Sclerosing Epithelioid Fibrosarcoma
Definition
- A rare malignant neoplasm composed of small round cells in nests and cords set in a hyalinized stroma
Diagnostic Criteria
- Nests and cords of uniform epithelioid cells
- Small to medium size
- Round, oval or angulated nuclei
- Scant clear to pale cytoplasm
- Hyalinized stroma
- May resemble osteoid
- Cartilaginous and osseous metaplasia may be seen
- May show focal myxoid change or calcification
- MUC4 positive (see Supplemental Studies)
- Variable cellularity
- May produce nodular or lobular pattern
- May have hemangiopericytomatous vessels
- May have vascular invasion
- Above pattern may merge with conventional fibrosarcoma pattern or with low grade fibromyxoid sarcoma pattern
- Based on some cases with mixed features and on cases that share the t(7;16) (q32-34;p11) characteristic of low grade fibromyxoid sarcoma, it has been suggested that these two neoplasms may represent ends of a common spectrum
- See separate entities:
Richard L Kempson MD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting/last update: 10/15/07, 1/22/14
