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Fasciitis nodularis pseudosarcomatosaM72.41

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Cutaneous pseudosarcomatous fibromatosis; Dermatofibroma pseudosarcomatous; Fasciitis nodular; Fasciitis nodularis pseudosarcomatosa; nodular fasciitis; Nodular Fasciitis; pseudosarcomatous dermatofibroma; Pseudosarcomatous dermatofibroma; Pseudosarcomatous fibromatosis; Subcutaneous pseudosarcomatous fibromatosis

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HistoryThis section has been translated automatically.

Konwaler, 1955

DefinitionThis section has been translated automatically.

Benign, rapidly growing subcutaneous tumor of unknown genesis originating from the fascia with a tendency to spontaneous regression, histologically reminiscent of a fibrosarcoma.

EtiopathogenesisThis section has been translated automatically.

Unclear; traumas? Molecular biology revealed a MYH9/USP6 fusion gene. This is probably a defect that promotes a reactive inflammatory process (Wil M et al. 2018).

ManifestationThis section has been translated automatically.

Occurs with about the same frequency in both sexes. Predominantly 20 to 50 years of age.

LocalizationThis section has been translated automatically.

Prefers extremities, especially forearms, less frequently on the trunk.

Clinical featuresThis section has been translated automatically.

Solitary, usually not > 2.0 cm, maximum up to 3.0 cm in size, subcutaneous, coarse, usually asymptomatic, often caked to the underlying tissue, easily delimited, rapidly growing, sometimes painful under pressure . Spontaneous regression has been observed in individual cases. S.a.u. Fasciitis ossificans; see also cranial fasciitis of childhood.

HistologyThis section has been translated automatically.

Connective tissue tumor originating in the fascial tissue and growing into the subcutaneous fatty tissue, rarely also into the musculature, which is rich in vessels and consists mainly of large, spindle-shaped fibroblasts and myofibroblasts with numerous mitoses. Few vascular incisions, partly with prominent endothelia. In varying density multinucleated giant cells, partly with bizarre nuclear formations. Underlaid is a loose myxoid stroma, which has a diagnostically significant fibrous structure.

Immunohistology: spindle cells = vimentin pos., muscle actin (HHF35 and alpha-SMA pos.); giant cells = S100 pos., muscle actin pos.

Differential diagnosisThis section has been translated automatically.

In case of subcutaneous localization, differentiation of fibrosarcoma, liposarcoma, atypical fibroxanthoma as well as malignant fibrous histiocytoma (rather in older patients).

TherapyThis section has been translated automatically.

If necessary, excision in healthy patients, waiting is also possible under regular clinical and sonographic examination and size control. No recurrence tendency.

Progression/forecastThis section has been translated automatically.

Spontaneous regression after a few months, no tendency to recur.

LiteratureThis section has been translated automatically.

  1. Fischer C (1992) Fasciitis nodularis pseudosarcomatosa - a contribution to the differential diagnosis of mesenchymal tumors. Act Dermatol 18: 189-190
  2. Handa Y et al (2003) Nodular fasciitis of the forehead in a pediatric patient. Dermatol Surgery 29: 867-868
  3. Kamiya H et al (2003) Nodular fasciitis of the cheek. Eur J Dermatol 13: 189-191
  4. Konwaler BE, Keasbery L, Kaplan L (1955) Subcutaneous pseudosarcomatous fibromatosis (fasciitis). At J Clin Pathol 25: 241-252
  5. Wilk M et al (2018) Mesenchymal and neuronal tumors. In: Braun-Falco`s Dermatology. Plewig G et al (Ed.). Springer publishing house, Heidelberg S 1893-1894

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer