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Stage Information
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
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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]
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Edwards MS, Hudgins RJ, Wilson CB, et al.: Pineal region tumors in children. J Neurosurg 68 (5): 689-97, 1988.
[PUBMED Abstract]
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Disclafani A, Hudgins RJ, Edwards MS, et al.: Pineocytomas. Cancer 63 (2): 302-4, 1989.
[PUBMED Abstract]
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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]
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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]
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