Malignant Peripheral Nerve Sheath Tumors (MPNST)

Alternative diagnostic terms
Malignant Schwannoma

Common anatomical locations
Trunk and extremities

Pathology comment
Often arises from a peripheral nerve, from a pre-existing benign nerve sheath tumor, or in a patient with neurofibromatosis type 1 (NF1). In the sporadic setting, diagnosis is most often based on the identification of Schwann cell differentiation (S100/SOX10 focal positivity) and/or loss of H3K27me3 expression in a soft tissue mass.

Source
Antonescu CR, Nielsen GP, Chi P. Malignant peripheral nerve sheath tumor. In: WHO Classification of Tumors Editorial Board. Soft tissue and bone tumors [Internet]. Lyon (France): International Agency for Research on Cancer; 2020 [2024 Dec 29]. (WHO Classification of Tumors Series, 5th ed.; vol. 3). Available from: https://tumorclassification.iarc.who.int/chapters/33

For diagnosis or treatment
Any center listed in the Sarcoma Centers Directory »

Patient advocacy
Any organization with an established program for patient triage, support, and education. See Patient Support Organizations »

Clinical trials
SARC Clinical Trials – None
Find other clinical studies registered on ClinicalTrials.gov

Alternative diagnostic terms
Malignant Melanotic Schwannian Tumor
Melanotic Schwannoma
Psammomatous Melanotic Schwannoma

Common anatomical locations
Spinal or autonomic nerves near the midlineer than upper extremities.

Pathology comment
Variably associated with Carney complex and frequently shows aggressive clinical behavior. PRKAR1A mutations and loss of PRKAR1A protein expression are seen in the overwhelming majority of c

Source
Antonescu CR, Folpe AL, Hameed M. Malignant melanotic nerve sheath tumor. In: WHO Classification of Tumors Editorial Board. Soft tissue and bone tumors [Internet]. Lyon (France): International Agency for Research on Cancer; 2020 [2024 Dec 29]. (WHO Classification of Tumors Series, 5th ed.; vol. 3). Available from: https://tumourclassification.iarc.who.int/login?redirecturl=%2Fchapters%2F33

For diagnosis or treatment
Any center listed in the Sarcoma Centers Directory »

Patient advocacy
Any organization with an established program for patient triage, support, and education. See Patient Support Organizations »

Clinical trials
SARC Clinical Trials – SARC032-SU2C Soft Tissue Sarcoma of the Extremity
Find other clinical studies registered on ClinicalTrials.gov

Alternative diagnostic terms
Schwannoma
Perineurioma
Granular Cell Tumor
Meningothelial Hamartoma
Benign Triton Tumor

Common anatomical locations
Pending

Pathology comment
Pending

Source
Pending

For diagnosis or treatment
Any center listed in the Sarcoma Centers Directory »

Patient advocacy
Any organization with an established program for patient triage, support, and education. See Patient Support Organizations »

Clinical trials
SARC Clinical Trials – None
Find other clinical studies registered on ClinicalTrials.gov