Low-grade fibromyxoid sarcoma of the extremities: A clinicopathologic study of 24cases and review of the literature

Andrea Sambri, Alberto Righi, Gianmarco Tuzzato, Davide Maria Donati, Giuseppe Bianchi

Research output: Contribution to journalArticle

Abstract

Low-grade fibromyxoid sarcoma (LGFMS) and hybrid sclerosing epithelioid fibrosarcoma (SEF)/LGFMS have a low potential for recurrence (10%) and metastasis (5%) but they are notorious for late occurring metastases. The aim of this study was to evaluate the outcome of LGFMS and review similar cases reported in the literature. We retrospectively evaluated 24 LGFMS operated at a single Institution. All cases were histologically revised. Mean age was 34 years (range, 8 to 74). Two cases presented areas of SEF (hybrid tumours). Three patients presented with metastasis at diagnosis. A strong cytoplasmic staining for MUC4 antibody was found in the majority of neoplastic cells. RT-PCR was feasible in 6 cases and it detected the presence of FUS-CREB3L2 fusion gene chimeric transcript. Mean follow-up was 44 months (range, 6 to 217). Two patients developed lung metastasis after 9 and 26 months respectively. Low-grade fibromyxoid sarcoma has a various histopathologic spectrum with few cases of LGFMS that share histopathologic resemblance with SEF, thereby reinforcing a possibility of a link within these two. It is of paramount importance an accurate and extensive sampling and examination of the whole specimen, in order to identify higher risk patients.

Original languageEnglish
Pages (from-to)219-225
Number of pages7
JournalPolish Journal of Pathology
Volume69
Issue number3
DOIs
Publication statusPublished - Nov 20 2018

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Sarcoma
Extremities
Fibrosarcoma
Neoplasm Metastasis
Gene Fusion
Staining and Labeling
Recurrence
Polymerase Chain Reaction
Lung
Antibodies
Neoplasms

Keywords

  • Evans tumour
  • Low-grade fibromyxoid sarcoma
  • Survival

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Low-grade fibromyxoid sarcoma of the extremities: A clinicopathologic study of 24cases and review of the literature. / Sambri, Andrea; Righi, Alberto; Tuzzato, Gianmarco; Donati, Davide Maria; Bianchi, Giuseppe.

In: Polish Journal of Pathology, Vol. 69, No. 3, 20.11.2018, p. 219-225.

Research output: Contribution to journalArticle

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