Trilateral retinoblastoma with suprasellar tumor and associated pineal cyst.
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.
Popovic
Maja Beck
MB
Pediatric Hematology Oncology Unit and Radiology Department, University Hospital CHUV, 1011 Lausanne, Switzerland. Maja.Beck-Popovic@chuv.ch
Diezi
Manuel
M
Kuchler
Henri
H
Abouzeid
Hana
H
Maeder
Philippe
P
Balmer
Aubin
A
Munier
Francis L
FL
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D016190:Carboplatin / Q000008:administration & dosage; D020863:Central Nervous System Cysts / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000556:secondary; D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage; D005134:Eye Neoplasms / Q000000981:diagnostic imaging* / Q000188:drug therapy; D006801:Humans; D008297:Male; D008577:Meningeal Neoplasms / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000556:secondary; D010870:Pineal Gland / Q000000981:diagnostic imaging*; D011859:Radiography; D012175:Retinoblastoma / Q000000981:diagnostic imaging* / Q000188:drug therapy; D013852:Thiotepa / Q000008:administration & dosage
D003520:Cyclophosphamide; D013852:Thiotepa; D016190:Carboplatin