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Chap 13 - FIBROBLASTIC AND MYOFIBROBLASTIC NEOPLASMS WITH MALIGNANT POTENTIAL

Published online by Cambridge University Press:  01 March 2011

By
Markku Miettinen
Edited by
Markku Miettinen
Markku Miettinen
Affiliation:
Armed Forces Institute of Pathology, Washington DC
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Summary

Fibroblastic and myofibroblastic neoplasms with malignant potential span a wide clinicopathological spectrum. Some of the entities listed here are nonmetastasizing and have recurrence potential only, whereas others have full metastatic potential. Solitary fibrous tumor is discussed in Chapter 12.

Dermatofibrosarcoma protuberans (DFSP) and its pigmented variant (Bednar tumor) are clinicopathologically distinctive CD34-positive fibroblastic tumors. Although typically they behave indolently if completely excised, they are capable of transformation to a more aggressive form that can metastasize. Giant cell fibroblastoma is the juvenile variant of DFSP, with a lower biologic potential.

Low-grade fibromyxoid sarcoma is a histologically distinctive tumor that can metastasize despite its bland appearance. This tumor also is capable of progressing to a more aggressive form.

Sclerosing epithelioid fibrosarcoma is the designation for a clinically heterogeneous group of tumors with histologically distinctive features, and some tumors in this category represent progressive forms of low-grade fibromyxoid sarcoma. Inflammatory fibrosarcoma belongs to the category of inflammatory myofibroblastic tumor and is discussed with fibroblastic tumors of children in Chapter 10.

Adult fibrosarcoma is the designation for a nonpleomorphic fibroblastic malignancy; this diagnosis is one of exclusion. This diagnosis is now rarely made because many tumors historically classified as adult fibrosarcomas are now diagnosed as monophasic synovial sarcoma, low-grade fibromyxoid sarcoma, desmoid fibromatosis, solitary fibrous tumor, and even benign conditions such as florid nodular fasciitis.

Acral myxoinflammatory fibroblastic sarcoma (inflammatory myxohyaline tumor of distal extremities) is a newly described fibroblastic lesion of low malignant potential.

Type
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Information
Modern Soft Tissue Pathology
Tumors and Non-Neoplastic Conditions
 , pp. 348 - 392
Publisher: Cambridge University Press
Print publication year: 2010

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