Cent Eur Neurosurg 2011; 72(4): 211-215
DOI: 10.1055/s-0029-1246137
Case Report
© Georg Thieme Verlag KG Stuttgart · New York

Intracranial Melanotic Schwannoma: A Case Report of Recurrence with Extra- and Intradural Manifestation Two Decades After Initial Diagnosis and Treatment

C. Ditz1 , K. Brunswig1 , C. Meyer1 , E. Reusche2 , G. Nowak1 , V. Tronnier1
  • 1UK-SH, Neurosurgery, Lübeck, Germany
  • 2UK-SH, Neuropathology, Lübeck, Germany
Further Information

Publication History

Publication Date:
22 April 2010 (online)


Melanotic schwannoma (MS) is a very rare nerve sheath tumor characterized by melanin-producing cells that display ultrastructural features of Schwann cells [24]. This type of tumor was first described in 1932 [17] and occurs primarily in the paraspinal region, originating from the spinal nerve roots or sympathetic ganglia. Craniofacial or intracranial locations are rare [15] [21]. However, extraneural locations such as skin, soft tissues, bone and viscera have also been described [5] [7]. In a minority of cases, MS may have multiple nodules [6] [26]. Although MS have been considered to be slow-growing, benign tumors, prognosis can be poor as a result of local recurrence or malignant progression [9]. Melanotic schwannoma occurs in relatively young adults (mean age 35 years) and have no sex predilection. Epidemically, two types of tumors can be distinguished: the sporadic variant and psammomatous melanotic schwannoma associated with the Carney complex, a rare, autosomal dominant hereditary syndrome characterized by variegated skin pigmentation, cardiac myxomas, endocrine overactivity, and nerve sheath tumors [2]. Psammoma bodies are present in more than 50% of MS. Immunohistochemistry reveals that S100, HMB-45 and vimentin are strongly expressed by most cells in MS [15] [18].


  • 1 Abbott Jr AE, Hill RC, Flynn MA. et al . Melanotic schwannoma of the sympathetic ganglia: pathological and clinical characteristics.  Ann Thorac Surg. 1990;  49 1006-1008
  • 2 Carney JA. Carney complex: the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas.  Semin Dermatol. 1995;  14 90-98
  • 3 Carney JA, Gordon H, Carpenter PC. et al . The complex of myxomas, spotty pigmentation, and endocrine overactivity.  Medicine (Baltimore). 1985;  64 270-283
  • 4 Carrasco CA, Rojas-Salazar D, Chiorino R. et al . Melanotic nonpsammomatous trigeminal schwannoma as the first manifestation of Carney complex: case report.  Neurosurgery. 2006;  59 E1334-1335 ; discussion E1335
  • 5 Claessens N, Heymans O, Arrese JE. et al . Cutaneous psammomatous melanotic schwannoma: non-recurrence with surgical excision.  Am J Clin Dermatol. 2003;  4 799-802
  • 6 Culhaci N, Dikicioglu E, Meteoglu I. et al . Multiple melanotic schwannoma.  Ann Diagn Pathol. 2003;  7 254-258
  • 7 Font RL, Truong LD. Melanotic schwannoma of soft tissues. Electron-microscopic observations and review of literature.  Am J Surg Pathol. 1984;  8 129-138
  • 8 Ghosh S, Chandy MJ. Solitary ectopic intracerebral schwannoma.  Br J Neurosurg. 1992;  6 163-166
  • 9 Goasguen O, Boucher E, Pouit B. et al . [Melanotic schwannoma, a tumor with a unpredictable prognosis: case report and review of the literature].  Neurochirurgie. 2003;  49 31-38
  • 10 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
  • 11 Huang CF, Kondziolka D, Flickinger JC. et al . Stereotactic radiosurgery for trigeminal schwannomas.  Neurosurgery. 1999;  45 11-16 ; discussion 16
  • 12 Huang HY, Park N, Erlandson RA. et al . Immunohistochemical and ultrastructural comparative study of external lamina structure in 31 cases of cellular, classical, and melanotic schwannomas.  Appl Immunohistochem Mol Morphol. 2004;  12 50-58
  • 13 Huang PP, Zagzag D, Benjamin V. Intracranial schwannoma presenting as a subfrontal tumor: case report.  Neurosurgery. 1997;  40 194-197
  • 14 Inui T, Morimoto T, Koshimae N. et al . Dura-based giant intracranial schwannoma in the middle fossa.  Neurol Med Chir (Tokyo). 2007;  47 367-370
  • 15 Jensen OA, Bretlau P. Melanotic schwannoma of the orbit. Immunohistochemical and ultrastructural study of a case and survey of the literature.  Apmis. 1990;  98 713-723
  • 16 Marton E, Feletti A, Orvieto E. et al . Dumbbell-shaped C-2 psammomatous melanotic malignant schwannoma. Case report and review of the literature.  J Neurosurg Spine. 2007;  6 591-599
  • 17 Miller W. Malignant melanotic tumor of ganglion cells arising from the thoracic sympathic ganglion.  J Pathol. 1932;  35 351-357
  • 18 Myers JL, Bernreuter W, Dunham W. Melanotic schwannoma. Clinicopathologic, immunohistochemical, and ultrastructural features of a rare primary bone tumor.  Am J Clin Pathol. 1990;  93 424-429
  • 19 Nishioka K, Abo D, Aoyama H. et al . Stereotactic radiotherapy for intracranial nonacoustic schwannomas including facial nerve schwannoma.  IJROBP. 2009;  75 1415-1419
  • 20 Oikawa A, Takeda N, Aoki N. et al . Schwannoma arising from the tentorium at an unusual location: case report.  Neurosurgery. 2002;  50 1352-1355
  • 21 Ranjan A, Chacko G, Chandi SM. Intracerebellar melanotic schwannoma: a case report.  Br J Neurosurg. 1995;  9 687-689
  • 22 Takei H, Schmiege L, Buckleair L. et al . Intracerebral schwannoma clinically and radiologically mimicking meningioma.  Pathol Int. 2005;  55 514-519
  • 23 Tan TC, Ho LC, Chiu HM. et al . Subfrontal schwannoma masquerading as meningioma.  Singapore Med J. 2001;  42 275-277
  • 24 Vallat-Decouvelaere AV, Wassef M, Lot G. et al . Spinal melanotic schwannoma: a tumour with poor prognosis.  Histopathology. 1999;  35 558-566
  • 25 Zabel A, Debus J, Thilmann C. et al . Management of benign cranial nonacoustic schwannomas by fractionated stereotactic radiotherapy.  IJC. 2001;  96 356-362
  • 26 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 1451-1461


Dr. C. Ditz

UK-SH Campus Lübeck Neurosurgery

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23538 Lübeck


Phone: +49/451/500 2076

Fax: + 49/451/500 6191

Email: claudia.ditz@gmx.de