Faculty Collaboration Database

Sclerosing poorly differentiated liposarcoma: clinicopathological, immunohistochemical and molecular analysis of a distinct morphological subtype of lipomatous tumour of soft tissue. Histopathology 2008 Feb;52(3):283-93

Date

02/14/2008

Pubmed ID

18269578

DOI

10.1111/j.1365-2559.2007.02936.x

Scopus ID

2-s2.0-38849095114 (requires institutional sign-in at Scopus site)   15 Citations

Abstract

AIMS: To present eight cases of a distinctive morphological subtype of lipomatous tumour of soft tissue.

METHODS AND RESULTS: The clinicopathological, immunohistochemical and molecular pathological features of these tumours were analysed. The tumours were characterized by dense stromal sclerosis containing singly scattered pleomorphic atypical cells with lipoblastic features. The tumours occurred in four women and four men, aged 39-90 years (mean = 63). They were located in the retroperitoneum, thigh, retropubic space, arm and spermatic cord. Four cases arose de novo and four cases presented as local recurrences of previously resected liposarcomas. MDM2 amplification and cyclophosphamide doxorubicin hydrochloride (adriamycin), vincristine and prednisolone (CHOP) translocation was studied in seven cases by fluorescence in situ hybridization. High-level amplification of MDM2 at 12q13-15 was observed in 4/7 cases. All cases were negative for the CHOP translocation; in one MDM2+ case, the CHOP gene showed amplification but no translocation. Three patients died from their tumours from 1 to 6 years after their last surgery with lung metastases.

CONCLUSIONS: The tumours described appear to represent an unusual morphological variant of poorly differentiated liposarcoma associated with aggressive behaviour, and may represent a common end-stage pathway for various types of liposarcoma.

Author List

Suster S, Morrison C

MESH terms used to index this publication - Major topics in bold

Adult
Aged
Aged, 80 and over
Fatal Outcome
Female
Humans
Immunoenzyme Techniques
In Situ Hybridization, Fluorescence
Liposarcoma
Male
Middle Aged
Neoplasm Recurrence, Local
Oncogene Proteins, Fusion
Proto-Oncogene Proteins c-mdm2
RNA-Binding Protein FUS
Sclerosis
Soft Tissue Neoplasms
Transcription Factor CHOP
Translocation, Genetic