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라트케 열낭종과 동반된 림프구성 뇌하수체염 1예

A case of lymphocytic hypophysitis associated with Rathke's cleft cyst

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초록

본 증례는 임상적, 방사선학적으로 뇌하수체선종에서 발생한 뇌하수체 졸중을 의심하여 경접형동 접근법을 통한 종괴의 제거를 시행한 결과 라트케 열낭종과 동반된 림프구성 뇌하수체염으로 진단된 예이다. 라트케 열낭종과 림프구성 뇌하수체염은 임상적으로나, 방사선학적으로 다른 뇌하수체의 병변과 감별하기가 매우 어렵다. 과도한 절제로 인한 뇌하수체 손상의 합병증을 예방하기 위해 수술 전 뇌하수체의 종괴의 감별진단에 있어 라트케 열낭종과 림프구성 뇌하수체염의 가능성을 항시 염두에 두어야할 것이다.

Abstract

Lymphocytic hypophysitis is a rare disorder characterized by focal or diffuse inflammatory infiltration and destruction of the pituitary gland. It has classically been described as affecting female patients during peripartum period and its pathogenesis has been attributed with autoimmunity. Pressure symptoms and visual impairment may arise from the sellar mass lesion, and mass effect, involvement of the pituitary stalk, and pituitary destruction may result in endocrine dysfunction. Rathke's cleft cyst is a cystic sellar or suprasellar lesion thought to originate from the remnants of Rathke's pouch. It is characteristically lined by a single layer of ciliated cuboidal or columnar epithelium. Although small asymptomatic Rathke's cleft cysts have been reported at autopsy in 2~26% of normal pituitary glands, symptomatic cysts are rare. In symptomatic Rathke's cleft cysts, the common presentations include headache, pituitary dysfunction, and visual loss caused by compression of the optic chiasm in the event of suprasellar extension. Recently we experienced a case of lymphocytic hypophysitis with Rathke's cleft cyst resected by transsphenoidal approach under the preoperative impression of pituitary adenoma. This coexistence is exceedingly rare event, and to our best knowledge, this may be the first case of lymphocytic hypophysitis with Rathke's cleft cyst in Korea.

참고문헌 (21)

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