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Malignant melanocytic neoplasm of the peripheral nerve sheath

MedGen UID:
99184
•Concept ID:
C0474847
•
Neoplastic Process
Synonyms: Malignant Melanocytic Neoplasm of Peripheral Nerve Sheath; malignant melanocytic neoplasm of peripheral nerve sheath; Malignant Melanocytic Neoplasm of the Peripheral Nerve Sheath; malignant melanocytic neoplasm of the peripheral nerve sheath; Malignant Melanocytic Peripheral Nerve Sheath Tumor; malignant melanocytic peripheral nerve sheath tumor; Malignant Melanotic Nerve Sheath Tumor; Malignant melanotic neurilemmoma; Malignant Melanotic Peripheral Nerve Sheath Tumor; malignant melanotic peripheral nerve sheath tumor; Malignant Melanotic Schwannian Tumor; Malignant melanotic schwannoma; Melanocytic MPNST; melanocytic MPNST; Melanotic malignant nerve sheath tumor; melanotic malignant nerve sheath tumor; Melanotic malignant peripheral nerve sheath tumor; melanotic malignant peripheral nerve sheath tumor; melanotic malignant peripheral nerve sheath tumor (morphologic abnormality); melanotic malignant peripheral nerve sheath tumour
SNOMED CT: Melanotic malignant nerve sheath tumor (404039004); Malignant melanotic schwannoma (404039004); Malignant melanotic neurilemmoma (404039004); Malignant melanotic schwannoma (253094006); Malignant melanotic neurilemmoma (253094006); Melanotic malignant peripheral nerve sheath tumor (253094006)
 
Monarch Initiative: MONDO:0003863

Definition

A rare variant of malignant peripheral nerve sheath tumor. It is characterized by the presence of malignant cells that contain melanin. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant melanocytic neoplasm of the peripheral nerve sheath

Recent clinical studies

Etiology

Loss of dimethylated H3K27 (H3K27me2) expression is not a specific marker of malignant peripheral nerve sheath tumor (MPNST): An immunohistochemical study of 137 cases, with emphasis on MPNST and melanocytic tumors.
Thangaiah JJ, Westling BE, Roden AC, Giannini C, Tetzlaff M, Cho WC, Folpe AL
Ann Diagn Pathol 2022 Aug;59:151967. Epub 2022 May 6 doi: 10.1016/j.anndiagpath.2022.151967. PMID: 35567887
Risk of Malignant Transformation Arising From Giant Congenital Melanocytic Nevi: A 20-year Single-center Study.
Kim JY, Jeon JH, Choi TH, Kim BJ
Dermatol Surg 2022 Feb 1;48(2):171-175. doi: 10.1097/DSS.0000000000003341. PMID: 34935754
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
A Preclinical Model of Malignant Peripheral Nerve Sheath Tumor-like Melanoma Is Characterized by Infiltrating Mast Cells.
Hölzel M, Landsberg J, Glodde N, Bald T, Rogava M, Riesenberg S, Becker A, Jönsson G, Tüting T
Cancer Res 2016 Jan 15;76(2):251-63. Epub 2015 Oct 28 doi: 10.1158/0008-5472.CAN-15-1090. PMID: 26511633
Intracranial Melanotic Schwannomas.
Spina A, Gagliardi F, Boari N, Terreni MR, Mortini P
J Neurol Surg A Cent Eur Neurosurg 2015 Sep;76(5):399-406. Epub 2015 May 8 doi: 10.1055/s-0034-1393926. PMID: 26302291

Diagnosis

SOX10.
Sy AL, Hoang MP
J Clin Pathol 2023 Oct;76(10):649-653. Epub 2023 Jun 19 doi: 10.1136/jcp-2023-208924. PMID: 37336549
WHO 2016 classification: changes and advancements in the diagnosis of miscellaneous primary CNS tumours.
Sahm F, Reuss DE, Giannini C
Neuropathol Appl Neurobiol 2018 Feb;44(2):163-171. doi: 10.1111/nan.12397. PMID: 28295484
Malignant gastrointestinal neuroectodermal tumor: a case report and review of the literature.
Alyousef MJ, Alratroot JA, ElSharkawy T, Shawarby MA, Al Hamad MA, Hashem TM, Alsayyah A
Diagn Pathol 2017 Mar 20;12(1):29. doi: 10.1186/s13000-017-0620-9. PMID: 28320420Free 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
Intracranial Melanotic Schwannomas.
Spina A, Gagliardi F, Boari N, Terreni MR, Mortini P
J Neurol Surg A Cent Eur Neurosurg 2015 Sep;76(5):399-406. Epub 2015 May 8 doi: 10.1055/s-0034-1393926. PMID: 26302291

