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Pheochromocytomas (PCC)/paragangliomas (PGL) are catecholamine (CA) -secreting neuroendocrine tumors, which are known as PPGL due to their histological and pathophysiological similarities. In addition to the typical triad of paroxysmal headache, palpitation, and sweating, PPGL may also be accompanied by symptoms and signs involving multiple organs and systems such as the cardiovascular system, digestive system, endocrine system, and nervous system. Currently, surgical resection is the first choice for PPGL. Safe and effective surgical management of complicated PPGL is the goal of clinical work. In this paper, we discuss this hot issue based on complicated PPGL cases, aiming to share our experience of the surgical management strategy of PPGL., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Wang, Zhao, Liao and Zhang.)

Pheochromocytoma/paraganglioma (PPGL) has a high genetic heterogeneity with 40% germline variants in known pathogenic genes. Data in Chinese on this aspect are scanty. To detect the genetic and clinical profile of Chinese PPGL patients, we examined the variants of 12 known germline pathogenic genes ( SDHA , SDHB , SDHC , SDHD , SDHAF2 , FH , VHL , RET , NF1 , MAX , TMEM127 , and KIF1B ) by next-generation sequencing and Sanger sequencing in 314 Chinese PPGL subjects. Twenty nine percent of Chinese PPGL patients had germline variants and SDHB was the most frequently mutated (14.6%). The most frequent SDHB variants were in exon 2, exon 7, and IVS 7. Pathogenic variants were more likely to occur in metastatic PPGL patients, paragangliomas, and patients under 30, with the ratio being 50.7% (35/69), 35.9% (56/156), and 49.5% (52/105), respectively. Our cohort included 314 patients from a single setting. The genetic and clinical features of Chinese PPGL patients were unique in some aspects compared to their non-Chinese counterparts. Identification of genotype-phenotype relation can serve as an effective tool for genetic prioritization and clinical decision-making., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2020 Ma, Li, Tong, Wang, Cui, Zhang, Zhang, Chen and Li.)

Objectives: To explore the feasibility and safety of retroperitoneal laparoscopic resection of paraganglioma (RLPG) in a large study population., Methods: In a six-year period, 49 patients with primary retroperitoneal paragangliomas (PG) underwent retroperitoneal laparoscopic surgery in a single center. Medical records were reviewed, and collected the following data, which were clinical characteristics, perioperative data (operative time, estimated blood loss, intraoperative hemodynamic changes, intraoperative and postoperative complications, and open conversions), and follow-up data (recurrence or distant metastases)., Results: All PGs were removed with negative tumor margin confirmed by postoperative histopathology. The operative time of RLPG was 101.59±31.12 minutes, and the estimated blood loss was 169.78±176.70ml. Intraoperative hypertensive and hypotensive episodes occurred in 25 cases and 27 cases, respectively. Two open conversions occurred. Two intraoperative complications occurred but were successfully managed endoscopically. Postoperative complications were minor and unremarkable. No local recurrence or distant metastasis were observed during the follow-up period., Conclusions: Our experience indicates the feasibility and safety of resection of PGs in a relatively large study population.

Objectives: To compare transumbilical laparoendoscopic single-site surgery with conventional laparoscopy for the resection of retroperitoneal paragangliomas., Methods: In the present case-control study, we compared 11 transumbilical laparoendoscopic single-site surgery procedures with 22 conventional laparoscopy procedures carried out to treat retroperitoneal paragangliomas between June 2004 and October 2013 at Peking Union Medical Hospital, Beijing, China. Operative time, estimated intraoperative blood loss, blood transfusions, intraoperative hypertension, intraoperative hypotension, highest intraoperative systolic blood pressure, lowest intraoperative systolic blood pressure, postoperative fatigue time, postoperative systematic inflammation reaction syndrome, 24-h postoperative visual analog scale scores, postoperative hospital stay duration, and postoperative complications were recorded and analyzed., Results: There were no significant differences in patient characteristics. All surgical procedures were successfully completed in both groups. Minor complications occurred in one case in the laparoendoscopic single-site surgery group and in two cases in the conventional laparoscopy group. In the laparoendoscopic single-site surgery group, the operative time was longer than that in the conventional laparoscopy group (P = 0.001). There were no significant differences in terms of intraoperative hypertension, intraoperative hypotension, highest intraoperative systolic blood pressure, lowest intraoperative systolic blood pressure, estimated blood loss, postoperative fatigue time, postoperative hospital stay or systematic inflammation reaction syndrome between the two groups. The 24-h postoperative visual analog scale score was lower in the laparoendoscopic single-site surgery group (P = 0.002). No recurrence or metastases were observed over the duration of the postoperative follow up., Conclusions: In properly selected patients, transumbilical laparoendoscopic single-site surgery is a feasible, safe and effective procedure for the surgical treatment of retroperitoneal paragangliomas., (© 2015 The Japanese Urological Association.)

We aimed to compare the safety and patient outcomes of retroperitoneal paraganglioma (PG) following the retroperitoneoscopic and transperitoneoscopic approaches based on large samples.Seventy-four patients with retroperitoneal PG undergoing laparoscopic resection from June 2004 to September 2013 were retrospectively included. The patients were divided into the retroperitoneal (n = 40) and transperitoneal (n = 34) groups. Demographic and perioperative data, including the operation time, estimated blood loss, incidence of intraoperative hypertension, bowel recovery day, postoperative hospital stay, and systemic inflammatory response syndrome (SIRS) were recorded.The retroperitoneal group showed a shorter operation time and earlier postoperative exsufflation time compared with the transperitoneal group (84 ± 28.5 minutes vs 115 ± 35.7 minutes and 1.7 ± 0.6 vs 2.3 ± 0.7 day, respectively; both P < 0.001). No significant differences in the baseline data were observed between 2 groups. All patients, except for 1 case of open conversion, underwent laparoscopic surgery. There were no patient deaths. Data analysis demonstrated no significant difference in the surgical blood loss, incidence of surgical blood pressure elevation, postoperative hospital stay, or incidence of SIRS between 2 groups.The operation time for the retroperitoneoscopic resection of retroperitoneal PG is shorter, and gastrointestinal functions improve more quickly compared to the transperitoneoscopic approach. This study may provide a valuable source of clinical information for clinicians in related fields.