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Start free with EleplanMalignant peripheral nerve sheath tumor with perineurial differentiation
ORPHA:252128Histopathological subtype
Also called Malignant perineurioma
What it is
Malignant peripheral nerve sheath tumor with perineurial differentiation is a rare soft tissue sarcoma composed predominantly of spindle-shaped neoplastic cells showing perineurial differentiation and displaying abundant cellular pleomorphism or anaplasia, frequent mitoses, tumor necrosis and high metastatic potential. It often presents as a soft, painless, solid mass in subcutaneous tissues of the trunk or limbs, but tumors have also been described in the facial area, mediastinum, retroperitoneum, pancreas, paravertebral column and the pelvic soft tissues. Frequent local recurrence and distant metastatic spread has been reported.
Key facts
- Classified as
- Histopathological subtype
Recorded for the broader condition
- Prevalence
- 1-9 / 100 000 (annual incidence)Malignant peripheral nerve sheath tumor
- Age of onset
- Adolescent, Adult, Childhood, Elderly, InfancyMalignant peripheral nerve sheath tumor
- Inheritance
- Not applicableMalignant peripheral nerve sheath tumor
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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