Keywords
Angiomatoid fibrous histiocytoma, metastasis, morphology, translocation, lung, retroperitoneal, treatment
Abstract
Introduction: Angiomatoid fibrous histiocytoma (AFH) is a rare soft-tissue neoplasm often arising in children and young adults. They commonly occur in somatic soft tissue, mainly the upper and lower extremities. AFH is a translocation associated neoplasm, with the majority of cases positive for EWSR1-CREB1 translocation. The tumor was initially described as “malignant” AFH. However, it has a relatively rare chance of metastasis. AFH shows a broad spectrum of morphological patterns, and metastatic lesions may or may not show malignant features.
Report: We report a case of a 12-year-old female who presented with a left supraclavicular mass that was initially diagnosed as a benign fibrous histiocytoma. The mass was excised with clear surgical margins. Six years later, the patient presented with multiple retroperitoneal masses and later succumbed to metastatic disease. Molecular studies confirmed an EWSR1-CREB1 fusion gene in the primary and metastatic lesions. The culmination of these features led to the diagnosis of a metastatic angiomatoid fibrous histiocytoma.
Conclusion: AFH is a rare neoplasm with a spectrum of morphological characteristics and a vast array of immunophenotypical profiles. This can make the diagnosis of AFH challenging. Few cases of metastatic AFH have been reported in literature. However, when these lesions do metastasize, they are often fatal. It is our hope that this report raises awareness of what remains an unmet need in the diagnosis and management of “angiomatoid fibrous histiocytoma with malignant features.”
Citation
Zvolanek L, Clay K, Tabet-Aoul A, Nguyen L, Andreadis A, et al. (2020) Malignant Angiomatoid Fibrous Histiocytoma: a separate entity or just the visible part of an iceberg? Case Report of Uncommon Tumor and Brief Review of Literature. JSM Clin Cytol Pathol 2: 5.