblastoma, is usually overexpressed because of chromosomal aberrations resulting in fusion genes. Myoepithelial tumors in soft tissue are morphologically similar to pleomorphic adenoma, but the genetic profiles of these tumors have not been fully examined. In the present study, we immunohistochemically evaluated the expression of PLAG1 in a series of 243 mesenchymal tumors. We determined that 14 tumors, including eight of 10 lipoblastomas, two of seven gastrointestinal stromal tumors, one of two angiomyofibroblastomas, one of five synovial sarcomas, one of seven leiomyomas and one of 12 myxofibrosarcomas were positive for PLAG1, whereas all seven soft tissue myoepitheliomas were PLAG1 negative. We examined two soft tissue myoepitheliomas, whose paraffin blocks were available, for fusion gene transcripts involving PLAG1 or HMGA2 specific for pleomorphic adenoma by a reverse transcription-polymerase chain reaction assay, and no fusion transcripts were detected. Our results suggest that soft tissue myoepithelioma may be a pathogenetically distinct tumor entity from pleomorphic adenoma based on the absence of PLAG1 overexpression and characteristic fusion genes. On the other hand, PLAG1 immunohistochemistry is useful for distinguishing lipoblastoma from other lipomatous tumors including liposarcoma. PMID:22192798

Matsuyama, Atsuji; Hisaoka, Masanori; Hashimoto, Hiroshi



A comparison of cytological and histopathological findings and role of immunostains in the diagnosis of soft tissue tumors  

PubMed Central

Background: Fine needle aspiration cytology (FNAC) has been employed as a useful technique for the initial diagnosis of soft tissue tumors (STT) as well for the identification of recurrent and metastatic cases. Aim: We conducted this study on soft tissue tumors to find the efficacy of FNAC and to finalize the histological diagnosis with immunostains. Materials and Methods: The present study was conducted on 126 patients of soft tissue tumors. FNAC and histopathology was performed in all the cases. Results: Hundred and five cases (83.3%) were diagnosed as benign and 21 cases (16.7%) as malignant. On FNAC, tumors were divided into six cytomorphological categories i.e. lipomatous, spindle cell, round cell, myxoid, pleomorphic and vascular tumors. Seventeen cases were inconclusive on cytology. In five cases, the type of malignancy was changed on histological examination. There were three false positive and two false negative cases giving a positive predictive value of 97.2 % in terms of malignancy, a sensitivity of 98.1% and a specificity of 96.7%. Conclusions: FNAC has a definite role in forming the initial diagnosis of STT, while histopathology with the aid of immunomarkers provides the final diagnosis.

Beg, Shaham; Vasenwala, Shaista M; Haider, Nazima; Ahmad, S Shamshad; Maheshwari, Veena; Khan, MA



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.

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



Castleman disease of the subcutis and underlying skeletal muscle: report of 6 cases.  


Castleman disease of the subcutis and skeletal muscle is rare. We have collected a series of 6 cases of extranodal Castleman disease, located in the subcutis and skeletal muscles of the extremities and trunk. Tumors from mediastinal and retroperitoneal soft tissue and those histologically involving peripheral or truncal lymph nodes were excluded. There were four females and two males; ages ranged from 18 to 37 years (mean, 26 years). Locations included thigh (n = 3), anterior chest (n = 2), and upper arm (n = 1). Sizes ranged from 4.0 to 6.0 cm (mean, 5.2 cm). All patients presented with localized disease. One patient had involvement of the mediastinum 1 year prior to the appearance of his soft tissue lesion. None of the patients demonstrated systemic involvement or signs of the POEMS syndrome. Histopathologically, all cases were classified as hyaline-vascular type (HVCD). In 3 cases, follicular dendritic cell dysplasia was observed. In 1 case, the dysplasia was marked. The subcutaneous lesion of 1 patient revealed a maze of capillaries set in a lipomatous background with occasional lymphoid follicles possessing hyalinized lymphocyte-depleted centers. This lesion probably represented incipient HVCD. Molecular biologic studies did not reveal the presence of Epstein-Barr virus or human herpesvirus-8 DNA in the lesional tissue. There also were no monoclonal rearrangements of IgH. Four patients with follow-up included 2 patients with no evidence of disease at 10 and 13 years, respectively, and 2 patients with local recurrence at 2 and 6 months, respectively. In conclusion, soft tissue Castleman disease is a disease of young patients with a female predominance and a propensity to involve the trunk and limbs. It can be of large size and is generally solitary. There may be mild to marked follicular dendritic cell dysplasia. The HVCD predominates in this location. These lesions are usually unassociated with POEMS, Epstein-Barr virus, human herpesvirus-8, or monoclonal rearrangements of IgH. PMID:15105643

Kazakov, Dmitry V; Fanburg-Smith, Julie C; Suster, Saul; Neuhauser, Thomas S; Palmedo, Gabriele; Zamecnik, Michal; Kempf, Werner; Michal, Michal



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


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



Contribution of US and CT for diagnosis of intraperitoneal focal fat infarction (IFFI): a pictorial review.  


