Papillary glioneuronal tumor

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Magnetic resonance imaging of an extensive papillary glioneuronal tumor.

A rare benign brain tumor that was included in the WHO classification of tumors of the central nervous system in 2007 and classified as grade I is referred to as papillary glioneuronal tumor . The entity was first described in 1998; Histopathologically similar tumors have previously been referred to as pseudopapillary ganglioglionic neurocytoma or pseudopapillary neurocytoma with glial differentiation .

Epidemiology and Clinic

Papillary glioneuronal tumors are very rare. They occur over a wide age range, with the median age at diagnosis being 27 years. The tumors are typically located in the temporal lobe area . In magnetic resonance imaging , glioneuronal papillary glioneuronal tumors appear as well-delimited masses with contrast medium uptake.

Neuropathology

Histopathology of the tumor with the formation of loose papillary structures. Hematoxylin-eosin stain .
Immunohistochemical staining of a papillary glioneuronal tumor for GFAP and synaptophysin.

Histopathologically, these are glioneuronal tumors in which GFAP- positive astrocytic tumor cells develop pseudopapillary structures around hyalinized blood vessels; in between there are groups of synaptophysin- positive neurocytic or ganglioid differentiated tumor cells.

Treatment and prognosis

After surgical removal of the tumor, recurrences are extremely rare.

Individual evidence

  1. ↑ Louis DN, Ohgaki H, Wiestler OD, et al. : The 2007 WHO classification of tumors of the central nervous system . In: Acta Neuropathol. . 114, No. 2, August 2007, pp. 97-109. doi : 10.1007 / s00401-007-0243-4 . PMID 17618441 . PMC 1929165 (free full text).
  2. ↑ Komori T, Scheithauer BW, Anthony DC, et al. : Papillary glioneuronal tumor: a new variant of mixed neuronal-glial neoplasm . In: Am. J. Surg. Pathol. . 22, No. 10, October 1998, pp. 1171-83. PMID 9777979 .
  3. ↑ Kim DH, Suh YL: Pseudopapillary neurocytoma of temporal lobe with glial differentiation . In: Acta Neuropathol. . 94, No. 2, August 1997, pp. 187-91. PMID 9255395 .