PALMAR WELL DIFFERENTIATED SPINDLE CELL LIPOSARCOMA: PRESENTATION OF A RARE TUMOR AT A RARE SITE

Hand Surgery ◽  
2013 ◽  
Vol 18 (01) ◽  
pp. 115-120 ◽  
Author(s):  
Sanjay D. Deshmukh ◽  
Harveen K. Gulati ◽  
Prashant Yadav ◽  
Pooja Naik
Keyword(s):  
World Literature ◽  
Spindle Cell ◽  
Soft Tissue Sarcomas ◽  
Low Grade ◽  
Aggressive Approach ◽  
Lipomatous Tumor ◽  
Synovial Sarcomas ◽  
Rare Site ◽  
Aggressive Tumors ◽  
Well Differentiated

The most common soft tissue sarcomas of hand are epitheloid sarcomas, synovial sarcomas and malignant fibrous histiocytomas which are high grade, aggressive tumors. Liposarcomas of the hand are extremely rare and to the best of our knowledge less than 20 cases have been reported so far in the literature. Well differentiated spindle cell liposarcoma is an extremely rare subtype of well differentiated liposarcoma/atypical lipomatous tumor which is different from the other subtypes clinicopathologically, genetically and prognostically. Palmar localization of this rare variant of liposarcoma at this site makes ours only the second case reported in the world literature so far. This recently described entity is low grade lesion and the hand surgeons need to adopt a less aggressive approach in the treatment of this subtype.

“Fat-Rich” (Spindle Cell-Poor) Variant of Atypical Spindle Cell/Pleomorphic Lipomatous Tumor: Striking Mimic of “Classical” Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma

2019 ◽  
Vol 27 (8) ◽  
pp. 868-871 ◽  
Author(s):  
David Creytens ◽  
Thomas Mentzel ◽  
Liesbeth Ferdinande ◽  
Joost van Gorp ◽  
Jo Van Dorpe ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Molecular Genetic ◽  
Low Grade ◽  
Tissue Mass ◽  
Atypical Lipomatous Tumor ◽  
Lipomatous Tumor ◽  
Multinucleated Cells ◽  
Pericytic Mimicry ◽  
Well Differentiated ◽  
The Right

We report the case of a “fat-rich” (spindle cell-poor) variant of an atypical spindle cell/pleomorphic lipomatous tumor in a 63-year-old female patient presenting with a firm, painless soft tissue mass on the right hip. Atypical spindle cell/pleomorphic lipomatous tumor is a very recently described low-grade adipocytic neoplasm, which occurs predominantly in adults with a predilection for the limbs and limb girdles. In the present case, the diagnosis of an atypical spindle cell/pleomorphic lipomatous tumor was challenging because the tumor was almost exclusively composed of an atypical adipocytic component (resembling “classical” atypical lipomatous tumor/well-differentiated tumor) with only very focal presence of the diagnostic atypical morphologic features (atypical spindle-shaped cells, floret-like multinucleated cells, and “bizarre” pleomorphic [multinucleated] cells). The possibility of a “classical” atypical lipomatous tumor/well-differentiated liposarcoma was ruled out by immunohistochemistry (lack of MDM2 expression and loss of Rb expression) and molecular genetic testing (no amplification of MDM2 and presence of monoallelic deletion of RB1). Another interesting morphologic observation in this case was the striking perivascular location of the atypical spindle/pleomorphic cells in some areas (so-called “pericytic mimicry”). To our knowledge, pericytic mimicry has not been reported in the setting of an atypical spindle cell/pleomorphic lipomatous tumor.

scholarly journals MDM2 Amplified Sarcomas: A Literature Review

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 496
Author(s):  
Raf Sciot
Keyword(s):  
Suppressor Gene ◽  
Negative Regulator ◽  
Low Grade ◽  
P53 Mutations ◽  
Lipomatous Tumor ◽  
Double Minute ◽  
Genetic Features ◽  
Murine Double Minute ◽  
Well Differentiated ◽  
Mdm2 Amplification

Murine Double Minute Clone 2, located at 12q15, is an oncogene that codes for an oncoprotein of which the association with p53 was discovered 30 years ago. The most important function of MDM2 is to control p53 activity; it is in fact the best documented negative regulator of p53. Mutations of the tumor suppressor gene p53 represent the most frequent genetic change in human cancers. By overexpressing MDM2, cancer cells have another means to block p53. The sarcomas in which MDM2 amplification is a hallmark are well-differentiated liposarcoma/atypical lipomatous tumor, dedifferentiated liposarcoma, intimal sarcoma, and low-grade osteosarcoma. The purpose of this review is to summarize the typical clinical, histopathological, immunohistochemical, and genetic features of these tumors.