The term IFFI--for intraperitoneal focal fat infarction--includes various acute abdominal clinical conditions in which focal fatty tissue necrosis represents the common pathologic denominator. Only differing by their various anatomical locations and dimensions, all cases nevertheless present rather similar clinical signs, aetiology, radiological features and prognosis. In clinical practise, most cases of IFFI concern torsion and/or infarction of the greater omentum or epiploic appendages. Rarer types of torsion and/or infarction of lipomatous appendages of the hepatic falciform ligament and of the lesser omentum have also been reported. Cases are finally described in the paediatric population. US and merely CT have been shown having a high sensitivity and specificity for the diagnosis of IFFI and in most cases the clinical evolution is spontaneously favourable. For these two reasons, the option of conservative treatment after specific imaging diagnosis now represents the other common denominator of IFFI. Such a safe and unambiguous imaging diagnosis of IFFI represents thus an important challenge for each abdominal radiologist with the intention of persuading the referent clinician to avoid unnecessary surgery for their patients. The aim of this pictorial review is to extensively explore not only the classical imaging findings of various types of IFFI but also to review the normal US and MDCT anatomy of the fatty abdominal structures being usually implicated in IFFI. More rare or atypical presentations are also illustrated as well as subacute findings and sequels. All reported patients were collected in our department during a 7-year-period and most were successfully treated conservatively. PMID:20957887

Coulier, B


Correlation between Clinicopathological Features and Karyotype in Spindle Cell Sarcomas  

PubMed Central

Soft-tissue tumors have proved to be a fruitful area for the identification of reproducible cytogenetic aberrations, especially among pediatric round-cell sarcomas and lipomatous tumors. Thus far, however, data regarding sarcomas of monomorphic spindle cell type have been limited and somewhat disappointing, with the notable exception of synovial sarcoma. As part of an ongoing international collaborative study, 130 karyotyped spindle-cell sarcomas were reviewed and classified histologically, without knowledge of the clinical and karyotypic data, with the aim of identifying objective correlations between morphology, karyotype, and clinical parameters. Clonal chromosomal abnormalities were identified in 82 cases studied (63%), but only in the group of synovial sarcomas was there clear correlation between the cytogenetic findings, in the form of a consistent t(X;18)(p11;q11), and morphology. Among leiomyosarcomas (41 cases) and malignant peripheral nerve sheath tumors (MPNSTs; 27 cases) as well as in individual examples of rarer entities, there was a general tendency for karyotypic complexity associated with frequent loss or rearrangement of chromosome arms 1p, 10p, 11q, 12q, 17p, and 22q. Rearrangements of 17q (the region of the NF1 gene) were seen in 9/27 (33%) of MPNSTs. Among nine cases of solitary fibrous tumor (in which previous cytogenetic data are very limited) no consistent aberrations were identified. We conclude that, with the exception of synovial sarcoma, most spindle-cell sarcomas share with pleomorphic sarcomas the tendency for karyotypic complexity. There was no indication (in most of these lesions) that detectable cytogenetic aberrations could either facilitate their diagnosis or help to determine prognosis. There is a clear need to further study and understand the significance of multiple chromosomal abnormalities in this group of mesenchymal neoplasms with the particular goal of determining their role in the process of tumor development.

Fletcher, Christopher D. M.; Dal Cin, Paola; de Wever, Ivo; Mandahl, Nils; Mertens, Fredrik; Mitelman, Felix; Rosai, Juan; Rydholm, Anders; Sciot, Raf; Tallini, Giovanni; van den Berghe, Herman; Vanni, Roberta; Willen, Helena



Lipoblastoma mimicking myxoid liposarcoma: a clinical report and literature review.  


