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

Kim, Yong Il; Jeon, Jae Seok; Kim, Young Jee; Park, Sang Won; Byun, Young Hye; Lee, Jong Yong; Kang, Hee In

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.