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]

• Clonus

  Bilateral and prolonged clonus could be elicited. The abdominal cutaneous reflexes were abolished. Finally, at general exploration, the infant presented abdominal distension due to paralytic ileus and urinary retention. [scielo.isciii.es]

• Meningism

  Meningeal carcinomatosis. Rev Neurol 2000; 31 (12) 1237-1241 15 Orrison W. Neurorradiología. 1era ed. Madrid: Harcourt; 2001 16 Girschick HJ, Klein R, Scheurlen WG, Kühl J. [thieme-connect.com]

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]

Supratentorial PNET's generally have a poorer prognosis than medulloblastomas, with a low 5-year survival rate. [radiopaedia.org]