Pituicytoma: a rare tumor

Diana Oliveira; Leonor Gomes; Dircea Rodrigues; Francisco Belo; Olinda Rebelo; Sandra Paiva; Carolina Moreno; Daniela Guelho; Ana Margarida Balsa; Nuno Rodrigues; Luis Cardoso; Diana Martins; Francisco Carrilho

doi:10.1530/endoabs.40.P21

Introduction: Pituicytoma is a low-grade glioma of the suprasellar and sellar regions that is rarely described (about 60 cases described in the literature). The clinical, laboratory and neuroradiological findings are not pathognomonic, and therefore definitive diagnosis is only possible after surgery and histopathological study. Total resection is the treatment of choice, since subtotal removal can often lead to recurrence or progression.

Case report: We report the case of a Caucasian male with no significant medical history who develops isolated frontal headache at the age of 48. The use of magnetic resonance imaging (MRI) revealed an expansive sellar and suprasellar lesion, with 14 mm, well-defined limits, bright homogeneous enhancement with gadolinium, associated with a slight deviation of the optic chiasm. Hormonal study was normal. The patient continued to be followed in Neurosurgery consultation and after 4 years presented complaints of gradually worsening visual disturbances, progressively decreased libido and erectile dysfunction. On visual field testing, a bitemporal hemianopia was noted. MRI revealed millimetrical increase of the tumor. He underwent transnasal/transsphenoidal surgery, which resulted in a partial removal due to difficulty in controlling bleeding. The histopathological examination showed a tumor composed of bipolar cells, with immunohistochemical positivity for vimentin and S100 protein, Ki-67 <2% – pituicytoma. The postoperative hormone study revealed panhypopituitarism. The control MRI detected significant residual tumor, so it was decided to propose the patient for radiosurgery treatment.

Conclusions: This pituicytoma case illustrates a diagnosis that, although rare, should not be excluded from the possibilities available before a suprasellar or sellar lesion that presents with certain cardinal radiological features. The persistence of significant residual tumor was due to the bleeding tendency of the lesion during surgery, which is frequent in pituicytomas. Awareness of the possibility of pituicytoma before surgery would be ideal for appropriate treatment planning, with possible preoperative embolization of the tumor.

Endocrine Abstracts

P21