We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
Rapidly fatal SMARCA4-deficient undifferentiated sarcoma originating from hybrid hemosiderotic fibrolipomatous tumor/pleomorphic hyalinizing angiectatic tumor of the foot.
- Authors
Agaimy, Abbas; Meidenbauer, Norbert; Sukov, William R.; Stoehr, Robert; Vieth, Michael; Roemer, Frank; Grützmann, Robert; Folpe, Andrew L.
- Abstract
Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft parts and hemosiderotic fibrolipomatous tumor (HFLT) are two rare low-grade locally recurring neoplasms with predilection for the foot/ankle. Recent studies support a close link between the two entities, and origin of PHAT from HFLT and occurrence of hybrid HFLT/PHAT have been documented. Both lesions often harbor TGFBR3 or MGEA5 rearrangements. Rare sarcomas originating from HFLT/PHAT have been reported, typically resembling myxofibrosarcoma or myxoinflammatory fibroblastic sarcoma. We describe a novel SMARCA4-deficient undifferentiated sarcoma with rhabdoid features originating from hybrid HFLT/PHAT in the foot of a 54-year-old male. The tumor pursued a highly aggressive course with rapid regrowth after resection and multiple metastases resulting in patient's death within 5 months, despite systemic chemotherapy. Immunohistochemistry revealed SMARCA4 loss in the undifferentiated sarcoma, but not in the HFLT/PHAT. Molecular testing confirmed TGFBR3/MGEA5 rearrangements. This report expands the phenotypes of sarcomas developing from pre-existing PHAT/HFLT.
- Subjects
POLYMORPHISM (Crystallography); SARCOMA; FIBROSARCOMA; PHENOTYPES; CANCER chemotherapy
- Publication
Virchows Archiv: European Journal of Pathology, 2022, Vol 480, Issue 5, p1115
- ISSN
0945-6317
- Publication type
Article
- DOI
10.1007/s00428-021-03167-6