Tumor Neuroectodermal Melanotic

OPM is a disease of unknown origin of a tumor nature with a predominant lesion of the central nervous system, which can occur in the form of glioma, arachnoid cyst, leukodystrophy, osteomyelitis or lethal syndrome. Histologically, it appears as a tumor of small dark brown cells with highly differentiated epithelial cells and melanin. It is quite rare in the world. According to some studies, the incidence of OPM ranges from 1 in 50 thousand to 1.5% of cases. There are no exact data on the prevalence in the population. According to ICD-10, ICD code 10-F43.0.

Causes of the disease

Not exactly established. Possibly develops as a result of malignant transformation of light primary and secondary neurogenic tumors. The causes of the appearance are considered to be a mutation in the S-adenosylmethionine gene: SAMHD1 (S-adenosylhomocysteine ​​deaminase): SAHA1 (succinyl-CoA: 4-hydroxy-3-oxo; dioxygenase protein) and CDKN2A

The manifestation of pathogenesis can be both chromosomal damage and hypoxic conditions arising due to insufficient oxygen in the brain tissues or lack of nutrition of the pituitary gland and