Rathke's cleft cyst and ACTH secreting pituitary microadenoma: simultaneous symptomatic

Case contributed by Dr Andrei Tsoriev

Presentation

Woman with Cushing disease for several months. High ACTH level in peripheral blood.

Patient Data

Age: 28
Gender: Female

MRI before and after contrast administration with dynamic scanning

Modality: MRI

Ovoid sharply marginated mass in the lower right part of adenohypophysis with low signal on T2-weighted and iso-slightly low signal on T1-weighted images. Laterally and slightly upper from the mass - low intensity on T1-weighted and isointense on T2-weighted images lesion, less than 4 mm in diameter. The latter is taking up contrast material slower, than anterior pituitery, which is consistent with microadenoma.  

Early,  20 seconds after contrast administration image from T1-weighted postcontrast series with dynamic scanning, red arrows indicate hypoenhancing microadenoma, green arrows indicate non-enhancing Rathke's cleft cyst.

Case Discussion

Pituitary adenomas rarely may exist simultaneously with a Rathke's cleft cyst (RCC). This is a case of a symptomatic ACTH-secreting pituitary microadenoma adenoma associated with a RCC.

RCCs have been discovered incidentally in 11-33% of all autopsies, and are associated with less than 2 percents of the pituitary adenoma cases. Secreting microadenomas assosiated with RCCS are even more rare, I found less than 40 such cases described in published literature.

In one series, published by Sumida er al. (2002), the frequency of the combination of Rathke's cleft cyst and pituitary  adenoma was 3.5 % for pituitary adenomas and 11% for Rathke's cleft cysts. Secreting adrenocorticotrophin adenomas were present in two patients and growth hormone in six, so the frequency of ACTH-secreting adenoma combined with Rathke's cleft cyst was about 0,5%.

This case is confirmed surgically and size and  localization of cyst and microadenoma at operation was in total agreement with preoperative MR-based localization. It is important to carefully seek for possible microadenomas in hormonal disorders of pituitary origin since many of them treated surgically and presurgical identification of lesion allows not to perform costly and invasive inferior petrous sinus blood sampling to lateralize tumor.

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Case Information

rID: 34272
Case created: 12th Feb 2015
Last edited: 4th Sep 2016
Inclusion in quiz mode: Included

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