A rare association between Rathke’s cyst and hypophysitis in a patient with delayed sex development and growth failure
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Daisuke Takahashi
Abstract
We report an 18-year-old Japanese male with a lack of secondary sex characterization and growth failure caused by a rare association between Rathke’s cyst and hypophysitis. He was referred to us because of delayed secondary sex characterization. Endocrinological examination showed panhypopituitarism, and the replacement of hydrocortisone, levothyroxine, and desmopressin acetate (DDAVP) was initiated. Brain magnetic resonance imaging (MRI) showed a suprasellar region and a swollen pituitary stalk. The mass was partially resected using the transsphenoidal approach. The pathological diagnosis was hypophysitis and Rathke’s cyst. Follow-up MRI performed 1 year after surgery showed that the size of sellar region had not changed. After surgery, in addition to pre-operative hormonal replacement, growth hormone and testosterone were initiated. Two years later, the size of sellar region remains unchanged.
In conclusion, while an association between Rathke’s cyst and hypophysitis is rare, we suggest that this condition should be included in differential diagnosis of the sellar region, even in adolescents.
References
1. Honegger J, Fahlbusch R, Bornemann A, Hensen J, Buchfelder M, et al. Lymphocytic and granulomatous hypophysitis: experience with nine cases. Neurosurgery 1999;40:713–22.10.1097/00006123-199704000-00010Search in Google Scholar
2. Flanagan DE, Ibrahim AE, Ellison DW, Armitage M, Gawne-Cain M, et al. Inflammatory hypophysitis – the spectrum of disease. Acta Neurochir (Wien) 2002;144:47–56.10.1007/s701-002-8273-5Search in Google Scholar
3. Leung GK, Lopes MB, Thorner MO, Vance ML, Laws ER Jr. Primary hypophysitis: a single-center experience in 16 cases. J Neurosurg 2004;101:262–71.10.3171/jns.2004.101.2.0262Search in Google Scholar
4. Gutenberg A, Hans V, Puchner MJ, Kreutzer J, Bruck W, et al. Primary hypophysitis: clinical-pathological correlations. Eur J Endocrinol 2006;155:101–7.10.1530/eje.1.02183Search in Google Scholar
5. Mukherjee JJ, Islam N, Kaltsas G, Lowe DG, Charlesworth M, et al. Clinical, radiological and pathological features of patients with Rathke’s cleft cysts: tumors that may recur. J Clin Endocrinol Metab 1997;82:2357–62.Search in Google Scholar
6. Shin JL, Asa SL, Woodhouse LJ, Smyth HS, Ezzat S. Cystic lesions of the pituitary: clinicopathological features distinguishing craniopharyngioma, Rathke’s cleft cyst, and arachnoid cyst. J Clin Endocrinol Metab 1999;84:3972–82.Search in Google Scholar
7. van Tijn DA, de Vijlder JJ, Vulsma T. Role of the thyrotropin-releasing hormone stimulation test in diagnosis of congenital central hypothyroidism in infants. J Clin Endocrinol Metab 2008;93:410–9.10.1210/jc.2006-2656Search in Google Scholar
8. Teramoto A, Hirakawa K, Sanno N, Osamura Y. Incidental pituitary lesions in 1,000 unselected autopsy specimens. Radiology 1994;193:161–4.10.1148/radiology.193.1.8090885Search in Google Scholar
9. Nakayama T, Tanaka A, Naritomi K, Yoshinaga S. Hyponatremia-induced metabolic encephalopathy caused by Rathke’s cleft cyst: a case report. Clin Neurol Neurosurg 1999;101:114–7.10.1016/S0303-8467(99)00016-5Search in Google Scholar
10. Asaeda M, Kurosaki M, Kambe A, Takenobu A, Horie Y, et al. MR imaging study of edema along the optic tract in patient with Rathke’s cleft cyst. No To Shinkei 2004;56:243–6.Search in Google Scholar
11. Daikokuya H, Inoue Y, Nemoto Y, Tashiro T, Shakudo M, et al. Rathke’s cleft cyst associated with hypophysitis: MRI. Neuroradiology 2000;42:532–4.10.1007/s002340000311Search in Google Scholar PubMed
12. Hama S, Arita K, Tominaga A, Yoshikawa M, Eguchi K, et al. Symptomatic Rathke’s cleft cyst coexisting with central diabetes insipidus and hypophysitis: case report. Endocr J 1999;46: 187–92.10.1507/endocrj.46.187Search in Google Scholar PubMed
13. Nishikawa T, Takahashi JA, Shimatsu A, Hashimoto N. Hypophysitis caused by Rathke’s cleft cyst. Neurol Med Chir (Tokyo) 2007;47:136–9.10.2176/nmc.47.136Search in Google Scholar PubMed
14. Schittenhelm J, Beschorner R, Psaras T, Capper D, Nagele 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 2008;31:157–63.10.1007/s10143-008-0120-1Search in Google Scholar PubMed
©2013 by Walter de Gruyter Berlin Boston
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- Review article
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- Images in pediatric endocrinology
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- A rare case of granuloma annulare in a 6-year-old child with type 1 diabetes
- Original articles
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- The relationship between adiposity and stature in prepubertal children with celiac disease
- Influence of micronutrient status and socioeconomic gradient on growth indices of 2–18-year-old Indian girls
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- The relation between salivary cortisol and the metabolic syndrome score in girls
- Association of vitamin D concentrations with adiposity indices among preadolescent children in Korea
- Association of serum resistin levels with metabolic syndrome and early atherosclerosis in obese Chinese children
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- Parental dyadic coping in families of children and adolescents with type 1 diabetes
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- Effect of insulin therapy on IGF-1 level in children with new-onset type 1 diabetes mellitus: a comparison between DKA and non-DKA
- Mutation analysis of the SRD5A2, AR and SF-1 genes in 52 Chinese boys with hypospadias
- Sexual development in prepubertal obese boys: a 4-year longitudinal study
- Genetic diagnosis of one family with incomplete clinical data
- Patient reports
- Complete androgen insensitivity syndrome and discordant Müllerian remnants: two cases with novel mutation in the androgen receptor
- Psychosexual outcomes in three siblings with partial androgen insensitivity syndrome: impact of nature versus nurture
- Ambiguous genitalia in a 48, XXYY newborn: a casual relationship or a coincidence?
- Gonadal function of beta-thalassemics following stem cell transplantation conditioned with myeloablative and reduced intensity regimens
- Significant testicular enlargement in a 6.5-year-old boy with monorchism and testicular microlithiasis
- Report of the first case of precocious puberty in Rett syndrome
- Type II vitamin D-dependent rickets with diabetic ketoacidosis
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- A rare association between Rathke’s cyst and hypophysitis in a patient with delayed sex development and growth failure
- Diabetes mellitus with Laron syndrome: case report
- Wegener granulomatosis as an uncommon cause of panhypopituitarism in childhood
- Rare cases of autoimmune hypothyroidism in young children
- Cushing syndrome related to leukemic infiltration of the central nervous system: a case report and a possible role of LIF
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- Letter to the Editors
- Homeostasis model assessment of insulin resistance may not be reliable to evaluate insulin sensitivity in young obese children
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