Intracranial Melanotic Schwannomas

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.