Fibrosarcoma C49.9

Very rare, malignant connective tissue tumour, usually originating from tendons or fasciae (rarely primarily originating from the cutis) and rapidly metastasising with proliferation of spindle-shaped cells.

Remark: The diagnosis "fibrosarcoma" was made more frequently in earlier years, but is now more in the background due to the more differentiated possibilities of subtle immunohistological tissue characterization. Today, <1% of adult fibroblastically differentiated sarcomas are diagnosed with fibrosarcoma. In this respect, the summary presented here refers to older scientific data.

Fast-growing, subcutaneous, usually painless, very coarse, with the underlay baked knots on the extremities; rather plate-like infiltrates on the trunk. Early ulceration on exit from the cutis, bluish-brownish surface. Early haematogenic metastasis mainly in the lungs.

1. Diaz-Cascajo C et al (2003) Fibroblastic/myofibroblastic sarcoma of the skin: a report of five cases. J Cutan Pathol 30: 128-134
2. Hill H (2006) Fibrohistiocytic skin tumors. J Dtsch Dermatol Ges 4: 544-555
3. Jurcic V et al (2002) Myxoinflammatory fibroblastic sarcoma: a tumor not restricted to acral sites. Ann Diagn Pathol 6: 272-280
4. Scott SM et al (1989) Soft Tissue Fibrosarcoma. Cancer 64: 925-931
5. Sheng WQ et al (2001) Congenital-infantile fibrosarcoma. A clinicopathologic study of 10 cases and molecular detection of the ETV6-NTRK3 fusion transcripts using paraffin-embedded tissues. At J Clin Pathol 115: 348-355