PMID- 16003734 OWN - NLM STAT- MEDLINE DCOM- 20060612 LR - 20090112 IS - 1545-5009 (Print) IS - 1545-5009 (Linking) VI - 46 IP - 7 DP - 2006 Jun TI - Benign pineal cysts in children with bilateral retinoblastoma: a new variant of trilateral retinoblastoma? PG - 755-61 AB - PURPOSE: Patients with hereditary retinoblastoma (Rb) develop in 4%-8% a malignant midline tumor called trilateral Rb (TRb). We report in this study on benign pineal cysts observed in patients investigated for TRb. PATIENTS AND METHODS: Between September 1990 and December 2001, 172 patients were screened for TRb. Ninty-five had bilateral, 77 unilateral disease. The median age at diagnosis of Rb was 7 months (range 1-26). Treatment included enucleation, local treatment with cryotherapy or photocoagulation, first-line chemotherapy (CT), thermo-chemotherapy (TCT), Ruthenium plaque, and, rarely, external beam radiation (EBR). RESULTS: TRb was found in 5/95 patients (5.3%) with bilateral disease. Interestingly, five other patients (5.3%) presented a pineal cyst on magnetic resonance imaging (MRI). No cysts were recorded in the 77 patients with unilateral disease. This difference was statistically significant (P < 0.05). The median age at diagnosis of the pineal cyst was 26 months (range 16-80), much younger than reported in literature for healthy children. Four of five patients with TRb died of the disease, while all the patients with pineal cysts remained stable and asymptomatic during a median follow-up of 41 months (range 37-54). CONCLUSIONS: This report describes benign cystic lesions of the pineal gland in patients with hereditary Rb, suggesting a benign variant of TRb. Underlying possible pathogenetic mechanisms are discussed. CI - (c) 2005 Wiley-Liss, Inc. FAU - Beck Popovic, Maja AU - Beck Popovic M AD - Pediatric Hematology-Oncology Unit, University Hospital, Lausanne, Switzerland. Maja.Beck-Popovic@hospvd.ch FAU - Balmer, Aubin AU - Balmer A FAU - Maeder, Philippe AU - Maeder P FAU - Braganca, Teresa AU - Braganca T FAU - Munier, Francis L AU - Munier FL LA - eng PT - Journal Article PL - United States TA - Pediatr Blood Cancer JT - Pediatric blood & cancer JID - 101186624 SB - IM MH - Brain Neoplasms/etiology/pathology MH - Central Nervous System Cysts/epidemiology/*etiology/*pathology MH - Child, Preschool MH - Humans MH - Infant MH - Magnetic Resonance Imaging MH - *Pineal Gland MH - Pinealoma/etiology/pathology MH - Prognosis MH - Retinal Neoplasms/*complications MH - Retinoblastoma/*complications MH - Switzerland/epidemiology MH - Tomography, X-Ray Computed EDAT- 2005/07/09 09:00 MHDA- 2006/06/13 09:00 CRDT- 2005/07/09 09:00 PHST- 2005/07/09 09:00 [pubmed] PHST- 2006/06/13 09:00 [medline] PHST- 2005/07/09 09:00 [entrez] AID - 10.1002/pbc.20464 [doi] PST - ppublish SO - Pediatr Blood Cancer. 2006 Jun;46(7):755-61. doi: 10.1002/pbc.20464.