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Mediastinal Malignant Peripheral Nerve Sheath Tumor

MedGen UID:
235312
•Concept ID:
C1334667
•
Neoplastic Process
Synonyms: Malignant Mediastinal Peripheral Nerve Sheath Neoplasm; Malignant Mediastinal Peripheral Nerve Sheath Tumor; Malignant Peripheral Nerve Sheath Neoplasm of Mediastinum; Malignant Peripheral Nerve Sheath Neoplasm of the Mediastinum; Malignant Peripheral Nerve Sheath Tumor of Mediastinum; Malignant Peripheral Nerve Sheath Tumor of the Mediastinum; Mediastinal MPNST; MPNST of Mediastinum; MPNST of the Mediastinum

Definition

A malignant peripheral nerve sheath tumor that arises from the mediastinum. It may be associated with neurofibromatosis. It often spreads to the lungs or pleura. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMediastinal Malignant Peripheral Nerve Sheath Tumor

Recent clinical studies

Diagnosis

A giant posterior mediastinal malignant peripheral nerve sheath tumor and benign neurofibroma in body surface: a case report.
Zhang Y, Cai H, Lv G, Li Y
BMC Surg 2021 Mar 10;21(1):128. doi: 10.1186/s12893-021-01122-5. PMID: 33691671Free PMC Article
Successful treatment with doxorubicin and ifosfamide for mediastinal malignant peripheral nerve sheath tumor with loss of H3K27me3 expression.
Seno N, Fukushima T, Gomi D, Kobayashi T, Sekiguchi N, Matsushita H, Ozawa T, Tsukahara Y, Mamiya K, Koizumi T, Sano K
Thorac Cancer 2017 Nov;8(6):720-723. Epub 2017 Sep 6 doi: 10.1111/1759-7714.12498. PMID: 28876532Free PMC Article

Therapy

Successful treatment with doxorubicin and ifosfamide for mediastinal malignant peripheral nerve sheath tumor with loss of H3K27me3 expression.
Seno N, Fukushima T, Gomi D, Kobayashi T, Sekiguchi N, Matsushita H, Ozawa T, Tsukahara Y, Mamiya K, Koizumi T, Sano K
Thorac Cancer 2017 Nov;8(6):720-723. Epub 2017 Sep 6 doi: 10.1111/1759-7714.12498. PMID: 28876532Free PMC Article

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