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Perineurioma

MedGen UID:
199712
•Concept ID:
C0751691
•
Neoplastic Process
Synonym: Perineuriomas
SNOMED CT: Perineurioma (404036006)
 
Monarch Initiative: MONDO:0019404
Orphanet: ORPHA85102

Definition

A rare benign tumor composed entirely of neoplastic perineurial cells. It may occur in the soft tissues, intraneurally or in mucosal sites. [from NCI]

Professional guidelines

PubMed

The differential diagnosis between sclerotic fibroma and sclerosing perineurioma: An unresolved challenge.
Saggini A, Baciorri F
J Cutan Pathol 2020 Feb;47(2):195-196. Epub 2019 Dec 9 doi: 10.1111/cup.13620. PMID: 31782178
Intramucosal fat is uncommon in large bowel polyps but raises three differential diagnoses.
Wong NACS, O'Mahony O
J Clin Pathol 2019 Aug;72(8):562-565. Epub 2019 May 16 doi: 10.1136/jclinpath-2019-205849. PMID: 31097491

Recent clinical studies

Etiology

Hybrid schwannoma-perineurioma frequently harbors VGLL3 rearrangement.
Dickson BC, Antonescu CR, Demicco EG, Leong DI, Anderson ND, Swanson D, Zhang L, Fletcher CDM, Hornick JL
Mod Pathol 2021 Jun;34(6):1116-1124. Epub 2021 Mar 1 doi: 10.1038/s41379-021-00783-0. PMID: 33649458Free PMC Article
Diagnostic Pathology of Tumors of Peripheral Nerve.
Belakhoua SM, Rodriguez FJ
Neurosurgery 2021 Feb 16;88(3):443-456. doi: 10.1093/neuros/nyab021. PMID: 33588442Free PMC Article
Extraneural perineurioma: CT and MRI imaging characteristics.
Broski SM, Littrell LA, Howe BM, Folpe AL, Wenger DE
Skeletal Radiol 2020 Jan;49(1):109-114. Epub 2019 Jul 2 doi: 10.1007/s00256-019-03264-3. PMID: 31267178
Intraneural perineurioma: a retrospective study of 19 patients.
Alkhaili J, Cambon-Binder A, Belkheyar Z
Pan Afr Med J 2018;30:275. Epub 2018 Aug 14 doi: 10.11604/pamj.2018.30.275.16072. PMID: 30637060Free PMC Article
The almost-invisible perineurioma.
Restrepo CE, Amrami KK, Howe BM, Dyck PJ, Mauermann ML, Spinner RJ
Neurosurg Focus 2015 Sep;39(3):E13. doi: 10.3171/2015.6.FOCUS15225. PMID: 26323815

Diagnosis

What is new in intraneural perineurioma?
Lenartowicz KA, Smith BW, Jack MM, Wilson TJ, Klein CJ, Amrami KK, Spinner RJ
Acta Neurochir (Wien) 2023 Nov;165(11):3539-3547. Epub 2023 Sep 9 doi: 10.1007/s00701-023-05765-6. PMID: 37684428
Gene fusions in superficial mesenchymal neoplasms: Emerging entities and useful diagnostic adjuncts.
Fischer GM, Papke DJ Jr
Semin Diagn Pathol 2023 Jul;40(4):246-257. Epub 2023 Apr 25 doi: 10.1053/j.semdp.2023.04.014. PMID: 37156707
Diagnostic Pathology of Tumors of Peripheral Nerve.
Belakhoua SM, Rodriguez FJ
Neurosurgery 2021 Feb 16;88(3):443-456. doi: 10.1093/neuros/nyab021. PMID: 33588442Free PMC Article
Aggressive fibromatosis.
Fisher C, Thway K
Pathology 2014 Feb;46(2):135-40. doi: 10.1097/PAT.0000000000000045. PMID: 24378386
Tumors and tumor-like lesions of peripheral nerves.
Woertler K
Semin Musculoskelet Radiol 2010 Nov;14(5):547-58. Epub 2010 Nov 11 doi: 10.1055/s-0030-1268073. PMID: 21072731

Therapy

Sodium Fluorescein as Intraoperative Visualization Tool During Peripheral Nerve Biopsies.
Pedro MT, Eissler A, Scheuerle A, Schmidberger J, Kratzer W, Wirtz CR, Antoniadis G, Koenig RW
World Neurosurg 2020 Jan;133:e513-e521. Epub 2019 Sep 21 doi: 10.1016/j.wneu.2019.09.081. PMID: 31550541
Reconstruction of the Shoulder Joint with a Custom-Made Ceramic Implant After a Total Scapulectomy: A Case Report.
Araki Y, Yoshida A, Tanzawa Y, Endo M, Kobayashi E, Kawai A
JBJS Case Connect 2018 Jan-Mar;8(1):e12. doi: 10.2106/JBJS.CC.17.00061. PMID: 29489523
Fluorescence in situ hybridization analysis is a helpful test for the diagnosis of dermatofibrosarcoma protuberans.
Karanian M, Pérot G, Coindre JM, Chibon F, Pedeutour F, Neuville A
Mod Pathol 2015 Feb;28(2):230-7. Epub 2014 Aug 1 doi: 10.1038/modpathol.2014.97. PMID: 25081750
Myxoid perineurioma in a transplanted kidney.
Gan VH, Wan WK, Tan YH
Transplant Proc 2014 Jan-Feb;46(1):286-9. doi: 10.1016/j.transproceed.2013.09.030. PMID: 24507070
Superficial acral fibromyxoma: a clinicopathologic and immunohistochemical analysis of 37 cases of a distinctive soft tissue tumor with a predilection for the fingers and toes.
Fetsch JF, Laskin WB, Miettinen M
Hum Pathol 2001 Jul;32(7):704-14. doi: 10.1053/hupa.2001.25903. PMID: 11486169

