| Objective:To retrospectively analyze the clinical data of 45 patients with pheochromocytoma and paraganglioma(PPGL)undergoing surgical treatment to summarize the clinical characteristics,diagnosis and treatment characteristics,and prognosis of PPGL.Methods:Collected the clinical case data of 45 patients who were diagnosed as PPGL patients through postoperative pathology in Dalian Central Hospital from January 2008to December 2020.Patients are divided into 2 groups according to the typical/atypical clinical symptoms:the typical symptom group,the clinical manifestations are"palpitations,headaches,hyperhidrosis,etc."caused by hypercatecholamineemia,and persistent/paroxysmal increases in blood pressure;symptoms Atypical group:no symptoms of hypercatecholamineemia and normal blood pressure.Record the patient’s general condition,clinical manifestations,blood pressure fluctuations,laboratory examinations,imaging examinations,treatment plans,pathological results,and prognostic follow-up data,and perform statistical analysis.SPSS 24.0 software was used for statistical analysis,and the comparison between groups of corresponding data types was carried out by using independent sample T test,non-parametric test,and chi-square test.P<0.05 means the difference is statistically significant.Results:(1)General conditions of patients:45 PPGL patients met the enrollment criteria,including 24 males(53.3%)and 21 females(46.7%),with an average age of 48.6±10.5years.Among them,patients aged 40-59 years have the largest number of cases(35cases),accounting for 77.8%of the total number of patients.A total of 7 patients with cardiovascular system abnormalities,with arrhythmia as the main manifestation;14patients with elevated blood sugar;2 patients with acute renal insufficiency;2 patients with lung infection;all improved after symptomatic treatment.(2)Clinical manifestations of patients:35 cases(77.8%)in the typical PPGL symptom group,of which 6 cases(13.3%)had the PPGL triad of"headache,palpitations,and hyperhidrosis",palpitations(20 cases),headaches(13)Excessive sweating(11 cases),the other manifestations may include vertigo,chest tightness,shortness of breath,abdominal pain,low back pain,nausea and vomiting,etc.;10 cases(22.2%)in the atypical symptom group are mild physical discomfort and no symptoms without requiring medical attention.A total of 28 patients(62.22%)had hypertension,with an average systolic blood pressure of 160.6±43.7 mm Hg and an average diastolic blood pressure of 95.2±23.9 mm Hg,including 16 cases(35.6%)of paroxysmal hypertension and 4 cases of persistent hypertension(8.9%),8 cases(17.8%)had paroxysmal exacerbations on the basis of persistent hypertension.There are even 12 patients who have experienced hypertensive crisis(4 cases of alternating hypertension and hypotension,8 cases of blood pressure exceeding 180/120mm Hg).(3)Laboratory examination results of patients:Compared with the typical group,there was no significant difference in the blood white blood cell,hemoglobin,liver function,kidney function,blood sugar,and ion level of the patients in the atypical group;24-hour urine vanillylmandelic acid(vanillylmandelic acid),VMA)was significantly lower(62.8±25.2umol/24h vs 145.1±39.4umol/24h,P<0.001).(4)Tumor location,size and relationship with symptoms:CT scans were performed for all patients,and the positive rate of localization diagnosis was 100%.There were 42 cases(93.3%)of adrenal tumors,including 2 cases(4.4%)on both sides and 23 cases on the left side.(51.1%),17 cases(37.8%)on the right side;3 cases(6.7%)with extra-adrenal tumors.Compared with the typical symptom group,the tumor diameter in the atypical symptom group was significantly larger(6.08±1.70cm vs 2.93±1.17cm,P<0.001),and the 24-hour urine VMA was significantly lower(62.8±25.2umol/24h vs 145.1±39.4umol/24h,P<0.001).(5)Treatment status:All patients underwent surgical treatment of PPGL,including 43 cases(95.56%)laparoscopic surgery and 2 cases(4.44%)open surgery.Statistics found that all patients(22 cases)who usedα-blockers before surgery could effectively reduce intraoperative blood pressure fluctuations(36.4%vs 73.9%,P=0.011),and reduce the chance of entering the ICU after surgery(4.5%vs 26.1)%,P=0.046).A subgroup analysis of patients in the atypical symptom group found that alpha-blockers were not used before surgery,the risk of blood pressure fluctuations during surgery was high(75%vs 0%),and the probability of entering ICU after surgery was high(12.5%vs0%).(6)Follow-up prognosis:A total of 35 patients in this study were followed up.A total of 3 cases developed distant metastases of PPGL(lung,bone,lymph nodes,etc.),and died after metastasis.The cause of death was multiple organ failure(2 cases).And cerebral hemorrhage caused by hypertension(1 case).During the follow-up,5 cases were diagnosed as hypertension again.There was no significant difference in prognosis between patients in the atypical symptom group and the typical group(P=0.632,χ~2=0.230).Conclusion:This study found that the age of onset of PPGL patients is mainly between40 and 59 years old,there is no gender difference,the specificity of clinical manifestations is low,and it is easy to be misdiagnosed and missed.Imaging examination is of great significance for tumor location and detection.Patients with atypical clinical symptoms have relatively large tumors.Regardless of whether the symptoms are typical or not,preoperative use ofα-blockers can effectively reduce intraoperative blood pressure fluctuations and reduce postoperative complications.The prognosis is also not related to symptoms.Therefore,clinically,patients should be actively screened for PPGL by imaging examination.After diagnosis,no matter whether the symptoms are typical or not,surgical resection should be adopted,and alpha blockers should be used before surgery to reduce the perioperative period.Complications can improve the quality of life of patients and prolong their survival. |
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