Intracranial melanotic schwannoma: a case report of recurrence with extra- and intradural manifestation two decades after initial diagnosis and treatment.

Abstract

Introduction & 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 fi rst 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] .

DOI: 10.1055/s-0029-1246137

4 Figures and Tables

Statistics

020040020132014201520162017
Citations per Year

151 Citations

Semantic Scholar estimates that this publication has 151 citations based on the available data.

See our FAQ for additional information.

Cite this paper

@article{Ditz2011IntracranialMS, title={Intracranial melanotic schwannoma: a case report of recurrence with extra- and intradural manifestation two decades after initial diagnosis and treatment.}, author={Claudia Ditz and Klaus Brunswig and Christian Meyer and Erich Reusche and Georg Nowak and Volker M. Tronnier}, journal={Central European neurosurgery}, year={2011}, volume={72 4}, pages={211-4} }