Intracranial Melanotic Schwannomas

Abstract

Study Aims Melanotic schwannomas (MSs) are an extremely rare variant of nerve sheath tumor. Lesions are characterized by melanin-producing cells that resemble ultrastructural features of Schwann cells. The main location is the paraspinal thoracic region, followed by other extraneural locations such as skin, soft tissues, bone, and viscera. Craniofacial and intracranial lesions are extremely rare. They may occur either sporadically or related to familiar syndromes, such as neurofibromatosis type II and Carney complex, a rare multisystemic autosomal dominant hereditary syndrome. Despite the benign histologic appearance, these tumors can recur or metastasize, even after a long time. We provide an overview of the epidemiological, clinical, radiologic, and histopathologic characteristics of intracranial MSs, with particular emphasis on diagnostic and therapeutic strategies and related clinical outcomes.

Material and Methods We performed a literature review on MSs (1932–2012) regarding intracranial and other localization. An illustrative case is reported.

Results To the best of our knowledge, 17 papers reporting 18 cases of intracranial MSs were previously published. All these studies are either case report or clinical series describing intracranial MSs. Therapeutic results and prognostic factors were reviewed.

Conclusion Radical surgical resection is considered the treatment of choice for MS, but treatment guidelines still do not exist. Radiotherapy seems to play an important role in reducing the risk of recurrence in the case of subtotal tumor resection. Despite the reported encouraging results, only anecdotal data are available in the pertinent literature. Future studies should focus on the role of radiotherapy as adjuvant treatment when radical surgical excision cannot be achieved.

