A case report of right retroperitoneal paraganglioma with Mayo grade Ⅳ inferior vena cava tumor thrombus

VernacularTitle:右侧腹膜后副神经节瘤合并Mayo Ⅳ级下腔静脉瘤栓1例报告
Author: Yongquan WANG ¹ ; Cong WANG ¹ ; Ling WEI ¹ ; Jun ZHENG ¹ ; Jian FU ¹ ; Linfei LI ¹
Author Information

1. 陆军军医大学第一附属医院泌尿外科，重庆　400038
Publication Type:Journal Article
Keywords: Paraganglioma; Tumor thrombus in inferior vena cava; Reconstruction of the inferior vena cava; SDHB gene germline mutation
From: Chinese Journal of Urology 2025;46(6):462-463
CountryChina
Language:Chinese
Abstract: Paraganglioma（PGL）is an extra-adrenal catecholamine-secreting endocrine tumor. We reported a young female patient who was admitted to the hospital with right flank pain and diagnosed with right retroperitoneal PGL with Mayo Ⅳ inferior vena cava tumor thrombus（double tumor thrombus）. After multidisciplinary consultation，right retroperitoneal PGL resection，right nephrectomy，inferior vena cava Mayo Ⅳ tumor thrombus resection and reconstruction were performed. Postoperative pathology showed that the primary tumor and inferior vena cava tumor thrombus was consistent with PGL. Genetic testing revealed a heterozygous germline nonsense mutation at c.69_70delinsTT（p.Q24*）locus of the SDHB gene in the patient. His mother was a carrier of the gene，but his father and sister did not carry the mutation. The patient recovered smoothly after surgery.