Elsevier

World Neurosurgery

Volume 110, February 2018, Pages 174-179
World Neurosurgery

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

https://doi.org/10.1016/j.wneu.2017.11.038Get rights and content

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.

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

References (35)

  • M. Alireza et al.

    Poor prognosis and challenging treatment of optic nerve malignant gliomas: literature review and case report series

    World Neurosurg

    (2017)
  • R.H. Mattson et al.

    Glioblastoma multiforme of the optic nerve

    JAMA

    (1966)
  • M.J. Taphoorn et al.

    Malignant optic glioma in adults. Case report

    J Neurosurg

    (1989)
  • N.M. Barbaro et al.

    Malignant optic glioma presenting radiologically as a “cystic” suprasellar mass: case report and review of the literature

    Neurosurgery

    (1982)
  • C. Woiciechowsky et al.

    Magnetic resonance imaging of a glioblastoma of the optic chiasm. Case report

    J Neurosurg

    (1995)
  • K.A. Lyapichev et al.

    Glioblastoma multiforme of the optic chiasm: a rare case of common pathology

    Surg Neurol Int

    (2016)
  • R. Brar et al.

    Glioblastoma of the optic pathways: an atypical case

    J Radiol Case Rep

    (2009)
  • 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.

    View full text