Primitive Neuroectodermal Tumors of the Central Nervous System Follow-up

Updated: May 31, 2018
  • Author: Subrata Ghosh, MD, MBBS, MS; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
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Further Outpatient Care

See the list below:

  • 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.


Further Inpatient Care

Patients with primitive neuroectodermal tumors (PNET) may need to be admitted for treatment of complications of surgery, chemotherapy, or tumor recurrence.



Complications may include the following:

  • Meningitis (postoperative)

  • Hydrocephalus

  • Immunosuppression due to chemotherapy and/or radiotherapy

  • Paralysis

  • Cranial nerve palsy

  • Hypothyroidism

  • Cognitive dysfunction

  • Growth retardation



The following factors worsen the prognosis:

  • Presence of metastases at diagnosis

  • Infiltrative nature, evidence of glial differentiation, and presence of TP53 mutation

  • Recent genomic study reveals that cases in the WNT group showed a slightly better survival with more favorable prognosis than those in the SHH or non-WNT/SHH group. [14]

  • 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.