Background: Craniopharyngiomas are rare, relatively benign, slowly growing intracranial tumors originating in pituitary gland embryonic tissue. They may present at any age, with two peaks of occurrence in children and in older adults. Clinical symptoms are the result of mass effects. Treatment options include surgery, radio- and chemotherapy and unfortunately are connected with a high ratio of postoperative pituitary insufficiency.
Aim: To present the long time observation, treatment modalities and outcomes in patients with childhood/adult onset of craniopharyngioma.
Methods: A retrospective analysis of 17 patients (11M/6W)was performed. The average follow-up time was 11.9 years (S.D. 8.3) with the longest observation lasting 31 years (6/17 patients with childhood onset).
Results: All patients were operated at a mean age (MA) of 28.4 years (S.D. 18.3), with 11/17 (64.7%) operated once and 6/17 (35.3%) requiring at least one re-operation. 8/17 (47.1%) patients underwent subsequent radiotherapy. In most patients the first symptoms were related to the tumor mass. All of the patients (17/17 100.0%) developed secondary hypothyroidism. Gonadal axis insufficiency was diagnosed in 16/17 patients (94.1%), while 82.4% (14/17) of the patients presented with secondary hypocortisolism. One patient (1/17, 5.9%) required transient substitution of glucocorticoids. Diabetes insipidus was found in 12/17 (70.6%) of cases. 16/17 (94.1%) of the patients had multiple hormonal deficiencies; one patient (1/17, 5.9%) was diagnosed with isolated thyroid axis insufficiency.
Conclusions: The diagnosis, management and treatment of craniopharyngiomas remains challenging. The diagnosis is usually delayed. The treatment leads to multiple hormonal deficiencies and a decreased QoL. Long term observations might help better the understanding of the disease and lead to the improvement of its management.
18 - 21 May 2019
European Society of Endocrinology