Keywords

melanotic schwannoma - Carney complex - schwannoma - melanocytic tumor

• References

• 1 Zhang HY, Yang GH, Chen HJ , et al. Clinicopathological, immunohistochemical, and ultrastructural study of 13 cases of melanotic schwannoma. Chin Med J (Engl) 2005; 118 (17) 1451-1461
• 2 Höllinger P, Godoy N, Sturzenegger M. Magnetic resonance imaging findings in isolated spinal psammomatous melanotic schwannoma. J Neurol 1999; 246 (11) 1100-1102
• 3 Beck DW, Menezes AH. Lesions in Meckel's cave: variable presentation and pathology. J Neurosurg 1987; 67 (5) 684-689
• 4 Buhl R, Barth H, Hugo HH, Mautner VF, Mehdorn HM. Intracranial and spinal melanotic schwannoma in the same patient. J Neurooncol 2004; 68 (3) 249-254
• 5 Carney JA. Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol 1990; 14 (3) 206-222
• 6 Carrasco CA, Rojas-Salazar D, Chiorino R, Venega JC, Wohllk N. Melanotic nonpsammomatous trigeminal schwannoma as the first manifestation of Carney complex: case report. Neurosurgery 2006; 59 (6) E1334-E1335 ; discussion E1335
• 7 Dastur DK, Sinh G, Pandya SK. Melanotic tumor of the acoustic nerve. J Neurosurg 1967; 27: 166-170
• 8 Díaz Beveridge R, Richart Aznar P, Núñez Lozano C, Chirivella Casanova M, Corbellas Aparicio M, Montalar Salcedo J. Orbital and myocardial metastases of a primary pulmonary melanotic schwannoma. Clin Transl Oncol 2010; 12 (7) 509-511
• 9 Ditz C, Brunswig K, Meyer C, Reusche E, Nowak G, Tronnier V. Intracranial melanotic schwannoma: a case report of recurrence with extra- and intradural manifestation two decades after initial diagnosis and treatment. Cent Eur Neurosurg 2011; 72 (4) 211-214
• 10 Earls JP, Robles HA, McAdams HP, Rao KC. General case of the day. Malignant melanotic schwannoma of the eighth cranial nerve. Radiographics 1994; 14 (6) 1425-1427
• 11 Er U, Kazanci A, Eyriparmak T, Yigitkanli K, Senveli E. Melanotic schwannoma. J Clin Neurosci 2007; 14 (7) 676-678
• 12 Jensen OA, Bretlau P. Melanotic schwannoma of the orbit. Immunohistochemical and ultrastructural study of a case and survey of the literature. APMIS 1990; 98 (8) 713-723
• 13 Mey KH, Buchwald C, Daugaard S, Prause JU. Sinonasal schwannoma—a clinicopathological analysis of five rare cases. Rhinology 2006; 44 (1) 46-52
• 14 Miller RT, Sarikaya H, Sos A. Melanotic schwannoma of the acoustic nerve. Arch Pathol Lab Med 1986; 110 (2) 153-154
• 15 Quencer RM, Stokes NA, Wolfe D, Page LK. Melanotic nerve sheath tumor of the gasserian ganglion and trigeminal nerve. AJR Am J Roentgenol 1979; 133 (1) 142-144
• 16 Ranjan A, Chacko G, Chandi SM. Intracerebellar melanotic schwannoma: a case report. Br J Neurosurg 1995; 9 (5) 687-689
• 17 Saint-Blancard P, Goasguen O, Kossowski M, Dulou R. A rare primary tumor of the cerebellopontine angle: melanotic schwannoma, a pigmented tumor with unpredictable prognosis [in French]. Rev Med Interne 2008; 29 (7) 587-590
• 18 Scheithauer BW, Erdogan S, Rodriguez FJ , et al. Malignant peripheral nerve sheath tumors of cranial nerves and intracranial contents: a clinicopathologic study of 17 cases. Am J Surg Pathol 2009; 33 (3) 325-338
• 19 Killeen RM, Davy CL, Bauserman SC. Melanocytic schwannoma. Cancer 1988; 62 (1) 174-183
• 20 Yokota H, Isobe K, Murakami M, Kubosawa H, Uno T. Dumbbell-shaped nonpsammomatous malignant melanotic schwannoma of the cervical spinal root. Spine J 2012; 12 (4) e14-e17
• 21 Millar WG. A malignant melanotic tumour of ganglion cells arising from a thoracic sympathetic ganglion. J Pathol Bacteriol 1932; 35: 351-357
• 22 Hodson JJ. An intra-osseous tumour combination of biological importance—invasion of a melanotic schwannoma by an adamantinoma. J Pathol Bacteriol 1961; 82: 257-266
• 23 Abbott Jr AE, Hill RC, Flynn MA, McClure S, Murray GF. Melanotic schwannoma of the sympathetic ganglia: pathological and clinical characteristics. Ann Thorac Surg 1990; 49 (6) 1006-1008
• 24 Torres-Mora J, Dry S, Li X, Binder S, Amin M, Folpe AL. 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 2014; 38 (1) 94-105
• 25 Vallat-Decouvelaere AV, Wassef M, Lot G , et al. Spinal melanotic schwannoma: a tumour with poor prognosis. Histopathology 1999; 35 (6) 558-566
• 26 Di Bella C, Declich P, Assi A , et al. Melanotic schwannoma of the sympathetic ganglia: a histologic, immunohistochemical and ultrastructural study. J Neurooncol 1997; 35 (2) 149-152
• 27 Myers JL, Bernreuter W, Dunham W. Melanotic schwannoma. Clinicopathologic, immunohistochemical, and ultrastructural features of a rare primary bone tumor. Am J Clin Pathol 1990; 93 (3) 424-429
• 28 Küsters-Vandevelde HV, van Engen-van Grunsven IA, Küsters B , et al. Improved discrimination of melanotic schwannoma from melanocytic lesions by combined morphological and GNAQ mutational analysis. Acta Neuropathol 2010; 120 (6) 755-764
• 29 Carney JA, Stratakis CA. Epithelioid blue nevus and psammomatous melanotic schwannoma: the unusual pigmented skin tumors of the Carney complex. Semin Diagn Pathol 1998; 15 (3) 216-224
• 30 Hoover JM, Bledsoe JM, Giannini C, Krauss WE. Intramedullary melanotic schwannoma. Rare Tumors 2012; 4 (1) e3
• 31 Stratakis CA, Horvath A. Carney complex. In: Pagon RA, Bird TD, Dolan CR, , et al., eds. GeneReviews [Internet]. Seattle, WA: University of Washington; 1993
• 32 Watson JC, Stratakis CA, Bryant-Greenwood PK , et al. Neurosurgical implications of Carney complex. J Neurosurg 2000; 92 (3) 413-418
