| BackgroundParagangliomas(PGL)are neuroendocrine tumors that originate from extraadrenal chromaffin tissue and secrete one or more catecholamines.Paragangliomas are thought to originate from parasympathetic and parasympathetic paraganglia.Paraganglioma usually occurs in the head and neck,retroperitoneum,abdomen,bladder and other parts.It is usually named according to the anatomical location of paraganglioma.Pheochromocytoma originating from retroperitoneum is called primary retroperitoneal paraganglioma.The initial symptoms and signs of primary retroperitoneal paraganglioma are mostly caused by excessive catecholamines secreted by tumors.However,its anatomical location is different from that of pheochromocytoma originating from adrenal gland.It is precisely because of its rarity and particularity that it is often misdiagnosed as retroperitoneal tumors of other nature,resulting in insufficient peritoneal treatment,especially before surgery.Preparing for the operation period may lead to serious complications during and after operation,such as excessive fluctuation of blood pressure before and during operation may lead to cerebral hemorrhage,and hypotension after operation may lead to cerebral infarction and myocardial infarction.Therefore,for primary retroperitoneal paraganglioma,which has a high rate of misdiagnosis and missed diagnosis and needs special preparedness during the perioperative period,how to reduce the misdiagnosis rate and improve perioperative preparation still need to be further explored and studied.ObjectiveTo discuss the diagnosis and treatment of primary retroperitoneal paraganglioma by analyzing the clinical features and treatment of primary retroperitoneal paraganglioma in the First Affiliated Hospital of Zhengzhou University in recent years.MethodA retrospective study was conducted on 42 cases of primary retroperitoneal paraganglioma confirmed by pathology in the First Affiliated Hospital of Zhengzhou University from November 2011 to November 2016.The epidemiology,etiology,clinical manifestations,imaging features,treatment methods,intraoperative conditions and prognosis of 42 cases of primary retroperitoneal paraganglioma were analyzed with relevant literature.The data were analyzed by SPSS22.0 software.Chi-square test was used for qualitative data,mean for non-normal distribution,Mann-Whitney U test was used for comparison between the two groups,P < 0.05 was statistically significant.ResultOf The incidence of primary retroperitoneal paraganglioma was higher in males than in females.Among 42 patients with primary retroperitoneal paraganglioma,17(17/42,40.48%)were females,25(25/42,59.52%)were males,and the ratio of females to males was 1:1.47.The age of the patients ranged from 4 to 77 years,with an average age of 44.67 + 13.73 years.Most of them were in urology: 33 cases(33/42,78.57%)were in urology,including 8 cases(8/42,19.05%)in hepatobiliary surgery and 1 case(1/42,2.38%)in vascular surgery.The proportion of patients in urology was higher than that in other departments.The misdiagnosis rate was high: the correct rate of preliminary diagnosis was only 54.76%,and the misdiagnosis rate was 45.24%.Among them,23 cases(23/42,54.76%)were initially diagnosed as retroperitoneal paraganglioma,13 cases(13/42,30.95%),2 cases(2/42,4.76%)were pancreatic tumors,2 cases(2/42,4.76%)were adrenal tumors,1 case(1/42,2.38%)was gastrointestinal stromal tumors,and 1 case(1/42,2.38%)was renal tumors.The symptoms were varied: headache/dizziness in 18 cases(18/42,42.86%),palpitation in 16 cases(16/42,38.10%),chest tightness in 8 cases(8/42,19.05%),panic in 10 cases(10/42,23.81%),fatigue in 5 cases(5/42,11.90%),blurred vision in 6 cases(6/42,14.29%),hypertension in 3 cases(3/42,7.17%),syncope in 1 case(1/42,2.38%).4 cases(4/42,9.52%)complained of low back pain,7 cases(7/42,16.67%)complained of abdominal pain,5 cases(5/42,11.90%)were found by physical examination and 1 case(1/42,2.38%)was admitted after trauma.Complicated symptoms: hypertension 23 cases(23/42,54.76%)diabetes 4 cases(4/42,9.52%),left kidney atrophy 1 case(1/42,2.38%),right kidney hydronephrosis 1 case(1/42,2.38%)and preoperative myocardial infarction 1 case(1/42,2.38%).Prevalence of tumors: 28 cases(28/42,66.67%)were located near the abdominal aorta(Zuckerkandl organ),5 cases(5/42,11.90%)near the inferior vena cava(inferior vena cava),5 cases(5/42,11.90%)around the kidney,3 cases(3/42,7.14%)behind the pancreas and duodenum,and 1 case(1/42,2.38%)at the lower pole of the kidney.Preoperative drug preparation and postoperative hemodynamic complications: 28 patients(28/42,66.67%)underwent preoperative drug preparation without any postoperative complications.Fourteen patients(14/42,33.33%)did not undergo preoperative drug preparation.One patient had cerebral infarction after operation.Four patients were sent to ICU because of the instability of hemodynamics after operation.All patients improved after conservative treatment.Treatment methods and surgical complications: 42 patients underwent surgical treatment,19 patients underwent open surgery,including 18 cases treated by laparoscopy(including retroperitoneal laparoscopy),3 cases treated by robotic Da Vinci,and 2 cases converted to open surgery.Among them,1 case had lymphatic leakage after laparoscopic treatment and improved after conservative treatment.42 cases were followed up,2 cases were lost,the follow-up time was 28-86 months,the average follow-up time was 52 months.Two patients died of multiple metastases of tumors 27 months and 34 months after operation,one patient died of cardio-cerebrovascular accident 19 months after operation,one patient had recurrence 12 months after operation,and one patient died of metastasis of tumors 17 months after operation.There was no significant difference in the prevalence and age of tumors between the functional group and the non-functional group(P > 0.05).The diameter of tumors in the non-functional group was significantly different(P = 0.037).The diameter of tumors in the non-functional group was larger than that in the functional group.The intraoperative maximum systolic pressure and blood pressure fluctuation were significantly different(P > 0.01).The intraoperative maximum systolic pressure and intraoperative blood pressure fluctuation were significantly higher than those in the functional group(P > 0.01).In non-functional group.Compared with the open group,there was no significant difference in the maximum systolic blood pressure,the maximum diameter of tumors and the fluctuation of blood pressure between the laparoscopic group and the open group(P > 0.05).There was no significant difference between preoperative preparation group and non-preoperative preparation group in maximum systolic blood pressure and blood pressure fluctuation(P > 0.05).Conclusion(1)According to the location of the tumors,CT or MRI should be highly alert when showing retroperitoneal tumors,especially those located at the distal end of the abdominal aorta from the beginning of the inferior mesenteric artery to the bifurcation of the abdominal aorta.Preoperative CTA or 3D imaging is helpful for preoperative evaluation of tumors.(2)Preoperative drug preparation is necessary.Although our study shows that intraoperative maximum systolic blood pressure and intraoperative blood pressure difference have nothing to do with preoperative preparation,it may be related to the function of tumors and the degree of intraoperative compression of tumors,or may lead to some research bias due to insufficient research samples.However,the preoperative drug preparation group can better prevent hemodynamic complications than the non-drug preparation group.(3)The maximum systolic blood pressure and intraoperative blood pressure fluctuation in the functional group were significantly higher than those in the non-functional group,while the diameter of tumors in the non-functional group was larger than that in the functional group.(4)There is no significant hemodynamic difference between laparoscopic surgery and open surgery.It is a better choice to use laparoscopic surgery in preoperative evaluation of those who can complete laparoscopic surgery. |
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