Clinicopathological and molecular analysis of multinodular and vacuolating neuronal tumors of the cerebrum.

Clinicopathological and molecular analysis of multinodular and vacuolating neuronal tumors of the cerebrum.

Hum Pathol. 2018 Dec 11;:

Authors: Choi E, Kim SI, Won JK, Chung CK, Kim SK, Choi SH, Choi S, Han B, Ahn B, Sun-Wha IM, Park SH

Abstract
Multinodular and vacuolating neuronal tumor (MVNT) of the cerebrum is a recently recognized rare neuronal tumor, and its pathogenesis is unclear. We analyzed 7 cases of histologically typical MVNT: six were adults [mean age: 43.0years (range: 23-56)] and one was a child (10-year-old). The most common symptoms were seizures (n=4) and headache (n=2). The tumors were supratentorial (temporal=5 and frontal lobes=2) in origin as reported. Vacuolated tumor cells were robustly positive for alpha-INA and Olig2 and at least partly positive for synaptophysin and MAP2, but negative for Neu-N. Two cases were positive for nestin and one for CD34. GFAP and vimentin were expressed in reactive astrocytes, but not in tumor cells. Negative results were obtained for p53, IDH-1, BRAFV600E, H3 K27M, EGFR, Lin28A, and L1CAM. ATRX, BRG1, INI-1, and TMHH were retained. The Ki-67 labeling index was very low (<1%) and pHH3 revealed no mitotic figure. Ultrastructural features of tumor cells were comparable with those of immature neuronal cells, with several intracytoplasmic myelin-like autophagosomes and pericellular vacuolization. No IDH1/IDH2 and BRAFV600E mutations were found upon direct sequencing. WES revealed FGFR2-ZMYND11 gene fusion in one case. After gross total resection, all patients were alive without seizures. There was no tumor recurrence during an average time period of 68months (range: 23-101months). The analysis of seven typical cases of MVNT suggested that these lesions may be clonal tumors because FGFR2-ZMYND11 fusion was found (one case).

PMID: 30550736 [PubMed - as supplied by publisher]

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