Abstract
Intracranial melanotic schwannomas (IMSch) are extremely rare nerve sheath tumors with features of Schwann cells that produce melanin. After a thorough review of the available literature since 1967, we report not only the 20th case of IMSch but a comprehensive modern-era analysis of radiographic and histological key-points to be considered when diagnosing and treating patients with this rare known entity. This is the case of a 43 years-old woman who presented with severe headaches 9 years ago (2008). At that time, MRI of the brain showed a 1.5 × 1.4 cm lesion at the level of the left cerebellar peduncle without any evidence of edema, mass effect or hydrocephalus. Given that the patient was neurologically intact, a conservative management with serial MRIs was recommended. Patient stopped following up due to the absence of symptoms. Over the course of the past year, patient noted mild left sided hearing loss and facial weakness, as well as some balance instability that progressed over the last 3 months. Given the presentation and progression of these signs and symptoms, a new MRI was performed in which considerable growth of the lesion was identified, measuring 2.5 × 2.8 × 2.6 cm with mass effect on the pons and the inferior fourth ventricle. She underwent a far lateral approach without a C1 hemilaminectomy for the resection of this lesion. Final pathology was consistent with a non-psammomatous melanotic schwannoma (NPMS) with areas of necrosis. Besides this case, only two other cases of IMSch with findings of necrosis have been reported in the literature, all of them reporting a subtotal resection. Evaluation of all previously reported cases of IMSch shows a male prevalence with a 1.6:1 male to female ratio. IMSch is radiographically T2 hypointense and can be differentiated from Schwannomas that are classically T2 hyperintense. In this case, only a subtotal resection was feasible due to the tumor’s overwhelming inherent attachment to vital structures such as cranial nerves (CN), brainstem, and vasculature. While MSch is considered histologically benign, several factors including localization, surrounding structures, the rate of growth, tumor volume resection and histological necrosis should be considered in determining prognosis and further adjuvant treatment planning.
Graphical Abstract
Similar content being viewed by others
References
Torres-Mora J, Dry S, Li X, Binder S, Amin M, Folpe AL (2014) Malignant melanotic schwannian tumor: a clinicopathologic, immunohistochemical, and gene expression profiling study of 40 cases, with a proposal for the reclassification of “melanotic schwannoma”. Am J Surg Pathol 38(1):94–105. doi: 10.1097/PAS.0b013e3182a0a150
Millar WG (1932) A malignant melanotic tumour of ganglion cells arising from a thoracic sympathetic ganglion. J Pathol 35(3):351–357
Arvanitis LD (2010) Melanotic schwannoma: a case with strong CD34 expression, with histogenetic implications. Pathol Res Pract 206(10):716–719. doi:10.1016/j.prp.2010.02.011
Burns DK, Silva FG, Forde KA, Mount PM, Clark HB (1983) Primary melanocytic schwannoma of the stomach. Evidence of dual melanocytic and schwannian differentiation in an extra-axial site in a patient without neurofibromatosis. Cancer 52(8):1432–1441
Höllinger P, Godoy N, Sturzenegger M (1999) Magnetic resonance imaging findings in isolated spinal psammomatous melanotic schwannoma. J Neurol 246(11):1100–1102
Marton E, Feletti A, Orvieto E, Longatti P (2007) Dumbbell-shaped C-2 psammomatous melanotic malignant schwannoma. Case report and review of the literature. J Neurosurg Spine 6(6):591–599. doi:10.3171/spi.2007.6.6.14
Myers JL, Bernreuter W, Dunham W (1990) Melanotic schwannoma. Clinicopathologic, immunohistochemical, and ultrastructural features of a rare primary bone tumor. Am J Clin Pathol 93(3):424–429
Shields JA, Font RL, Eagle RC Jr, Shields CL, Gass JD (1994) Melanotic schwannoma of the choroid. Immunohistochemistry and electron microscopic observations. Ophthalmology 101(5):843–849
Spina A, Gagliardi F, Boari N, Terreni MR, Mortini P (2015) Intracranial melanotic schwannomas. J Neurol Surg A 76(5):399–406. doi:10.1055/s-0034-1393926
Er U, Kazanci A, Eyriparmak T, Yigitkanli K, Senveli E (2007) Melanotic schwannoma. J Clin Neurosci 14(7):676–678
Beck DW, Menezes AH (1987) Lesions in Meckel’s cave: variable presentation and pathology. J Neurosurg 67(5):684–689
Buhl R, Barth H, Hugo HH, Mautner VF, Mehdorn HM (2004) Intracranial and spinal melanotic schwannoma in the same patient. J Neurooncol 68(3):249–254
Carney JA (1990) Psammomatous melanotic schwannoma: a distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol 14(3):206–222
Carrasco CA, Rojas-Salazar D, Chiorino R, Venega JC, Wohllk N (2006) Melanotic nonpsammomatous trigeminal schwannoma as the first manifestation of Carney complex: case report. Neurosurgery 59(6):E1334
