Abstract
Background
To develop an effective therapeutic strategy for patients with diencephalic pilocytic astrocytomas (PA), we must understand the long-term effects and problems of conventional treatments.
Methods
We performed a retrospective follow-up of four infants with PA in the diencephalon.
Conclusion
The initial treatment consisted of partial tumor removal and/or external radiation. The mean progression-free duration after the initial treatment was 108.5 months. All patients received a second course of radiation to treat recurrent tumors. These conventional treatments produced relatively long survival. Three patients died 105, 202, and 379 months after the initial treatment. One patient is still alive at 249 months after the initial treatment. However, mental retardation was obvious in all four patients 6 to 12 years after the first irradiation and within 2 years of the second course of irradiation. Therefore, new therapeutic strategies must focus on delaying the timing of radiation until the patients are at least 5 years old and avoiding a second course of radiation to treat recurrence.
References
Balkhoyor KB, Bernstein M (2000) Involution of diencephalic pilocytic astrocytoma after partial resection. Report of two cases in adults. J Neurosurg 93:484–486
Brown MT, Friedman HS, Oakes WJ, Boyko OB, Hockenberger B, Schold SC (1993) Chemotherapy for pilocytic astrocytomas. Cancer 71:3165–3172
Burger PC, Scheithauer BW, Paulus W, Szymas J, Giannini C, Kleihues P (2000) Pilocytic astrocytomas. In: Kleihues P, Cavenee WK (eds) Tumours of the nervous system. IARC, Lyon, pp 45–51
Burr IM, Slonim AE, Danish RK, Gadoth N, Butler IJ (1976) Diencephalic syndrome revisited. J Pediatr 88:439–444
Drake JM, Joy M, Goldenberg A, Kreindler D (1991) Computer- and robot- assisted resection of thalamic astrocytomas in children. Neurosurgery 29:27–31
Duffner PK, Cohen ME, Thompson PR, Lansky SB (1985) The long-term effects of cranial radiation on the central nervous system. Cancer 56:1841–1847
Fishman MA, Peake GT (1970) Paradoxical growth in a patient with the diencephalic syndrome. Pediatrics 45:973–982
Gravey M, Packer RJ (1996) An integrated approach to the treatment of chiasmatic-hypothalamic gliomas. J Neurooncol 28:167–183
Hoffman HJ, Humphreys RP, Drake JM, Rutka JT, Becker LE, Jenkin D, Greenberg M (1993) Optic pathway/hypothalamic gliomas: a dilemma in management. Pediatr Neurosurg 19:186–195
Mulhern RK, Hancock J, Fairclough D, Kun L (1992) Neuropsychological status of children treated for brain tumors: a critical review and integrative analysis. Med Pediatr Oncol 20:181–191
Namba S, Nishimoto A, Yagyu Y (1985) Diencephalic syndrome of emaciation (Russell's syndrome). Long-term survival. Surg Neurol 23:581–588
Packer RJ (2000) Chemotherapy: low-grade gliomas of the hypothalamus and thalamus. Pediatr Neurosurg 32:259–263
Packer RJ, Savino PJ, Bilaniuk LT, Zimmerman RA, Schatz NJ, Rosenstock JG, Nelson DS, Jarrett PD, Bruce DA, Schut L (1983) Chiasmatic gliomas of childhood. A reappraisal of natural history and effectiveness of cranial irradiation. Childs Brain 10:393–403
Packer RJ, Ater J, Allen J, Phillips P, Geyer R, Nicholson HS, Jakacki R, Kurcynski E, Needle M, Finlay J, Reaman G, Boyett JM (1997) Carboplatin and vincristine chemotherapy for children with newly diagnosed progressive low grade gliomas. J Neurosurg 86:747–754
Prados MD, Edwards MS, Rabbitt J, Lamborn K, Davis RL, Levin VA (1997) Treatment of pediatric low-grade gliomas with a nitrosourea-based multiagent chemotherapy regimen. J Neurooncol 32:235–241
Rajakulasingam K, Cerullo LJ, Raimondi AJ (1979) Childhood moyamoya syndrome. Postradiation pathogenesis. Childs Brain 5:467–475
Richards GE (1980) Effects of irradiation on the hypothalamic and pituitary regions. In: Gilbert HA, Kogan AR (eds) Radiation damage to the nervous system. Raven Press, New York, pp 175–180
Somaza SC, Kondziolka D, Lunsford LD, Flickinger JC, Bissonette DJ, Albright AL (1996) Early outcomes after stereotactic radiosurgery for growing pilocytic astrocytomas in children. Pediatr Neurosurg 25:109–115
Sutton LN, Molloy PT, Sernyak H, Goldwein J, Phillips PL, Rorke LB, Moshang T Jr, Lange B, Packer RJ (1995) Long-term outcome of hypothalamic/chiasmatic astrocytomas in children treated with conservative surgery. J Neurosurg 83:583–589
Wisoff JH, Abbott R, Epstein F (1990) Surgical management of exophytic chiasmatic-hypothalamic tumors of childhood. J Neurosurg 73:661–667
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A part of this study, comprising three cases and a shorter follow-up period, was previously published in Japanese with an English abstract: Arita K et al (1993) The long-term prognosis of diencephalic syndrome (in Japanese). Shoni No Noshinkei (Tokyo) 18:181–189
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Arita, K., Kurisu, K., Sugiyama, K. et al. Long-term results of conventional treatment of diencephalic pilocytic astrocytoma in infants. Childs Nerv Syst 19, 145–151 (2003). https://doi.org/10.1007/s00381-002-0705-x
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DOI: https://doi.org/10.1007/s00381-002-0705-x