RESUMO
To evaluate the efficacy of a 2-drug chemotherapy regimen without external-beam radiotherapy [EBRT] and/or without enucleation in bilateral retinoblastoma. From 1996 to 2010, 79 patients were diagnosed with bilateral RB and were eligible for chemotherapy. Chemotherapy was administered prior to and/or following local therapy to the eye. All patients received 3 cycles of chemo-reduction with carboplatin and vincristine, additional cycles of the same or other chemotherapy, local therapy, EBRT and enucleation were determined according to re-evaluation by the ophthalmologist. Advanced disease was seen in 115 [79%] eyes [group IV and V: 96, Group D and E: 19] out of 146 affected eyes. Tumor response after chemotherapy was observed in 78 patients [98.7%]; complete response in 25 [32.1%], partial response in 49 [62.8%] Four [5.1%] had progressive disease. A total of 50 [63.3%] patients required EBRT; 38 for persistent disease, 4 for progressive disease, 2 for new lesions, 2 for re-activation and 4 for disease control. Enucleation was required in 15 [19%]. Secondary malignancies occurred in two patients who underwent EBRT; one osteogenic sarcoma and one rhabdomyosarcoma then later osteogenic sarcoma. The 10 year overall survival was 96.3% with a median follow-up time of 3.124 +/- 0.536 years [95%CI: 2.074-4.174]. The 2-drug chemotherapy regimen combined with local therapy appears to be adequate therapy for low stage disease but not in patients with advanced disease. The occurrence of secondary cancers in this group of patients is worrisome further highlighting the deleterious effects of EBRT
RESUMO
Intracranial germ cell tumors [GCTs] are not a common disease. We reviewed the experience of a single institution to determine the variables that affect treatment outcome. A retrospective review of patients with the diagnosis of intracranial germ cell tumors treated in a single institution [KFSHRC] during the period from March 1985 to December 2007. Fifty-seven patients with the diagnosis of intracranial GCT were recorded in the KFSHRC Tumor Registry during the period from 1985 to 2007. Seven patients with a pineal region tumor treated as germinomas in the earlier years without a tissue diagnosis were excluded. This retrospective study was restricted to the remaining 50 patients with a tissue or marker diagnosis: 31 germinomas and 19 non-germinomatous germ cell tumors [NGGCTs]. The 10-year overall survival [OS], event-free survival [EFS] and relapse-free survival [RFS] were 87%, 88% and 96% for patients with germinoma, with a median follow-up of 4.5 [range 2-17] years, compared with 26%, 29% and 46% for patients with NGGCT with a median follow-up of 3 [range 1.5-13] years. For NGGCT, variables favorably influencing OS were younger age [< 16 y vs >/= 16 y, P=.01], higher radiation dose [>50 Gy vs 1990 vs <1990 P=.002]. Tissue diagnosis of GCTs is mandatory prior to treatment except for patients with elevated markers. In germinoma, localized radiotherapy [RT] for M0 patients may be adequate. Long-term follow-up is needed to define the benefit of adding chemotherapy. For NGGCT, the use of combined modality treatment and RT dose >50 Gy are important factors that influence the outcome. Second-look surgery and resection of residual/refractory tumors is always recommended