Fibrosarcoma C49.9

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 29.10.2020

Synonym(s)

fibroblastic sarcoma; fibroma sarcomatodes; Fibromyxosarcoma; fibrosarcoma; Myxosarcoma; Sarcoma anaplastic; Sarcoma fibroblastic; Sarcoma Fibrosarcoma; Spindle cell sarcoma

Definition
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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.

Etiopathogenesis
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Primary cutaneous fibrosarcomas develop preferentially in long existing atrophic scars, e.g. in lupus erythematosus, burns, radiodermatitis chronica.

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Internal medicine

Cyclophosphamide

Cytostatic, immunosuppressive.

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Manifestation
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Possible at any age. Age peak around the age of 40.

Localization
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Especially extremities (thighs); possible everywhere.

Clinical features
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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.

Histology
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Densely packed, atypical, fish-like, spindle-shaped cells arranged in fascicular patterns, numerous mitoses. Negative for CD34. Highly differentiated tumors are less cell-deficient and richer in collagen fibers than low differentiated ones. The more pronounced the anaplasia of the cells, the more they lose their spindle shape. Occasionally secretion of a myxoid substance (fibromyxosarcoma).

Differential diagnosis
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Dermatofibrosarcoma protuberans; malignant fibrous histiocytoma; fasciitis nodularis pseudosarcomatosa; desmoid tumor; leiomyosarcoma; undifferentiated spinocellular carcinoma.

Radiation therapy
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The fibrosarcoma responds little to radiotherapy.

Internal therapy
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Chemotherapy: Effectiveness of adjuvant cytostatic therapy ( vincristine, doxorubicin, cyclophosphamide) has not been proven to date. Treatment in collaboration with oncologists.

Operative therapie
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Early radical excision with a safety margin of at least 2 cm leads to fewer local recurrences. However, the metastasis potential and thus survival are determined by the degree of differentiation of the tumor cells.

Progression/forecast
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5-year survival rate 50%, prognosis of childhood fibrosarcoma is significantly better than that of adults.

Literature
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Diaz-Cascajo C et al (2003) Fibroblastic/myofibroblastic sarcoma of the skin: a report of five cases. J Cutan Pathol 30: 128-134
Hill H (2006) Fibrohistiocytic skin tumors. J Dtsch Dermatol Ges 4: 544-555
Jurcic V et al (2002) Myxoinflammatory fibroblastic sarcoma: a tumor not restricted to acral sites. Ann Diagn Pathol 6: 272-280
Scott SM et al (1989) Soft Tissue Fibrosarcoma. Cancer 64: 925-931
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