Abstract
Five cases of pilomyxoid astrocytoma (PmA) characterized by a monophasic pattern with a myxoid background were selected for a clinicopathological study from 23 cases previously diagnosed as pilocytic astrocytoma (PA). All PmA patients were either infants or young children (mean age 2.1 years), and all tumors were located in the optic chiasm/hypothalamus region. All cases received chemotherapy, which reduced tumor size, and the location of the tumor became confined to the optic chiasm. In two cases, tumor recurrence occurred 3 and 7 years after chemotherapy. Histology of the recurrent tumors showed the biphasic pattern of classical PA. Hence, we conclude that PmA might be an infantile form of PA and speculate that a subset of PmA in the optic pathway/hypothalamus originates from the optic chiasm, possibly derived from radial glia existing in the embryonic optic chiasm.
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Arslanoglu A, Cirak B, Horska A, Okoh J, Tihan T, Aronson L, Avellino AM, Burger PC, Yousem DM (2003) MR imaging characteristics of pilomyxoid astrocytomas. AJNR Am J Neuroradiol 24:1906–1908
Brown MT, Friedman HS, Oakes WJ, Boyko OB, Hockenberger B, Schold SC Jr (1993) Chemotherapy for pilocytic astrocytomas. Cancer 71:3165–3172
Burger PC, Cohen KJ, Rosenblum MK, Tihan T (2000) Pathology of diencephalic astrocytomas. Pediatr Neurosurg. 32:214–219
Burger PC, Scheithauer BW, Paulus W, Szymas J, Giannini C, Kleihues P (2000) Pilocytic astrocytoma. In: Kleihues P, Cavenee WK (eds), Pathology and Genetics: Tumours of the Nervous System. WHO Classification of Tumours, IARC, Lyon, pp 45–51
Burger PC, Scheithauer BW, Vogel FS (2002) Surgical Pathology of the Nervous System and its Coverings. 4th edn, Churchill Livingston, New York, pp 203–215
Cottingham SL, Boesel CP, Yates AJ (1996) Pilocytic astrocytoma in infants: A distinctive histological pattern. J Neuropathol Exp Neurol 55:654
Fernandez C, Figarella-Branger D, Girard N, Bouvier-Labit C, Gouvernet J, Paz Paredes A, Lena G (2003) Pilocytic astrocytomas in children: prognostic factors—a retrospective study of 80 cases. Neurosurgery 53:544–553; discussion 554–555
Fuller CE, Frankel B, Smith M, Rodziewitz G, Landas SK, Caruso R, Schelper R (2001) Suprasellar monomorphous pilomyxoid neoplasm: an ultastructural analysis. Clin Neuropathol 20:256–262
Giannini C, Scheithauer BW, Burger PC, Christensen MR, Wollan PC, Sebo TJ, Forsyth PA, Hayostek CJ (1999) Cellular proliferation in pilocytic and diffuse astrocytomas. J Neuropathol Exp Neurol 58:46–53
Gottfried ON, Fults DW, Townsend JJ, Couldwell WT (2003) Spontaneous hemorrhage associated with a pilomyxoid astrocytoma. J Neurosurg 99:416–420
Ironside JW, Moss TH, Louis DN, Lowe JS, Weller RO (eds) (2003) Diagnostic Pathology of Nervous System Tumours. Churchill Livingstone, London, pp 58–64
Jaenisch W, Schreiber D, Martin H, Gerlach H (1985) Dienzephale pilozytische Astrozytome mit Krankheitsbeginn im Saeuglingsalter. Biologisches Verhalten und pathomorphologische Befunde bei 11 Kindern. Zbl allg Pathol pathol Anat 130:31–43
Kato T, Sawamura Y, Tada M, Ikeda J, Ishii N, Abe H (1998) Cisplatin/vincristine chemotherapy for hypothalamic/visual pathway astrocytomas in young children. J Neurooncol 37:263–270
Komotar RJ, Burger PC, Carson BS, Brem H, Olivi A, Goldthwaite PT, Tihan T (2004). Pilocytic and pilomyxoid hypothalamic/chiasmatic astrocytomas. Neurosurgery 54:72–79; discussion 79–80.
Krieger MD, Gonzalez-Gomez I, Levy ML, McComb JG (1997) Recurrence patterns and anaplastic change in a long-term study of pilocytic astrocytomas. Pediatr Neurosurg 27:1–11
Mason CA, Sretavan DW (1997) Glia, neurons, and axon pathfinding during optic chiasm development. Curr Opin Neurobiol 7:647–653
Nishio S, Takesita I, Fukui M, Yamashita M, Tateishi J (1988) Anaplastic evolution of childhood optico-hypothalamic pilocytic astrocytoma: Report of an autopsy case. Clin Neuropathol 7:254–258
Perilongo G, Carollo C, Salviati L, Murgia A, Pillon M, Basso G, Gardiman M, Laverda A (1997) Diencephalic syndrome and disseminated juvenile pilocytic astrocytomas of the hypothalamic-optic chiasm region. Cancer 80:142–146
Petito C (2003) Suprasellar monomorphous pilomyxoid gliomas. AJNR Am J Neuroradiol 24:1931–1932
Tihan T, Fisher PG, Kepner JL, Godfraind C, McComb RD, Goldthwaite PT, Burger PC (1999) Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome. J Neuropathol Exp Neurol 58:1061–1068
Tokoro Y (1959) Glioma of the optic chiasm. In: Brain Tumor, Igaku-shoin, Tokyo, pp 622–628
Wilson WB, Feinsod M, Hoyt WF, Nielsen SL (1976) Malignant evolution of childhood chiasmal pilocytic astrocytoma. Neurology 26:322–325
Acknowledgements
We thank Mr. M. Shimizu, Mr. J. Moriya, Mr. K. Marukawa, Ms. T. Kurokawa, and Ms. Y. Saito for their technical assistance. This study was supported in part by grants from the Ministry of Education, Science, Sports and Culture, and the Ministry of Health, Labour and Welfare, Japan.
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This study was supported by grants from Core Research for Evolutional Science and Technology (CREST), Japan Science and Technology (JST) Corporation.
H. Sawa is a member of the 21st Century COE Program for Zoonosis Control
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Chikai, K., Ohnishi, A., Kato, T. et al. Clinico-pathological features of pilomyxoid astrocytoma of the optic pathway. Acta Neuropathol 108, 109–114 (2004). https://doi.org/10.1007/s00401-004-0858-7
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DOI: https://doi.org/10.1007/s00401-004-0858-7