Case ReportSmall Cell Glioblastoma of the Sella Turcica Region: Case Report and Review of the Literature
Introduction
Glioblastoma is the most common primary central nervous system (CNS) neoplasm of neural epithelial origin. Despite decades of research, the prognosis of affected patients remains extremely poor. The frontal, parietal, and temporal lobes are the most commonly affected anatomic locations. The sella turcica is one of the most anatomically complex regions of the CNS, which harbors various structures with diverse histomorphology; pathologic lesions of these diverse tissues often have similar imaging manifestations. Gliomas of the sella turcica region are rarely encountered but may originate from the glial cells of the optic nerve or chiasm, the hypothalamus, or the pituitary or pituitary stalk; in some cases, the origin may not be clearly discernible.1, 2, 3 Most of these gliomas are low-grade tumors; World Health Organization grade IV gliomas are extremely rare.4, 5 In our retrospective case series of 27 sellar gliomas reported over a period of 15 years,6 only 1 case of glioblastoma was recorded; this particular case manifested a rare disease pattern of a small cell glioblastoma with a very high cellular proliferation. In this report, we provide a detailed description of this case, for which the initial differential diagnosis included sellar chordoma, pituitary adenoma, craniopharyngioma, germ cell tumor, lymphoma, glioma, and metastatic lesion based on imaging examination. A review of all reported cases is also provided to further illustrate the features of these cases.
Section snippets
Clinical Presentation
A 42-year-old woman was admitted to our department with a 1-month history of right frontotemporal headache with projectile vomiting and a 3-month history of amenorrhea and lactation. During the course of the disease, the patient experienced progressive worsening of symptoms of diabetes insipidus with a daily urine output of approximately 4000 mL. Physical examination revealed tachycardia (96 beats/minute), thyroid tenderness, decreased visual acuity, bitemporal visual deficit, and signs of
Discussion
Gliomas occurring in the sella turcica region represent a relatively uncommon subgroup of CNS neural epithelial tumors. Most of the tumors in the region are optic and/or chiasmatic gliomas, which constitute approximately 2% of all brain tumors; most of these occur in pediatric patients and are low-grade gliomas.7 Malignant optic gliomas are very rare and typically affect adult patients. Only 46 clearly documented cases are on record, of which 25 were World Health Organization grade IV
Conclusions
Glioblastoma in the sella turcica region is very rare. Optic or optic chiasmatic gliomas account for most malignant gliomas in this region. However, in a very small proportion of these tumors (as illustrated in the present case), a direct origin in the optic pathway is not found. Such tumors are suspected to originate from the hypothalamic-pituitary axis, and these patients typically present with endocrinologic abnormalities. MRI may facilitate the exclusion of optic gliomas in these cases, as
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
Shuanglin Deng and Linlin Liu are co–first authors.