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OBJECTIVE: To clarify the outcome of all cases of Rathke's cleft cysts (RCC) treated surgically and followed up in Oxford during a long-term period. SUBJECTS AND METHODS: The records of all patients with RCC seen in the Department of Endocrinology between January 1978 and June 2009 were reviewed. RESULTS: A total of 33 patients (20 females, median age 43 years) were identified. At presentation, major visual field defects were detected in 58% of patients and gonadotrophin, ACTH and TSH deficiency in 60, 36 and 36% of patients respectively. Desmopressin treatment was required in 18% of patients. Treatment consisted of cyst evacuation combined with or without biopsy/removal of the wall. Post-operatively, visual fields improved in 83% of patients with impairment, whereas there was no reversal of ACTH or TSH deficiency or of diabetes insipidus. All but one subject had imaging follow-up during a mean period of 48 months (range 2-267). Cyst relapse was detected in 22% of patients at a mean interval of 29 months (range 3-48 months); in 57% of them, the recurrence was symptomatic. Relapse-free rates were 88% at 24-months and 52% at 48-months follow-up. At last assessment, at least quadrantanopia was reported in 19% of patients, gonadotrophin, ACTH and TSH deficiency in 50, 42 and 47% of patients respectively. Desmopressin treatment was required in 39% of patients. CONCLUSIONS: In this study of patients with RCC and long-term follow-up, we showed a considerable relapse rate necessitating long-term monitoring. Surgical intervention is of major importance for the restoration of visual field defects, but it does not improve endocrine morbidity, which in the long-term affects a substantial number of patients.

Original publication

DOI

10.1530/EJE-11-0142

Type

Journal article

Journal

Eur J Endocrinol

Publication Date

07/2011

Volume

165

Pages

33 - 37

Keywords

Adenoma, Adolescent, Adrenocorticotropic Hormone, Adult, Aged, Aged, 80 and over, Central Nervous System Cysts, Child, Deamino Arginine Vasopressin, Diabetes Insipidus, Female, Follicle Stimulating Hormone, Humans, Luteinizing Hormone, Male, Middle Aged, Neoplasm Recurrence, Local, Pituitary Neoplasms, Thyrotropin, Treatment Outcome, Visual Fields