Rathke Cleft Cyst with a Coexisting Gonadotropin Producing Pituitary Adenoma

Abstract

Rathke cleft cyst is thought to arise from incomplete obliteration of the lumen of Rathke pouch. The cells of the anterior pituitary lobe, from which pituitary adenomas develop, are also derived from the cells of Rathke pouch. Although Rathke cleft cyst and pituitary adenoma have a shared ancestry, they rarely occur coincidentally. Rathke cleft cysts have been found incidentally in 11%-33% of post-mortem examinations, and were associated with 1.7%-2.1% of the pituitary adenoma cases. These coexisting lesions are difficult to diagnose pre-operatively due to the variable signal intensity and position of the Rathke cleft cysts. Treatment of these lesions involves surgical resection to decrease mass effect and medical management to normalize hormonal imbalances. To our best knowledge, only 42 cases of Rathke cleft cyst with a coexisting pituitary adenoma have been reported in the English or Japanese literatures, but none of the coexisting pituitary adenoma is gonadotropin producing. We would like to report a case of Rathke cleft cyst with a coexisting gonadotropin producing adenoma that was successfully treated by endoscopic endonasal transsphenoidal approach with the removal of the sellar lesion and temporary hormone replacement.

En Plaque Pituitary Adenoma within a Rathke's Cleft Cyst: Report of Three Cases

“…However, cases such as ours in which MRI showed a large cystic mass with a contrast-enhanced cyst wall and without an obvious solid part are extremely rare. 9,10 According to Binning et al, contrast enhancement of the RCC wall is actually due to enhancement of the adjacent normal pituitary gland. 19 In our cases a part of the cyst wall was thicker, and these parts were found as en plaque-shaped pituitary adenomas intraoperatively.…”

“…[3][4][5][6][7][8] A recent literature review disclosed < 50 detailed cases. 9,10 In this report, we present three cases of en plaque pituitary adenoma within an RCC. Diagnostic and therapeutic difficulties caused by the intracystic location and the en plaque configuration of the adenomas are also discussed.…”