我们描述了一例继发于转移性肾细胞癌、表现为顽固性腹泻的系统性 AA 淀粉样变性的尸检病例。严重腹泻是诊断AA型淀粉样变性的主要症状。没有表现出继发于肾细胞癌的 AA 淀粉样变性中常见的肾脏症状，因为双侧肾切除术后的血液透析已在 9 年前开始。由于身体状况不佳，无法进行以 AA 淀粉样变性为解决方案的转移性肿瘤治疗，患者在诊断后 5 个月死亡。尸检结果显示，在包括肠道在内的多个器官中都发现了 AA 淀粉样蛋白沉积。转移性肿瘤在组织学上与肾细胞癌的转移相一致。没有其他原因导致慢性炎症，例如炎症性关节炎。我们得出的结论是，肾细胞癌转移性肿瘤引起的慢性炎症是系统性 AA 淀粉样变性的主要原因。肠 AA 淀粉样变性伴吸收不良是死亡原因。临床医生应记住，实体器官恶性肿瘤可能是 AA 淀粉样变性的原因，而肾细胞癌是最常见的癌原因。该病例特别具有启发性，因为无尿的血液透析患者可能会错过淀粉样变性的进展，而胃肠道症状可能是系统性淀粉样变性的主要指标。包括活检在内的内镜检查对于淀粉样变性的诊断和早期治疗非常重要。© 2023 作者。由巴塞尔 S. Karger AG 出版。

We describe an autopsied case of systemic AA amyloidosis secondary to metastatic renal cell carcinoma presenting intractable diarrhea. Severe diarrhea was the major symptom for the diagnosis of AA amyloidosis. No renal symptoms which are common in AA amyloidosis secondary to renal cell carcinoma were shown because hemodialysis following bilateral nephrectomy had already been started 9 years before. Treatment against metastatic tumors as a solution of AA amyloidosis could not be performed because of bad performance status and the patient died 5 months after the diagnosis. Autopsy findings revealed that AA amyloid deposition was seen in multi-organs including the intestine. The metastatic tumors were histologically compatible as metastasis of renal cell carcinoma. There was no other cause of chronic inflammation such as inflammatory arthritis. We concluded that chronic inflammation provoked by the metastatic tumors of renal cell carcinoma was a major cause of systemic AA amyloidosis. Intestinal AA amyloidosis with malabsorption was the cause of death. Clinicians should keep it in mind that solid organ malignancy can be a cause of AA amyloidosis and renal cell carcinoma is the most common carcinomatous cause. This case is particularly instructive in that progression of amyloidosis may be missed in hemodialysis patients with anuria and that gastrointestinal symptoms can be the primary indicators of systemic amyloidosis. Endoscopic examination including biopsy is important for the diagnosis and early treatment of amyloidosis.© 2023 The Author(s). Published by S. Karger AG, Basel.