Malignant Ameloblastoma, Spindle Cell Variant

2003 ◽  
Vol 127 (3) ◽  
pp. 352-355
Author(s):  
Richard J. Zarbo ◽  
Mark T. Marunick ◽  
Robert Johns
Keyword(s):  
Spindle Cell ◽  
Intracranial Extension ◽  
Low Grade ◽  
Epithelial Proliferation ◽  
American Girl ◽  
Spindle Cell Proliferation ◽  
Histologic Pattern ◽  
Cell Variant ◽  
Well Differentiated ◽  
African American Girl

Abstract In this report, we document the histologic and clinical features of a previously undefined spindle cell variant of ameloblastoma that eventually behaved in a malignant fashion during a protracted course. The predominant histologic pattern was a well-differentiated, cellular, spindled epithelial proliferation arising in the maxilla of a 14-year-old African American girl. Over 19 years, the patient experienced numerous local recurrences, metastases to distant bones after 15 years, and finally bulky local recurrence with intracranial extension resulting in death. This ameloblastic malignancy histologically simulates a low-grade true sarcoma or an ameloblastic sarcoma, but differs in that the extensive spindle cell proliferation is epithelial, characterized by strong cytokeratin immunoreactivity and negative vimentin staining.

scholarly journals Well-Differentiated Liposarcoma of The Larynx: A Case Report and Review of Literature

2016 ◽  
Vol 9 (1) ◽  
pp. 85-89
Author(s):  
Svetlana A. Mateva ◽  
Margarita R. Nikolova ◽  
Alexandar V. Valkov ◽  
Margarita R. Nikolova
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
World Health ◽  
Low Grade ◽  
Review Of Literature ◽  
Grade Malignancy ◽  
Well Differentiated ◽  
Health Organization ◽  
Histologic Types

Summary Liposarcoma is one of the most common soft tissue sarcomas in adults with a relative incidence amongst other sarcomas ranging from 9.8% to 16%. It usually locates in the limbs and retroperitoneum. Primary liposarcomas of the larynx and hypopharynx are rare, comprising less than 20% of all head and neck liposarcomas. According to World Health Organization, these tumors are divided into four histologic types, and well-differentiated liposarcoma is the most common one. It is a tumor of low-grade malignancy that may recur locally, but does not metastasize. We present a case of laryngopharyngeal well- differentiated liposarcoma in an old patient with two previous removals. We also discuss recently published cases with this unusual location of liposarcoma.

scholarly journals FluorescenceIn SituHybridization forMDM2Amplification as a Routine Ancillary Diagnostic Tool for Suspected Well-Differentiated and Dedifferentiated Liposarcomas: Experience at a Tertiary Center

Sarcoma ◽  
2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Khin Thway ◽  
Jayson Wang ◽  
John Swansbury ◽  
Toon Min ◽  
Cyril Fisher
Keyword(s):  
Gene Amplification ◽  
Diagnostic Tool ◽  
Spindle Cell ◽  
Significant Group ◽  
Lipomatous Tumor ◽  
Tertiary Center ◽  
Benign Histology ◽  
Well Differentiated ◽  
Correlation Rate

Background. The assessment ofMDM2gene amplification by fluorescencein situhybridization (FISH) has become a routine ancillary tool for diagnosing atypical lipomatous tumor (ALT)/well-differentiated liposarcoma and dedifferentiated liposarcoma (WDL/DDL) in specialist sarcoma units. We describe our experience of its utility at our tertiary institute.Methods. All routine histology samples in whichMDM2amplification was assessed with FISH over a 2-year period were included, and FISH results were correlated with clinical and histologic findings.Results. 365 samples from 347 patients had FISH forMDM2gene amplification. 170 were positive (i.e., showedMDM2gene amplification), 192 were negative, and 3 were technically unsatisfactory. There were 122 histologically benign cases showing a histology:FISH concordance rate of 92.6%, 142 WDL/DDL (concordance 96.5%), and 34 cases histologically equivocal for WDL (concordance 50%). Of 64 spindle cell/pleomorphic neoplasms (in which DDL was a differential diagnosis), 21.9% showedMDM2amplification. Of the cases with discrepant histology and FISH, all but 3 had diagnoses amended following FISH results. For discrepancies of benign histology but positive FISH, lesions were on average larger, more frequently in “classical” (intra-abdominal or inguinal) sites for WDL/DDL and more frequently core biopsies. Discrepancies of malignant histology but negative FISH were smaller, less frequently in “classical” sites but again more frequently core biopsies.Conclusions. FISH has a high correlation rate with histology for cases with firm histologic diagnoses of lipoma or WDL/DDL. It is a useful ancillary diagnostic tool in histologically equivocal cases, particularly in WDL lacking significant histologic atypia or DDL without corresponding WDL component, especially in larger tumors, those from intra-abdominal or inguinal sites or core biopsies. There is a significant group of well-differentiated adipocytic neoplasms which are difficult to diagnose on morphology alone, in which FISH forMDM2amplification is diagnostically contributory.

