Abstract
Background
Symptomatic Rathke’s cleft cyst is usually accompanied by a long history of headache, visual disturbance, and hypopituitarism; however, rare cases present with acute onset and the clinical features in such cases remain uncertain. We report herein the clinical features of Rathke’s cleft cyst with acute onset and discuss the clinical significance.
Method
In this study, we defined acute onset as the clinical course with clinical symptoms within a 7-day history. From among 35 cases of symptomatic Rathke’s cleft cyst that were pathologically diagnosed at Fukuoka University Hospital between 1990 and 2009, five cases presented with acute onset. The symptoms, endocrinological findings, MR image findings, and pathological findings of these cases were analyzed retrospectively.
Findings
Mean age was 56.8 years. Initial symptoms included headache (n = 3), general malaise (n = 2), polyuria (n = 2), and fever (n = 1). MR imaging revealed an intrasellar cystic lesion with suprasellar extension in all cases and showed rim enhancement in three cases. All cases were treated by transsphenoidal surgery. Pathological findings included hemorrhage (n = 2), hypophysitis (n = 2), and abscess formation in the cyst (n = 1). Postoperatively, all symptoms, except for hypopituitarism, improved in all cases.
Conclusions
Rathke’s cleft cysts sometimes present with acute onset, and the presentation is consistent with the features of pituitary apoplexy caused by pituitary adenoma. Although pituitary apoplexy due to hemorrhage, inflammation, or infection due to an underlying Rathke’s cleft cyst is difficult to diagnose pre-operatively, Rathke’s cleft cyst should be included in the differential diagnosis, and early surgical treatment is needed, as for pituitary apoplexy caused by pituitary adenoma.
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References
Albini CH, MacGillivray MH, Fisher JE, Voorhess ML, Klein DM (1988) Triad of hypopituitarism, granulomatous hypophysitis, and ruptured Rathke's cleft cyst. Neurosurgery 22:133–136
Benveniste RJ, King WA, Walsh J, Lee JS, Naidich TP, Post KD (2004) Surgery for Rathke cleft cysts: technical considerations and outcomes. J Neurosurg 101:577–584
Binning MJ, Liu JK, Gannon J, Osborn AG, Couldwell WT (2008) Hemorrhagic and nonhemorrhagic Rathke cleft cysts mimicking pituitary apoplexy. J Neurosurg 108:3–8
Bognar L, Szeifert GT, Fedorcsak I, Pasztor E (1992) Abscess formation in Rathke's cleft cyst. Acta Neurochir (Wien) 117:70–72
Celikoglu E, Boran BO, Bozbuga M (2006) Abscess formation in Rathke's cleft cyst. Neurol India 54:213–214
Daikokuya H, Inoue Y, Nemoto Y, Tashiro T, Shakudo M, Ohata K (2000) Rathke's cleft cyst associated with hypophysitis: MRI. Neuroradiology 42:532–534
Hama S, Arita K, Tominaga A, Yoshikawa M, Eguchi K, Sumida M, Inai K, Nishisaka T, Kurisu K (1999) Symptomatic Rathke's cleft cyst coexisting with central diabetes insipidus and hypophysitis: case report. Endocr J 46:187–192
Israel ZH, Yacoub M, Gomori JM, Dotan S, Fellig Y, Shoshan Y, Spektor S (2000) Rathke's cleft cyst abscess. Pediatr Neurosurg 33:159–161
Kim JE, Kim JH, Kim OL, Paek SH, Kim DG, Chi JG, Jung HW (2004) Surgical treatment of symptomatic Rathke cleft cysts: clinical features and results with special attention to recurrence. J Neurosurg 100:33–40
Kimura H, Fukushima T, Matsuda T, Tomonaga M (1994) Abscess formation in a Rathke's cleft cyst. No To Shinkei 46:392–395
