P05.07 * A LOWER-DOSE CISPLATIN-ETOPOSIDE REGIMEN FOR CHILDREN WITH HYPOTHALAMIC/CHIASMATIC TUMOR AND DIENCEPHALIC CACHEXIA

Abstract

Diencephalic Syndrome (DS) is a rare but potentially lethal condition, which may complicate the clinical course of infants with hypothalamic/chiasmatic tumors, usually low-grade gliomas, either pilocytic (WHO-grade I) or pilomyxoid (WHO-grade II) astrocytomas. The mean survival of DS patients who do not receive any treatment after diagnosis, is usually less than 12 months. Yet, effective therapy is hampered by the tumor location preventing its complete resection, and by significant neurocognitive and endocrinological sequelae associated with potentially curative radiotherapy. The aim of our study was to induce an objective tumor response and to obtain a rapid weight recovery, using ten three-day courses of reduced dose cisplatin-etoposide. Between 2004 and 2009, 8 pediatric patients with DS due to a hypothalamic tumor and a median age at diagnosis of 6.5 months (range: 4-60 months) received 10 monthly courses of cisplatin (25 mg/m2/d on days 1 to 3) and etoposide (100 mg/m2/d on days 1 to 3). All patients had a rapid weight recovery and a tumor response in 6 (75%;partial response in four and minimal response in two), while the remaining two had a stable disease at completion of treatment. Mean time to weight recovery was 6 months (range, 5 - 7 months) for pilomyxoid astrocytoma patients, and 3.3 months (range, 3 - 4 months) for those with pilocytic astrocytoma. DS patients who received nutritional support (enteral or parenteral nutrition) had a mean time of weight recovery of 5 months (range: 3 - 7 months), while children who were able to assume high energy diet by oral route had a mean time of weight recovery of 8.66 months (range 3 - 19 months). After a follow-up time ranging from 22 to 89 months (median, 38 months), all patients are alive. In conclusion, a chemotherapy program with ten three-day courses of reduced dose cisplatin-etoposide, according to a LGG type schedule, seems appropriate and effective in far most cases of DS, with lower ototoxicity and myelosuppression compared with other LGG regimens.