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Adult malignant schwannoma

MedGen UID:
75845
•Concept ID:
C0278622
•
Neoplastic Process
Synonyms: Adult Malignant Neoplasm of Peripheral Nerve Sheath; adult malignant neoplasm of peripheral nerve sheath; Adult Malignant Neoplasm of the Peripheral Nerve Sheath; adult malignant neoplasm of the peripheral nerve sheath; Adult Malignant Neurilemmoma; adult malignant neurilemmoma; Adult Malignant Peripheral Nerve Sheath Neoplasm; adult malignant peripheral nerve sheath neoplasm; Adult Malignant Peripheral Nerve Sheath Tumor; adult malignant peripheral nerve sheath tumor; adult malignant peripheral nerve sheath tumour; Adult Malignant Schwannoma; adult malignant schwannoma; Adult Malignant Tumor of Peripheral Nerve Sheath; adult malignant tumor of peripheral nerve sheath; Adult Malignant Tumor of the Peripheral Nerve Sheath; adult malignant tumor of the peripheral nerve sheath; adult malignant tumour of peripheral nerve sheath; adult malignant tumour of the peripheral nerve sheath; Adult MPNST; adult MPNST
 
Monarch Initiative: MONDO:0004545

Definition

A malignant peripheral nerve sheath tumor occurring during adulthood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdult malignant schwannoma

Professional guidelines

PubMed

The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
Overview of primary adult retroperitoneal tumours.
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
Intradural spinal tumors in adults-update on management and outcome.
Ottenhausen M, Ntoulias G, Bodhinayake I, Ruppert FH, Schreiber S, Förschler A, Boockvar JA, Jödicke A
Neurosurg Rev 2019 Jun;42(2):371-388. Epub 2018 Feb 17 doi: 10.1007/s10143-018-0957-x. PMID: 29455369

Recent clinical studies

Etiology

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356
Surgery of the lateral skull base: a 50-year endeavour.
Zanoletti E, Mazzoni A, Martini A, Abbritti RV, Albertini R, Alexandre E, Baro V, Bartolini S, Bernardeschi D, Bivona R, Bonali M, Borghesi I, Borsetto D, Bovo R, Breun M, Calbucci F, Carlson ML, Caruso A, Cayé-Thomasen P, Cazzador D, Champagne PO, Colangeli R, Conte G, D'Avella D, Danesi G, Deantonio L, Denaro L, Di Berardino F, Draghi R, Ebner FH, Favaretto N, Ferri G, Fioravanti A, Froelich S, Giannuzzi A, Girasoli L, Grossardt BR, Guidi M, Hagen R, Hanakita S, Hardy DG, Iglesias VC, Jefferies S, Jia H, Kalamarides M, Kanaan IN, Krengli M, Landi A, Lauda L, Lepera D, Lieber S, Lloyd SLK, Lovato A, Maccarrone F, Macfarlane R, Magnan J, Magnoni L, Marchioni D, Marinelli JP, Marioni G, Mastronardi V, Matthies C, Moffat DA, Munari S, Nardone M, Pareschi R, Pavone C, Piccirillo E, Piras G, Presutti L, Restivo G, Reznitsky M, Roca E, Russo A, Sanna M, Sartori L, Scheich M, Shehata-Dieler W, Soloperto D, Sorrentino F, Sterkers O, Taibah A, Tatagiba M, Tealdo G, Vlad D, Wu H, Zanetti D
Acta Otorhinolaryngol Ital 2019 Jun;39(SUPPL. 1):S1-S146. doi: 10.14639/0392-100X-suppl.1-39-2019. PMID: 31130732Free PMC Article
Pediatric neurofibrosarcoma.
Neville H, Corpron C, Blakely ML, Andrassy R
J Pediatr Surg 2003 Mar;38(3):343-6; discussion 343-6. doi: 10.1053/jpsu.2003.50105. PMID: 12632346
Plexiform schwannoma.
Megahed M
Am J Dermatopathol 1994 Jun;16(3):288-93. doi: 10.1097/00000372-199406000-00011. PMID: 7943637
Extraskeletal osteosarcoma.
Chung EB, Enzinger FM
Cancer 1987 Sep 1;60(5):1132-42. doi: 10.1002/1097-0142(19870901)60:5<1132::aid-cncr2820600536>3.0.co;2-l. PMID: 3475157

