Final Diagnosis -- Gangliocytoma

DIAGNOSIS: GANGLIOCYTOMA

DISCUSSION:

Ganglion cell tumors (GCT) are rather common low grade neoplasms found in patients with epilepsy and representing up to 1.3% of brain tumors (5). Over 80% of tumors are discovered during the first three decades of life with a male-to-female ratio of 1.1:1-1.9:1 (3), (7). Many of the tumors arise in the temporal lobe, but other sites are not spared (1). A classic cyst with an enhancing mural nodule appearance simplifies the diagnostic challenge (8), effectively limiting the differential diagnosis to such entities as pilocytic astrocytoma, pleomorphic xanthoastrocytoma (PXA), hemangioblastoma, extraventricular neurocytoma and ependymoma. PXA and GCT share not only desmoplasia, but also a variable proportion of a neuronal component (2). PXA differs in having mostly fascicular architecture, presence of xanthic cells and extreme nuclear pleomorphism. The prognosis of GCT is generally favorable, except for a fraction of neoplasms featuring a component of frankly anaplastic astrocytes. Even in such cases the outlook mostly depends on the extent of surgical removal (6). Both neural and astrocytic components are clonal (9), but the histogenesis of GCT is unclear. Frequent association of GCT with focal microdysgenesis suggests a dysembryoplastic component (4).

REFERENCES:

  1. Celli P, Scarpinati M, Nardacci B, Cervoni L, Cantore GP (1993)Gangliogliomas of the cerebral hemispheres. Report of 14 cases with long-term follow-up and review of the literature. Acta Neurochir (Wien) 125: 52-7.
  2. Giannini C, Scheithauer BW, Lopes MB, Hirose T, Kros JM, VandenBerg SR. (2002) Immunophenotype of pleomorphic xanthoastrocytoma. Am J Surg Pathol 26: 479-85.
  3. Hirose T, Schneithauer BW, Lopes MB, Gerber HA, Altermatt HJ, VandenBerg SR (1997) Ganglioglioma: an ultrastructural and immunohistochemical study. Cancer 79: 989-1003.
  4. Prayson RA, Khajavi K, Comair YG (1995) Cortical architectural abnormalities and MIB1 immunoreactivity in gangliogliomas: a study of 60 patients with intracranial tumors. J Neuropathol Exp Neurol 54: 513-20.
  5. Russell DS, Rubinstein LJ (1989) Pathology of tumours of the nervous system. Williams & Wilkins: Baltimore.
  6. Selch MT, Goy BW, Lee SP, El-Sadin S, Kincaid P, Park SH (1998) Gangliogliomas: experience with 34 patients and review of the literature. Am J Clin Oncol 21: 557-64.
  7. Wolf HK, Muller MB, Spanle M, Zentner J, Schramm J, Wiestler OD (1994) Ganglioglioma: a detailed histopathological and immunohistochemical analysis of 61 cases. Acta Neuropathol (Berl) 88: 166-73.
  8. Zentner J, Wolf HK, Ostertun B, Hufnagel A, Campos MG, Solymosi L (1994) Gangliogliomas: clinical, radiological, and histopathological findings in 51 patients. J Neurol Neurosurg Psychiatry 57: 1497-502.
  9. Zhu JJ, Leon SP, Folkerth RD, Guo SZ, Wu JK, Black PM (1997) Evidence for clonal origin of neoplastic neuronal and glial cells in gangliogliomas. Am J Pathol 151: 565-71.

Contributed by Jorge E. Dotto, Joachim Baehring, Joseph M. Piepmeier, Sergei I. Bannykh



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