| Objective Analyze the clinical manifestations,diagnostic methods,intraoperative blood pressure and follow-up of pheochromocytoma / paraganglioma(PPGL),explore the influencing factors of PPGL diagnosis and treatment,improve clinicians’ awareness of PPGL,and reduce misdiagnosis and mortality.Methods Retrospective analysis of the medical records of 141 patients diagnosed with PPGL admitted to the General Hospital of Tianjin Medical University from January2015 to December 2019.The main clinical manifestations,related biochemical and imaging examinations,treatment plans,and follow-up were analyzed.The preoperative examination,preoperative medication,and intraoperative blood pressure were compared between the small diameter group and the large diameter group,the typical symptom group and the atypical symptom group.Results The 141 patients with PPGL were divided into 62 males(44%)and 79 females(56%),male: female = 1: 1.27.The average time from onset to treatment was 6months.The age of visit was(49.98±14.53)years.119 patients were first diagnosed in urology,7 were first diagnosed in vascular surgery,5 were first diagnosed in endocrinology,and 4 were first diagnosed in hepatobiliary and pancreatic surgery,3were first diagnosed in cardiology,and the remaining cases were first diagnosed in neurology,otolaryngology,head and neck surgery,and other departments.67 patients had no clinical symptoms,46 patients had at least one of headache,palpitations,and sweating at the time of onset,and the remaining symptoms were diverse and complicated.PPGL patients have the highest proportion of patients with hypertension,followed by diabetes.Tumors most commonly located in the adrenal glands and extra-adrenal tumors most commonly located in the abdominal cavity.The tumor diameter of PPGL patients ranged from 0.8cm to 25 cm,with an average diameter(5.35±3.33)cm.There were 34 cases with tumor diameter ≤3cm,70 cases with 3cm<diameter ≤6cm,and 37 cases with tumor diameter> 6cm.106 patients underwent urinary vanillyl mandelic acid examination,50 were above the upper limit of normal;128 patients had blood potassium examination,and14 patients had a lower potassium limit than normal;Supine aldosterone and renin were examined in 87 patients,aldosterone in 19 patients was higher than the normal upper limit,renin in 31 patients was higher than the normal upper limit.Cortisol and adrenocorticotropic hormone were examined in 90 patients,cortisol in 45 patients were higher than the normal upper limit,adrenocorticotropic hormone in 26 was higher than the normal upper limit,urinary cortisol was higher than the normal upper limit in 10 patients.Plasma lipoprotein phospholipase A2 in 19 patients was higher than the normal upper limit of all 32 patients.All 4 patients’ blood and urine MNs were positive.19 patients underwent adrenal CT examination,the positive rate was100%,45 underwent abdominal CT,with a positive rate of 97.8%,38 did MRI,with a positive rate of 100%,88 underwent abdominal ultrasound,with a positive rate of93.2%.13 patients underwent PET-CT,and the positive rate was 100%.141 patients had pathological results,and Ki-67 positive rates of 96% patients were described≤10%.Syn in 115 cases and Cg A in 128 case were all positive.57 Melan A,42 Calretinin,35 EMA,33 CD56 were described negative.In all 97 cases’ S-100,92 patients were positive.35 CK were negative in 38 cases.92 patients received alpha blockers treatment before surgery,of which 71 took terazosin or doxazosin,10 took phenoxybenzamine,11 took both phenoxybenzamine and doxazosin.42 patients had intraoperative hypertension,and the mean systolic blood pressure during the operation was 215 ± 35 mm Hg.Postoperative follow-up was performed on 138 patients.7 patients(5.0%)had recurrence after surgery and returned to hospital for treatment.1 patient(0.7%)died of tumor metastasis and 3patients were lost to follow-up.The patients were divided into different groups,the typical group was more likely to have complications(diabetes,coronary heart disease,cerebrovascular disease,etc.)than the atypical group(P <0.05);the typical group was more likely to use alpha blockers before surgery than the atypical group(P <0.01).The large diameter group were more prone to have intraoperative blood pressure fluctuations,and the fluctuation values and the percentage of alpha blockers used before surgery were higher than those in the small diameter group(P <0.05).Conclusion The most common first contact department for PPGL patients is urology,and the most common reason for visiting patients is adrenal incidentalomas and other parts’ tumours.In addition to hypertension,headaches,palpitations,and sweating,there are many atypical symptoms such as dizziness,blurred vision,abdominal pain,nausea,and fatigue in PPGL patients.PPGL patients have the complex and diverse clinical symptoms,if patients with the above symptoms are accompanied by adrenal incidentalomas at same time,clinicians should be alert to the occurrence of PPGL.。Patients with small tumor diameter always have atypical symptoms,low biochemical test positive rate,and are difficulty in identifying,which requires more attention from clinicians.Clinicians should develop individualized follow-up plans for patients with genetic syndrome to reduce the chances of recurrence and metastasis.It is suggested that urology strengthen multi-disciplinary cooperation with endocrinology and other departments to implement more accurate medical decisions for PPGL patients. |
