Primitive Neuroectodermal Tumors of the Central Nervous System Treatment & Management

  • Author: Subrata Ghosh, MD, MBBS; Chief Editor: Tarakad S Ramachandran, MBBS, FRCP(C), FACP   more...
 
Updated: Aug 29, 2011
 

Medical Care

  • Preoperative administration of steroids can help to alleviate some of the signs and symptoms of primitive neuroectodermal tumors (PNET) by reducing peritumoral edema.[1]
  • Radiation therapy
    • Radiation therapy, usually given adjuvantly, should be performed under the direction of a radiation oncologist.
    • Many series report a clear, dose-dependent relationship between postoperative radiation and local tumor control.
    • Adjuvant radiotherapy alone, with posterior fossa doses of 5000 cGy and neuraxis doses of 3000 cGy, results in a 5-year event-free survival rate of 50-70%. Lower than standard doses of radiation therapy, at least without chemotherapy, are less effective.
    • Craniospinal axis radiation is used for patients with spinal dissemination.
    • Newer methods, including stereotactic radiosurgery and high fractionation radiotherapy, are being evaluated.[8] These methods limit the dose of radiation to the local sites and avoid potential adverse effects in children, including cognitive dysfunction or delay in growth, that are seen frequently with conventional radiotherapy.
  • Chemotherapy
    • Chemotherapy should be administered under the direction of a medical oncologist.
    • Varying combinations of drugs used in these tumors include, but are not limited to, lomustine (CCNU), vincristine, cisplatin, etoposide (VP-16), and cyclophosphamide. Several trials, including those from the Pediatric Oncology Group (POG) and Children's Cancer Group (CCG), are underway, evaluating varying combinations of chemotherapy with and without radiotherapy. The best results, so far, have come from the Children's Hospital of Pennsylvania study, reporting a 5-year event-free survival rate of 80% among 51 patients.
    • CCNU has been shown to be of limited benefit, particularly in high-risk cases.
    • High-dose chemotherapy with stem cell rescue is being tried to improve survival and outcome.
Next

Surgical Care

  • The goals of surgery are to achieve radical tumor resection, when possible, and to restore normal CSF outflow.
  • For persistent lesions, second-look surgery is recommended to remove residual tumor.
  • Available studies have failed to show a significant advantage, in terms of event-free survival, of total resection as compared to near-total and less-aggressive resections.
  • Permanent CSF diversion in the form of ventriculoperitoneal shunt is required in as many as 30% of these cases.
Previous
Next

Consultations

  • Neurosurgeon
  • Neurologist/pediatric neurologist
  • Radiation oncologist
  • Medical oncologist
Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Subrata Ghosh, MD, MBBS  Staff Physician, Division of Neurosurgery, St. Luke's Episcopal Hospital, Texas Medical Center, Houston; Assistant Professor of Neurosurgery, Baylor College of Medicine

Subrata Ghosh, MD, MBBS is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, Congress of Neurological Surgeons, and Texas Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Draga Jichici, MD, FRCP, FAHA  Associate Clinical Professor, Department of Neurology and Critical Care Medicine, McMaster University School of Medicine, Canada

Draga Jichici, MD, FRCP, FAHA is a member of the following medical societies: American Academy of Neurology, Canadian Congress of Neurological Sciences, Canadian Congress of Neurological Sciences, Canadian Congress of Neurological Sciences, Canadian Critical Care Society, Canadian Medical Protective Association, Canadian Neurocritical Care Society, Neurocritical Care Society, Royal College of Physicians and Surgeons of Canada, and Society of Critical Care Medicine (USA)

Disclosure: Nothing to disclose.

Specialty Editor Board

Roberta J Seidman, MD  Associate Professor of Clinical Pathology, Stony Brook University; Director of Neuropathology, Department of Pathology, Stony Brook University Medical Center

Roberta J Seidman, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuropathologists, New York Association of Neuropathologists (The Neuroplex), and Suffolk County Society of Pathologists

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Jorge C Kattah, MD  Head, Associate Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria

Jorge C Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences

Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

Chief Editor

Tarakad S Ramachandran, MBBS, FRCP(C), FACP  Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, FRCP(C), FACP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine

Disclosure: Abbott Labs None None; Teva Marion None None; Boeringer-Ingelheim Honoraria Speaking and teaching

References

  1. Mueller S, Chang S. Pediatric brain tumors: current treatment strategies and future therapeutic approaches. Neurotherapeutics. Jul 2009;6(3):570-86. [Medline].

