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十二指肠神经鞘瘤伴发膜性肾病罕见病例报告。

Duodenal Schwannoma as a Rare Association With Membranous Nephropathy: A Case Report.

机构信息

Department of Medicine, University of Hawaii John A. Burns School of Medicine, Honolulu, HI.

Brown School of Public Health, Washington University in St. Louis, MO.

出版信息

Am J Kidney Dis. 2019 Feb;73(2):278-280. doi: 10.1053/j.ajkd.2018.09.003. Epub 2018 Nov 16.

DOI:10.1053/j.ajkd.2018.09.003
PMID:30454884
Abstract

Membranous nephropathy (MN) associated with malignancies is a well-known entity. However, its association with benign neoplasm is not broadly recognized. A 69-year-old man with recurrent nephrotic syndrome presented with pedal edema and proteinuria of 5 months' duration. Laboratory results showed hypoalbuminemia and hyperlipidemia. Proteinuria was estimated to be protein excretion of 3.5g/d. Studies were negative for viral hepatitis, syphilis, human immunodeficiency virus, autoimmune diseases, and paraproteinemia. Kidney biopsy disclosed MN with negative phospholipase A receptor (PLAR) staining, favoring a secondary form of MN. Computed tomography detected a 7.6-cm duodenal schwannoma. Elective surgical resection was performed. Pathologic study showed that THSD7A (thrombospondin type 1 domain-containing 7A) was positive in both glomeruli and schwannoma. Commonly, secondary MN is related to underlying conditions, including lupus, hepatitis, and neoplasm, and can be medication induced. The risk for developing a concomitant neoplasm among patients with PLAR-negative MN is up to 12 times higher than in the general population. Most of these neoplasms are malignancies, and the presence of autoantibodies directed at similar tissue targets is hypothesized as the potential mechanism. In our case, THSD7A may be the autoantibody that has linked the schwannoma and the development of MN. Although benign tumors rarely produce renal manifestations, effective treatment may lead to resolution of nephrotic syndrome.

摘要

膜性肾病(MN)与恶性肿瘤相关是一个众所周知的事实。然而,它与良性肿瘤的关联并未被广泛认识。一名 69 岁男性因复发性肾病综合征就诊,表现为足肿和 5 个月的蛋白尿。实验室结果显示低白蛋白血症和高脂血症。蛋白尿估计为 3.5g/d 的蛋白排泄量。研究排除了病毒性肝炎、梅毒、人类免疫缺陷病毒、自身免疫性疾病和副蛋白血症。肾脏活检显示 MN 伴阴性磷脂酶 A 受体(PLAR)染色,提示为继发性 MN。计算机断层扫描发现一个 7.6cm 的十二指肠神经鞘瘤。行择期手术切除。病理研究显示,THSD7A(血栓素 A 型 1 结构域包含 7A)在肾小球和神经鞘瘤中均为阳性。通常,继发性 MN 与潜在疾病有关,包括狼疮、肝炎和肿瘤,并可能由药物引起。PLAR 阴性 MN 患者并发肿瘤的风险比普通人群高 12 倍。这些肿瘤大多数为恶性肿瘤,推测针对类似组织靶标的自身抗体是潜在机制。在我们的病例中,THSD7A 可能是将神经鞘瘤和 MN 联系起来的自身抗体。尽管良性肿瘤很少引起肾脏表现,但有效的治疗可能会导致肾病综合征的缓解。

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