Original Article Submitted: 1.11.2014 Accepted: 9.12.2014 Conflict of interest None.
DOI: 10.1111/ddg.12600
Angiomatoid fibrous histiocytoma – case series with emphasis on a late fibrotic variant
Michael Wilk1, Bettina G. Zelger2, Maria Debiec-Rychter 3, Raf Sciot 4, Bernhard Zelger5 (1) Dermatohistological Laboratory, P.O. Box 4145, 90021 Nuernberg, Germany (2) Institute of Pathology, Medical University Innsbruck, Innsbruck, Austria (3) Department of Human Genetics, Catholic University Leuven, University Hospital Gasthuisberg, Leuven, Belgium (4) Department of Pathology, Catholic University Leuven, University Hospital Gasthuisberg, Leuven, Belgium (5) Clinical Department of Dermatology & Venereology, Medical University Innsbruck, Innsbruck, Austria This study was in part presented at the 2011 annual meeting of the Arbeitsgemeinschaft für Dermatologische Histologie (ADH) of the Deutsche Dermatologische Gesellschaft (DDG) in Munich, Germany.
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.
Introduction Angiomatoid fibrous histiocytoma (AFH) is a low-grade malignant subcutaneous neoplasm frequently arising on the extremities of children or young adults [1–4]. Rarely, however, other sites may also be affected [5–7]. In a number of cases, systemic symptoms may be present, such as elevated erythrocyte sedimentation rate and C-reactive protein, anemia (14–24 %), weight loss (4–12 %), fever (2–15 %), and rarely increased gamma globulins [1–3, 6, 8–10]. Local recurrence
may occur in 2–12 % and metastases in
DOI: 10.1111/ddg.12600
Angiomatoid fibrous histiocytoma – case series with emphasis on a late fibrotic variant
Michael Wilk1, Bettina G. Zelger2, Maria Debiec-Rychter 3, Raf Sciot 4, Bernhard Zelger5 (1) Dermatohistological Laboratory, P.O. Box 4145, 90021 Nuernberg, Germany (2) Institute of Pathology, Medical University Innsbruck, Innsbruck, Austria (3) Department of Human Genetics, Catholic University Leuven, University Hospital Gasthuisberg, Leuven, Belgium (4) Department of Pathology, Catholic University Leuven, University Hospital Gasthuisberg, Leuven, Belgium (5) Clinical Department of Dermatology & Venereology, Medical University Innsbruck, Innsbruck, Austria This study was in part presented at the 2011 annual meeting of the Arbeitsgemeinschaft für Dermatologische Histologie (ADH) of the Deutsche Dermatologische Gesellschaft (DDG) in Munich, Germany.
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.
Introduction Angiomatoid fibrous histiocytoma (AFH) is a low-grade malignant subcutaneous neoplasm frequently arising on the extremities of children or young adults [1–4]. Rarely, however, other sites may also be affected [5–7]. In a number of cases, systemic symptoms may be present, such as elevated erythrocyte sedimentation rate and C-reactive protein, anemia (14–24 %), weight loss (4–12 %), fever (2–15 %), and rarely increased gamma globulins [1–3, 6, 8–10]. Local recurrence
may occur in 2–12 % and metastases in
