First Report of Coexistence of Two Ectopic Pituitary Tumors: Rathkle Cleft Cyst and Silent Adrenocorticotropic Hormone Adenoma

BACKGROUND: Rathke cleft cysts (RCCs) and pituitary adenomas (PAs) are thought to have a common embryonic ancestry; however, PAs with a concomi­tant RCC inside the sella turcica are rarely observed. Ectopic pituitary tumors are also rare.

CASE DESCRIPTION: We present the case of a 65-year-old woman with an ectopic RCC in the sphenoid sinus and outside the sella turcica concomitant with an adrenocorticotropic hormone (ACTH)-staining, clinically silent PA. The patient had headache but no endocrine or visual disturbances. Preoperative magnetic resonance imaging revealed infrasellar cystic lesion in the sphenoid sinus with erosion of the clivus and intact sellar floor. The patient underwent gross total microsurgical resection through the transnasal route with an un­eventful postoperative course.

CONCLUSIONS: To our knowledge, this is the first reported ectopic RCC located outside the sella turcica with a concomitant ACTH-staining PA. This also appears to be the first ACTH-staining adenoma concomitant with RCC reported in the literature, regardless of location, not presenting with Cushing disease. This case shows that we can now include pituitary adenoma with or without a concomitant RCC in the differential diagnosis of processes in the sphenoid sinus. As both PAs and RCCs are benign sellar lesions, surgical management of a concomitant occurrence of these tumors mainly depends on the size of the le­sions and their clinical manifestations. For patients with PA and concomitant RCC, surgical resection should be considered, as there is an approximatrely 20% recurrence rate of the cyst after resection and the possibility of future clival erosion, if left untreated.