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Rathke’s cleft cysts with significant squamous metaplasia—high risk of postoperative deterioration and close origins to craniopharyngioma

  • Clinical Article - Brain Tumors
  • Published:
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Abstract

Background

Rathke’s cleft cyst (RCC) with significant squamous and/or stratified epithelium including smooth transition from single cuboidal to squamous epithelium (tRCC) is rare and possibly represents an intermediate form to craniopharyngioma.

Methods

Twelve patients with histologically confirmed tRCC were retrospectively investigated from a series of 167 cases of RCC and 96 cases of craniopharyngiomas. Clinical data were reviewed, and immunohistochemistry findings for cytokeratins and β-catenin were examined.

Results

All lesions were located in the sella turcica with marked extension to suprasellar cistern. Six of the 12 patients had suffered postoperative re-enlargement, and three of these six patients required more than two additional operations and irradiation. CAM5.2 was positive in the glandular epithelium in all tRCCs and focally positive in the squamous epithelium of all these tRCCs. 34βE12 was positive in the squamous epithelium in all tRCCs and focally positive in the glandular epithelium in all but one tRCC. The findings of cytokeratin expression of tRCCs were very similar to those of craniopharyngioma. β-Catenin showed nuclear translocation in five cases. All patients with nuclear translocation of β-catenin suffered postoperative re-enlargement.

Conclusions

tRCC carries an extremely high risk of re-enlargement. Cytokeratin expression resembles that in craniopharyngioma, which might indicate a very close origin of these pathologies. Nuclear translocation of β-catenin may be related to the aggressive clinical course.

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Disclosure

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this article.

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Authors and Affiliations

  1. Department of Neurosurgery, Kohnan Hospital, 4-20-1 Nagamachiminami, Taihaku-ku, Sendai, Miyagi, 982-8523, Japan

    Yoshikazu Ogawa

  2. Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan

    Mika Watanabe

  3. Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan

    Teiji Tominaga

Authors
  1. Yoshikazu Ogawa
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  2. Mika Watanabe
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  3. Teiji Tominaga
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Corresponding author

Correspondence to Yoshikazu Ogawa.

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Comment

This is an interesting series of unusual Rathke’s cleft cysts that recurred as part of a large series of RCCs. Although the position of the cyst in the sella above the anterior gland is probably the most likely indicator of the potential for regrowth, the key point here is that these cysts also had unusual histological features. There is increasing evidence for a transitional pathology between RCCs and craniopharyngeomas, which this article supports. Of interest is that fractionated radiation also helped control these lesions where recurrence occurred, albeit with the high price of induced hypopituitarism.

Michael Powell,

London, UK

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Ogawa, Y., Watanabe, M. & Tominaga, T. Rathke’s cleft cysts with significant squamous metaplasia—high risk of postoperative deterioration and close origins to craniopharyngioma. Acta Neurochir 155, 1069–1075 (2013). https://doi.org/10.1007/s00701-012-1593-9

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  • DOI: https://doi.org/10.1007/s00701-012-1593-9

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