Successful treatment with a low-dose cisplatin--etoposide regimen for patients with diencephalic syndrome.
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/m(2)/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.
Department of Pediatric Hematology Oncology, A.O.U. Meyer Children's Hospital, viale G.Pieraccini 24, 50139, Florence, Italy. i.sardi@meyer.it
Iacopo
I
Sardi
Cecilia
C
Bresci
Elisabetta
E
Schiavello
Veronica
V
Biassoni
Valentina
V
Fratoni
Stefania
S
Cardellicchio
Lorenzo
L
Genitori
Maurizio
M
Aricò
Maura
M
Massimino
D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001254:Astrocytoma; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D004334:Drug Administration Schedule; D005047:Etoposide / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D006728:Hormones; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000188:drug therapy*; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D013002:Somatomedins / Q000378:metabolism
D000970:Antineoplastic Agents; D006728:Hormones; D013002:Somatomedins; D005047:Etoposide; D002945:Cisplatin