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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

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant perineurioma
Follow this link to review classifications for Malignant perineurioma in Orphanet.

Professional guidelines

PubMed

Improta L, Tzanis D, Bouhadiba T, Abdelhafidh K, Bonvalot S
Eur J Surg Oncol 2020 Sep;46(9):1573-1579. Epub 2020 May 21 doi: 10.1016/j.ejso.2020.04.054. PMID: 32600897
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: 31504802Free PMC Article
Ly KI, Blakeley JO
Med Clin North Am 2019 Nov;103(6):1035-1054. doi: 10.1016/j.mcna.2019.07.004. PMID: 31582003

Recent clinical studies

Etiology

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

Diagnosis

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
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
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
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
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

Prognosis

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
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

Clinical prediction guides

Sato K, Ueda Y, Miwa S, Yokogawa A, Ozaki M, Katsuda S
Pathol Int 2008 Nov;58(11):718-22. doi: 10.1111/j.1440-1827.2008.02299.x. PMID: 18844938
Suster D, Plaza JA, Shen R
Ann Diagn Pathol 2005 Aug;9(4):197-201. doi: 10.1016/j.anndiagpath.2005.04.016. PMID: 16084452

Supplemental Content

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