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
