Neurocytoma

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Central neurocytoma. Magnetic resonance tomography (T1 weighting) with heterogeneous weak contrast medium uptake of the tumor.
Central neurocytoma. Magnetic resonance tomography (T2 weighting).

A rare non-malignant brain tumor is called a neurocytoma . Since recurrences can occur, the tumor is classified as grade II according to the WHO classification of tumors of the central nervous system . The WHO classification distinguishes the central neurocytoma , which is typically located intraventricularly in the area of ​​the foramen Monroi , from the rarer extraventricular neurocytoma , which has no relation to the ventricular system of the brain.

Epidemiology and Clinic

Neurocytomas are rare. Since it was first described in 1982, some 100 cases have been reported worldwide. The central neurocytoma, due to its intraventricular location, typically leads to an obstruction of the CSF outflow and is clinically noticeable as hydrocephalus . In imaging, neurocytomas often appear as cystic masses that take up variable contrast media.

Neuropathology

Neurocytoma histopathology. The relatively monomorphic neurocytically differentiated tumor cells form fine neuropil-like processes and islands. Hematoxylin-eosin stain . Magnification 200x
Immunohistochemistry. The clear staining of the cell nuclei with an antibody directed against NeuN proves the neuronal differentiation of the tumor cells. 400x magnification

The relatively monomorphic neurocytically differentiated tumor cells form fine neuropil-like processes and islands. With the expression of neuronal markers such as Synaptophysin and NeuN, a neuronal differentiation of the tumor cells can be demonstrated immunohistochemically , which allows a differentiation from other neuroectodermal tumors such as ependymoma .

Treatment and prognosis

After complete neurosurgical resection of the tumor, the prognosis is generally favorable for both central neurocytomas and extraventricular neurocytomas; a 5-year survival rate of 81% was given for central neurocytomas . In addition to an incomplete resection, increased proliferative activity in particular has been associated with an increased tendency to relapse; Such tumors are also referred to as atypical neurocytomas in the literature. In this case, the further course of the disease can, under certain circumstances, be favorably influenced by adjuvant radiation .

literature

Individual evidence

  1. ^ J. Hassoun, D. Gambarelli, F. Grisoli, W. Pellet, G. Salamon, JF Pellissier, M. Toga: Central neurocytoma. An electron-microscopic study of two cases. In: Acta Neuropathol 1982; 56, pp 151-156. PMID 7064664
  2. DJ Brat, BW Scheithauer, CG Eberhart, PC Burger: Extraventricular neurocytomas: pathologic features and clinical outcome. In: Am J Surg Pathol . 2001 Oct; 25 (10), pp. 1252-1260. PMID 11688459
  3. ^ SE Schild, BW Scheithauer, MG Haddock, D. Schiff, PC Burger, WW Wong, MK Lyons: Central neurocytomas. In: Cancer . 1997; 79, pp. 790-795. PMID 9024717
  4. D. Rades, SE Schild: Treatment recommendations for the various subgroups of neurocytomas. In: J Neurooncol. 2006; 77 (3), pp. 305-309. PMID 16575540
  5. JL Leenstra, FJ Rodriguez, CM Frechette, C. Giannini, SL Stafford, BE Pollock, SE Schild, BW Scheithauer, RB Jenkins, JC Buckner, PD Brown: Central neurocytoma: management recommendations based on a 35-year experience. In: Int J Radiat Oncol Biol Phys . 2007 Mar 15; 67 (4), pp. 1145-1154. PMID 17187939