• 33 Culhaci N, Dikicioĝlu E, Meteoğlu I, Boylu S. Multiple melanotic schwannoma. Ann Diagn Pathol 2003; 7 (4) 254-258
• 34 Santaguida C, Sabbagh AJ, Guiot MC, Del Maestro RF. Aggressive intramedullary melanotic schwannoma: case report. Neurosurgery 2004; 55 (6) 1430
• 35 Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM. The pathobiologic spectrum of Schwannomas. Histol Histopathol 2003; 18 (3) 925-934
• 36 Shields LB, Glassman SD, Raque GH, Shields CB. Malignant psammomatous melanotic schwannoma of the spine: a component of Carney complex. Surg Neurol Int 2011; 2: 136
• 37 Zonenshayn M, Edgar MA, Lavyne MH. Removal of a lumbar melanotic schwannoma via the far-lateral approach in a patient with Carney complex. Case report. J Neurosurg 2000; 92 (2, Suppl) 241-245
• 38 Agaimy A, Märkl B, Kitz J , et al. Peripheral nerve sheath tumors of the gastrointestinal tract: a multicenter study of 58 patients including NF1-associated gastric schwannoma and unusual morphologic variants. Virchows Arch 2010; 456 (4) 411-422
• 39 Aryanpur J, Long DM. Schwannoma of the medulla oblongata. Case report. J Neurosurg 1988; 69 (3) 446-449
• 40 Celli P, Trillò G, Ferrante L. Spinal extradural schwannoma. J Neurosurg Spine 2005; 2 (4) 447-456
• 41 Claessens N, Heymans O, Arrese JE, Garcia R, Oelbrandt B, Piérard GE. Cutaneous psammomatous melanotic schwannoma: non-recurrence with surgical excision. Am J Clin Dermatol 2003; 4 (11) 799-802
• 42 De Cerchio L, Contratti F, Fraioli MF. Dorsal dumb-bell melanotic schwannoma operated on by posterior and anterior approach: case report and a review of the literature. Eur Spine J 2006; 15 (Suppl. 05) 664-669
• 43 Dhouib M, Briki S, Ben Mahfoudh K , et al. Melanotic schwannoma of the temporozygomatic region [in French]. Rev Stomatol Chir Maxillofac 2007; 108 (2) 139-142
• 44 Erlandson RA. Melanotic schwannoma of spinal nerve origin. Ultrastruct Pathol 1985; 9 (1–2) 123-129
• 45 Goasguen O, Boucher E, Pouit B, Soulard R, Le Charpentier M, Pernot P. Melanotic schwannoma, a tumor with a unpredictable prognosis: case report and review of the literature [in French]. Neurochirurgie 2003; 49 (1) 31-38
• 46 Grätz KW, Makek M, Sailer HF. Malignant melanotic schwannoma of the oral cavity. Int J Oral Maxillofac Surg 1991; 20 (4) 236-238
• 47 Guthikonda M. Melanotic schwannoma of the gasserian ganglion: case report. Skull Base Surg 1998; 8 abstract 14
• 48 Liessi G, Barbazza R, Sartori F, Sabbadin P, Scapinello A. CT and MR imaging of melanocytic schwannomas; report of three cases. Eur J Radiol 1990; 11 (2) 138-142
• 49 Lowman RM, Livolsi VA. Pigmented (melanotic) schwannomas of the spinal canal. Cancer 1980; 46 (2) 391-397
• 50 Mouchaty H, Conti R, Buccoliero AM, Conti P. Intramedullary melanotic schwannoma of the conus medullaris: a case report. Spinal Cord 2008; 46 (10) 703-706
• 51 Peltier J, Page C, Toussaint P, Bruniau A, Desenclos C, Le Gars D. Melanocytic schwannomas. Report of three cases. Neurochirurgie 2005; 51 (3–4 Pt 1) 183-189
• 52 Sola-Pérez J, Pérez-Guillermo M, Bas-Bernal A, Giménez-Bascuñana A, Montes-Clavero C. Melanocytic schwannoma: the cytologic aspect in fine-needle aspiration cytology (FNAC): report of a case located in the spinal cord. Diagn Cytopathol 1994; 11 (3) 291-296
• 53 Tawk RG, Tan D, Mechtler L, Fenstermaker RA. Melanotic schwannoma with drop metastases to the caudal spine and high expression of CD117 (c-kit). J Neurooncol 2005; 71 (2) 151-156
• 54 Welling LC, Guirado VM, Tessari M , et al. Spinal melanotic schwannomas. Arq Neuropsiquiatr 2012; 70 (2) 156-157
• 55 Zhao QH, Zhi S, Wang Z, Tian JW. Psammomatous melanotic schwannoma with cystic changes from old hemorrhages in the cervical spinal canal: a case report. Orthop Surg 2011; 3 (2) 143-146
• 56 Smith AB, Rushing EJ, Smirniotopoulos JG. Pigmented lesions of the central nervous system: radiologic-pathologic correlation. Radiographics 2009; 29 (5) 1503-1524
• 57 Riffaud L, Morandi X, Massengo S, Carsin-Nicol B, Heresbach N, Guegan Y. MRI of intramedullary spinal schwannomas: case report and review of the literature. Neuroradiology 2000; 42 (4) 275-279
• 58 Piedra MP, Scheithauer BW, Driscoll CL, Link MJ. Primary melanocytic tumor of the cerebellopontine angle mimicking a vestibular schwannoma: case report. Neurosurgery 2006; 59 (1) E206 ; discussion E206
• 59 Seifert V. Clinical management of petroclival meningiomas and the eternal quest for preservation of quality of life: personal experiences over a period of 20 years. Acta Neurochir (Wien) 2010; 152 (7) 1099-1116
• 60 Flickinger JC, Burton S. Radiotherapy of cranial nerve schwannomas. In: Régis J, Roche PH, , eds. Modern Management of Acoustic Neuroma. Basel, Switzerland: Karger; 2008: 238-246
• 61 Williams JA. Fractionated stereotactic radiotherapy for acoustic neuromas: preservation of function versus size. J Clin Neurosci 2003; 10 (1) 48-52
• 62 Fuentes S, Arkha Y, Pech-Gourg G , et al. Management of large vestibular schwannomas by combined surgical resection and Gamma Knife radiosurgery. In: Régis J, Roche PH, , eds. Modern Management of Acoustic Neuroma. Basel, Switzerland: Karger; 2008: 79-82
• 63 Wowra B, Tonn JC. Tumors of the cranial nerves. In: Tonn JC, Westphal M, Rutka JT, , et al, eds. Neuro-Oncology of CNS Tumors. Berlin, Germany: Springer-Verlag; 2006: 217-234