Abstract
Herein, we report on a lymphocytic hypophysitis related to a ruptured Rathke’s cleft cyst which is not associated with pregnancy. A 45-year-old woman initially presented with headache and temporary double vision followed by amenorrhea. Preoperative imaging showed an intra- and suprasellar cystic mass. Complete resection of the tumor mass was performed via a transnasal, transseptal approach. Pathological examination displayed lymphocytic infiltrates within fibrotic tissue and residual pituitary cells accompanied by epithelial tissue of a Rathke’s cleft cyst. The strongest inflammatory reaction was observed at the site of disrupture of the cyst integrity, suggesting that high protein levels from ruptured Rathke’s cleft cyst might have triggered a lymphocytic hypophysitis. Our review of the literature provides further insights regarding the clinical behavior and different histological types of the lesions as well as the inflammatory changes that can occur in Rathke’s cleft cysts.
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Ahmadi J, Destian S, Apuzzo ML, Segall HD, Zee CS (1992) Cystic fluid in craniopharyngiomas: MR imaging and quantitative analysis. Radiology 182:783–785
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
Asaeda M, Kurosaki M, Kambe A, Takenobu A, Horie Y, Yamane Y, Murakami Y, Watanabe T (2004) MR imaging study of edema along the optic tract in patient with Rathke’s cleft cyst. No To Shinkei 56:243–246
Bellastella A, Bizzarro A, Coronella C, Bellastella G, Sinisi AA, De Bellis A (2003) Lymphocytic hypophysitis: a rare or underestimated disease. Eur J Endocrinol 149:363–376
Bottazzo GF, Pouplard A, Florin-Christensen A, Doniach D (1975) Autoantibodies to prolactin-secreting cells of human pituitary. Lancet 2:97–101
Bullmann C, Faust M, Hoffmann A, Heppner C, Jockenhovel F, Muller-Wieland D, Krone W (2000) Five cases with central diabetes insipidus and hypogonadism as first presentation of neurosarcoidosis. Eur J Endocrinol 142:365–372
Byun WM, Kim OL, Kim D (2000) MR imaging findings of Rathke’s cleft cysts: significance of intracystic nodules. Am J Neuroradiol 21:485–488
Caturegli P, Newschaffer C, Olivi A, Pomper MG, Burger PC, Rose NR (2005) Autoimmune hypophysitis. Endocr Rev 26:599–614
Collin P, Kaukinen K, Valimaki M, Salmi J (2002) Endocrinological disorders and celiac disease. Endocr Rev 23:464–483
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
Donadieu J, Rolon MA, Thomas C, Brugieres L, Plantaz D, Francois Emile J, Frappaz D, David M, Brauner R, Genereau T, Debray D, Cabrol S, Barthez MA, Hoang-Xuan K, Polak M, French LCH Study Group (2004) Endocrine involvement in pediatric-onset Langerhans’ cell histiocytosis: a population based study. J Pediatr 144:344–350
Fehn M, Bettendorf M, Ludecke DK, Sommer C, Saeger W (1999) Lymphocytic hypophysitis masking a suprasellar germinoma in a 12-year-old girl—a case report. Pituitary 1:303–307
Flanagan DE, Ibrahim AE, Ellison DW, Armitage M, Gawne-Cain M, Lees PD (2002) Inflammatory hypophysitis—the spectrum of disease. Acta Neurochir 144:47–56
Folkerth RD, Price DL Jr, Schwartz M, Black PM, De Girolami U (1998) Xanthomatous hypophysitis. Am J Surg Pathol 22:736–741
Goyal M, Kucharczyk W, Keystone E (2000) Granulomatous hypophysitis due to Wegener's granulomatosis. Am J Neuroradiol 21:1466–1469
Graziani N, Dufour H, Figarella-Branger D, Donnet A, Bouillot P, Grisoli F (1995) Do the suprasellar neurenteric cyst, the Rathke cleft cyst and the colloid cyst constitute a same entity. Acta Neurochir 133:174–180
Gutenberg A, Hans V, Puchner MJ, Kreutzer J, Bruck W, Caturegli P, Buchfelder M (2006) Primary hypophysitis: clinical–pathological correlations. Eur J Endocrinol 155:101–107
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
Hama S, Arita K, Nishisaka T, Fukuhara T, Tominaga A, Sugiyama K, Yoshioka K, Equichi K, Sumida M, Heike Y, Kurisu K (2002) Changes in the epithelium of Rathke cleft cyst associated with inflammation. J Neurosurg 96:209–216
Hansen I, Petrossians P, Thiry A, Flandroy P, Gaillard RC, Kovacs K, Claes F, Stevenaert A, Piguet P, Beckers A (2001) Extensive inflammatory pseudotumor of the pituitary. J Clin Endocrinol Metab 86:4603–4610
Hashimoto K, Takao T, Makino S (1997) Lymphocytic adenohypophysitis and lymphocytic infundibuloneurohypophysitis. Endocr J 44:1–10
Hashimoto K, Asaba K, Tamura K, Takao T, Nakamura T (2002) A case of lymphocytic infundibuloneurohypophysitis associated with systemic lupus erythematosus. Endocr J 49:605–610
Hassoun P, Anayssi E, Salti I (1985) A case of granulomatous hypophysitis with hypopituitarism and minimal pituitary enlargement. J Neurol Neurosurg Psychiatry 48:949–951