Lipoblastoma is an uncommon benign lipomatous tumor, occurring typically in children less than 3 years of age. The magnetic resonance image (MRI) is a useful tool for diagnosis of lipoblastoma; its imaging typically shows high-intensity signals on both T1-weighted (T1-W) and T2 weighted (T2-W) images. Here, we present a 12-year-old female patient with a painless mass on the anterior right shoulder. MRI showed the mass with low-intensity signals on T1-W and high-intensity signals on T2-W images. Because of the atypical age and MRI findings, it was difficult to make a conclusive diagnosis of the tumor as lipoblastoma preoperatively. Histopathological examination of the excised tumor showed spindle-shaped or stellate cells embedded in the myxoid matrix, and a few small irregular clusters of mature fat cells that are separated by connective tissue septa. There were some immature, lipoblast-like cells dispersed. These findings are consistent with lipoblastoma, and myxoid liposarcoma was considered as one of the differential diagnosis. We finally diagnosed the tumor as a lipoblastoma for the reasons that there were many mature fat cells and no atypical cells for a myxoid liposarcoma. The postoperative course was uneventful and no recurrence was observed 5 years after the operation. The patient presented is worthy of note due to the unusual characteristics of the tumor. Even in the case of adolescent or older patients with atypical imaging, lipoblastoma should be considered as one of differential diagnosis. PMID:21212605

Nagano, Akihito; Ohno, Takatoshi; Nishimoto, Yutaka; Hirose, Yoshinobu; Miyake, Satoru; Shimizu, Katsuji



Impact of molecular analysis on the final sarcoma diagnosis: a study on 763 cases collected during a European epidemiological study.  


Sarcomas are rare, heterogenous, and often difficult to classify. A large proportion of sarcomas are associated with specific molecular genetic lesions such as translocations, mutations, and amplifications, which are helpful in the diagnosis of individual cases. However, the exact impact of molecular genetics on the final diagnosis of sarcomas is unknown. In this study, all soft tissue and visceral sarcomas arising in patients living in 3 European regions in 2 countries (representing 13 million inhabitants) were collected and reviewed during 2 consecutive years. A molecular analysis was performed for all suspicions of sarcomas with specific genetic lesions [mutations of KIT/PDGFRA in gastrointestinal stromal tumors (GISTs), reciprocal translocation, or amplification of MDM2 in atypical lipomatous tumors, well-differentiated liposarcoma-dedifferentiated liposarcoma (ALT/WDLPS-DDLPS)]. To evaluate the impact of molecular tests, a premolecular analysis diagnosis was proposed with 3 categories of certainty: certain, probable, or possible. A molecular analysis was performed in 763/1484 tumors corresponding to 295 cases in which GIST was suspected, 248 sarcomas with a suspicion of translocation, and 220 cases in which ALT/WDLPS-DDLPS was suspected. Molecular analysis was found to be useful (confirms a probable diagnosis) in 11 (4%) GISTs, 62 (26%) suspicions of translocation, and 66 (31%) suspicions of ALT/WDLPS-DDLPS; and necessary (allows a possible diagnosis) in 2 (<1%) GISTs, 31 (12%) suspicions of translocation, and 19 (9%) suspicions of ALT/WDLPS-DDLPS. This study performed in an epidemiological setting demonstrates the significant impact of molecular analysis on the final sarcoma diagnosis and favors such an analysis on any tumor with a suspicion of a specific genomic abnormality and for which the diagnosis is uncertain. PMID:23774173

Neuville, Agnes; Ranchère-Vince, Dominique; Dei Tos, Angelo Paolo; Montesco, Maria Cristina; Hostein, Isabelle; Toffolatti, Luisa; Chibon, Frédéric; Pissaloux, Daniel; Alberti, Laurent; Decouvelaere, Anne-Valérie; Albert, Sabrina; Rossi, Carlo Riccardo; Blay, Jean-Yves; Coindre, Jean-Michel



Scope of FNAC in the diagnosis of soft tissue tumors-A study from a tertiary cancer referral center in India  

