Pituicitoma in a patient with neurofibromatosis type 1

Pituicytoma is a rare low degree tumor that arises from pituicytes in the infundibulum and posterior pituitary gland. As they do not have characteristic radiological features, they can be misdiagnosed as pituitary adenoma, meningioma, or craniopharyngioma. Clinically, patients can present with hormonal disturbances, headaches and visual field defects. A 46 year old male who presented with visual field defects and symptoms suggestive of panhypopituitarism for the past 3 years, is described. As a personal history, he had neurofibromatosis type 1 and his daughter, with the same disease, who has been operated of astrocytoma. Upon clinical assessment, the patient's ophthalmic examination was bitemporal hemianoptia, and hormonal evaluation confirmed hypopituitarism. Pituitary magnetic resonance imaging showed a suprasellar homogeneous enhancing post-gadolinium mass causing chiasmatic displacement. Transnasal transsphenoidal resection was performed. Postoperatively, the patient developed diabetes insipidus. Histopathology showed patterns consistent with pituicytoma. and immunohistochemistry confirmed it. Total resection remains the goal of treatment.