Extraventricular neurocytoma of the sellar region: case report and literature review

INTRODUCTION: The extraventricular neurocytoma of the sellar region (EVNSR) is a rare disease, it is difficult to make exact diagnosis of and operate on patients. Retrospectively analysed the clinical manifestations, image features, therapy methods and outcomes among patients with EVNSR, to investigate the epidemiological characteristics, image features, diagnosis, treatment and prognosis. CASE DESCRIPTION: A 25-year-old man man with 7-month worsening vision of left eye, was confirmed EVNSR after subtotally resection from the neurosurgical department of Deji hospital. DISCUSSION AND EVALUATION: Nine cases of EVNSR were reported from this article and elsewhere. Ages of these patients were ranging from 25 to 66 (with an average of 45.67). The male–female ratio was 1–2. All EVNSR patients had visual damage. Images showed the tumors were in the sellar and suprasellar regions. Preoperatively, all patients were misdiagnosed as other diseases: such as pituitary tumor, craniopharyngioma, and meningioma. For tumor removal treatment, five patients received transpterional approach, one received subfrontal approach and three received transnostril–transsphenoidal approach. EVNSR was confirmed by pathological tests. The tumor was completely removed in one patient. During the 12–24 month postoperative follow up period, the recurrence or metastasis of the tumor was found in two patients. CONCLUSIONS: EVNSR is a rare disease. It occurs mostly in middle-aged women. EVNSR is likely to be misdiagnosed as pituitary adenoma preoperatively. The histological examination would help confirm the diagnosis. Using transpterional approach to remove tumor will help the prognosis, especially among patients with normal pituitary function. Both postoperative radiotherapy and long-term follow-up are recommended.