Pojskić Mirza, Zbytek Blazej, Beckford Neal S, Boop Frederick A, Arnautović Kenan I
Department of Neurosurgery, University of Marburg, Marburg, Germany.
Department of Pathology and Laboratory Medicine; Center for Adult Cancer Research; University of Tennessee Health Science Center, Memphis, Tennessee.
World Neurosurg. 2017 Aug;104:1048.e1-1048.e7. doi: 10.1016/j.wneu.2017.05.070. Epub 2017 May 19.
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.
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.
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.
拉克氏囊肿（RCC）和垂体腺瘤（PA）被认为具有共同的胚胎起源；然而，蝶鞍内伴有RCC的PA很少见。异位垂体肿瘤也很罕见。
我们报告一例65岁女性，其蝶窦内及蝶鞍外存在异位RCC，并伴有促肾上腺皮质激素（ACTH）染色的临床无症状PA。患者有头痛，但无内分泌或视觉障碍。术前磁共振成像显示蝶窦内鞍下囊性病变，斜坡骨质侵蚀，鞍底完整。患者经鼻入路接受了全切除显微手术，术后恢复顺利。
据我们所知，这是首例报道的位于蝶鞍外的异位RCC并伴有ACTH染色的PA。这似乎也是文献中首例报道的无论位置如何、不伴有库欣病的与RCC相关的ACTH染色腺瘤。该病例表明，在蝶窦病变的鉴别诊断中，我们现在可以将伴有或不伴有RCC的垂体腺瘤纳入考虑。由于PA和RCC均为蝶鞍良性病变，这些肿瘤同时发生时的手术治疗主要取决于病变大小及其临床表现。对于患有PA并伴有RCC的患者，应考虑手术切除，因为囊肿切除后复发率约为20%，如果不治疗，未来有斜坡骨质侵蚀的可能。
