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Melanotic psammomatous malignant peripheral nerve sheath tumor

MedGen UID:
730167
•Concept ID:
C1321709
•
Neoplastic Process
Synonyms: Malignant Melanotic Psammomatous Peripheral Nerve Sheath Tumor; Melanocytic Psammomatous Malignant Peripheral Nerve Sheath Tumor; melanocytic psammomatous malignant peripheral nerve sheath tumor; melanocytic psammomatous malignant peripheral nerve sheath tumour; Melanocytic Psammomatous MPNST; melanocytic psammomatous MPNST; Melanotic MPNST; Melanotic Psammomatous Malignant Peripheral Nerve Sheath Tumor; melanotic psammomatous malignant peripheral nerve sheath tumor; Melanotic psammomatous MPNST
SNOMED CT: Melanotic psammomatous MPNST (19897006); Melanotic MPNST (19897006)
 
Monarch Initiative: MONDO:0003862

Definition

A malignant peripheral nerve sheath tumor characterized by the presence of malignant cells that contain melanin and formation of psammoma bodies. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMelanotic psammomatous malignant peripheral nerve sheath tumor

Recent clinical studies

Diagnosis

Melanotic schwannoma arising in association with nevus of Ota: 2 cases suggesting a shared mechanism.
Trufant JW, Brenn T, Fletcher CD, Virata AR, Cook DL, Bosenberg MW
Am J Dermatopathol 2009 Dec;31(8):808-13. doi: 10.1097/DAD.0b013e3181accd0e. PMID: 19786855
Is clear cell sarcoma a malignant form of psammomatous melanotic schwannoma? Case report.
Sengöz A, Taşdemiroğlu E, Togay H
Neurosurg Focus 2006 Dec 15;21(6):E11. doi: 10.3171/foc.2006.21.6.3. PMID: 17341045
The pathobiologic spectrum of Schwannomas.
Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM
Histol Histopathol 2003 Jul;18(3):925-34. doi: 10.14670/HH-18.925. PMID: 12792904

Prognosis

Melanotic schwannoma arising in association with nevus of Ota: 2 cases suggesting a shared mechanism.
Trufant JW, Brenn T, Fletcher CD, Virata AR, Cook DL, Bosenberg MW
Am J Dermatopathol 2009 Dec;31(8):808-13. doi: 10.1097/DAD.0b013e3181accd0e. PMID: 19786855
Is clear cell sarcoma a malignant form of psammomatous melanotic schwannoma? Case report.
Sengöz A, Taşdemiroğlu E, Togay H
Neurosurg Focus 2006 Dec 15;21(6):E11. doi: 10.3171/foc.2006.21.6.3. PMID: 17341045
The pathobiologic spectrum of Schwannomas.
Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM
Histol Histopathol 2003 Jul;18(3):925-34. doi: 10.14670/HH-18.925. PMID: 12792904

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