Dastur DK, Sinh G, Pandya S (1967) Melanotic tumor of the acoustic nerve: case report. J Neurosurg 27(2):166–170
Díaz Beveridge R, Richart Aznar P, Núñez Lozano C, Chirivella Casanova M, Corbellas Aparicio M, Montalar Salcedo J (2010) Orbital and myocardial metastases of a primary pulmonary melanotic schwannoma. Clin Transl Oncol 12(7):509–511
Ditz C, Brunswig K, Meyer C, Reusche E, Nowak G, Tronnier V (2011) Intracranial melanotic schwannoma: a case report of recurrence with extra-and intradural manifestation two decades after initial diagnosis and treatment. Cent Eur Neurosurg 72(04):211–215
Earls JP, Robles HA, McAdams H, Rao K (1994) General case of the day. Malignant melanotic schwannoma of the eighth cranial nerve. Radiographics 14(6):1425–1427
Jensen O, Bretlau P (1990) Melanotic schwannoma of the orbit. Apmis 98(7–12):713–723
Mey K, Buchwald C, Daugaard S, Prause J (2006) Sinonasal schwannoma—a clinicopathological analysis of five rare cases. Rhinology 44(1):46
Miller R, Sarikaya H, Sos A (1986) Melanotic schwannoma of the acoustic nerve. Arch Pathol Lab Med 110(2):153–154
Quencer RM, Stokes NA, Wolfe D, Page LK (1979) Melanotic nerve sheath tumor of the gasserian ganglion and trigeminal nerve. Am J Roentgenol 133(1):142–144
Ranjan A, Chacko G, Chandi S (1995) Intracerebellar melanotic schwannoma: a case report. Br J Neurosurg 9(5):687–690
Saint-Blancard P, Goasguen O, Kossowski M, Dulou R (2008) A rare primary tumor of the cerebellopontine angle: melanotic schwannoma, a pigmented tumor with unpredictable prognosis. La Revue de medecine interne 29(7):587–590
Scheithauer BW, Erdogan S, Rodriguez FJ, Burger PC, Woodruff JM, Kros JM, Gokden M, Spinner RJ (2009) Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases. Am J Surg Pathol 33(3):325–338
Zhang HY, Yang GH, Chen HJ, Wei B, Ke Q, Guo H, Ye L, Bu H, Yang K, Zhang YH (2005) Clinicopathological, immunohistochemical, and ultrastructural study of 13 cases of melanotic schwannoma. Chin Med J 118(17):1451–1461
Culhaci N, Dikicioglu E, Meteoglu I, Boylu S (2003) Multiple melanotic schwannoma. Ann Diagn Pathol 7(4):254–258
Atlas SW, Grossman RI, Gomori JM, Guerry D, Hackney DB, Goldberg HI, Zimmerman RA, Bilaniuk LT (1987) MR imaging of intracranial metastatic melanoma. J Comput Assist Tomogr 11(4):577–582
Tawk RG, Tan D, Mechtler L, Fenstermaker RA (2005) Melanotic schwannoma with drop metastases to the caudal spine and high expression of CD117 (c-kit). J Neurooncol 71(2):151–156. doi:10.1007/s11060-004-9301-0
Vallat-Decouvelaere AV, Wassef M, Lot G, Catala M, Moussalam M, Caruel N, Mikol J (1999) Spinal melanotic schwannoma: a tumour with poor prognosis. Histopathology 35(6):558–566
Benet A, Rincon-Torroella J, Vivas-Buitrago T, Quiñones-Hinojosa A (2016) Far lateral approach. In: Quinones-Hinojosa A (ed) Video Atlas of neurosurgery, contemporary tumor and skull base surgery. Elsevier, New York, pp 137–146
Font RL, Truong LD (1984) Melanotic schwannoma of soft tissues. Electron-microscopic observations and review of literature. Am J Surg Pathol 8(2):129–138
Nitzan E, Pfaltzgraff ER, Labosky PA, Kalcheim C (2013) Neural crest and Schwann cell progenitor-derived melanocytes are two spatially segregated populations similarly regulated by Foxd3. Proc Natl Acad Sci USA 110(31):12709–12714
Thomas AJ, Erickson CA (2009) FOXD3 regulates the lineage switch between neural crest-derived glial cells and pigment cells by repressing MITF through a non-canonical mechanism. Development 136(11):1849–1858. doi:10.1242/dev.031989
Napoli I, Noon LA, Ribeiro S, Kerai AP, Parrinello S, Rosenberg LH, Collins MJ, Harrisingh MC, White IJ, Woodhoo A, Lloyd AC (2012) A central role for the ERK-signaling pathway in controlling schwann cell plasticity and peripheral nerve regeneration in vivo. Neuron 73(4):729–742. doi:10.1016/j.neuron.2011.11.031
Adameyko I, Lallemend F, Aquino JB, Pereira JA, Topilko P, Müller T, Fritz N, Beljajeva A, Mochii M, Liste I, Usoskin D, Suter U, Birchmeier C, Ernfors P (2009) Schwann cell precursors from nerve innervation are a cellular origin of melanocytes in skin. Cell 139(2):366–379. doi:10.1016/j.cell.2009.07.049
Rizvi TA, Huang Y, Sidani A, Atit R, Largaespada DA, Boissy RE, Ratner N (2002) A novel cytokine pathway suppresses glial cell melanogenesis after injury to adult nerve. J Neurosci 22(22):9831–9840
Hoover JM, Bledsoe JM, Giannini C, Krauss WE (2012) Intramedullary melanotic schwannoma. Rare Tumors 4(1):e3. doi:10.4081/rt.2012.e3
Merat R, Szalay-Quinodoz I, Laffitte E, Kaya G (2015) Psammomatous melanotic schwannoma: a challenging histological diagnosis. Dermatopathology 2(3):67–70. doi:10.1159/000442708
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Mahato, D., Vivas-Buitrago, T., Gassie, K. et al. Intracranial melanotic schwannomas: a rare variant with unusual adherent features. J Neurooncol 136, 299–306 (2018). https://doi.org/10.1007/s11060-017-2653-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11060-017-2653-z