Prognosis

Clinical and Radiological Follow-up of Intraneural Perineuriomas.
Wilson TJ, Amrami KK, Howe BM, Spinner RJ
Neurosurgery 2019 Dec 1;85(6):786-792. doi: 10.1093/neuros/nyy476. PMID: 30481319
The almost-invisible perineurioma.
Restrepo CE, Amrami KK, Howe BM, Dyck PJ, Mauermann ML, Spinner RJ
Neurosurg Focus 2015 Sep;39(3):E13. doi: 10.3171/2015.6.FOCUS15225. PMID: 26323815
MRI in hypertrophic mono- and polyneuropathies.
De Smet K, De Maeseneer M, Talebian Yazdi A, Stadnik T, De Mey J
Clin Radiol 2013 Mar;68(3):317-22. Epub 2012 Sep 7 doi: 10.1016/j.crad.2012.07.014. PMID: 22959851
Perineurioma: a distinctive and underrecognized peripheral nerve sheath neoplasm.
Macarenco RS, Ellinger F, Oliveira AM
Arch Pathol Lab Med 2007 Apr;131(4):625-36. doi: 10.5858/2007-131-625-PADAUP. PMID: 17425397
The WHO classification of tumors of the nervous system.
Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, Cavenee WK
J Neuropathol Exp Neurol 2002 Mar;61(3):215-25; discussion 226-9. doi: 10.1093/jnen/61.3.215. PMID: 11895036

Clinical prediction guides

GAB1::ABL1 fusions define a distinctive soft tissue neoplasm, with variable perineurial differentiation, and a predilection for children and young adults.
Agaimy A, Perret R, Demicco EG, Gross J, Liu YJ, Azmani R, Engelmann C, Schubart C, Seppet J, Stoehr R, Le Loarer F, Dickson BC
Genes Chromosomes Cancer 2023 Aug;62(8):449-459. Epub 2023 Feb 21 doi: 10.1002/gcc.23131. PMID: 36744864
Association of intraneural perineurioma with neurofibromatosis type 2.
Pendleton C, Spinner RJ, Dyck PJB, Mauermann ML, Ladak A, Restrepo CE, Baheti S, Klein CJ
Acta Neurochir (Wien) 2020 Aug;162(8):1891-1897. Epub 2020 Jun 11 doi: 10.1007/s00701-020-04439-x. PMID: 32529330
Soft tissue perineurioma and other unusual tumors in a patient with neurofibromatosis type 1.
Schaefer IM, Ströbel P, Thiha A, Sohns JM, Mühlfeld C, Küffer S, Felmerer G, Stepniewski A, Pauli S, Agaimy A
Int J Clin Exp Pathol 2013;6(12):3003-8. Epub 2013 Nov 15 PMID: 24294391Free PMC Article
Peripheral tumor and tumor-like neurogenic lesions.
Abreu E, Aubert S, Wavreille G, Gheno R, Canella C, Cotten A
Eur J Radiol 2013 Jan;82(1):38-50. Epub 2011 May 10 doi: 10.1016/j.ejrad.2011.04.036. PMID: 21561733
The WHO classification of tumors of the nervous system.
Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, Cavenee WK
J Neuropathol Exp Neurol 2002 Mar;61(3):215-25; discussion 226-9. doi: 10.1093/jnen/61.3.215. PMID: 11895036

Recent systematic reviews

Hybrid tumors with perineurioma components: a systematic review of the literature and illustrative case.
Lenartowicz KA, Monie DD, Amrami KK, Klein CJ, Giannini C, Spinner RJ
Acta Neurochir (Wien) 2023 Apr;165(4):935-945. Epub 2022 Nov 18 doi: 10.1007/s00701-022-05413-5. PMID: 36396843Free PMC Article
Clinical Features, Natural History, and Outcomes of Intraneural Perineuriomas: A Systematic Review of the Literature.
Lenartowicz KA, Goyal A, Mauermann ML, Wilson TJ, Spinner RJ
World Neurosurg 2021 Oct;154:120-131.e8. Epub 2021 Jul 17 doi: 10.1016/j.wneu.2021.07.042. PMID: 34284162
Modern treatment of perineuriomas: a case-series and systematic review.
Uerschels AK, Krogias C, Junker A, Sure U, Wrede KH, Gembruch O
BMC Neurol 2020 Feb 13;20(1):55. doi: 10.1186/s12883-020-01637-z. PMID: 32054523Free PMC Article
Intraneural perineurioma: a systematic review with illustrative cases.
Boyanton BL Jr, Jones JK, Shenaq SM, Hicks MJ, Bhattacharjee MB
Arch Pathol Lab Med 2007 Sep;131(9):1382-92. doi: 10.5858/2007-131-1382-IPASRW. PMID: 17824794

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