Craniopharyngioma : A Review of 14 Cases at Siriraj Hospital

Abstract

Objective: 1) To characterize clinical presentations of craniophryngioma in the hypothalamic- pituitary region 2) To examine both pre-treatment and post-treatment endocrine dysfunction of patients with craniopharyngioma.
Methods: Medical records of patients with craniopharyngiomas from the Endocrine Division, Department of Medicine from 1997 to 2005 (9 years) were retrospectively reviewed. Data were expressed as mean ± SD (range) or percent as appropriate.
Results: There were 14 cases (7 males and 7 females) with histological-proven diagnosis of craniopharyngioma. Their mean age was 32.3 ± 13.0 years (15-56). The major distinguishing feature leading to diagnosis was visual field defects (85.7%), and a quarter of them were blind. Other distinguishing symptoms were headaches (78.6%), and menstrual disorders (28.6%). Pre-operative evaluations of their pituitary function revealed hypogonadotropic hypogonadism (55.6%), diabetes insipidus (DI) (42.9%), secondary adrenal insufficiency (18%) and elevated prolactin levels (36.4%) with a mean level of 36.1 ± 39.2 ng/ml (12-138). All tumors were confirmed by pituitary CT or MRI. Mean tumor size was 3.83 ± 1.3 cm. (2-6), and hydrocephalus was found in 54.5% of all patients. Craniotomy was performed in 13 cases with one transphenoidal surgery. Post-operative endocrine evaluations revealed more frequent hypogonadotropic hypogonadism (90%), both secondary adrenal insufficiency and diabetes insipidus (85.7%). 50% of DI cases were permanent DI. Secondary hypothyroidism was found post-operatively (78%). All cases of elevated serum prolactin levels became normal post-operatively and 44% of all patients had visual field improvement within a follow up period of 2.1 ± 2.3 years (0-7).
Conclusion: Craniopharyngioma is associated with high morbidity pre and post-operation. Visual field tests should be carefully evaluated in all patients with headaches to make early diagnosis in order to improve the treatment outcome.