Angiomatoid fibrous histiocytoma - case series with emphasis on a late fibrotic variant

Summary Background Angiomatoid fibrous histiocytoma (AFH) is a rare, low‐grade malignant, subcutaneous neoplasm in children or young adults. Methods AFHs in different disease stages were studied histologically, in part, also immunohistologically, and by fluorescence in situ hybridization. Results De...

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Bibliographic Details
Published in:Journal der Deutschen Dermatologischen Gesellschaft 2015-05, Vol.13 (5), p.441-448
Main Authors: Wilk, Michael, Zelger, Bettina G., Debiec-Rychter, Maria, Sciot, Raf, Zelger, Bernhard
Format: Article
Language:English
Subjects:
Adult
Aged
Biomarkers, Tumor - metabolism
Child
Child, Preschool
Diagnosis, Differential
Female
Fibrosis - classification
Fibrosis - metabolism
Fibrosis - pathology
Histiocytoma, Malignant Fibrous - metabolism
Histiocytoma, Malignant Fibrous - pathology
Humans
Male
Skin Neoplasms - classification
Skin Neoplasms - metabolism
Skin Neoplasms - pathology
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Summary:Summary Background Angiomatoid fibrous histiocytoma (AFH) is a rare, low‐grade malignant, subcutaneous neoplasm in children or young adults. Methods AFHs in different disease stages were studied histologically, in part, also immunohistologically, and by fluorescence in situ hybridization. Results Depending on the degree of fibrosclerosis, nine AFH were divided into the following categories: classic type (n = 3): well‐defined subcutaneous lesions composed of multinodular spindle to epithelioid (histiocytoid) cells surrounding a pseudoangiomatous space filled with blood. Peripherally, there is a fibrous pseudocapsule and an inflammatory infiltrate. Early sclerotic type (n = 4): the fibrous capsule extends more to the inner circle of the lesion, focally replacing the cellular neoplastic component and pseudoangiomatous spaces. Late sclerotic type (n = 2): the architecture of AFH with its zonal arrangement of an outer fibrous and inner cellular component is largely replaced by fibrosis occluding the pseudovascular space in the center of the lesion. Immunohistochemistry was available in 5/9 cases with positivity for EMA (5/5), desmin (3/5), caldesmon (1/2), and CD99 (2/5). One of two cases tested displayed EWSR1 rearrangement. Conclusion Late‐stage AFH may present with marked fibrosis obscuring the real nature of the lesion and may easily be misinterpreted by the unwary as a harmless fibrotic condition.
ISSN:1610-0379
1610-0387
DOI:10.1111/ddg.12600