Cervical Angiomatoid Fibrous Histiocytoma

Background: Angiomatoid fibrous histiocytoma (AFH) is a rare type of sarcoma with low-grade malignancy that usually occurs in young subjects. AFH is uncommon in the head and neck region. Methods: We describe an exceptional case of localization in the neck. This is the first report of a rare variant of AFH presenting in a 42-year-old woman. The tumor was situated posterior to the carotid artery adhering to the prevertebral plane and invading the sympathetic cervical chain. In this setting, we decided to perform a surgical exploration with frozen section biopsy. The frozen section revealed a fusiform tumor proliferation resembling a sarcoma. A FISH study concluded on the presence of a EWSR1 22q (22) gene rearrangement. Furthermore, the immunohistochemical study revealed anti-EMA positive cells. The final pathological description concluded on the presence of an AFH, which was excised by surgery alone. Results: After a 2 year follow-up period, the patient is free of disease. Angiomatoid fibrous histiocytoma (AFH) is a rare sarcoma subtype, and mis diagnosis can lead to its over treatment. A precise description of the pathological response and a multidisciplinary discussion can lead to a correct decision. Conclusion: AFH is a tumor with local evolution, and surgery is the mainstay of its management. Complete local excision is recommended to hamper local recurrences. This record was migrated from the OpenDepot repository service in June, 2017 before shutting down.