Small Cell Glioblastoma of the Sella Turcica Region: Case Report and Review of the Literature.

BACKGROUND: Glioblastomas in the sellar region are very rare; in most cases, the tumor originates from the optic nerve or optic chiasm. Only 4 cases of sellar glioblastoma with a nonoptic origin have been reported. We present such a case with detailed clinical, imaging, and histopathologic information. We also review similar published cases.

CASE DESCRIPTION: A 42-year-old woman presented with endocrinologic abnormalities, including amenorrhea and lactation, symptoms of diabetes insipidus, and signs of elevated intracranial pressure. Magnetic resonance imaging showed a giant, heterogeneously enhancing lesion involving the intrasellar, parasellar, and suprasellar regions, with hypercellularity and signs of infiltration of adjacent structures. Intraoperative examination revealed the tumor to be independent from the optic pathways, but it showed infiltration of the hypothalamic region. Histopathologic examination demonstrated uniformly packed small cells and negative staining for glial fibrillary acidic protein, which was consistent with a diagnosis of small cell glioblastoma.

CONCLUSIONS: This is the first report of a small cell glioblastoma in the sella turcica region. Glioblastomas in the sellar region with no clear evidence of an optic origin should be viewed as an independent disease entity. The typical characteristics of this tumor indicate it should be considered a rare subtype of glioblastoma. Further accumulation of experience is needed to better differentiate these cases and to offer optimal treatment.