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Plexiform schwannoma

MedGen UID:
277972
•Concept ID:
C1370659
•
Neoplastic Process
Synonyms: Plexiform neurilemmoma; Plexiform Neurilemmoma; plexiform neurilemmoma; Plexiform Neurinoma; plexiform neurinoma; Plexiform Schwannoma; plexiform schwannoma; plexiform schwannoma (morphologic abnormality)
SNOMED CT: Plexiform neurilemmoma (404025004); Plexiform neurilemmoma (253087008); Plexiform schwannoma (253087008); Plexiform schwannoma (404025004)
 
Monarch Initiative: MONDO:0002559

Definition

A schwannoma characterized by a plexiform or multinodular growth pattern. It usually arises from the skin or subcutaneous tissues in the extremities, trunk, and head and neck. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPlexiform schwannoma

Professional guidelines

PubMed

Neurofibromatosis- and schwannomatosis-associated tumors: Approaches to genetic testing and counseling considerations.
Goetsch Weisman A, Weiss McQuaid S, Radtke HB, Stoll J, Brown B, Gomes A
Am J Med Genet A 2023 Oct;191(10):2467-2481. Epub 2023 Jul 24 doi: 10.1002/ajmg.a.63346. PMID: 37485904
Surgical management of peripheral nerve sheath tumours in children, with special consideration of neurofibromatoses.
Zipfel J, Al-Hariri M, Gugel I, Haas-Lude K, Grimm A, Warmann S, Krimmel M, Mautner VF, Tatagiba M, Schuhmann MU
Childs Nerv Syst 2020 Oct;36(10):2433-2442. Epub 2020 Jun 6 doi: 10.1007/s00381-020-04703-6. PMID: 32506255Free 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

Recent clinical studies

Etiology

Giant intradural plexiform schwannoma of the lumbosacral spine - a case report and literature review.
Yu D, Choi JH, Jeon I
BMC Musculoskelet Disord 2020 Jul 11;21(1):454. doi: 10.1186/s12891-020-03492-y. PMID: 32652976Free PMC Article
Plexiform schwannoma of the foot: a review of the literature and case report.
Jacobson JM, Felder JM 3rd, Pedroso F, Steinberg JS
J Foot Ankle Surg 2011 Jan-Feb;50(1):68-73. Epub 2010 Sep 15 doi: 10.1053/j.jfas.2010.07.002. PMID: 20833568
Plexiform schwannoma: a clinicopathologic overview with emphasis on the head and neck region.
Berg JC, Scheithauer BW, Spinner RJ, Allen CM, Koutlas IG
Hum Pathol 2008 May;39(5):633-40. doi: 10.1016/j.humpath.2007.10.029. PMID: 18439936
Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative analysis with the superficial variety.
Agaram NP, Prakash S, Antonescu CR
Am J Surg Pathol 2005 Aug;29(8):1042-8. PMID: 16006798
Plexiform schwannoma.
Megahed M
Am J Dermatopathol 1994 Jun;16(3):288-93. doi: 10.1097/00000372-199406000-00011. PMID: 7943637

Diagnosis

Pelvic Plexiform Schwannoma on 18F-FDG PET/MRI.
Zeng Y, Luo J, Nie J, Leng X
Clin Nucl Med 2022 Jan 1;47(1):63-65. doi: 10.1097/RLU.0000000000003684. PMID: 34874350
Plexiform Schwannoma of the Oral Cavity: Report of Eight Cases and a Review of the Literature.
Chi AC, Neville BW, Cheng L
Head Neck Pathol 2021 Mar;15(1):288-297. Epub 2020 Apr 8 doi: 10.1007/s12105-020-01159-7. PMID: 32270393Free PMC Article
An update on the CNS manifestations of neurofibromatosis type 2.
Coy S, Rashid R, Stemmer-Rachamimov A, Santagata S
Acta Neuropathol 2020 Apr;139(4):643-665. Epub 2019 Jun 4 doi: 10.1007/s00401-019-02029-5. PMID: 31161239Free PMC Article
Plexiform schwannoma.
Terasaki K, Mera Y, Uchimiya H, Katahira Y, Kanzaki T
Clin Exp Dermatol 2003 Jul;28(4):372-4. doi: 10.1046/j.1365-2230.2003.01286.x. PMID: 12823295
Plexiform schwannoma.
Megahed M
Am J Dermatopathol 1994 Jun;16(3):288-93. doi: 10.1097/00000372-199406000-00011. PMID: 7943637

