Abstract

Castleman's disease, a benign lymphoproliferative disorder, may be seen as a self-limited, curable unifocal process, or highly aggressive multicentric disease frequently resulting in death despite aggressive management. Non-Hodgkin's lymphoma has been known to arise within the context of Castleman's disease, usually when multicentric. Hodgkin's lymphoma, however, can also arise within the context of Castleman's disease, but this is a rare process. We report a case of unifocal Castleman's disease (plasma cell variant) occurring concurrently with Hodgkin's disease in the neck of a young woman. The presentation, workup, pathologic evaluation, and management of a young woman diagnosed with Castleman's disease occurring concurrently with Hodgkin's disease in the neck is presented and discussed. A 32-year-old woman with a 5-year history of unifocal right cervicoparotid Castleman's disease (plasma cell variant) underwent right functional neck dissection and superficial parotidectomy for cosmetic and functional purposes. Pathologic and immunohistochemical analysis confirmed Hodgkin's lymphoma occurring in a background of the plasma cell variant of Castleman's disease. The patient subsequently underwent external beam radiation therapy as definitive management for her early-stage Hodgkin's lymphoma. Castleman's disease can occur as an isolated regional process in the head and neck. Furthermore, lymphoma (and specifically Hodgkin's lymphoma) can develop within regionally isolated cervical Castleman's disease. Although complete surgical excision of unifocal Castleman's disease is curative, the management of lymphoma occurring within the context of the Castleman's disease warrants a standard lymphoma workup and management strategy.

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