Popovic Maja Beck, Diezi Manuel, Kuchler Henri, Abouzeid Hana, Maeder Philippe, Balmer Aubin, Munier Francis L
Pediatric Hematology Oncology Unit and Radiology Department, University Hospital CHUV, 1011 Lausanne, Switzerland.
J Pediatr Hematol Oncol. 2007 Jan;29(1):53-6. doi: 10.1097/MPH.0b013e3180308782.
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.
三边性视网膜母细胞瘤(TRb)是一种著名的综合征,它将遗传性视网膜母细胞瘤(Rb)与通常发生于松果体区域、极少发生于鞍上或鞍旁部位的颅内神经母细胞瘤相关联。大多数情况下,它在Rb诊断后发生。由于继发脊髓播散,其预后通常是致命的。松果体囊肿最近被报道为TRb的一种良性变异型。我们报告了一名12个月大男孩TRb的不寻常表现,其患有广泛双侧Rb、巨大鞍上肿瘤、松果体囊肿和软脑膜疾病。本文讨论了这种“四边性”Rb的特殊特征。