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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Follow-up

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.

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Further Inpatient Care

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

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Complications

Complications may include the following:

  • Meningitis (postoperative)

  • Hydrocephalus

  • Immunosuppression due to chemotherapy and/or radiotherapy

  • Paralysis

  • Cranial nerve palsy

  • Hypothyroidism

  • Cognitive dysfunction

  • Growth retardation

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Prognosis

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).

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Patient Education

Patients (and parents) should be referred for psychosocial counseling.

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