A Case of Primary Sellar Neuroblastoma

Abstract

Background The primary intracranial development of neuroblastomas is rare and they generally arise in the supratentorial parenchyma, or the paraventricular region. Neuroblastomas originated in the sellar region are even rarer; only 10 cases have been reported previously. The effective management of these tumors is based on surgery. But adjuvant treatment is not consensual, largely depends on a patient's conditions and aggressiveness of histopathological features. We are reporting a new case of primary sellar neuroblastoma presenting as a sellar mass lesion mimiking a pituitary macroadenoma. Clinical case A 36-year-old woman presented to our hospital with progressive headache without visual disturbance. The sellar MRI showed an intrasellar mass with suprasellar extension and left cavernous sinus invasion. It had a maximum diameter of 2.5cm and wall enhancement with cystic change, which mimicked pituitary macroadenoma. Laboratory findings revealed slightly increased polactin which is related to stalk compression, and the other endocrine evaluation showed no evidence of pituitary hypersecretion. The patient underwent transsphenoidal resection of the mass. Histological examination confirmed the diagnosis of neuroblastoma by numerous primitive blast cells with mature ganglion cells and positive immunohistochemistry stain of chromogranin and synaptophysin. Postoperatively, headache improved and prolactin level dropped to normal values. After the operation and histological confirmation, there was no significant abnormal uptake in 131I-MIBG scan, especially in the brain and abdomen. And then no other adjuvant therapy was done. Conclusion This is the first case of primary sellar neuroblastoma in Korea. Although, the primary sellar neuroblastomas are extremely rare, we should be considered in the differential diagnosis of patients with sellar lesions.