Ізольована меланотична шваннома
ORPHA:590539· ICD-10 D36.1· Isolated melanotic schwannoma
Визначення(English summary)
A rare nervous system tumor characterized by cells phenotypically representing Schwann cells but containing melanosomes and expressing melanoma markers. The entity occurs as a non-psammomatous (typically affecting spinal nerves) or a psammomatous (also involving nerves of the intestinal tract and heart) variant. About 50% of psammomatous tumors are associated with Carney complex. Slightly over 10% of melanotic schwannomas follow a malignant course.
- Поширеність
- Unknown
- Вік початку
- Adolescent, Adult, Childhood, Elderly