High prevalence of adrenal insufficiency at diagnosis and headache recovery in surgically resected Rathke’s cleft cysts—a large retrospective single center study
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Rathke’s cleft cysts (RCC) are lesions that arise from Rathke’s pouch. Though frequently incidental, resulting symptoms in a minority of cases are indicators for surgical resection, which may prove beneficial.
To characterize a cohort of surgically-resected RCC cases at Oregon Health & Science University; tabulate associated hormonal imbalances and symptoms, possible symptom reversal with surgery, determine recurrence risk; identify predictors of recurrence and headache improvement.
Electronic records of all RCC resected cases (from 2006–2016; 11 years) were retrospectively reviewed. Patients had been evaluated by one neuroendocrinologist using a uniform protocol.
A pathological RCC diagnosis was established in 73 of 814 (9%) surgical pituitary cases. The RCC cohort was 77% (n = 56/73) female, mean age was 39.5 ± 14.9 years at first surgery, and at presentation headache was reported in 88% and visual defects/diplopia in 18% of patients. Initial RCC maximum diameter was 1.3 ± 0.7 cm. The most frequent hormonal deficit was cortisol; 24% of patients had a new adrenal insufficiency (AI) diagnosis, however, 36% also had AI at 3 months post-operatively. Mean follow up was 4.0 ± 4.5 years. Two-thirds of patients (41/62) had headache improvement 3 months post-operatively. Post-operative imaging revealed no residual cyst in 58% (38/65). In those patients with no residual RCC, 29% had recurrence and 71% had long lasting cure. From the 42% (27/65) of patients with residual cyst on post-operative imaging; 59% (16/27) remained stable, 26% (7/27) progressed and 15% (4/27) regressed.
Symptomatic RCC present mostly in women, with a high proportion reporting headaches. Prevalence of AI at diagnosis is high. Surgery may not achieve adrenal axis recovery, but renders a high percentage of headache improvement. Approximately 25% of RCC will recur by 4 years postoperatively. Clinicians should cautiously screen patients with symptomatic RCC, regardless of lesion size for AI.
KeywordsRathke’s cleft cysts Pituitary cyst Pituitary lesions Adrenal insufficiency Transsphenoidal surgery Headache
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
Informed consent was waived in this institutional approved retrospective study.
- 3.G. Childs, Pituitary Gland (Cell Types, Mediators, Development), in Squire L (ed): Encyclopedia of Neuroscience. California: Academic Press 2009, Vol 7, pp 719–726Google Scholar
- 17.M. Lin, M.A. Wedemeyer, D. Bradley, D.A. Donoho, V.L. Fredrickson, M.H. Weiss, J.D. Carmichael, G. Zada, Long-term surgical outcomes following transsphenoidal surgery in patients with Rathke’s cleft cysts. J. Neurosurg. 1–7, https://doi.org/10.3171/2017.11.JNS171498 (2018)
- 30.J. Schittenhelm, R. Beschorner, T. Psaras, D. Capper, T. Nagele, R. Meyermann, W. Saeger, J. Honegger, M. Mittelbronn, Rathke’s cleft cyst rupture as potential initial event of a secondary perifocal lymphocytic hypophysitis: proposal of an unusual pathogenetic event and review of the literature. Neurosurg. Rev. 31, 157–163 (2008)CrossRefPubMedGoogle Scholar