Sitovskaya D A, Verbitsky O P, Petrova Yu A, Sokolova T V, Zabrodskaya Yu M
Prof. A.L. Polenov Russian Research Institute of Neurosurgery - Branch V.A. Almazov National Medical Research Center of the Ministry of Health of Russia, St. Petersburg, Russia.
City Mariinskaya Hospital, St. Petersburg, Russia.
Arkh Patol. 2022;84(1):27-32. doi: 10.17116/patol20228401127.
Primary melanocytic tumors of the central nervous system (CNS) are extremely rare and account for only 1% of all melanomas and 0.05% of primary brain tumors. In case of diffusely invasive lesions of the pia mater with a tumor of melanocytic origin (without signs of extracranial metastases), the tumors are classified as primary diffuse meningeal melanomatosis (PDMM). The latter is an extremely rare subtype of CNS malignant tumor with an incidence rate of 1 in 20 million people. Despite the development of neuroimaging techniques, today a morphological examination remains the main and most accurate method for verifying CNS melanocytic tumors. The paper describes a fatal case of PMMD manifested as epileptic syndrome, with a rapidly progressive course.
中枢神经系统(CNS)原发性黑素细胞肿瘤极为罕见,仅占所有黑色素瘤的1%和原发性脑肿瘤的0.05%。如果软脑膜出现弥漫性浸润性病变且伴有黑素细胞起源的肿瘤(无颅外转移迹象),则这些肿瘤被归类为原发性弥漫性脑膜黑素瘤病(PDMM)。后者是一种极为罕见的中枢神经系统恶性肿瘤亚型,发病率为两千分之一。尽管神经影像学技术有所发展,但如今形态学检查仍然是确诊中枢神经系统黑素细胞肿瘤的主要且最准确的方法。本文描述了一例表现为癫痫综合征、病程快速进展的致命性PMMD病例。