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De novo MYH9 mutation in congenita scalp hemangioma
Journal article   Open access   Peer reviewed

De novo MYH9 mutation in congenita scalp hemangioma

Elena I. Fomchenko, Daniel Duran, Sheng Chih Jin, Weilai Dong, E. Zeynep Erson-Omay, Prince Antwi, August Allocco, Jonathan R. Gaillard, Anita Huttner, Murat Gunel, …
Cold Spring Harbor molecular case studies, v 4(4), 002998
01 Aug 2018
PMID: 29903892
url
https://doi.org/10.1101/mcs.a002998View
Published, Version of Record (VoR) Open

Abstract

Life Sciences & Biomedicine Medicine, Research & Experimental Research & Experimental Medicine Science & Technology
Congenital hemangiomas are tumor-like vascular malformations with poorly understood pathogenesis. We report the case of a neonate with a massive congenital scalp hemangioma that required urgent neurosurgical removal on the second day of life because of concern for high-flow arteriovenous shunting. Exome sequencing identified a rare damaging de novo germline mutation in MYH9 (c.5308C>T, p.[Arg1770Cys]), encoding the MYH9 nonmuscle myosin IIA. MYH9 has a probability of loss-of-function intolerance (pLI) score of >0.99 and is highly intolerant to missense variation (z score = 5.59). The p.(Arg1770Cys) mutation substitutes an evolutionarily conserved amino acid in the protein's critical myosin tail domain and is predicted to be highly deleterious by SIFT, PolyPhen-2, MetaSVM, and CADD. MYH9 is a known regulator of cytokinesis, VEGF-regulated angiogenesis, and p53-dependent tumorigenesis. These findings reveal a novel association of germline de novo MYH9 mutation with congenital hemangioma.

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