Diagnosis

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
Overview of primary adult retroperitoneal tumours.
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
Intradural spinal tumors in adults-update on management and outcome.
Ottenhausen M, Ntoulias G, Bodhinayake I, Ruppert FH, Schreiber S, Förschler A, Boockvar JA, Jödicke A
Neurosurg Rev 2019 Jun;42(2):371-388. Epub 2018 Feb 17 doi: 10.1007/s10143-018-0957-x. PMID: 29455369
Malignant intracerebellar schwannoma.
Maiuri F, Colella G, D'Acunzi G, De Caro Mdel B
J Neurooncol 2004 Jan;66(1-2):191-5. doi: 10.1023/b:neon.0000013476.26986.7a. PMID: 15015786
Pediatric neurofibrosarcoma.
Neville H, Corpron C, Blakely ML, Andrassy R
J Pediatr Surg 2003 Mar;38(3):343-6; discussion 343-6. doi: 10.1053/jpsu.2003.50105. PMID: 12632346

Therapy

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356
Laparoscopic partial splenectomy.
Uranues S, Grossman D, Ludwig L, Bergamaschi R
Surg Endosc 2007 Jan;21(1):57-60. Epub 2006 Oct 9 doi: 10.1007/s00464-006-0124-2. PMID: 17031738
Posterior mediastinal masses.
Saenz NC, Schnitzer JJ, Eraklis AE, Hendren WH, Grier HE, Macklis RM, Shamberger RC
J Pediatr Surg 1993 Feb;28(2):172-6. doi: 10.1016/s0022-3468(05)80268-4. PMID: 8437074
Postirradiation neurofibrosarcoma.
Ducatman BS, Scheithauer BW
Cancer 1983 Mar 15;51(6):1028-33. doi: 10.1002/1097-0142(19830315)51:6<1028::aid-cncr2820510610>3.0.co;2-3. PMID: 6821867
Neurofibrosarcoma.
Storm FK, Eilber FR, Mirra J, Morton DL
Cancer 1980 Jan 1;45(1):126-9. doi: 10.1002/1097-0142(19800101)45:1<126::aid-cncr2820450122>3.0.co;2-u. PMID: 6766081

Prognosis

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
Intraocular schwannoma.
You JY, Finger PT, Iacob C, McCormick SA, Milman T
Surv Ophthalmol 2013 Jan-Feb;58(1):77-85. Epub 2012 Sep 14 doi: 10.1016/j.survophthal.2012.04.003. PMID: 22981398
Intravascular schwannoma.
Gaudi S, Mills O, Goyette EF, Morgan MB
Am J Dermatopathol 2011 Dec;33(8):850-4. doi: 10.1097/DAD.0b013e3181f5df90. PMID: 22042258
Pediatric neurofibrosarcoma.
Neville H, Corpron C, Blakely ML, Andrassy R
J Pediatr Surg 2003 Mar;38(3):343-6; discussion 343-6. doi: 10.1053/jpsu.2003.50105. PMID: 12632346

