Primitive Neuroectodermal Tumors of the Central Nervous System Follow-up
- Author: Subrata Ghosh, MD, MBBS; Chief Editor: Tarakad S Ramachandran, MBBS, FRCP(C), FACP more...
Further Inpatient Care
Patients with primitive neuroectodermal tumors (PNET) may need to be admitted for treatment of complications of surgery, chemotherapy, or tumor recurrence.
Further Outpatient Care
- Radiation therapy
- Chemotherapy
- Follow-up care: Serial neuroimaging is used on an individual basis depending upon each patient's initial extent of disease and the extent of resection achieved. In general, MRI is indicated every 3 months for 6 months, followed by every 6 months and then every year. After 5 years, imaging intervals can be prolonged to 5 or 10 years as appropriate.
Complications
- Meningitis (postoperative)
- Hydrocephalus
- Immunosuppression due to chemotherapy and/or radiotherapy
- Paralysis
- Cranial nerve palsy
- Hypothyroidism
- Cognitive dysfunction
- Growth retardation
Prognosis
- The following factors worsen the prognosis:
- Presence of metastases at diagnosis
- Infiltrative nature, evidence of glial differentiation, and presence of TP53 mutation
- Unfavorable location that prevents complete resection: Failure at the primary site continues to be the predominant barrier to cure in patients with medulloblastoma.
- Younger age at presentation: Age older than 4 years at the time of initial diagnosis is associated with more favorable prognosis than age younger than 4 years.
- In recent series of low-risk cases, the 5-year survival rate has been reported to be 60-80% (or even higher).
- Many tumors relapse at a period equal to the age at diagnosis plus 9 months (the Collin law).
Patient Education
Patients (and parents) should be referred for psychosocial counseling.
Mueller S, Chang S. Pediatric brain tumors: current treatment strategies and future therapeutic approaches. Neurotherapeutics. Jul 2009;6(3):570-86. [Medline].
Kleihues P, Burger PC, Scheithauer BW. The new WHO classification of brain tumours. Brain Pathol. Jul 1993;3(3):255-68. [Medline].
Huppmann AR, Orenstein JM, Jones RV. Cerebellar medulloblastoma in the elderly. Ann Diagn Pathol. Feb 2009;13(1):55-9. [Medline].
Gulino A, Arcella A, Giangaspero F. Pathological and molecular heterogeneity of medulloblastoma. Current Opinions in Oncology. November 2008;20(6):668-75.
Guessous F, Li Y, Abounader R. Signalling pathways in Medulloblastoma. Journal of Cell Physiology. December 2008;217(3):577-583.
Roussel MF, Robinson G. Medulloblastoma: advances and challenges. F1000 Biol Rep. 2011;3:5. [Medline]. [Full Text].
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].
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].
Albright AL, Pollack IF, Adelson PD. Principles and Practice of Pediatric Neurosurgery. 1st ed. New York: Thieme;1999: 591-608.
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].
Graham DI, Lantos PL. Greenfield's Neuropathology. Vol 2. 6th ed. New York: Oxford University Press; 1997:698-710.
Kay A, et al. Brain tumors. First ed. New York: Churchill Livingstone; 1997:561-574.
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.
Kun LE. Brain tumors. Challenges and directions. Pediatr Clin North Am. Aug 1997;44(4):907-17. [Medline].
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].
Rood BR, Macdonald TJ, Packer RJ. Current treatment of medulloblastoma: recent advances and future challenges. Semin Oncol. Oct 2004;31(5):666-75. [Medline].
Russell DS, et al. Pathology of Tumors of the Nervous System. 4th ed. Baltimore: Williams & Wilkins; 1977:203-26.

