범뇌하수체저하증, 요붕증을 보인 원발성 육아종성 뇌하수체염 1예
Primary Granulomatous Hypophysitis Presenting with Panhypopituitarism and Central Diabetes Insipidus
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초록
본 증례에서는 기저 질환이 없던 48세 여자 환자가 두통, 피로함, 오심, 희발 월경, 다뇨 증상을 호소하였고 호르몬 검사와 영상 검사 결과 범뇌하수체 기능 저하증과 중심성 요붕증을 동반하는 뇌하수체 병변으로 진단하였다. 6개월간스테로이드 및 호르몬 요법을 시행했으나 두통이 지속되고종괴의 크기도 변하지 않아 접형동을 통한 종괴 절제술을시행하였다. 수술 후 원발성 육아종성 뇌하수체염으로 확진할 수 있었으며 현재 호르몬 보충 요법을 유지하면서 외래에서 경과관찰 중이다.
Abstract
Primary granulomatous hypophysitis is a rare inflammatory disorder of the pituitary gland and patients commonly present with symptoms of sellar compression and hypopituitarism. A 48-year-old woman was admitted due to headache and fatigue. Magnetic resonance imaging showed a 21 × 18 × 13-mm round sellar mass with a thickened pituitary stalk. The endocrinological examination revealed panhypopituitarism and diabetes insipidus. Suspecting hypophysitis, the patient was given steroid and hormone replacement therapy. Six months later, she continued to complain of severe headaches and nausea. Computed tomography showed no significant change in the sellar mass. Subsequently, transsphenoidal surgery was performed. The pathological examination revealed granulomatous changes with multinucleated giant cells and primary granulomatous hypophysitis was diagnosed. Her headache resolved, but the pituitary functions did not improve. This is the first reported case in Korea of primary granulomatous hypophysitis with dysfunction of anterior and posterior pituitary gland, including the stalk, without optic chiasm compression.
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