Endocrine Abstracts

Introduction: Craniopharyngiomas (CP) are benign tumors that arise from remnants of the Rathke’s pouch. Classically, they are classified as cystic or calcified tumors. The presence of hormonal receptors in CP has been reported in in vitro studies but only eight cases growing during pregnancy have been published in literature.

Case report: We report a 32-year-old woman who was initially diagnosed with a prolactin secreting tumor with rapid enlargement during pregnancy that eventually was found to be an aggressive CP. Pregnancy was achieved with IVF. The patient had a medical history of hyperprolactinemia treated with cabergoline and a pituitary tumor of 9 mm. She consulted at 27 weeks of gestation because of visual impairment. A pituitary MRI showed a cystic pituitary tumor of 22 mm in maximum diameter with suprasellar extension and compression of the optic apparatus. A second 8 mm tumor was described in the floor of the fourth ventricle. Visual assessment had demonstrated no deficit before pregnancy. By the 33rd week of gestation the subject had right lateral homonymous hemianopsia with a decreasing visual acuity that resulted in blindness of the left eye and diabetes insipidus; a caesarean section was performed at that time. The newborn was healthy. Neurosurgery was performed postpartum by a sub-frontal approach. The pathology confirmed an adamantinomatous type CP. The second 8 mm tumor was an ependymoma grade 2.

Immunohistological staining demonstrated epithelial growth factor receptors in 100%, progesterone receptors in 10% and estrogen receptors in 5% of tumor cells. Ki-67 was <1%.

Conclussion: IVF allows pregnancy in infertile population. Only eight cases of CP growing during pregnancy have been reported. We have demonstrated expression of hormonal receptors that may explain the aggressive behaviour of CP during pregnancy. This is the only report with an associated ependymoma.