Journal Mobile Options
Table of Contents
Vol. 46, No. 5, 2010
Issue release date: April 2011
Section title: Case Report
Pediatr Neurosurg 2010;46:381–384
(DOI:10.1159/000322896)

Medulloblastoma with Extensive Nodularity Undergoing Post-Therapeutic Maturation to a Gangliocytoma: A Case Report and Literature Review

Chelliah D. · Mensah Sarfo-Poku C. · Stea B.D. · Gardetto J. · Zumwalt J.
aSchool of Medicine and bSchool of Public Health, Loma Linda University, Loma Linda, Calif., and Departments of cRadiation Oncology and dPathology, University of Arizona, Tuscon, Ariz., USA

Do you have an account?

Register and profit from personalized services (MyKarger) Login Information

Please create your User ID & Password





Contact Information









I have read the Karger Terms and Conditions and agree.

Register and profit from personalized services (MyKarger) Login Information

Please create your User ID & Password





Contact Information









I have read the Karger Terms and Conditions and agree.

To view the fulltext, please log in

To view the pdf, please log in

Buy

  • FullText & PDF
  • Unlimited re-access via MyKarger (new!)
  • Unrestricted printing, no saving restrictions for personal use
  • Reduced rates with a PPV account
read more

Direct: USD 33.00
Account: USD 23.00

Select

Rent/Cloud

  • Rent for 48h to view
  • Buy Cloud Access for unlimited viewing via different devices
  • Synchronizing in the ReadCube Cloud
  • Printing and saving restriction apply

Rental: USD 8.50
Cloud: USD 20.00

Select

Subscribe

  • Automatic perpetual access to all articles of the subscribed year(s)
  • Unlimited re-access via Subscriber Login or MyKarger
  • Unrestricted printing, no saving restrictions for personal use
read more

Subcription rates


Select


Article / Publication Details

First-Page Preview
Abstract of Case Report

Received: 9/27/2010
Accepted: 6/11/2010
Published online: 3/9/2011

Number of Print Pages: 4
Number of Figures: 3
Number of Tables: 0

ISSN: 1016-2291 (Print)
eISSN: 1423-0305 (Online)

For additional information: http://www.karger.com/PNE

Abstract

We present a report of a 12-year-old boy diagnosed with medulloblastoma at 22 months of age. A gross total resection was performed followed by adjuvant systemic chemotherapy due to his young age; however, the tumor recurred locally in the posterior fossa 7 months later. The recurrent tumor was excised and he received craniospinal radiation with a boost given to the posterior fossa followed by high-dose chemotherapy. He remained disease free for approximately 10 years without major neurologic deficit and only mild cognitive impairment. A routine follow-up MRI of the brain revealed an enhancing mass. The patient underwent surgical debulking and pathological examination revealed no residual immature medulloblastoma cells but instead mature ganglion cells, consistent with a gangliocytoma. The apparent maturation of primitive medulloblastoma cells is a rare phenomenon, which may have ensued from the long-term effects of adjuvant therapies inducing advanced cellular maturation.


Article / Publication Details

First-Page Preview
Abstract of Case Report

Received: 9/27/2010
Accepted: 6/11/2010
Published online: 3/9/2011

Number of Print Pages: 4
Number of Figures: 3
Number of Tables: 0

ISSN: 1016-2291 (Print)
eISSN: 1423-0305 (Online)

For additional information: http://www.karger.com/PNE


Copyright / Drug Dosage

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 or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
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 goverment 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.

