biostudies-literatureUnknown5(6)Yamaguchi Y<h4>Introduction</h4><i>SDH</i> Gene mutation is known to be a common cause of pheochromocytoma/paraganglioma and renal cell carcinoma. Here, we report a case of succinate dehydrogenase B-deficient paraganglioma, which has a high risk of metastasis and recurrence, complicated by succinate dehydrogenase-deficient renal cell carcinoma, which is rare and accounts for approximately 0.1% of all renal cell carcinomas.<h4>Case presentation</h4>A 50-year-old man underwent en bloc resection of a retroperitoneal tumor and the right kidney for retroperitoneal paraganglioma and right renal tumor. Both tumors showed negative expressions of succinate dehydrogenase B in immunostaining. The patient was diagnosed with succinate dehydrogenase-deficient paraganglioma and succinate dehydrogenase-deficient renIJU case reports480-483https://www.ebi.ac.uk/biostudies/studies/S-EPMC9626355biostudies-literatureSuccinate dehydrogenase-deficient malignant paraganglioma complicated by succinate dehydrogenase-deficient renal cell carcinoma.PMC9626355Tanaka HMatsuoka YUehara SYoshida SFujii YNakamura YTakemoto AFukuda SYokoyama MYamaguchi YfalseSuccinate dehydrogenase-deficient malignant paraganglioma complicated by succinate dehydrogenase-deficient renal cell carcinoma.<h4>Introduction</h4><i>SDH</i> Gene mutation is known to be a common cause of pheochromocytoma/paraganglioma and renal cell carcinoma. Here, we report a case of succinate dehydrogenase B-deficient paraganglioma, which has a high risk of metastasis and recurrence, complicated by succinate dehydrogenase-deficient renal cell carcinoma, which is rare and accounts for approximately 0.1% of all renal cell carcinomas.<h4>Case presentation</h4>A 50-year-old man underwent en bloc resection of a retroperitoneal tumor and the right kidney for retroperitoneal paraganglioma and right renal tumor. Both tumors showed negative expressions of succinate dehydrogenase B in immunostaining. The patient was diagnosed with succinate dehydrogenase-deficient paraganglioma and succinate dehydrogenase-deficient ren2022-01-01T00:00:00Z2022 Nov2025-04-27T01:47:36.368Z2025-04-06T18:19:44.033ZS-EPMC96263553634117910.1002/iju5.12520