scholarly journals Paravertebral Well-Differentiated Liposarcoma with Low-Grade Osteosarcomatous Component: Case Report with 11-Year Follow-Up, Radiological, Pathological, and Genetic Data, and Literature Review

2017 ◽  
Vol 2017 ◽  
pp. 1-6
Author(s):  
Nicolas Macagno ◽  
Stéphane Fuentes ◽  
Gonzague de Pinieux ◽  
André Maues de Paula ◽  
Sébastien Salas ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Soft Tissue Sarcomas ◽  
Fish Analysis ◽  
Low Grade ◽  
Central Mass ◽  
Divergent Differentiation ◽  
Paravertebral Muscles ◽  
Well Differentiated ◽  
The Right

Despite being one of the most frequent soft-tissue sarcomas, well-differentiated liposarcoma has never been reported near the spine. The authors present the case of a 67-year-old man with progressive history of back pain. Physical examination revealed a mass located within the right paravertebral muscles. MR and CT imaging showed a heavily ossified central mass surrounded by a peripheral fatty component. No connection with the underlying bone was detected on imagery and during surgery. After surgical resection, histopathological examination revealed a tumor harboring combined features of well-differentiated liposarcoma and low-grade osteosarcoma. Tumor cells displayed overexpression of MDM2, CDK4, and P16 by immunohistochemistry and CGH revealed amplification of 12q13-15 as the only genetic imbalance. MDM2 FISH analysis was performed but was inconclusive. The pathological, immunohistochemical, and genetic features, the differential diagnoses, and the therapeutic management of this unusual tumor are discussed. No complementary treatment was performed initially. Following first treatment, two recurrences occurred 6 and 9 years later, both displaying histological features similar to the first occurrence. Radiotherapy was started after the second recurrence. Follow-up shows no evidence of disease 11 years after initial diagnosis. This case was unusual due to the paravertebral location of the tumor and its divergent differentiation.

Leiomyosarcoma of the urinary bladder: a clinicopathological study of 34 cases

2010 ◽  
Vol 63 (8) ◽  
pp. 708-713 ◽  
Author(s):  
Matthew R Lindberg ◽  
Cyril Fisher ◽  
Khin Thway ◽  
Dengfeng Cao ◽  
John C Cheville ◽  
...  
Keyword(s):  
Urinary Bladder ◽  
Adverse Outcome ◽  
Soft Tissue Sarcomas ◽  
Mitotic Rate ◽  
Low Grade ◽  
Grading Systems ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Degree Of Differentiation

BackgroundLeiomyosarcomas of the urinary bladder (LMS-UB) are rare, usually aggressive neoplasms. Owing to their rarity, only a limited number of cases with clinical follow-up information have been published. There is no current consensus on LMS-UB grading, and it is unknown whether the widely accepted Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) and National Cancer Institute (NCI) grading systems of soft-tissue sarcomas are applicable to LMS-UB.MethodsThe authors studied 34 well-characterised LMS-UB and compared the prognostic power of the FNCLCC and NCI systems with that of one published grading scheme for LMS-UB (Mayo). All available slides from 34 LMS-UB were retrieved and evaluated with regards to degree of differentiation, mitotic rate/10 high-powered fields (HPF), and % necrosis. Cases were graded using published criteria for the FNCLCC, NCI and Mayo schemes. Follow-up information was obtained.ResultsThe tumours occurred in 17 females and 17 males, ranging from 31 to 91 years (median 65), and measured 2–12 cm in size. One tumour was well differentiated, 17 tumours were moderately differentiated, and 16 tumours were poorly differentiated. Mitotic rates ranged from 1 to >30/10 HPF (median 12/10 HPF), and tumours showed 0–60% necrosis (median 25%). FNCLCC grades were 1 (3), 2 (12) and 3 (19). NCI grades were 1 (2), 2 (11) and 3 (21). Mayo grades were low (7) and high (27). FNCLCC and NCI grades were identical in 23/34 cases (68%). Four cases were FNCLCC/NCI grade 2 or 3 and Mayo low-grade. Clinical follow-up was available for 25 of 34 patients (74%). Clinical follow-up of ≥12 months was available for 17 of these 25 cases (68%) with a median follow-up duration of 52 months (range 12–120 months). Adverse outcome was seen in nine of these 17 patients (53%). Seven of the eight cases (88%) with a clinical follow-up duration of <12 months died of their disease. Overall, adverse outcome was documented in 16 of 25 (64%) cases. Metastatic disease was seen in 13 of 25 (52%) cases, with the lungs being the most common site of metastasis (62%). Adverse outcome was noted in 15 of 23 (65%) of FNCLCC grade 2 or 3 LMS-UB, as compared with zero of two (0%) FNCLCC grade 1 tumours (p=0.15), in 15 of 23 (65%) NCI grade 2 or 3 LMS-UB, versus zero of two (0%) NCI grade 1 sarcomas (p=0.17) and in 13 of 20 (65%) Mayo high grade LMS-UB, as opposed to two of five (40%) low-grade lesions (all results not statistically significant).ConclusionsThe authors conclude that LMS-UB occurs in older adults of either sex and is characterised by aggressive behaviour, with adverse outcome in >60% of cases. Certain advantages of the FNCLCC system may support its more widespread adoption for future studies.

Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma With Features Mimicking Spindle Cell Lipoma

2019 ◽  
Vol 28 (3) ◽  
pp. 336-340 ◽  
Author(s):  
Judith A. S. Jebastin ◽  
Kyle D. Perry ◽  
Dhananjay A. Chitale ◽  
Michael P. Mott ◽  
Jessica Sanchez ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Spindle Cell Lipoma ◽  
Rb1 Gene ◽  
Prognostic Features ◽  
Atypical Lipomatous Tumor ◽  
Lipomatous Tumor ◽  
Well Differentiated ◽  
Mdm2 Amplification ◽  
Lipomatous Tumors

Atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDL) and spindle cell lipoma are lipomatous tumors with distinct clinical, molecular, and prognostic features. Although histological and immunophenotypic features can overlap between ALT/WDL and spindle cell lipoma, the oncogenesis and clinical behavior are markedly different. In borderline cases, molecular analysis for MDM2 or CDK4 amplification can aid in distinguishing ALT/WDL from spindle cell lipoma. Although dedifferentiated liposarcoma has been reported to harbor both MDM2 amplification and loss of the RB1 region, we are not aware of a reported RB1 loss in well-differentiated ALT/WDL. In this article, we present a 69-year-old woman with a lipomatous tumor in the gluteal region that histologically, immunohistochemically, and molecularly mimicked spindle cell lipoma (with positive immunohistochemical staining for CD34 and loss of the RB1 gene region), yet harbored amplification of MDM2 and CDK4 confirmed by fluorescence in situ hybridization, supporting classification as ALT/WDL. This case strengthens the argument that in atypical clinical contexts, molecular studies for MDM2/CDK4 should be considered in tumors resembling spindle cell lipoma.

Retroperitoneal Well-Differentiated Liposarcoma With Uterine-Type Leiomyomatous Differentiation: A First Case Report With Literature Analysis of Soft Tissue Sarcomas With Dual Lipomatous and Low-Grade Smooth Muscle Differentiation

2019 ◽  
Vol 27 (7) ◽  
pp. 798-803
Author(s):  
Tien Anh N. Tran ◽  
Sebastian de La Fuente
Keyword(s):  
Smooth Muscle ◽  
Mitotic Activity ◽  
Soft Tissue Sarcomas ◽  
Diagnostic Errors ◽  
Muscle Differentiation ◽  
Low Grade ◽  
Supporting Evidence ◽  
Spindle Cell Proliferation ◽  
Smooth Muscle Differentiation ◽  
Well Differentiated

The occurrence of smooth muscle differentiation in a liposarcoma is a very uncommon phenomenon, even in dedifferentiated liposarcomas. In dedifferentiated liposarcomas, the leiomyosarcomatous component frequently displays high-grade cytologic features, increased mitotic activity, and tumor necrosis. Even more unusual are rare reported cases of low-grade smooth muscle differentiation in atypical lipomatous tumors/well-differentiated liposarcomas (WDLS). The current case describes a 39-year-old female with a large retroperitoneal WDLS harboring a well-demarcated mass composed of benign-appearing smooth muscle fascicles completely lacking cytologic atypia and mitotic activity. In conjunction with the immunopositivity for estrogen and progesterone receptors, the morphology of this nodule was highly reminiscent of a uterine-type leiomyoma. Of note, the lipomatous component largely displayed a lipoma-like appearance with only rare foci of mildly atypical spindle cell proliferation among the adipocytes and few fibrous septae harboring atypical stromal cells. Immunohistochemical and fluorescence in situ hybridization studies revealed MDM2 gene amplification in both the lipomatous and leiomyoma-like areas, thus confirming the diagnosis of a WDLS with smooth muscle differentiation. A literature review on the subject of sarcomas with dual adipocytic and low-grade smooth muscle differentiation provided sufficient supporting evidence to categorize the tumor as a WDLS with “leiomyomatous” differentiation. Pathologists should be aware of the occurrence of uterine-type leiomyomatous differentiation in retroperitoneal WDLS to avoid potential diagnostic errors.

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