Abstract
We report a 13-month-old boy with diencephalic syndrome (DS) due to an optico-hypothalamic juvenile pilocytic astrocytoma (JPA). Massive neuroaxis dissemination was identified at diagnosis. He received 6 courses of combined conventional-dose chemotherapy consisting of carboplatin (CBDCA), etoposide (VP-16), and cyclophosphamide (CPA) followed by high-dose chemotherapy with CBDCA, CPA, and ranimustine (MCNU) and peripheral blood stem cell transplantation (PBSCT). This treatment produced tumor regression in both intracranial and spinal lesions and remarkable improvement of DS. The rare combination of DS and symptomatic neuroaxis dissemination of JPA at diagnosis suggests that the behavior of some of these tumors is more aggressive and resistant to conventional-dose chemotherapy than is that of JPA without DS manifestation and dissemination.
Similar content being viewed by others
References
Packer RJ, Ater J, Allen J, Phillips P, Geyer R, Nicholsson S, Jakacki R, Kurczynski E, Needle M, Finlay J, Reaman G, Boyett J: Carboplatin and vincristine chemotherapy for children with newly diagnosed progression of low-grade gliomas. J Neurosurg 86: 747–754, 1997
Sutton LN, Molloy PT, Sernyak H, Goldwein J, Phillips P, Borke L, Moshang T, Lange B, Packer R: Long-term outcome of hypothalamic/chiasmatic astrocytomas in children treated with conservative surgery. J Neurosurg 83: 583–589, 1995
Cohen ME, Mahoney DH Jr, Duffner PK, Gener L, Jamea HE, Langston JW, Fontanesi J, Friedman HS: Evaluation of optic pathway tumors: natural history and treatment. Pediatric Oncology Group (POG). Neurology 36: 505, 1994
Russel A:Adiencephalic syndrome of emaciation in infancy and childhood. Arch Dis Child 26: 274–279, 1951
Burr IM, Slonim AE, Danish RK, Gadoth N, Butler IJ: Diencephalic syndrome revisited. J Pediatr 88: 439–444, 1976
Packer RJ, Vezina G: Pediatric glial neoplasms including brain-stem gliomas. Semin Oncol 21: 260–272, 1994
Greens D, Woods M: A 4-month-old boy with severe emaciation, normal linear growth, and a happy affect. Curr Opin Pediatr 8: 50–57, 1996
Perilongo G, Carollo C, Salviati L, Murgia A, Pillon M, Basso G, Gardiman M, Laverda A: Diencephalic syndrome and disseminated juvenile pilocytic astrocytomas of the hypothalamic-optic chiasm region. Cancer 80: 142–146, 1997
Pollack IF, Hurtt M, Pang D, Albright L: Dissemination of low grade intracranial astrocytomas in children. Cancer 73: 2869–2878, 1994
Rodriguez LA, Edwards MSB, Levin VA: Management of hypothalamic gliomas in children: an analysis of 33 cases. Neurosurg 26: 242–247, 1990
Gropman AL, Packer RJ, Nicholson HS, Vezina LG, Jakacki R, Geyer R, Olson JM, Phillips P, Needle M, Broxson EH, Reaman G, Finlay J:Treatment of diencephalic syndrome with chemotherapy. Growth, tumor response, and long term control. Cancer 83: 166–172, 1998
Gajjar A, Bhargava R, Jenkins J, Heideman R, Sanford R, Langston J, Walter A, Kuttesch J, Muhlbauer M, Kun L: Low-grade astrocytomas with neuroaxis dissemination at diagnosis. J Neurosurg 83: 67–71, 1995
Civitello LA, Packer RJ, Rorke LB, Siegel K, Sutton LN, Schun L: Leptomeningeal dissemination of low-grade gliomas in childhood. Neurology 38: 562–566, 1988
Mamelak A, Prados MD, Obana MG, Cogen PH, Edwards MS:Treatment options and prognosis for multicentric juvenile pilocytic astrocytoma. J Neurosurg 81: 24–30, 1994
Wisoff JH, Abbott JR, Epstein F: Surgical management of exophytic chiasmatic hypothalamic tumors of childhood. J Neurosurg 73: 661–667, 1990
Kato T, Sawamura Y, Tada M, Ikeda J, Ishii N, Abe H: Cisplatin/vincristine chemotherapy for hypothalamic/visual pathway astrocytomas in young children. J Neuro-Oncol 37: 263–270, 1998
Moghrabi A, Feidmen HS, Burger PC, Tien R, Oakes J: Carboplatin treatment of progressive optic pathway gliomas to delay radiotherapy. J Neurosurg 79: 223–227, 1993
Nishio S, Morioka T, Takeshita I, Shono T, Inamura T, Fujiwara S, Fukui M: Chemotherapy for progressive pilocytic astrocytomas in the chiasmo-hypothamalic regions. Clin Neurol Neurosurg 97: 300–306, 1995
McCowage G, Tien R, Mclendon R, Felsberg G, Fuchs H, Graham M, Kurtzberg J, Moghrabi A, Ferrel L, Kerby T, Duncan-Brown M, Stewart E, Robertson P, Colvin M, Golembe B, Binger D, Friedman H: Successful treatment of childhood pilocytic astrocytomas metastatic to the leptomeninges with high-dose cyclophosphamide. Med Pediatr Oncol 27: 32–39, 1996
Tamura M, Zama A, Kurihara H, Fujimaki H, Imai H, Kano T, Saitoh F: Management of recurrent pilocytic astrocytoma with leptomeningeal dissemination in children. Child's Nerv Syst 14: 617–622 1998
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kageji, T., Nagahiro, S., Horiguchi, H. et al. Successful high-dose chemotherapy for widespread neuroaxis dissemination of an optico-hypothalamic juvenile pilocytic astrocytoma in an infant: a case report. J Neurooncol 62, 281–287 (2003). https://doi.org/10.1023/A:1023363122666
Issue Date:
DOI: https://doi.org/10.1023/A:1023363122666