U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Melanotic neurilemmoma

MedGen UID:
266292
•Concept ID:
C1306247
•
Neoplastic Process
Synonyms: Melanocytic Neurilemmoma; melanocytic neurilemmoma; Melanocytic Schwannoma; melanocytic schwannoma; Melanotic Neurilemmoma; melanotic neurilemmoma; Melanotic Neurinoma; melanotic neurinoma; Melanotic Schwannoma; Melanotic schwannoma; melanotic schwannoma; melanotic schwannoma (morphologic abnormality); MSCHW; Pigmented Neurilemmoma; pigmented neurilemmoma; Pigmented schwannoma; Pigmented Schwannoma; pigmented schwannoma
SNOMED CT: Melanotic neurilemmoma (404024000); Melanotic schwannoma (404024000); Melanotic schwannoma (253088003); Melanotic neurilemmoma (253088003); Pigmented schwannoma (253088003)
 
Monarch Initiative: MONDO:0002558

Definition

A rare circumscribed, non-encapsulated and grossly pigmented nerve sheath tumor. It is composed of cells with the immunophenotypic and electron microscopic features of Schwann cells which contain melanosomes and are positive for melanoma markers. It usually involves spinal nerve roots but may occur in other locations. It may be associated with PRKAR1A gene mutation and Carney complex. Malignant behavior has been reported in a significant number of patients. [from NCI]

Recent clinical studies

Etiology

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
Melanotic schwannoma: an 11-year case series.
Khoo M, Pressney I, Hargunani R, Tirabosco R
Skeletal Radiol 2016 Jan;45(1):29-34. Epub 2015 Sep 19 doi: 10.1007/s00256-015-2256-8. PMID: 26386847
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
Melanotic tumors of the nervous system are characterized by distinct mutational, chromosomal and epigenomic profiles.
Koelsche C, Hovestadt V, Jones DT, Capper D, Sturm D, Sahm F, Schrimpf D, Adeberg S, Böhmer K, Hagenlocher C, Mechtersheimer G, Kohlhof P, Mühleisen H, Beschorner R, Hartmann C, Braczynski AK, Mittelbronn M, Buslei R, Becker A, Grote A, Urbach H, Staszewski O, Prinz M, Hewer E, Pfister SM, von Deimling A, Reuss DE
Brain Pathol 2015 Mar;25(2):202-8. Epub 2014 Dec 15 doi: 10.1111/bpa.12228. PMID: 25399693Free PMC Article
Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems.
Rodriguez FJ, Folpe AL, Giannini C, Perry A
Acta Neuropathol 2012 Mar;123(3):295-319. Epub 2012 Feb 12 doi: 10.1007/s00401-012-0954-z. PMID: 22327363Free PMC Article

Diagnosis

Peripheral nerve tumors.
Giannini C, Righi A
Handb Clin Neurol 2024;201:251-271. doi: 10.1016/B978-0-323-90108-6.00016-8. PMID: 38697744
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
What's new in nerve sheath tumors.
Meyer A, Billings SD
Virchows Arch 2020 Jan;476(1):65-80. Epub 2019 Nov 9 doi: 10.1007/s00428-019-02671-0. PMID: 31707590
Pathology of Melanotic Schwannoma.
Alexiev BA, Chou PM, Jennings LJ
Arch Pathol Lab Med 2018 Dec;142(12):1517-1523. Epub 2018 Jan 26 doi: 10.5858/arpa.2017-0162-RA. PMID: 29372846
Carney complex.
Espiard S, Bertherat J
Front Horm Res 2013;41:50-62. Epub 2013 Mar 19 doi: 10.1159/000345669. PMID: 23652670

Therapy

Psammomatous melanotic schwannoma - a rare neck lump.
Morgan RV, Simpson A, Vaghela KR, Quick T
Ann R Coll Surg Engl 2021 Apr;103(4):e124-e126. Epub 2021 Mar 8 doi: 10.1308/rcsann.2020.7083. PMID: 33682474Free PMC Article
18F-FDG PET/CT imaging for aggressive melanotic schwannoma of the L3 spinal root: A case report.
Shen XZ, Wang W, Luo ZY
Medicine (Baltimore) 2021 Feb 26;100(8):e24803. doi: 10.1097/MD.0000000000024803. PMID: 33663098Free PMC Article
Clinical outcomes following resection of giant spinal schwannomas: a case series of 32 patients.
J Neurosurg Spine 2017 Apr;26(4):494-500. Epub 2017 Jan 13 doi: 10.3171/2016.9.SPINE16778. PMID: 28084933
Melanotic schwannoma of the cervical spine progressing with pulmonary metastasis: case report.
Faria MH, Dória-Netto RH, Osugue GJ, Queiroz Lde S, Chaddad-Neto FE
Neurol Med Chir (Tokyo) 2013;53(10):712-6. Epub 2013 Sep 27 doi: 10.2176/nmc.cr2012-0203. PMID: 24077273Free PMC Article
Primary melanocytic tumor of the cerebellopontine angle mimicking a vestibular schwannoma: case report.
Piedra MP, Scheithauer BW, Driscoll CL, Link MJ
Neurosurgery 2006 Jul;59(1):E206; discussion E206. doi: 10.1227/01.NEU.0000219236.48886.44. PMID: 16823290

Prognosis

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
Carney complex.
Espiard S, Bertherat J
Front Horm Res 2013;41:50-62. Epub 2013 Mar 19 doi: 10.1159/000345669. PMID: 23652670
Melanotic schwannoma.
Er U, Kazanci A, Eyriparmak T, Yigitkanli K, Senveli E
J Clin Neurosci 2007 Jul;14(7):676-8. doi: 10.1016/j.jocn.2006.03.010. PMID: 17532504
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
Melanotic schwannoma.
McGavran WL 3rd, Sypert GW, Ballinger WE
Neurosurgery 1978 Jan-Feb;2(1):47-51. doi: 10.1227/00006123-197801000-00010. PMID: 683481

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
Peripheral Nerve Sheath Tumors of Head and Neck: Imaging-Based Review of World Health Organization Classification.
Abdel Razek AAK, Gamaleldin OA, Elsebaie NA
J Comput Assist Tomogr 2020 Nov/Dec;44(6):928-940. doi: 10.1097/RCT.0000000000001109. PMID: 33196600
Malignant melanotic schwannian tumor: a clinicopathologic, immunohistochemical, and gene expression profiling study of 40 cases, with a proposal for the reclassification of "melanotic schwannoma".
Torres-Mora J, Dry S, Li X, Binder S, Amin M, Folpe AL
Am J Surg Pathol 2014 Jan;38(1):94-105. doi: 10.1097/PAS.0b013e3182a0a150. PMID: 24145644
Carney complex.
Espiard S, Bertherat J
Front Horm Res 2013;41:50-62. Epub 2013 Mar 19 doi: 10.1159/000345669. PMID: 23652670
Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems.
Rodriguez FJ, Folpe AL, Giannini C, Perry A
Acta Neuropathol 2012 Mar;123(3):295-319. Epub 2012 Feb 12 doi: 10.1007/s00401-012-0954-z. PMID: 22327363Free PMC Article

Recent systematic reviews

L3 rootlet recurrent melanocytic schwannoma - case report and literature review.
Georgiev GK, Todorov IV, Shamov TP, Krushovenski IV, Fakirova AD, Ilcheva BN, Georgiev IT, Georgiev ND, Prandjev VS, Eftimov TN
Folia Med (Plovdiv) 2021 Jun 30;63(3):448-456. doi: 10.3897/folmed.63.e55295. PMID: 34196157

Supplemental Content

Table of contents

    Clinical resources

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...