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Sclerosing epithelioid fibrosarcoma.
James W Patterson1, Georgi Tchernev2, Anastasiya Atanasova Chokoeva3,4
1Department of Pathology, University of Virginia Health System, 1215 Lee Street, 800214, 22908, Charlottesville, VA, USA.
Wiener Medizinische Wochenschrift (1946)
|September 16, 2016
Summary
Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue tumor. This case highlights SEF
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue tumor.
- SEF is part of a spectrum including low-grade fibromyxoid sarcoma (LGFMS) and hyalinizing spindle cell tumor with giant rosettes (HSCTGR).
- Understanding SEF's relationship with LGFMS is crucial for diagnosis and treatment.
Observation:
- A 55-year-old woman presented with a soft tissue mass in her right proximal thigh.
- Biopsy findings were consistent with sclerosing epithelioid fibrosarcoma (SEF).
- The tumor exhibited overlapping histopathologic features and MUC4 immunopositivity with LGFMS.
Findings:
- Genetic analysis suggests EWSR1 and CREB3L1 rearrangements are predominant in pure SEF.
- FUS and CREB3L2 gene rearrangements, typical of LGFMS, are found in hybrid tumors and LGFMS relapses with SEF morphology.
- Distinguishing between SEF and LGFMS based on genetic profiles is important for accurate classification.
Implications:
- This case contributes to the understanding of SEF's distinct genetic profile.
- Differentiating SEF from LGFMS has implications for prognosis and therapeutic strategies.
- Further research into the molecular basis of SEF and related tumors is warranted.
