Primary Sellar Rhabdomyosarcoma Arising in Association With a Pituitary Adenoma.

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Primary Sellar Rhabdomyosarcoma Arising in Association With a Pituitary Adenoma.

Int J Surg Pathol. 2016 Dec;24(8):753-756

Authors: Duncan VE, Nabors LB, Warren PP, Conry RM, Willey CD, Perry A, Riley KO, Hackney JR

Abstract
Intrasellar rhabdomyosarcoma is rare, and when arising in collusion with an unrelated entity, can present a diagnostic pitfall. We describe a case of a 56-year-old woman who presented with right eye ptosis and a pituitary mass, which was resected and diagnosed as atypical pituitary adenoma. Because of residual disease and atypical pathology, the patient received adjuvant radiation. Tumor recurrence was noted after 5 months, and a second resection was performed. Nests of atypical pituitary adenoma cells within an exuberant spindle cell stroma were noted, with areas of presumed radiation necrosis. A second recurrence prompted a third resection. After expert consultation and additional immunohistochemical stains for muscle markers, a diagnosis was rendered of primary rhabdomyosarcoma arising in association with a pituitary adenoma. Despite its rarity, a high index of suspicion for rhabdomyosarcoma arising within a pituitary adenoma should be maintained in cases of atypical pituitary adenoma with a stromal response.

PMID: 27422470 [PubMed - indexed for MEDLINE]

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