Histiocytoma malignant fibrous C49.-

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Fibroxanthosarcoma; malignant fibrous histiocytoma

History
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O'Brien and Stout, 1964

Definition
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Malignant soft tissue sarcoma with cells that combine characteristics of histiocytes and fibroblasts Occurrence in the skin is rather rare; often originates from the fibrous connective tissue of the fascia and muscles and from bone tissue.

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Classification
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Histologically, the following forms are distinguished between Enzinger and Weiss:

Pleomorphic type (73% of cases)
Myxoid type (19.5%)
Giant cell type (3%)
Xanthogranulomatous-angiomatoid type (2.5%)
Inflammatory type (2%).

Manifestation
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Occurrence mainly 50th-70th LJ. Men are affected twice as often as women.

Localization
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Mainly gluteal region, lower extremity, more rarely upper extremity or also peritoneal cavity.

Clinical features
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Little characteristic clinical picture. Formation of a greyish-white or yellow to reddish-brown, coarse, usually broadly ulcerated nodule in the cutis and subcutis. All tumour types can infiltrate not only the subcutis but also the musculature and periosteum.

Histology
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There are storiform pleomorphic, myxoid, large cell and inflammatory types. Depending on the type, there are differently configured cells with vesicular, hyperchromatic nuclei and pleomorphic histiocytes with prominent nucleoli and vacuolated cytoplasm. Numerous atypical mitoses. In addition, bizarre mono- or multinuclear giant cells are often detectable.
Immunohistologically, the diversity of the cell types involved is shown. Vimentin is positive. Visualization of histiocytes (MAC387 neg., CD68 neg., alpha-antichymotrypsin neg/pos.) as well as lysosomal histiocytes, myofibroblasts (alpha-SAM pos., muscle actin (HHF35) pos.) and dendritic cells (FXIII positive).

Differential diagnosis
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Clinical and histological: S.u. fibroxanthoma, atypical

Radiation therapy
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Postoperative radiotherapy with electron accelerators improves the prognosis.

Internal therapy
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High-dose chemotherapy ( doxorubicin, dactinomycin; possibly also cyclophosphamide and vincristine). S.a.u. cytostatics. Relatively good results have recently also been described with preoperative chemotherapy with doxorubicin and radiation for malignant histiocytomas of the extremities.

Operative therapie
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Radical excision. After histological confirmation, if necessary, resection with a safety margin of 2 cm and removal of the entire subcutis. Lymph node removal. High local recurrence rate despite adequate measures (up to 70%; especially in myxoid forms).

Progression/forecast
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It's not a good time. The local recurrence rate is 19-31%. The metastasis is both lymphogenic and hematogenic. Metastasis rate: up to 30%. Remote metastasis varies according to the form. Average 5-year survival rate is 75% for the storiform-pleomorphic type. It is significantly worse in the inflammatory type.

Note(s)
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Many authors postulate an identity of malignant fibrous histiocytoma (MFH) and undifferentiated pleomorphic sarcoma (UPS). Histologically it is not possible to distinguish between MFH and UPS. The atypical fibroxanthoma is again considered to be a (superficial) dermal variant of this tumor species.

Literature
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Aoe K et al (2003) Malignant fibrous histiocytoma of the lung. Anticancer Res 23: 3469-3474
Berth-Jones J et al (1990) Cutaneous malignant fibrous histicytoma. Ann Derm Venerol 70: 254-256
Chang P et al (1994) Malignant fibrous histiocytoma of the skin. Int J Dermatol 33: 50-51
Mentzel T (2002) Cutaneous mesenchymal neoplasms versus mesenchymal neoplasms of subcutaneous and deep soft tissue. Similarities and differences. Pathologist 23: 97-106
O'Brien JE, Stout AP (1964) Malignant fibrous xanthoma. Cancer 17: 1445-1458
Wanebo HJ et al (1995) Preoperative regional therapy for extremity sarcoma - a tricenter update. Cancer 75: 2299-2306
Wiriosuparto S et al (2003) Malignant fibrous histiocytoma, giant cell type, of the breast mimicking metaplastic carcinoma. A case report. Acta Cytol 47: 673-678