Ectopic schwannoma of the sellar region in a 1-year-old child: A case report and review of literature

Background: Schwannomas are cranial and spinal nerves' sheath tumors accounting for up to 8% of all intracranial neoplasms. Although typical intracranial schwannomas originate from Schwann cells surrounding cranial nerves, ectopic schwannomas are not associated with a known cranial nerve or have an unknown origin. The location of schwannomas may impose clinical challenges. Sellar region schwannomas are rare whether it is ectopic or not. Herein, we report a pediatric case of a 1-year-old female with ectopic, intra-supra sellar with a literature review. We report the first case of juvenile ectopic schwannoma in the sellar region.

Methods: A PubMed Medline database search was performed by the following combined formula of medical subject headings (MESH) terms and keywords: ((sella turcica [MeSH Terms]) OR (sella*[Title/Abstract]) OR (ectopic [Title/Abstract]) AND ((neurilemmoma [MeSH Terms]) OR (schwannoma [Title/Abstract]) OR (neuroma [Title/Abstract]) OR (neurinoma [Title/Abstract])).

Results: Total results of 206 articles were obtained. In exclusion of intraparenchymal and intraventricular schwannomas, only 34 articles remained. Thirty-nine cases were included in 34 articles. According to the reported cases, intrasellar schwannomas are more common in elderly individuals in an average of 49.5 years (range: 19- 79 years). They have a good prognosis and affect males to females equally (20:19).

Conclusion: Ectopic schwannoma sited in the sellar region is rare. It is the first case to be reported in the pediatric age group with a literature review. This lesion should be highlighted and included in the differential diagnosis of sellar mass.