  2. Kleihues P, Burger PC, Scheithauer BW. The new WHO classification of brain tumours. Brain Pathol. Jul 1993;3(3):255-68. [Medline].

  3. Huppmann AR, Orenstein JM, Jones RV. Cerebellar medulloblastoma in the elderly. Ann Diagn Pathol. Feb 2009;13(1):55-9. [Medline].

  4. Gulino A, Arcella A, Giangaspero F. Pathological and molecular heterogeneity of medulloblastoma. Current Opinions in Oncology. November 2008;20(6):668-75.

  5. Guessous F, Li Y, Abounader R. Signalling pathways in Medulloblastoma. Journal of Cell Physiology. December 2008;217(3):577-583.

  6. Roussel MF, Robinson G. Medulloblastoma: advances and challenges. F1000 Biol Rep. 2011;3:5. [Medline]. [Full Text].

  7. Crawford JR, Rood BR, Rossi CT, Vezina G. Medulloblastoma associated with novel PTCH mutation as primary manifestation of Gorlin syndrome. Neurology. May 5 2009;72(18):1618. [Medline].

  8. Allen JC, Donahue B, DaRosso R, Nirenberg A. Hyperfractionated craniospinal radiotherapy and adjuvant chemotherapy for children with newly diagnosed medulloblastoma and other primitive neuroectodermal tumors. Int J Radiat Oncol Biol Phys. Dec 1 1996;36(5):1155-61. [Medline].

  9. Albright AL, Pollack IF, Adelson PD. Principles and Practice of Pediatric Neurosurgery. 1st ed. New York: Thieme;1999: 591-608.

  10. Goldwein JW, Radcliffe J, Johnson J, et al. Updated results of a pilot study of low dose craniospinal irradiation plus chemotherapy for children under five with cerebellar primitive neuroectodermal tumors (medulloblastoma). Int J Radiat Oncol Biol Phys. Mar 1 1996;34(4):899-904. [Medline].

  11. Graham DI, Lantos PL. Greenfield's Neuropathology. Vol 2. 6th ed. New York: Oxford University Press; 1997:698-710.

  12. Kay A, et al. Brain tumors. First ed. New York: Churchill Livingstone; 1997:561-574.

  13. Kleihues P, Cavanee WK. Pathology and genetics of tumours of the nervous system. Lyon, France: International Agency for Research on Cancer (IARC); 1997:49-55.

  14. Kun LE. Brain tumors. Challenges and directions. Pediatr Clin North Am. Aug 1997;44(4):907-17. [Medline].

  15. Prados MD, Wara W, Edwards MS, et al. Treatment of high-risk medulloblastoma and other primitive neuroectodermal tumors with reduced dose craniospinal radiation therapy and multi-agent nitrosourea-based chemotherapy. Pediatr Neurosurg. Oct 1996;25(4):174-81. [Medline].

  16. Rood BR, Macdonald TJ, Packer RJ. Current treatment of medulloblastoma: recent advances and future challenges. Semin Oncol. Oct 2004;31(5):666-75. [Medline].

  17. Russell DS, et al. Pathology of Tumors of the Nervous System. 4th ed. Baltimore: Williams & Wilkins; 1977:203-26.

 
Previous
Next
 
Cerebellar medulloblastoma. This MRI (axial view, T2-weighted image) demonstrates the heterogeneity of the tumor.
Cerebellar medulloblastoma. This sagittal view MRI without contrast demonstrates characteristic midline cerebellar location with mild obstructive hydrocephalus.
Cerebellar medulloblastoma. This axial view CT scan with contrast shows a partially enhancing mass arising in the midline from cerebellum and filling the fourth ventricle.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.