Long-term outcomes of adult-onset craniopharyngioma

July 29, 2023

Though benign by histopathology, craniopharyngiomas can carry high morbidity due to tumor proximity to the pituitary gland, hypothalamus, third ventricle, vision and eye movement nerves, and blood vessels. Therapies, including surgery, radiation and, more recently, targeted medical therapy, are effective, but development of hypopituitarism and even hypothalamic syndrome is possible.

Craniopharyngiomas typically occur in teenagers, but they also occur in people in their 50s. However, little has been published on the factors that predict postoperative weight changes in adults until now. Prerna Dogra, M.B.B.S., an endocrinology fellow at Mayo Clinic in Rochester, Minnesota, says, "We analyzed data in 91 patients with adult-onset craniopharyngioma with initial treatment and greater than six months of follow-up at Mayo Clinic. Of these patients, 44% were women, and the mean age at diagnosis was 48.2 ± 18 years."

At presentation, 16% had secondary adrenal insufficiency, 23% had secondary hypothyroidism, 47% had secondary hypogonadism and 29% were growth hormone deficient. Thirteen percent had three or more deficiencies. The mean weight and BMI at presentation were 87.5 ± 22.6 kg and 29.4 ± 5.8 kg/m2, respectively. By BMI criteria, 41% patients were classified as obese, 39% were overweight and 20% were of healthy weight. No sex differences were observed. Study findings were published in a 2022 issue of Endocrine.

Jamie J. Van Gompel, M.D., a neurosurgeon at Mayo Clinic in Rochester, Minnesota, notes: "As expected, the majority (74 patients) had an adamantinomatous tumor subtype, and the mean maximum tumor diameter was 2.6 ± 1.0 cm. Initial surgical procedure entailed gross total resection in 44 patients (49%), partial resection in 45 patients (50%) and P32 injection in one patient. Tumor recurrence occurred in 26% of patients. Radiotherapy was administered to 47% patients either as adjuvant treatment following surgery or as primary treatment for recurrent disease."

Dana Erickson, M.D., an endocrinologist at Mayo Clinic in Rochester, Minnesota, says, "During a mean follow-up of 100.3 ± 69.5 months, 72% patients had three or more anterior pituitary hormone deficiencies and 57 patients (63%) had diabetes insipidus (DI)."

Weight measurement at last follow-up was significantly higher than that before surgery (mean difference 9.5 ± 14.8 kg), with a higher mean percentage increase in weight seen in those with lower preoperative BMI: healthy weight (20.7 ± 18%) versus overweight (13.3 ± 18.0%) versus obese (6.4 ± 15%). At the last follow-up, the prevalence of obesity increased significantly (62% versus 40.5%). Weight gain of 5% or more occurred in 55 patients (65%), with a mean increase of 17.6 ± 10.6 kg. Patients with DI at last follow-up had a higher BMI compared with those without DI (33 ± 6.6 versus 30 ± 6.2 kg/m2).

Dr. Dogra continues: "The mean total weight gain was not associated with sex, preoperative BMI category, tumor size, histologic subtype, gross total resection, postoperative grade of hypothalamic injury, radiotherapy use, or type or number of pituitary hormone deficiencies."

Dr. Erickson says, "What is concerning is the observation that all-cause mortality was 12%, with the average age of death 71.9 years compared with the average U.S. life expectancy of 77.7 years, according to the Centers for Disease Control and Prevention in 2020. But there were no documented deaths secondary to tumor progression. Clinicians should proactively and aggressively address metabolic issues, including weight gain and cardiovascular risk factors, and treat hypopituitarism in these patients."

Future studies addressing possible oxytocin deficiency and molecular targets for hypothalamic obesity might offer new options to further improve outcomes for these patients.

For more information

Dogra P, et al. Long-term outcomes in patients with adult-onset craniopharyngioma. Endocrine. 2022;78:123.

Refer a patient to Mayo Clinic.