PubMed Central

Background Fine needle aspiration cytology (FNAC) forms one of the first diagnostic tools in the evaluation of tumors. Its role in diagnosing soft tissue tumors (STT) has been fairly documented, as well as debated. Present study was aimed at evaluating its scope in diagnosing 127 cases of soft tissue tumors. Methods Conventional Pap and MGG staining was available in all the cases. Immunocytochemistry (ICC) was performed in 15 cases. Histopathological details were available in 115 cases. Results 50% cases were referred for a primary diagnosis, while 26.8% & 22.8% cases were evaluated for recurrent and metastatic lesions, respectively. Extremities were the commonest sites. On FNAC, 101 cases (79.5%) were labeled as malignant, whereas 10 cases (7.9%) were labeled as benign. The remaining 16 cases (11%) were not categorized and were labeled as 'unsure/not specified'. Histopathological confirmation in 115 cases, gave a diagnostic accuracy of 98%, with a positive predictive value of 98% in malignant cases and a negative predictive value of 100% in benign cases. Two cases were false positive. Among the various cytological categories, 60 cases (47.2%) were of spindle cell type, followed by 32 (25.2%) of round cell type and 14 cases (11%) of lipomatous type. Other 12 cases (9.4%) were of pleomorphic type; 7 (5.5%) cases of epithelioid type and remaining 2 cases were of myxoid type. All the round cell, pleomorphic and myxoid type of tumors were sarcomas, whereas 73.3% cases of spindle cell type were labeled as 'malignant'. Exact cytological sub typing was offered in 58 cases, with rhabdomyosarcoma (RMS) as the most frequently sub typed tumor. The two false positive malignant cases were of fibromatosis and a pigmented schwannoma, on biopsy. Out of 28 metastatic lesions, lymph nodes were the commonest site for metastasis, with epithelioid tumors that formed highest percentage of metastatic cases. Conclusion FNAC is fairly specific and sensitive in STT diagnoses for primary, recurrent and metastatic lesions. The cytological types, especially round cell and pleomorphic sarcomas, can be quickly identified. Clinicopathological correlation with ICC as an adjunct, are valuable in exact sub typing.

Rekhi, Bharat; Gorad, Biru D; Kakade, Anagha C; Chinoy, RF



Omental lipoblastoma.  


A large intra-abdominal mass was discovered in a 6-month-old boy during a routine well-child examination. Imaging studies revealed a solid mass which appeared to arise from the left lobe of the liver, extending caudally and filling the entire pelvis. At the time of surgical excision, the mass was found to be unassociated with the liver, but was instead localized to the omentum. Histologic examination revealed adipocytes of varying stages of maturation arranged in a lobular architecture, consistent with a lipoblastoma. This unusual tumor is only the eighth reported omental lipoblastoma [J. Hicks, A. Dilley, D. Patel, J. Barrish, S. Zhu, M. Brandt, Lipoblastoma and lipoblastomatosis in infancy and childhood: histologic, ultrastructural, and cytogenetic features. Ultrastruct. Pathol. 25 (2001) 321-333; J. Harrer, G. Hammon, T. Wagner, M. Bolkenius, Lipoblastoma and lipoblastomatosis: a report of two cases and review of the literature. Eur. J. Pediatr. Surg. 11 (2001) 342-349; S. Weiss and J. Goldblum, Enzinger and Weiss's Soft Tissue Tumors, fourth ed., Mosby, St. Louis, MO, 2001, pp. 601-605, 670-686; S. Soin, S. Andronikou, R. Lisle, K. Platt, K. Lakhoo, Omental lipoblastoma in a child; diagnosis based in CT density measurements. J. Pediatr. Hematol. Oncol. 28(1) (2006) 57-58; A. Prando, S. Wallace, J.L. Marins, R.M. Pereira, E.R. de Oliveira, M. Alvarenga, Sonographic features of benign intraperitoneal lipomatous tumors in children-report of 4 cases. Pediatr. Radiol. 20(8) (1990) 571-574; C. Blank, E. Schoenmakers, P. Rogalla, E. Huys, A. Van Rijk, N. Drieschner, J. Bullerdiek, Intragenic breakpoint within RAD51L1 in a t(6;14)(p21.3;q24) of a pulmonary chondroid hamartoma. Cytogenet. Cell Genet. 95 (2001) 17-19; S. Ingraham, R. Lynch, S. Kathiresan, A. Buckler, A. Menon, hREC2, a RAD51-like gene, is disrupted by t(12;14)(q15;q24.1) in a uterine leiomyoma. Cancer Genet. Cytogenet. 115 (1999) 56-61]. Cytogenetics revealed a karyotype of 46,XY,t(8;14)(q13;q24). While lipoblastomas characteristically involve 8q, only one prior case has been reported with 14q24 as its fusion partner [M. He, K. Das, M. Blacksin, J. Benevenia, M. Hameed, A translocation involving the placental growth factor gene is identified in an epithelioid hemangioendothelioma. Cancer Genet. Cytogenet. 168 (2006) 150-154]. We report this unique case of an omental lipoblastoma with a focus on its unusual karyotype, as well as its differentiation from myxoid liposarcoma. PMID:18276084

Koplin, Stephanie A; Twohig, Matthew H; Lund, Dennis P; Hafez, G Reza



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)