Presentation
Abstract Primitive neuroectodermal tumors (PNET) are malignant tumors exceptionally present in adulthood, especially those with supratentorial location. [thieme-connect.com]
Clinical presentation is non-specific and cannot be distinguished from other aggressive intracranial masses. Symptoms of raised intracranial pressure, seizures and focal neurology may be present. [radiopaedia.org]
Purely IPNETs are tumors presenting mainly in children and young adults (age from 3 months to 29 years; mean 12.9 years). There are not evident sex differences (ratio female: male 1.25: 1). [scielo.isciii.es]
Entire Body System
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Weakness
The neurological examination revealed paraparesis (grade of weakness 3/5 at hips; 2/5, at knees; and 1/5, at ankles). His knee and ankle reflexes were bilaterally brisk. [scielo.isciii.es]
Neurologic
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Seizure
Symptoms of raised intracranial pressure, seizures and focal neurology may be present. Supratentorial PNETs are composed of primitive undifferentiated neuroepithelial cells, and are WHO grade IV tumors. [radiopaedia.org]
Treatment
At seven reported cases of purely IPNETs in which chemotherapy or a combination of radiotherapy and chemotherapy were administered either as primary treatment or as treatment after progression, recurrence or intracranial dissemination, temporary clinical [scielo.isciii.es]
Leptomeningeal and/or subarachnoid seeding are common, necessitating imaging and treatment of the whole neuraxis. [radiopaedia.org]
Prognosis
Supratentorial PNET's generally have a poorer prognosis than medulloblastomas, with a low 5-year survival rate. [radiopaedia.org]