Clinical prediction guides

Malignant peripheral nerve sheath tumour of the oral and maxillofacial region-A systematic review.
Schuch LF, Kirschnick LB, de Arruda JAA, Klein IP, Silveira FM, Vasconcelos ACU, Santos-Silva AR, Lopes MA, Carrard VC, Vargas PA, Martins MAT, Wagner VP, Martins MD
Oral Dis 2022 Nov;28(8):2072-2082. Epub 2021 Aug 12 doi: 10.1111/odi.13982. PMID: 34333825
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
Surgery of the lateral skull base: a 50-year endeavour.
Zanoletti E, Mazzoni A, Martini A, Abbritti RV, Albertini R, Alexandre E, Baro V, Bartolini S, Bernardeschi D, Bivona R, Bonali M, Borghesi I, Borsetto D, Bovo R, Breun M, Calbucci F, Carlson ML, Caruso A, Cayé-Thomasen P, Cazzador D, Champagne PO, Colangeli R, Conte G, D'Avella D, Danesi G, Deantonio L, Denaro L, Di Berardino F, Draghi R, Ebner FH, Favaretto N, Ferri G, Fioravanti A, Froelich S, Giannuzzi A, Girasoli L, Grossardt BR, Guidi M, Hagen R, Hanakita S, Hardy DG, Iglesias VC, Jefferies S, Jia H, Kalamarides M, Kanaan IN, Krengli M, Landi A, Lauda L, Lepera D, Lieber S, Lloyd SLK, Lovato A, Maccarrone F, Macfarlane R, Magnan J, Magnoni L, Marchioni D, Marinelli JP, Marioni G, Mastronardi V, Matthies C, Moffat DA, Munari S, Nardone M, Pareschi R, Pavone C, Piccirillo E, Piras G, Presutti L, Restivo G, Reznitsky M, Roca E, Russo A, Sanna M, Sartori L, Scheich M, Shehata-Dieler W, Soloperto D, Sorrentino F, Sterkers O, Taibah A, Tatagiba M, Tealdo G, Vlad D, Wu H, Zanetti D
Acta Otorhinolaryngol Ital 2019 Jun;39(SUPPL. 1):S1-S146. doi: 10.14639/0392-100X-suppl.1-39-2019. PMID: 31130732Free PMC Article
Intravascular schwannoma.
Gaudi S, Mills O, Goyette EF, Morgan MB
Am J Dermatopathol 2011 Dec;33(8):850-4. doi: 10.1097/DAD.0b013e3181f5df90. PMID: 22042258
Postirradiation neurofibrosarcoma.
Ducatman BS, Scheithauer BW
Cancer 1983 Mar 15;51(6):1028-33. doi: 10.1002/1097-0142(19830315)51:6<1028::aid-cncr2820510610>3.0.co;2-3. PMID: 6821867

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
Malignant peripheral nerve sheath tumour of the oral and maxillofacial region-A systematic review.
Schuch LF, Kirschnick LB, de Arruda JAA, Klein IP, Silveira FM, Vasconcelos ACU, Santos-Silva AR, Lopes MA, Carrard VC, Vargas PA, Martins MAT, Wagner VP, Martins MD
Oral Dis 2022 Nov;28(8):2072-2082. Epub 2021 Aug 12 doi: 10.1111/odi.13982. PMID: 34333825
Composite phaeochromocytomas-a systematic review of published literature.
Dhanasekar K, Visakan V, Tahir F, Balasubramanian SP
Langenbecks Arch Surg 2022 Mar;407(2):517-527. Epub 2021 Mar 2 doi: 10.1007/s00423-021-02129-5. PMID: 33651160Free PMC Article
Malignant peripheral nerve sheath tumors of the eighth cranial nerve arising without prior irradiation.
Carlson ML, Jacob JT, Habermann EB, Glasgow AE, Raghunathan A, Link MJ
J Neurosurg 2016 Nov;125(5):1120-1129. Epub 2016 Jan 8 doi: 10.3171/2015.7.JNS151056. PMID: 26745487
Histopathologic classification of parapharyngeal space tumors: a case series and review of the literature.
Locketz GD, Horowitz G, Abu-Ghanem S, Wasserzug O, Abergel A, Yehuda M, Fliss DM
Eur Arch Otorhinolaryngol 2016 Mar;273(3):727-34. Epub 2015 Feb 24 doi: 10.1007/s00405-015-3545-5. PMID: 25708410

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