High-risk pregnancy complicated by craniopharyngioma: diagnosis in the context of visual impairment and tumor resection during pregnancy following IVF.

Abstract

Summary: This report describes the case of a 37-year-old woman diagnosed with a craniopharyngioma during pregnancy. The patient initially presented with visual impairment at 15 weeks of gestation, and MRI revealed a cystic suprasellar tumor. Endocrine evaluation indicated central hypothyroidism, and central adrenal insufficiency could not be definitively ruled out based on a basal 08:00 h cortisol, as the patient was pregnant and treatment with hydrocortisone was initiated empirically. Hydrocortisone 10 mg/day and levothyroxine 25 µg/day were initiated, but rapid visual deterioration and polyuria by 21 weeks necessitated surgical intervention. She underwent successful treatment with endoscopic transsphenoidal surgery during the second trimester. Adrenal function was assessed on postoperative day 7 based on baseline values, but hydrocortisone was maintained given the risk during pregnancy. At 6 weeks after surgery, pituitary hormones were reassessed, and hydrocortisone, levothyroxine, and desmopressin were continued. Arginine vasopressin deficiency was diagnosed based on polyuria, hypotonic urine, and response to desmopressin, as formal testing was high risk both before and after surgery. Postoperatively, endocrine status was monitored, and pregnancy progressed uneventfully. She underwent an elective cesarean section at 38 weeks of gestation. At 6 months postpartum, MRI revealed residual tumor along the pituitary stalk extending from the right optic chiasm. At 9 months postpartum, the patient had persistent central hypothyroidism, hypogonadism, and newly diagnosed adult GH deficiency. Normal adrenal function allowed discontinuation of hydrocortisone. GH therapy was planned pending tumor assessment. This case underscores the importance of a multidisciplinary approach involving obstetrics, neurosurgery, and endocrinology in managing craniopharyngiomas during pregnancy.

Learning points: Regardless of pregnancy planning, central hypogonadism should always be investigated with a brain MRI to determine its etiology, including the potential presence of a craniopharyngioma or other sellar/parasellar lesions. If a craniopharyngioma enlarges during pregnancy and causes visual impairment, surgical intervention can be performed through multidisciplinary collaboration. The mode of delivery in patients with panhypopituitarism should be carefully determined through multidisciplinary consultation between obstetricians and endocrinologists, with consideration of planned cesarean section as a potential option.