An 8-year-old boy with neurofibromatosis type 1 (NF1) and a biopsy-proven juvenile pilocytic astrocytoma of the hypothalamic/chiasmatic region was followed with serial MRIs over 4 years. Spontaneous tumor regression was followed by progression and biopsy; 6 months later, the tumor regressed again. This bimodal regression is rare, but highlights the variable natural history of low-grade gliomas in children with NF1 and the difficulty in evaluating response of such tumors to therapy.

1.
Borit A, Richardson EP: The biological and clinical behavior of pilocytic astrocytomas of the optic pathways. Brain 1982;105:161–187.
2.
Liu GT, Lessell S: Spontaneous visual improvement in chiasmal gliomas. Am J Ophthalmol 1992;114:193–201.
3.
Hoyt WF, Baghdassarian SA: Optic glioma of childhood. Natural history and rationale for conservative management. Br J Ophthalmol 1969;53:793–798.
4.
Leisti EL, Pyhtinen J, Poyhonen M: Spontaneous decrease of a pilocytic astrocytoma in neurofibromatosis type 1. Am J Neuroradiol 1996;17:1691–1694.
5.
Parazzini C, Triulzi F, Bianchini E, Agnetti V, Conti M, Zanolini C, Maninetti MM, Rossi LN, Scotti G: Spontaneous involution of optic pathway lesions in neurofibromatosis type 1: Serial contrast MR evaluation. Am J Neuroradiol 1995;16:1711–1718.
6.
Gottschalk S, Tavakolian R, Buske A, Tinschert S, Lehmann R: Spontaneous remission of chiasmatic/hypothalamic masses in neurofibromatosis type 1: Report of two cases. Neuroradiology 1999;41:199–201.
7.
Venes JL, Latack J, Kandt RS: Postoperative regression of opticochiasmatic astrocytoma: A case for expectant therapy. Neurosurgery 1984;15:421–423.
8.
Takeuchi H, Kabuto M, Sato K, Kubota T: Chiasmal gliomas with spontaneous regression: Proliferation and apoptosis. Childs Nerv Syst 1997;13:229–233.
9.
Rao S, Constantini S, Gomori JM, Siegal T, Epstein F: Spontaneous involution of an intra-axial brainstem lesion: A case report. Pediatr Neurosurg 1995;23:279–282.
10.
Lazareff JA, Suwinski R, De Rosa R, Olmstead CE: Tumor volume and growth kinetics in hypothalamic-chiasmatic pediatric low grade gliomas. Pediatr Neurosurg 1999;30:312–319.
11.
Kernan JC, Horgan MA, Piatt JH, D’Agostino A: Spontaneous involution of a diencephalic astrocytoma. Pediatr Neurosurg 1998;29:149–153.
12.
Lenard HG, Engelbrecht V, Janssen G, Wechsler W, Tautz C: Complete remission of a diffuse pontine glioma. Neuropediatrics 1998;29:328–330.
13.
Hoffman HJ, Soloniuk DS, Humphreys RP, Drake JM, Becker LE, De Lima BO, Piatt JH Jr: Management and outcome of low-grade astrocytomas of the midline in children: A retrospective review. Neurosurgery 1993;33:964–971.
14.
Packer RJ, Savino PJ, Bilaniuk LT, Zimmerman RA, Schatz NJ, Rosenstock JG, Nelson DS, Jarrett PD, Bruce DA, Schut L: Chiasmatic gliomas of childhood. A reappraisal of natural history and effectiveness of cranial irradiation. Childs Brain 1983;10:393–403.
15.
Shuper A, Horev G, Kornreich L, Michowiz S, Weitz R, Zaizov R, Cohen IJ: Visual pathway glioma: An erratic tumor with therapeutic dilemmas. Arch Dis Child 1997;76:259–263.
16.
Perilongo G, Moras P, Carollo C, Battistella A, Clementi M, Laverda A, Murgia A: Spontaneous partial regression of low-grade glioma in children with neurofibromatosis-1: A real possibility. J Child Neurol 1999;14:352–356.
17.
Brzowski AE, Bazan C, Mumma JV, Ryan SG: Spontaneous regression of optic glioma in a patient with neurofibromatosis. Neurology 1992;42:679–681.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.