Malignant perineurioma

MedGen UID:: 220432
•Concept ID:: C1266188
•: Neoplastic Process

Synonyms:	Malignant Perineurioma; malignant perineurioma; Malignant peripheral nerve sheath neoplasm with perineurial differentiation; Malignant Peripheral Nerve Sheath Tumor with Perineurial Differentiation; Malignant peripheral nerve sheath tumor with perineurial differentiation; malignant peripheral nerve sheath tumor with perineurial differentiation; malignant peripheral nerve sheath tumour with perineurial differentiation; MPNST (malignant peripheral nerve sheath tumor) with perineurial differentiation; Perineurial Malignant Peripheral Nerve Sheath Tumor; perineurial malignant peripheral nerve sheath tumor; perineurial malignant peripheral nerve sheath tumour; Perineurioma, malignant; perineurioma, malignant
SNOMED CT:	Perineurioma, malignant (128796000); Malignant peripheral nerve sheath neoplasm with perineurial differentiation (761958009); Malignant peripheral nerve sheath tumor with perineurial differentiation (761958009); Malignant perineurioma (761958009); Malignant peripheral nerve sheath neoplasm with perineurial differentiation (128796000); MPNST (malignant peripheral nerve sheath tumor) with perineurial differentiation (128796000)

Monarch Initiative:	MONDO:0016751
Orphanet:	ORPHA252128

Definition

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. [from ORDO]

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVMalignant perineurioma

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Neoplasm
      - Malignant peripheral nerve sheath tumor
        Malignant perineurioma

Follow this link to review classifications for Malignant perineurioma in Orphanet.

Professional guidelines

PubMed

Revised diagnostic criteria for neurofibromatosis type 1 and Legius syndrome: an international consensus recommendation.

Legius E, Messiaen L, Wolkenstein P, Pancza P, Avery RA, Berman Y, Blakeley J, Babovic-Vuksanovic D, Cunha KS, Ferner R, Fisher MJ, Friedman JM, Gutmann DH, Kehrer-Sawatzki H, Korf BR, Mautner VF, Peltonen S, Rauen KA, Riccardi V, Schorry E, Stemmer-Rachamimov A, Stevenson DA, Tadini G, Ullrich NJ, Viskochil D, Wimmer K, Yohay K; International Consensus Group on Neurofibromatosis Diagnostic Criteria (I-NF-DC), Huson SM, Evans DG, Plotkin SR
Genet Med 2021 Aug;23(8):1506-1513. Epub 2021 May 19 doi: 10.1038/s41436-021-01170-5. PMID: 34012067 Free PMC Article

EANO guideline on the diagnosis and treatment of vestibular schwannoma.

Goldbrunner R, Weller M, Regis J, Lund-Johansen M, Stavrinou P, Reuss D, Evans DG, Lefranc F, Sallabanda K, Falini A, Axon P, Sterkers O, Fariselli L, Wick W, Tonn JC
Neuro Oncol 2020 Jan 11;22(1):31-45. doi: 10.1093/neuonc/noz153. PMID: 31504802 Free PMC Article

The Diagnosis and Management of Neurofibromatosis Type 1.

Ly KI, Blakeley JO
Med Clin North Am 2019 Nov;103(6):1035-1054. doi: 10.1016/j.mcna.2019.07.004. PMID: 31582003

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Recent clinical studies

Etiology

Reverse adipofascial flap after resection of a malignant perineurioma of the forearm.

Takahashi M, Kasai T, Nishisho T, Takai M, Endo H, Hirose T, Sairyo K
Orthopedics 2014 Jul;37(7):e661-4. doi: 10.3928/01477447-20140626-65. PMID: 24992065

See all (1)

Diagnosis

Sporadic Malignant Perineurioma: A Rare Diagnosis Among Malignant Peripheral Nerve Sheath Tumors.

Everson MC, Pendleton C, Jack MM, Smith BW, Carter JM, Spinner RJ
World Neurosurg 2021 May;149:e36-e41. Epub 2021 Feb 27 doi: 10.1016/j.wneu.2021.02.099. PMID: 33647483

Malignant perineurioma (malignant peripheral nerve sheath tumor with perineural differentiation).

Mitchell A, Scheithauer BW, Doyon J, Berthiaume MJ, Isler M
Clin Neuropathol 2012 Nov-Dec;31(6):424-9. doi: 10.5414/NP300423. PMID: 22762889

Evaluation of perineurial differentiation in epithelioid sarcoma.

Smith ME, Awasthi R, O'Shaughnessy S, Fisher C
Histopathology 2005 Dec;47(6):575-81. doi: 10.1111/j.1365-2559.2005.02298.x. PMID: 16324194

Malignant peripheral nerve sheath tumor with perineurial differentiation: "malignant perineurioma".

Rosenberg AS, Langee CL, Stevens GL, Morgan MB
J Cutan Pathol 2002 Jul;29(6):362-7. doi: 10.1034/j.1600-0560.2001.290607.x. PMID: 12135468

Malignant peripheral nerve sheath tumor with perineurial cell differentiation (malignant perineurioma).

Zámecník M, Michal M
Pathol Int 1999 Jan;49(1):69-73. doi: 10.1046/j.1440-1827.1999.00815.x. PMID: 10227727

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Prognosis

Sporadic Malignant Perineurioma: A Rare Diagnosis Among Malignant Peripheral Nerve Sheath Tumors.

Everson MC, Pendleton C, Jack MM, Smith BW, Carter JM, Spinner RJ
World Neurosurg 2021 May;149:e36-e41. Epub 2021 Feb 27 doi: 10.1016/j.wneu.2021.02.099. PMID: 33647483

Low-grade malignant perineurioma of the paravertebral column, transforming into a high-grade malignancy.

Karaki S, Mochida J, Lee YH, Nishimura K, Tsutsumi Y
Pathol Int 1999 Sep;49(9):820-5. doi: 10.1046/j.1440-1827.1999.00948.x. PMID: 10504555

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