Recurrent paraneoplastic nephrotic syndrome; insights from a Lynch syndrome patient with multiple malignancies.

IF 1.8 4区 医学 Q3 GENETICS & HEREDITY
Myrthe A de Jong, Marije Slingerland, Lukas J A C Hawinkels, Maartje Nielsen, Augustinus S L P Crobach, Eveline S M de Jonge-Muller, Antonius J Rabelink, Alexandra M J Langers
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引用次数: 0

Abstract

Nephrotic syndrome is a common clinical presentation of glomerulopathy. A glomerulopathy as a paraneoplastic manifestation caused by underlying malignancy is rare. In patients with a solid tumor, membranous nephropathy is the most frequent paraneoplastic glomerulopathy. We present a case of recurrent paraneoplastic nephrotic syndrome caused by minimal change disease in a patient with Lynch syndrome. Over the years, a decrease in creatinine clearance and nephrotic-range proteinuria repeatedly functioned as a warning signal for underlying malignancies; consecutively, a colon adenocarcinoma, renal cell carcinoma and gastric adenocarcinoma were diagnosed. After treatment of the malignancies the nephrotic syndrome resolved without immunosuppressive therapy. Our patient also developed a primary lung carcinoma thrice, which did not cause an exacerbation of the minimal change disease. To further elucidate the mechanism behind the development of this phenomenon, we performed immunohistochemical analysis for vascular endothelial growth factor (VEGF) on the different tumor specimens. We found a high VEGF expression in the gastro-intestinal tumors, whereas the VEGF expression in the lung tumors was low, suggesting an association between VEGF expression and the development of paraneoplastic minimal change disease. This case report not only underlines the importance of considering a malignancy as a cause for (recurrent) nephrotic syndrome, especially in patients with an increased risk of developing malignancies like Lynch syndrome patients, but also suggests a role for VEGF in the pathogenesis of paraneoplastic minimal change disease.

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来源期刊
Familial Cancer
Familial Cancer 医学-遗传学
CiteScore
4.10
自引率
4.50%
发文量
36
审稿时长
6-12 weeks
期刊介绍: In recent years clinical cancer genetics has become increasingly important. Several events, in particular the developments in DNA-based technology, have contributed to this evolution. Clinical cancer genetics has now matured to a medical discipline which is truly multidisciplinary in which clinical and molecular geneticists work together with clinical and medical oncologists as well as with psycho-social workers. Due to the multidisciplinary nature of clinical cancer genetics most papers are currently being published in a wide variety of journals on epidemiology, oncology and genetics. Familial Cancer provides a forum bringing these topics together focusing on the interests and needs of the clinician. The journal mainly concentrates on clinical cancer genetics. Most major areas in the field shall be included, such as epidemiology of familial cancer, molecular analysis and diagnosis, clinical expression, treatment and prevention, counselling and the health economics of familial cancer.
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