Endocrine Abstracts

Background: Rathke cleft cysts (RCC) are benign, epithelial-lined cystic lesions arising from remnants of Rathke's pouch. Although reported in 13-22% of normal autopsies, they are rarely symptomatic. Their outcome after surgery remains unclear.

Aim: To analyse the clinical/laboratory features and treatment outcome of patients who presented to the Departments of Endocrinology and Neurosurgery with symptomatic RCC (histologically confirmed) between 1977-2003.

Patients and Methods: Ten patients were identified (6 males; median age 36 years, range 9-63). The median follow-up from presentation was 40 months (6-216).

Results: The commonest presenting manifestations were headaches (80%) and visual field defects (70%). The median duration of symptoms was 13 months (4-120). 70% of the tumours were intra- and extrasellar, 20% extrasellar and 10% intrasellar. ACTH deficiency was found in 6/8(75%) patients, TSH deficiency in 6/8(75%), FSH/LH deficiency in 6/9(67%), DI in 2/9(22%) and hyperprolactinaemia in 6/8(75%) (195-3731 mU/L). The pre-operative diagnosis was consistent with craniopharyngioma in 4/7(57%) or pituitary adenoma in 3/7(43%). Drainage of the cyst fluid with biopsy or removal of a larger portion of the cyst wall was performed in all patients (transsphenoidally 80% - by craniotomy 20%). DI was the commonest post-operative complication (67%). In one case a co-existent chromophobe adenoma was identified. Post-operatively there was no reversal of pre-existing hormone deficits. One patient received adjuvant radiotherapy. Two patients (20%) had symptomatic recurrence(s) (one 44 and 128 months and a second 36 months after operation). The peri-operative mortality was 0%. By the end of the follow-up GH deficiency was found in 4/4(100%), ACTH deficiency in 9/10(100%), FSH/LH deficiency in 7/8(88%), TSH deficiency in 8/10(80%) and DI in 6/10(60%).

Conclusions: In this series with one of the longest follow-up periods reported we show that symptomatic RCCs are associated with significant irreversible endocrinopathy. Their recurrence rate after surgery is not negligible necessitating careful follow-up.