Skip to main content

Advertisement

Log in

Pediatric symptomatic Rathke cleft cyst compared with cystic craniopharyngioma

  • Original Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Introduction

Symptomatic Rathke cleft cysts (RCCs) are rarely detected in neuroradiological screening and are less commonly found in children than in adults. However, when RCCs are observed in children, it is important to carefully distinguish a RCC from a cystic craniopharyngioma (CP) even if surgically treated or conservatively followed up.

Methods

We conducted a retrospective review of clinical data from 11 patients with symptomatic RCCs whose ages were under 18 years and compared the data with data from 15 age- and sex-matched patients with cystic CP who were treated at our institute.

Results

The mean age of the patients with RCCs was 12.2 years (range, 6–18). There were six males and five females. As initial symptoms, nine patients presented with headache, while two each had impaired visual function, diabetes insipidus, and activity loss. The 14 patients with CP suffered from impaired visual function. Magnetic resonance imaging (MRI) mainly showed hyperintensity on T1-weighted images (WIs) and hypointensity on T2-WI in patients with RCC. However, patients with CP had characteristic hyperintensity on T2-WI. The average maximum diameter of the RCCs was 19.0 mm on average (range, 8–33 mm). The RCCs were thus significantly smaller than CPs (34.9 mm; range, 21–54 mm). The RCCs were usually oval or dumbbell-shaped and regular in appearance, while the larger CPs were lobular and irregular. A preoperative endocrinological evaluation revealed insufficiencies in four axes in five patients with RCC. Postoperative endocrinological status improved in three patients, remained unchanged in three, and worsened in one. The gonadotropin axis was damaged in a majority (nine) of the patients with CP preoperatively. Postoperative evaluation revealed deficits in five axes in 14 patients with CP, which is a significantly different trend than observed in patients with RCC. Eight patients underwent surgical procedures (transsphenoidal surgery (TSS) in four, craniotomy in four). Two of these patients experienced a recurrence of the cysts. One of these patients subsequently underwent two craniotomies followed by radiation and other underwent TSS. Among the three conservatively treated patients, two experienced a transient worsening of their symptoms along with cyst enlargement. However, none of the three conservatively treated patients required an operation.

Conclusions

When RCCs become symptomatic in children, the most common symptom they lead to is headache. The cysts are commonly small, regular, and oval in shape. Hypointensity of cyst contents on MRI is a characteristic of RCCs, which distinguishes them from CPs. Surgical intervention can be effective and lead to the relief of symptoms without a high rate of complications. However, there seems to be a relatively high recurrence rate following surgery. Thus, if the patient’s symptoms remain minor, the surgical treatment option should be used only when prudent, as the patient’s symptoms may improve over time.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Cohan P, Foulad A, Esposito F et al (2004) Symptomatic Rathke’s cleft cyt: a report of 24 cases. J Endocrinol Invest 27:943–948

    Article  CAS  PubMed  Google Scholar 

  2. Voelker JL, Campbell RL, Muller J (1991) Clinical, radiographic, and pathological features of symptomatic Rathke’s cleft cysts. J Neurosurg 74:535–544

    Article  CAS  PubMed  Google Scholar 

  3. Fager CA, Carter H (1966) Intrasellar epithelial cysts. J Neurosurg 24:77–81

    Article  CAS  PubMed  Google Scholar 

  4. Yoshida J, Kobayashi T, Kageyama N et al (1977) Symptomatic Rathke’s cleft cyst. Morphological study with light and electron microscopy and tissue culture. J Neurosurg 47:451–458

    Article  CAS  PubMed  Google Scholar 

  5. Baskin DS, Wilson CB (1984) Transsphenoidal treatment of non-neoplastic intrasellar cysts. A report of 38 cases. J Neurosurg 60:8–13

    Article  CAS  PubMed  Google Scholar 

  6. Steinberg GK, Koenig GH, Golden JB (1982) Symptomatic Rathke’s cleft cyst. Report of two cases. J Neurosurg 56:290–295

    Article  CAS  PubMed  Google Scholar 

  7. Zada G (2011) Rathke cleft cysts: a review of clinical and surgical management. Neurosurg Focus 31, E1

    Article  PubMed  Google Scholar 

  8. Wait SD, Garrett MP, Little AS et al (2010) Endocrinopathy, vision, headache, and recurrence after transsphenoidal surgery for Rathke cleft cysts. Neurosurgery 67:837–843

    Article  PubMed  Google Scholar 

  9. Jagannathan J, Dumont AS, Jane JA Jr et al (2005) Pediatric sellar tumors: diagnostic procedures and management. Neurosurg Focus 18, E6

    Article  PubMed  Google Scholar 

  10. Monzavi R, Kelly DF, Geffner ME (2004) Rathke’s cleft cyst in two girls with precocious puberty. J Pediatr Endocrinol Metab 17:781–785

    Article  PubMed  Google Scholar 

  11. Setian N, Aguiar CH, Galvão JA et al (1999) Rathke’s cleft cyst as a cause of growth hormone deficiency and micropenis. Childs Nerv Syst 15:271–273

