Description of Pregnancies and Their Impact on the Craniopharyngioma

Craniopharyngiomas are benign, slow-growing epithelial tumors that develop from the pituitary stem or pituitary gland in the sellar and/or parasellar region. They account for 2-4% of intracranial tumours and 5.6-15% of childhood tumours. The age distribution at diagnosis of these tumors is bimodal, with a first peak of incidence in children aged 5 to 14, and a second peak in adults aged 50 to 74. Despite the benign nature of these lesions, the quality of life of affected patients is most often impaired, due to endocrine (panhypopituitarism, hypothalamic obesity), visual and neuro-intellectual sequelae associated with the tumor itself and/or its treatment, as well as a high rate of local recurrence.

Treatment is mainly surgical, with the aim of removing as much of the tumour as possible and reducing the rate of local recurrence. However, this procedure has a high morbidity and mortality rate, without preventing a significant risk of recurrence (up to 62% at 10 years). Treatment is often complemented by radiotherapy or proton therapy to improve tumor control. Craniopharyngioma patients have an overall mortality rate 3 to 5 times higher than that observed in the general population. Increased morbidity is also observed in relation to hypopituitarism, hypothalamic lesions, visual and neurocognitive deficits, reduced quality of life and the development of cardiovascular risk factors.

The literature describing pregnancy in women with craniopharyngioma and the impact on the tumor is almost non-existent. A case report published in 2002 relates the story of a patient whose craniopharyngioma was discovered following visual disturbances and operated on at the age of 8; she presented with secondary panhypopituitarism, other complications are not mentioned. She became pregnant at the age of 34 after 8 cycles of gonadotropin stimulation. The pregnancy was uneventful and she delivered at term after spontaneous labor a eutrophic daughter weighing 3,450 g. She did not lactate. During pregnancy, it was necessary to increase the doses of L-thyroxine, hydrocortisone and desmopressin, which were resumed at the usual doses 3 weeks after delivery. No mention was made of changes in tumour volume, visual disturbances, neurocognitive disorders or weight gain during pregnancy. The largest study described 6 women, mean age 24, who had had a craniopharyngioma in childhood. Four of them had at least one pituitary deficiency, half of them had had induced pregnancies; there appeared to be no obstetric complications, no description was made of tumor evolution, hormone substitutions, weight.

The aim of this study is to carry out a monocentric study on patients followed in the endocrinology and reproductive medicine department of the Pitié-Salpêtrière hospital.

This department is the constituent center of the CRESCENDO CRMR, which includes craniopharyngiomas, and one of the reference endocrinology departments for craniopharyngiomas in Ile-de-France, so it has the expertise and one of the largest populations of patients with this rare pathology in the region.