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Childhood Supratentorial Primitive Neuroectodermal Tumors and Pineoblastoma Treatment (PDQ®)     
Last Modified: 04/09/2008
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Poorly differentiated small round cell tumors of the cerebrum have been referred to as cerebral neuroblastoma and as primitive neuroectodermal tumors. These tumors in the pineal body have classically been called pineoblastomas. Histologically, these tumors may be similar to cerebellar medulloblastoma with varying proportions of features that suggest astrocytic or ependymal differentiation. Genetic aberrations of supratentorial brain tumors appear to be different from those of infratentorial primitive neuroectodermal tumors (PNET, medulloblastoma).[1] Tumors may spread throughout the subarachnoid space. Every patient with a primitive neuroectodermal tumor or with pineoblastoma should be evaluated with diagnostic imaging of the spinal cord and whole brain. The most sensitive method currently available for evaluating spinal cord subarachnoid metastasis is spinal magnetic resonance imaging performed with gadolinium. Cerebrospinal fluid should be examined cytologically for tumor cells. There is no generally accepted staging system for supratentorial primitive neuroectodermal tumors and for pineoblastomas, but the staging system used for medulloblastoma is conventionally used for tumor dissemination. Prognosis is probably related to the extent of disease both at diagnosis and after surgery.[2-5] Patients with cystic tumors may fare better.  [Note: 2-year survival rate: 30% to 50%]

References

  1. Russo C, Pellarin M, Tingby O, et al.: Comparative genomic hybridization in patients with supratentorial and infratentorial primitive neuroectodermal tumors. Cancer 86 (2): 331-9, 1999.  [PUBMED Abstract]

  2. Edwards MS, Hudgins RJ, Wilson CB, et al.: Pineal region tumors in children. J Neurosurg 68 (5): 689-97, 1988.  [PUBMED Abstract]

  3. Disclafani A, Hudgins RJ, Edwards MS, et al.: Pineocytomas. Cancer 63 (2): 302-4, 1989.  [PUBMED Abstract]

  4. D'Andrea AD, Packer RJ, Rorke LB, et al.: Pineocytomas of childhood. A reappraisal of natural history and response to therapy. Cancer 59 (7): 1353-7, 1987.  [PUBMED Abstract]

  5. Abay EO 2nd, Laws ER Jr, Grado GL, et al.: Pineal tumors in children and adolescents. Treatment by CSF shunting and radiotherapy. J Neurosurg 55 (6): 889-95, 1981.  [PUBMED Abstract]