Angiomatoid fibrous histiocytoma: A review with recent genetic findings
ABSTRACT Angiomatoid fibrous histiocytoma is a neoplasm of intermediate biologic potential most often arising in the extremities of children and young adults. Its rarity may lead to misdiagnosis as either a reactive lesion or a benign or higher-grade tumor. Originally described as a type of malignant fibrous histiocytoma, its differentiation remains enigmatic, with precise histogenesis still only hypothesized. Its morphology is distinct, as a circumscribed lesion with sheets of bland spindle to ovoid cells, peripheral lymphoplasmacytic infiltrate, and blood-filled cystic cavities, and half of the cases show strong desmin expression. Cytogenetically, 2 specific translocations, t(12:16)(q13:p11) and more recently t(12:22)(q13:q12), have been characterized. The literature on angiomatoid fibrous histiocytoma is reviewed, particularly with regard to recent molecular genetic developments and differentiation, and its morphology, immunohistochemistry, and differential diagnosis are summarized.
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ABSTRACT: Angiomatoid fibrous histiocytoma is an uncommon soft-tissue tumor of intermediate malignancy that is often misdiagnosed initially. As there is not one immunohistochemical marker that consequently stains positive or negative for angiomatoid fibrous histiocytoma, molecular diagnostics are becoming more widely used. So far three translocations have been reported to arise in angiomatoid fibrous histiocytoma: FUS-ATF1, EWSR1-CREB1, or EWSR1-ATF1. We present a case of angiomatoid fibrous histiocytoma on the upper arm of a 40-year-old female, which was initially misdiagnosed as metastatic melanoma in a lymph node. Revision of the pathology revealed an angiomatoid fibrous histiocytoma, which was later confirmed by a EWSR1-CREB1 translocation with molecular diagnostics. Furthermore, we review the relevant literature and provide an overview of all available case reports in the past ten years. This case report illustrates the importance for pathologists of knowing the typical pathology features of AFH and integrating immunohistochemical and molecular findings in order to prevent overdiagnosis of lymph node metastasis of a malignancy.12/2012; 2012:291623. DOI:10.1155/2012/291623
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ABSTRACT: Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of low malignant potential. The tumor is mostly seen in the deep dermis and subcutis of the lower extremities in children and young adults. Histologically, the tumor forms lobulated sheets of plump round to spindle cells surrounded by a fibrous pseudocapsule and lymphoid cuff. The cytogenetic and molecular hallmarks of AFH are not well defined. Only 4 of 30 reported cases of AFH have had karyotypic information. We present a case of AFH in the inguinal region of a 12-year-old girl. The tumor showed characteristic histological features, t(2;22)(q33;q12.2), and EWSR gene rearrangement by fluorescence in situ hybridization.Pediatric and Developmental Pathology 08/2008; 12(2):143-6. DOI:10.2350/08-04-0460.1 · 0.86 Impact Factor
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ABSTRACT: We report a case of AFH presenting as an asymptomatic subcutaneous nodule on the arm of a 6-year-old boy. AFH is a fibrohistiocytic tumor of intermediate malignancy. Predominantly seen in children and young adults, AFH presents as a deep dermal or subcutaneous nodule usually on the extremities. The histology is characterized by a fibrous capsule, surrounding lymphocytic infiltrate and blood-filled cystic spaces lined by flattened tumor cells. AFH cells express desmin, epithelial membrane antigen, and CD 68 in over 60% of cases; they are negative for myogenin, MYOD1, and endothelial markers. Rate of local recurrence is 2% to 20%. The metastatic rate is 1%. Management is with wide surgical excision and careful follow-up.Pediatric Dermatology 09/2009; 26(5):636-8. DOI:10.1111/j.1525-1470.2009.01007.x · 1.52 Impact Factor