CAJ | 학술논문

目的探讨原发性色素性结节状肾上腺皮质病(PPNAD)的临床表现及诊治方法.方法 PPNAD患者1例,男性,52岁.因血压升高1年余入院.库欣征面容.血、尿皮质醇升高,大、小剂量地塞米松抑制试验均不被抑制.B超检查示双侧肾上腺区未见肿块回声.CT检查左侧肾上腺可见1.6 cm×2.0 cm肿块,右侧正常.检索国内外医学数据库相关文献并复习. 结果患者行腹腔镜下左肾上腺切除术.术后病理标本切面中央见灰黑色细带,镜下示肾上腺皮质各带均增生、网状带细胞胞质内可见脂褐素颗粒.结合临床诊断为PPNAD.术后患者血压降至正常.目前仍在随访. 结论 PPNAD临床较罕见,其症状体征可不典型,影像学检查多无特异性,临床上极易误诊.由于该病部分可合并多发性内分泌肿瘤综合征,故需对其加强认识.
목적 탐토원발성색소성결절상신상선피질병(PPNAD)적림상표현급진치방법.방법 PPNAD환자1례,남성,52세.인혈압승고1년여입원.고흔정면용.혈、뇨피질순승고,대、소제량지새미송억제시험균불피억제.B초검사시쌍측신상선구미견종괴회성.CT검사좌측신상선가견1.6 cm×2.0 cm종괴,우측정상.검색국내외의학수거고상관문헌병복습. 결과 환자행복강경하좌신상선절제술.술후병리표본절면중앙견회흑색세대,경하시신상선피질각대균증생、망상대세포포질내가견지갈소과립.결합림상진단위PPNAD.술후환자혈압강지정상.목전잉재수방. 결론 PPNAD림상교한견,기증상체정가불전형,영상학검사다무특이성,림상상겁역오진.유우해병부분가합병다발성내분비종류종합정,고수대기가강인식.
Objective To investigate the clinical features and the management of primary pigmented nodular adrenocortical disease PPNAD) and to evaluate its relationship with Carney complex. Methods One case of PPNAD reported. The patient was a 52 years oldmale. He was hospitalized because of hypertension for one year. The patient had a Cushing's face with elevated plasma and urine cortisone levels which could not be suppressed by both low dose and high dose dexamethasone tests. Ultrasonography howed normal bilateral adrenal glands. CT scan found a 1.6 cm × 2.0 cm mass in the left adrenal gland and normal on tralateral adrenal gland. Results The patient had accepted left laparoscopic adrenalectomy. The pathological examination onfirmed the diagnosis of PPNAD. Micro scopic study showed that there were black-gray spots in the center of the specimen. Hyperplasia was found in all the three adrenal zones. Lipofuscin was observed in the cytoplasm of reticular zone cells. The patient's blood pressure had returned to normal level after the surgery. Conclusions PPNAD is a rare type of ypercortisolism. As there is no specific feature in clinical manifestation and radiological examination of this disease, it is very easy to make a misdiagnosis in clinical practice. PPNAD itself can be the comorbidity of Carney complex, careful differentiation is needed.