    Article  CAS  PubMed  Google Scholar 

  12. Aho CJ, Liu C, Zelman V et al (2005) Surgical outcomes in 118 patients with Rathke cleft cysts. J Neurosurg 102:189–193

    Article  PubMed  Google Scholar 

  13. Benveniste RJ, King WA, Walsh J, Lee JS et al (2004) Surgery for Rathke cleft cysts: technical considerations and outcomes. J Neurosurg 101:577–584

    Article  PubMed  Google Scholar 

  14. Jahangiri A, Molinaro AM, Tarapore PE et al (2011) Rathke cleft cysts in pediatric patients: presentation, surgical management, and postoperative outcomes. Neurosurg Focus 31, E3

    Article  PubMed  Google Scholar 

  15. Zada G, Ditty B, McNatt SA et al (2009) Surgical treatment of Rathke cleft cysts in children. Neurosurgery 64:1132–1137

    Article  PubMed  Google Scholar 

  16. Choi SH, Kwon BJ, Na DG et al (2007) Pituitary adenoma, craniopharyngioma, and Rathke cleft cyst involving both intrasellar and suprasellar regions: differentiation using MRI. Clin Radiol 62:453–462

    Article  CAS  PubMed  Google Scholar 

  17. Takanashi J, Tada H, Barkovich AJ et al (2005) Pituitary cysts in childhood evaluated by MR imaging. AJNR Am J Neuroradiol 26:2144–2147

    PubMed  Google Scholar 

  18. Fan J, Qi S, Peng Y et al (2014) An isolated primary Rathke’s cleft cyst in the cerebellopontine angle. J Neurosurg 121:846–850

    Article  PubMed  Google Scholar 

  19. Dastoli PA, Nicácio JM, Silva NS et al (2011) Cystic craniopharyngioma: intratumoral chemotherapy with alpha interferon. Arq Neuropsiquiatr 69:50–55

    Article  PubMed  Google Scholar 

  20. Saeki N, Hoshi S, Sunada S et al (2002) Correlation of high signal intensity of the pituitary stalk in macroadenoma and postoperative diabetes insipidus. Am J Neuroradiol 23:822–827

    PubMed  Google Scholar 

  21. Sato K, Oka H, Utsuki S et al (2006) Ciliated craniopharyngioma may arise from Rathke cleft cyst. Clin Neuropathol 25:25–28

    CAS  PubMed  Google Scholar 

  22. Ogawa Y, Watanabe M, Tominaga T (2013) Rathke’s cleft cysts with significant squamous metaplasia—high risk of postoperative deterioration and close origins to craniopharyngioma. Acta Neurochir (Wien) 155:1069–1075

    Article  Google Scholar 

  23. Shin JL, Asa SL, Woodhouse LJ et al (1999) Cystic lesions of the pituitary: clinicopathological features distinguishing craniopharyngioma, Rathke’s cleft cyst, and arachnoid cyst. J Clin Endocrinol Metab 84:3972–3982

    CAS  PubMed  Google Scholar 

  24. Zada G, Lin N, Ojerholm E, Ramkissoon S et al (2010) Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships. Neurosurg Focus 28, E4

    Article  PubMed  Google Scholar 

  25. Kim JE, Kim JH, Kim OL et al (2004) Surgical treatment of symptomatic Rathke cleft cysts: clinical features and results with special attention to recurrence. J Neurosurg 100:33–40

    Article  PubMed  Google Scholar 

  26. Sartoretti-Schefer S, Wichmann W, Aguzzi A et al (1997) MR differentiation of adamantinous and squamous-papillary craniopharyngiomas. Am J Neuroradiol 18:77–87

    CAS  PubMed  Google Scholar 

  27. Hayashi Y, Tachibana O, Muramatsu N et al (1999) Rathke cleft cyst: MR and biomedical analysis of cyst content. J Comput Assist Tomogr 23:34–38

    Article  CAS  PubMed  Google Scholar 

  28. Tominaga JY, Higano S, Takahashi S (2003) Characteristics of Rathke’s cleft cyst in MR imaging. Magn Reson Med Sci 2:1–8

    Article  PubMed  Google Scholar 

  29. Binning MJ, Liu JK, Gannon J et al (2008) Hemorrhagic and nonhemorrhagic Rathke cleft cysts mimicking pituitary apoplexy. J Neurosurg 108:3–8

    Article  PubMed  Google Scholar 

  30. Naylor MF, Scheithauer BW, Forbes GS et al (1995) Rathke cleft cyst: CT, MR, and pathology of 23 cases. J Comput Assist Tomogr 19:853–859

    Article  CAS  PubMed  Google Scholar 

  31. Ross DA, Norman D, Wilson CB (1992) Radiological characteristics and results of surgical management of Rathke’s cysts in 43 patients. Neurosurgery 30:173–179

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Yasuhiko Hayashi.

Ethics declarations

Conflict of interest

All authors have no conflict of interests.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Hayashi, Y., Kita, D., Fukui, I. et al. Pediatric symptomatic Rathke cleft cyst compared with cystic craniopharyngioma. Childs Nerv Syst 32, 1625–1632 (2016). https://doi.org/10.1007/s00381-016-3160-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-016-3160-9

Keywords

Navigation