melanotic neurilemmoma
Findings
No curated finding names melanotic neurilemmoma yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A rare circumscribed, non-encapsulated and grossly pigmented nerve sheath tumor. It is composed of cells with the immunophenotypic and electron microscopic features of Schwann cells which contain melanosomes and are positive for melanoma markers. It usually involves spinal nerve roots but may occur in other locations. It may be associated with PRKAR1A gene mutation and Carney complex. Malignant behavior has been reported in a significant number of patients.
Definition from the Mondo Disease Ontology (MONDO:0002558), read 2026-09-29. CC BY 4.0.
Where it sits
- A kind of
- Narrower terms (2)
Other names
7 names
Resolves to: melanotic neurilemmoma
- Also called
- melanocytic neurilemmomamelanocytic schwannomamelanotic neurinomamelanotic schwannomamelanotic schwannoma (morphologic abnormality)pigmented neurilemmomapigmented schwannoma