Therapy

Gastric plexiform schwannoma in association with neurofibromatosis type 2.
Kudose S, Kyriakos M, Awad MM
Clin J Gastroenterol 2016 Dec;9(6):352-357. Epub 2016 Sep 30 doi: 10.1007/s12328-016-0687-y. PMID: 27696205
Plexiform schwannoma: a clinicopathologic overview with emphasis on the head and neck region.
Berg JC, Scheithauer BW, Spinner RJ, Allen CM, Koutlas IG
Hum Pathol 2008 May;39(5):633-40. doi: 10.1016/j.humpath.2007.10.029. PMID: 18439936

Prognosis

Challenging Management of Plexiform Schwannoma and Plexiform Neurofibroma.
Nicoli TK, Saat R, Tarkkanen J, Kinnunen I, Mäkitie AA, Jero J
J Craniofac Surg 2022 May 1;33(3):803-808. Epub 2021 Dec 2 doi: 10.1097/SCS.0000000000008381. PMID: 34855632
Giant intradural plexiform schwannoma of the lumbosacral spine - a case report and literature review.
Yu D, Choi JH, Jeon I
BMC Musculoskelet Disord 2020 Jul 11;21(1):454. doi: 10.1186/s12891-020-03492-y. PMID: 32652976Free PMC Article
An update on the CNS manifestations of neurofibromatosis type 2.
Coy S, Rashid R, Stemmer-Rachamimov A, Santagata S
Acta Neuropathol 2020 Apr;139(4):643-665. Epub 2019 Jun 4 doi: 10.1007/s00401-019-02029-5. PMID: 31161239Free PMC Article
Diagnostic and Prognostic Relevance of the Cutaneous Manifestations of Neurofibromatosis Type 2.
Plana-Pla A, Bielsa-Marsol I, Carrato-Moñino C; en representación del grupo de Centros, Servicios y Unidades de Referencia (CSUR) en Factomatosis
Actas Dermosifiliogr 2017 Sep;108(7):630-636. Epub 2017 Feb 22 doi: 10.1016/j.ad.2016.12.007. PMID: 28237041
Plexiform schwannoma of the foot: a review of the literature and case report.
Jacobson JM, Felder JM 3rd, Pedroso F, Steinberg JS
J Foot Ankle Surg 2011 Jan-Feb;50(1):68-73. Epub 2010 Sep 15 doi: 10.1053/j.jfas.2010.07.002. PMID: 20833568

Clinical prediction guides

Plexiform Schwannoma of Brachial Plexus in Axilla: A Rare Case Report.
Xiao Y, Feng SQ, Luo T, Zan YS, Li L, Shen LQ
J Musculoskelet Neuronal Interact 2023 Sep 1;23(3):371-376. PMID: 37654224Free PMC Article
Skin lesions in neurofibromatosis type 2: diagnostic and prognostic significance of cutaneous (plexiform) schwannomas.
Plana-Pla A, García B, Munera-Campos M, Catasus N, Serra Arenas E, Blanco I, Castellanos Perez E, Bielsa I; NF2 Multidisciplinary Clinics HUGTiP-ICO-IGTP
J Eur Acad Dermatol Venereol 2022 Sep;36(9):1632-1640. Epub 2022 May 21 doi: 10.1111/jdv.18190. PMID: 35490384Free PMC Article
Giant intradural plexiform schwannoma of the lumbosacral spine - a case report and literature review.
Yu D, Choi JH, Jeon I
BMC Musculoskelet Disord 2020 Jul 11;21(1):454. doi: 10.1186/s12891-020-03492-y. PMID: 32652976Free PMC Article
Low-fat, plexiform spindle cell lipoma of the lip expressing S100 protein: a neural tumor simulator.
Val-Bernal JF, Cagigal ML, Mazorra R
Rom J Morphol Embryol 2018;59(1):385-390. PMID: 29940654
Plexiform schwannoma.
Megahed M
Am J Dermatopathol 1994 Jun;16(3):288-93. doi: 10.1097/00000372-199406000-00011. PMID: 7943637

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