Kurisaka M, Fukui N, Sakamoto T, Mori K, Okada T, Sogabe K (1998) A case of Rathke's cleft cyst with apoplexy. Childs Nerv Syst 14:343–347
Murakami M, Nishioka H, Izawa H, Ikeda Y, Haraoka J (2008) Granulomatous hypophysistis associated with Rathke's cleft cyst: a case report. Minim Invasive Neurosurg 51:169–172
Nishikawa T, Takahashi JA, Shimatsu A, Hashimoto N (2007) Hypophysitis caused by Rathke's cleft cyst. Case report. Neurol Med Chir (Tokyo) 47:136–139
Nishioka H, Haraoka J, Izawa H, Ikeda Y (2006) Headaches associated with Rathke's cleft cyst. Headache 46:1580–1586
Nishioka H, Haraoka J, Izawa H, Ikeda Y (2006) Magnetic resonance imaging, clinical manifestations, and management of Rathke's cleft cyst. Clin Endocrinol (Oxf) 64:184–188
Nishioka H, Ito H, Miki T, Hashimoto T, Nojima H, Matsumura H (1999) Rathke's cleft cyst with pituitary apoplexy: case report. Neuroradiology 41:832–834
Obenchain TG, Becker DP (1972) Abscess formation in a Rathke's cleft cyst. Case report. J Neurosurg 36:359–362
Onesti ST, Wisniewski T, Post KD (1990) Pituitary hemorrhage into a Rathke's cleft cyst. Neurosurgery 27:644–646
Pawar SJ, Sharma RR, Lad SD, Dev E, Devadas RV (2002) Rathke's cleft cyst presenting as pituitary apoplexy. J Clin Neurosci 9:76–79
Roncaroli F, Bacci A, Frank G, Calbucci F (1998) Granulomatous hypophysitis caused by a ruptured intrasellar Rathke's cleft cyst: report of a case and review of the literature. Neurosurgery 43:146–149
Rosales MY, Smith TW, Safran M (2004) Hemorrhagic Rathke's cleft cyst presenting as diplopia. Endocr Pract 10:129–134
Schittenhelm J, Beschorner R, Psaras T, Capper D, Nagele T, Meyermann R, Saeger W, Honegger J, Mittelbronn M (2008) 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
Sonnet E, Roudaut N, Meriot P, Besson G, Kerlan V (2006) Hypophysitis associated with a ruptured Rathke's cleft cyst in a woman, during pregnancy. J Endocrinol Investig 29:353–357
Sonntag VK, Plenge KL, Balis MS, Raudzens PA, Hodak JA, Clark RJ, Waggener JD (1983) Surgical treatment of an abscess in a Rathke's cleft cyst. Surg Neurol 20:152–156
Thomas N, Wittert GA, Scott G, Reilly PL (1998) Infection of a Rathke's cleft cyst: a rare cause of pituitary abscess. Case illustration. J Neurosurg 89:682
Wearne MJ, Barber PC, Johnson AP (1995) Symptomatic Rathke's cleft cyst with hypophysitis. Br J Neurosurg 9:799–803
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Comment
In this report, in the 10-year series of 35 Rathke's cleft cysts treated at Fukuoka University, five cases that presented with pituitary apoplexy are presented. The finding is that a lot of individuals carry Rathke's cleft cysts; some of these cysts became symptomatic due to their mass effect. Only a few of these cysts present with pituitary apoplexy. In these cases, hemorrhage, hypophysitis, or abscess is the cause of the sudden onset. For clinical symptoms and treatment, the Rathke's cleft cyst becomes the secondary pathology.
For these rare pathologies with even more uncommon presentation, the retrospective analysis still remains our only source of information. So, it is important to publish series of this kind.
Jens Lehmberg
Munich, Germany
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Komatsu, F., Tsugu, H., Komatsu, M. et al. Clinicopathological characteristics in patients presenting with acute onset of symptoms caused by Rathke’s cleft cysts. Acta Neurochir 152, 1673–1678 (2010). https://doi.org/10.1007/s00701-010-0687-5
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DOI: https://doi.org/10.1007/s00701-010-0687-5