Trilateral retinoblastoma with suprasellar tumor and associated pineal cyst.

J Pediatr Hematol Oncol

Pediatric Hematology Oncology Unit and Radiology Department, University Hospital CHUV, 1011 Lausanne, Switzerland.

Published: January 2007

Trilateral retinoblastoma (TRb) is a well-known syndrome associating hereditary retinoblastoma (Rb) with an intracranial neuroblastic tumor arising usually in the pineal region, rarely at the suprasellar or parasellar site. It develops in most cases after diagnosis of Rb. The outcome is usually fatal because of secondary spinal dissemination. Pineal cysts have recently been reported as a benign variant of TRb. We report the unusual presentation of a TRb in a 12-month-old boy with extensive bilateral Rb, a voluminous suprasellar tumor, pineal cyst, and leptomeningeal disease. The special features of this "quadrilateral" Rb are discussed.

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http://dx.doi.org/10.1097/MPH.0b013e3180308782DOI Listing

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