Department of Pathology and Medical Biology, University Medical Center Groningen, Groningen, the Netherlands.
Department of Pathology and Medical Biology, University Medical Center Groningen, Groningen, the Netherlands.
Am J Kidney Dis. 2017 Apr;69(4):546-549. doi: 10.1053/j.ajkd.2016.09.027. Epub 2016 Dec 23.
A 52-year-old woman with widely disseminated medullary thyroid carcinoma developed nephrotic syndrome and slowly decreasing kidney function. A kidney biopsy was performed to differentiate between malignancy-associated membranous glomerulopathy and tyrosine kinase inhibitor-induced focal segmental glomerulosclerosis. Surprisingly, the biopsy specimen revealed diffuse glomerular deposition of amyloid that was proved to be derived from the calcitonin hormone (Acal), produced by the medullary thyroid carcinoma. This amyloid was also present in an abdominal fat pad biopsy. Although local ACal deposition is a characteristic feature of medullary thyroid carcinoma, the systemic amyloidosis involving the kidney that is presented in this case report has not to our knowledge been described previously and may be the result of long-term high plasma calcitonin levels. Our case illustrates that systemic calcitonin amyloidosis should be considered in the differential diagnosis of proteinuria in patients with medullary thyroid carcinoma.
一位 52 岁女性患有广泛播散性甲状腺髓样癌,出现肾病综合征和肾功能逐渐下降。进行肾活检以区分与恶性肿瘤相关的膜性肾小球病变和酪氨酸激酶抑制剂诱导的局灶节段性肾小球硬化症。令人惊讶的是,活检标本显示出弥漫性肾小球淀粉样物质沉积,经证实源自降钙素激素(Acal),由甲状腺髓样癌产生。这种淀粉样物质也存在于腹部脂肪垫活检中。虽然局部 Acal 沉积是甲状腺髓样癌的特征性表现,但本例报告中涉及肾脏的全身性淀粉样变性在我们的知识范围内尚未被描述过,可能是由于长期高血浆降钙素水平所致。我们的病例表明,在甲状腺髓样癌患者的蛋白尿鉴别诊断中应考虑全身性降钙素淀粉样变性。