Hayashi Y, Tachibana O, Muramatsu N, Tsuchiya H, Tada M, Arakawa Y, Suzuki M, Yamashita J (1999) Rathke cleft cyst: MR and biomedical analysis of cyst content. J Comput Assist Tomogr 23:34–38
Heshmati HM, Kujas M, Casanova S, Wollan PC, Racadot J, Van Effenterre R, Derome PJ, Turpin G (1998) Prevalence of lymphocytic infiltrate in 1400 pituitary adenomas. Endocr J 45:357–361
Honegger J, Fahlbusch R, Bornemann A, Hensen J, Buchfelder M, Muller M, Nomikos P (1997) Lymphocytic and granulomatous hypophysitis: experience with nine cases. Neurosurgery 40:713–722
Houdouin L, Polivka M, Henegar C, Blanquet A, Delalande O, Mikol J (2003) Pituitary germinoma and lymphocytic hypophysitis: a pitfall. Report of two cases. . Ann Pathol 23:349–354
Huang YY, Lin SF, Dunn P, Wai YY, Hsueh C, Tsai JS (2005) Primary pituitary lymphoma presenting as hypophysitis. Endocr J 52:543–549
Leung GK, Lopes MB, Thorner MO, Vance ML, Laws ER (2004) Primary hypophysitis: a single-center experience in 16 cases. J Neurosurg 101:262–271
Macaulay RJ (1997) Ruptured Rathke’s cleft cyst: a possible cause of Tolosa–Hunt syndrome. Clin Neuropathol 16:98–102
Mirzai H, Demir MA, Gözüpek G, Temiz C, Selcuki M (2004) Rathke’s cleft cyst rupture as the cause of a granulomatous reaction: a case report. Turkiye klinikleri J Med Sci 24:421–424
Nagasaka S, Kuromatsu C, Wakisaka S, Kitamura K, Matsushima T (1981) Rathke’s cleft cyst. Surg Neurol 15:402–405
Nakayama Y, Tanaka A, Naritomi K, Yoshinaga S (1999) Hyponatriemia-induced metabolic encephalopathy caused by Rathke’s cleft cyst: a case report. Clin Neurol Neurosurg 101:114–117
Nishikawa T, Takahashi JA, Shimatsu A, Hashimoto N (2007) Hypophysitis caused by Rathke’s cleft cyst. Case report. Neurol Med Chir 47:136–139
O’Dwyer DT, Clifton V, Hall A, Smith R, Robinson PJ, Crock PA (2002) Pituitary autoantibodies in lymphocytic hypophysitis target both gamma- and alpha-Enolase—a link with pregnancy. Arch Physiol Biochem 110:94–98
Oka H, Kawano N, Suwa T, Yada K, Kan S, Kameya T (1994) Radiological study of symptomatic Rathke’s cleft cysts. Neurosurgery 35:632–636
Paulus W, Honegger J, Keyvani K, Fahlbusch R (1999) Xanthogranuloma of the sellar region: a clinicopathogical entity different from adamantinous craniopharyngeoma. Acta Neuropathol 97:371–382
Puchner MJ, Ludecke DK, Saeger W (1994) The anterior pituitary lobe in patients with cystic craniopharyngiomas: three cases of associated lymphocytic hypophysitis. Acta Neurochir 126:38–43
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
Ross DA, Norman D, Wilson CB (1992) Radiologic characteristics and results of surgical management of Rathke’s cysts in 43 patients. Neurosurgery 30:173–178
Sautner D, Saeger W, Ludecke DK, Jansen V, Puchner MJ (1995) Hypophysitis in surgical and autoptical specimens. Acta Neuropathol 90:637–644
Scherbaum WA, Schrell U, Gluck M, Fahlbusch R, Pfeiffer EF (1987) Autoantibodies to pituitary corticotropin-producing cells: possible marker for unfavourable outcome after pituitary microsurgery for Cushing’s disease. Lancet 1:1394–1398
Simmonds M (1917) Über das Vorkommen von Riesenzellen in der Hypophyse. Virchows Arch 223:281–290
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 Invest 29:353–357
Sumida M, Uozumi T, Mukada K, Arita K, Kurisu K, Eguchi K (1994) Rathke cleft cysts: correlation of enhanced MR and surgical findings. Am J Neuroradiol 15:525–532
Supler ML, Mickle JP (1992) Lymphocytic hypophysitis: report of a case in a man with cavernous sinus involvement. Surg Neurol 37:472–476
Takahashi JA, Hojo M, Nishikawa T, Mori H, Hirano A, Hashimoto N (2004) Inflammatory pituitary gland changes in cases of Rathke’s cleft cyst associated with panhypopituitarism. No Shinkei Geka 13:565–571
Vidal S, Rotondo F, Horvath E, Kovacs K, Scheithauer BW (2002) Immunocytochemical localization of mast cells in lymphocytic hypophysitis. Am J Clin Pathol 117:478–483
Voelker JL, Campbell RL, Muller J (1991) Clinical, radiographic, and pathological features of symptomatic Rathke’s cleft cysts. J Neurosurg 74:535–544
Wearne MJ, Barber PC, Johnson AP (1995) Symptomatic Rathke’s cleft cyst with hypophysitis. Br J Neurosurg 9:799–803
Yuyama R, Kojima H, Mizutani T, Suzuki Y, Miki Y (2002) Secondary panhypophysitis caused by rupture of Rathke’s cleft cyst: case report. No Shinkei Geka 30:95–99
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Schittenhelm, J., Beschorner, R., Psaras, T. et al. 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). https://doi.org/10.1007/s10143-008-0120-1
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DOI: https://doi.org/10.1007/s10143-008-0120-1