Successful treatment with a low-dose cisplatin-etoposide regimen for patients with Diencephalic syndrome

Iacopo Sardi, Cecilia Bresci, Elisabetta Schiavello, Veronica Biassoni, Valentina Fratoni, Stefania Cardellicchio, Lorenzo Genitori, Maurizio Aricò, Maura Massimino

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Diencephalic syndrome (DS) is a rare but rapidly fatal condition, usually occurring during the first year of life, as a result of a hypothalamic/ chiasmatic tumor. The purpose of this study was to induce an objective tumor response and to achieve rapid weight recovery by using ten three-day courses of reduced-dose cisplatin-etoposide. Between 2004 and 2009, eight pediatric patients with DS as a result of an hypothalamic tumor and with a median age at diagnosis of 6.5 months (range 4-60 months) received 10 monthly courses of cisplatin (25 mg/m2/day on days 1-3) and etoposide (100 mg/m 2/day on days 1-3). Under chemotherapy, rapid weight recovery was observed for all patients; tumor response was observed for six (75 %; partial response in four and minimum response in two). The other two had 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. For DS patients who received nutritional support (enteral or parenteral nutrition) the mean time for weight recovery was 5 months (range 3-7 months) whereas children who were able to orally ingest a high-energy diet had a mean time for weight recovery of 8.66 months (range 3-19 months). After follow-up ranging from 22 to 89 months (median 38 months) all patients are alive. A low-dose cisplatin-etoposide regimen is highly effective regarding tumor response and treatment of DS symptoms/cachexia without causing significant side-effects.

Original languageEnglish
Pages (from-to)375-383
Number of pages9
JournalJournal of Neuro-Oncology
Issue number2
Publication statusPublished - Sep 2012


  • Cachexia
  • Chemotherapy
  • Diencephalic syndrome
  • Hypothalamic tumor
  • Russell syndrome

ASJC Scopus subject areas

  • Clinical Neurology
  • Cancer Research
  • Oncology
  • Neurology


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