Therapy

Reexcision Perineural Invasion and Epithelial Sheath Neuroma Possibly on a Spectrum of Postinjury Reactive Hyperplasia Mediated by IL-6.
Wang JY, Nuovo G, Kline M, Magro CM
Am J Dermatopathol 2017 Jan;39(1):49-52. doi: 10.1097/DAD.0000000000000671. PMID: 27749287
The development of two different malignancies in a patient with large congenital melanocytic nevus.
Ambros T, Furian R, Riccardi F
Pediatr Dermatol 2011 Nov-Dec;28(6):729-731. Epub 2011 Sep 25 doi: 10.1111/j.1525-1470.2011.01398.x. PMID: 21950562

Prognosis

Risk of Malignant Transformation Arising From Giant Congenital Melanocytic Nevi: A 20-year Single-center Study.
Kim JY, Jeon JH, Choi TH, Kim BJ
Dermatol Surg 2022 Feb 1;48(2):171-175. doi: 10.1097/DSS.0000000000003341. PMID: 34935754
Malignant gastrointestinal neuroectodermal tumor: a case report and review of the literature.
Alyousef MJ, Alratroot JA, ElSharkawy T, Shawarby MA, Al Hamad MA, Hashem TM, Alsayyah A
Diagn Pathol 2017 Mar 20;12(1):29. doi: 10.1186/s13000-017-0620-9. PMID: 28320420Free 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
Intracranial Melanotic Schwannomas.
Spina A, Gagliardi F, Boari N, Terreni MR, Mortini P
J Neurol Surg A Cent Eur Neurosurg 2015 Sep;76(5):399-406. Epub 2015 May 8 doi: 10.1055/s-0034-1393926. PMID: 26302291
The pathobiologic spectrum of Schwannomas.
Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM
Histol Histopathol 2003 Jul;18(3):925-34. doi: 10.14670/HH-18.925. PMID: 12792904

Clinical prediction guides

Plexiform Melanocytic Schwannoma: Report of a Second Case and Overview of a Rare Entity.
Ranson M, Lai J, Van Brenk B, McCalmont TH, Walsh NM
Am J Dermatopathol 2022 Dec 1;44(12):943-947. Epub 2022 Nov 2 doi: 10.1097/DAD.0000000000002311. PMID: 36395450
PRAME expression in spindle cell melanoma, malignant peripheral nerve sheath tumour, and other cutaneous sarcomatoid neoplasms: a comparative analysis.
Hrycaj SM, Szczepanski JM, Zhao L, Siddiqui J, Thomas DG, Lucas DR, Patel RM, Harms PW, Bresler SC, Chan MP
Histopathology 2022 Dec;81(6):818-825. Epub 2022 Sep 28 doi: 10.1111/his.14797. PMID: 36102613Free PMC Article
Malignant gastrointestinal neuroectodermal tumor: a case report and review of the literature.
Alyousef MJ, Alratroot JA, ElSharkawy T, Shawarby MA, Al Hamad MA, Hashem TM, Alsayyah A
Diagn Pathol 2017 Mar 20;12(1):29. doi: 10.1186/s13000-017-0620-9. PMID: 28320420Free 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
Diagnostic implications of podoplanin expression in peripheral nerve sheath neoplasms.
Jokinen CH, Dadras SS, Goldblum JR, van de Rijn M, West RB, Rubin BP
Am J Clin Pathol 2008 Jun;129(6):886-93. doi: 10.1309/M7D5KTVYYE51XYQA. PMID: 18480004

Recent systematic reviews

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

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