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首页> 外文期刊>Sao Paulo Medical Journal >Primary pigmented nodular adrenocortical disease associated with Carney complex: case report and literature review
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Primary pigmented nodular adrenocortical disease associated with Carney complex: case report and literature review

机译：原发性色素性结节性肾上腺皮质疾病与卡尼复杂体：病例报告和文献复习

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CONTEXT: Carney complex (CNC), a familial multiple neoplasm syndrome with dominant autosomal transmission, is characterized by tumors of the heart, skin, endocrine and peripheral nervous system, and also cutaneous lentiginosis. This is a rare syndrome and its main endocrine manifestation, primary pigmented nodular adrenal disease (PPNAD), is an uncommon cause of adrenocorticotropic hormone-independent Cushing's syndrome. CASE REPORT: We report the case of a 20-year-old patient with a history of weight gain, hirsutism, acne, secondary amenorrhea and facial lentiginosis. Following the diagnosing of CNC and PPNAD, the patient underwent laparoscopic bilateral adrenalectomy, and she evolved with decreasing hypercortisolism. Screening was also performed for other tumors related to this syndrome. The diagnostic criteria, screening and follow-up for patients and affected family members are discussed.
机译：背景：卡尼复合体（CNC）是一种以常染色体显性遗传为主的家族性多发性肿瘤综合症，其特征是心脏，皮肤，内分泌和周围神经系统的肿瘤，以及皮肤慢性变。这是一种罕见的综合征，其主要内分泌表现是原发性色素性结节性肾上腺疾病（PPNAD），是不依赖促肾上腺皮质激素的库欣综合征的罕见原因。病例报告：我们报告了一名20岁的患者，该患者有体重增加，多毛症，痤疮，继发性闭经和面部乳腺病的病史。诊断为CNC和PPNAD后，患者接受了腹腔镜双侧肾上腺切除术，并且随着皮质醇过多症的发展而逐渐发展。还对与该综合征相关的其他肿瘤进行了筛查。讨论了患者和受影响家庭成员的诊断标准，筛查和随访。

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