References

  1. Becker LE, Hinton D: Primitive neuroectodermal tumors of the central nervous system. Hum Pathol 1983;14:538–550.
  2. Herman MM, Rubinstein LJ: Divergent glial and neuronal differentiation in cerebellar medulloblastoma in an organ culture system in vitro occurrence of synaptic ribbons. Acta Neuropathol 1984;65:10–24.
  3. Giangaspero F, Eberhart CG, Haapasalo H, Pietsch T, Wiestler OD, Ellison DW: Medulloblastoma; in Louis DN, Ohgaki H, Wiestler OD (eds): Pathology and Genetics of Tumours of the Nervous System. World Health Organization Classification of Tumours. Lyon, IARC Press, 2007, pp 132–140.
  4. Pearl GS, Takei Y: Cerebellar ‘neuroblastoma’: nosology as it relates to medulloblastoma. Cancer 1981;47:772–779.
  5. Shin WY, Laufer H, Lee YC, et al: Fine structure of a cerebellar neuroblastoma. Acta Neuropathol 1978;42:11–13.
  6. Warzog R, Janisch W, Lang G: Morphology and biology of cerebellar neuroblastomas. J Neurooncol 1983;1:373–379.
  7. Yagishita S, Itoh Y, Chiba Y, et al: Morphological investigations on cerebellar ‘neuroblastoma’ group. Acta Neuropathol 1982;56:22–28.
  8. Giangaspero F, Girogio P, Fondelli MP, Brisigotti M, Carollo C, Burnelli R, Burger P, Garre ML: Medulloblastoma with extensive nodularity: a variant with favorable prognosis. J Neurosurg 1999;91:971–977.
  9. McManamy CS, Pears J, Weston CL, et al: Nodule formation and desmoplasia in medulloblastomas: defining the nodular/desmoplastic variant and its biological behavior. Brain Pathol 2007;17:151–164.
  10. Hellbusch L, Robinson M, McComb R, Gordon B, Meyer R: Medulloblastoma with extensive nodularity: a report of two cases. Pediatr Neurosurg 2008;44:430–432.
  11. Cai DX, Mafra M, Schmidt RE, Scheithauer BW, Park TS, Perry A: Medulloblastomas with extensive posttherapy neuronal maturation. Report of two cases. J Neurosurg 2000;93:330–334.
  12. Kudo M, Shimizu M, Akutsu Y, Imaya H, Chen MN, Miura M: Ganglioglial differentiation in medulloblastoma. Acta Pathol Jpn 1990;40:50–56.
  13. Kubota KC, Itoh T, Yamada Y, Yamaguchi S, Ishida Y, Nakasu Y, Watanabe R, Ito I, Sawamura Y, Matsuno Y, Nagashima K: Melanocytic medulloblastoma with ganglioneurocytomatous differentiation: a case report. Neuropathology 2009;29:72–77.
  14. Chandarevian JD, Montes IL, German AM, Freeman CR: Maturation of cerebellar neuroblastoma into ganglioneuroma with melanosis: histologic immunocytochemical and ultrastructural study. Cancer 1987;59:69–76.
  15. Gulino A, Arcella A, Giangaspero F: Pathological and molecular heterogeneity of medulloblastoma. Curr Opin Oncol 2008;20:668–675.
  16. Kenney AM, Rowitch DH: Sonic hedgehog promotes G(1) cyclin expression and sustained cell cycle progression in mammalian neuronal precursors. Mol Cell Biol 2000;20:9055–9067.
  17. Gilbertson RJ, Ellison DW: The origins of medulloblastoma subtypes. Annu Rev Pathol 2008;3:341–365.
  18. Eberhardt CG, Kepner JL, Goldthwaite PT, et al: Histopathologic grading of medulloblastomas: a Pediatric Oncology Group study. Cancer 2002;94:552–560.
  19. Garre ML, Cama A, Bagnasco F, Morana G, Giangaspero F, Brisigotti M, Gambini C, Forni M, Rossi A, Haupt R, et al: Medulloblastoma variants: age-dependent occurrence and relation to Gorlin syndrome – A new clinical perspective. Clin Cancer Res 2009;15:2463–2471.
  20. Aliani S, Brunner J, Graf N, et al: Medulloblastoma with extensive nodularity in nevoid basal cell carcinoma syndrome. Med Pediatr Oncol 2003;30:266–267.

    External Resources

  21. Tortori-Donati P, Rossi A, Biancheri R, et al: Brain tumors; in Tortori-Donati P, Rossi A (eds): Pediatric Neuroradiology: Brain. Berlin, Springer, 2005, vol 1, pp 329–436.
  22. Walter AW, Mulhern RK, Gajjar A, Heideman RL, Reardon D, Sanford RA, Xiong X, Kun LE: Survival and neurodevelopmental outcome in young children with medulloblastoma at St. Jude’s Research Hospital. J of Clin Oncol 1999;17:3720–3728.
  23. Suresh TN, Santosh V, Yasha TC, Anandh B, Mohanty A, Indiradevi B, Sampath S, Shankar SK: Medulloblastoma with extensive nodularity: a variant occurring in the very young-clinicopathological and immunohistochemical study of four cases. Childs Nerv Syst 2004;20:55–60.
  24. Lang FF, Epstein FJ, Ransohoff J, Allen JC, Wisoff J, Abbott IR, et al: Central nervous system gangliogliomas. Part 2. Clinical outcome. J Neurosurg 1993;79:867–873.