| Objective:Adrenal medullary tumors have a low incidence rate,and their clinical manifestations are complex and diverse,which is prone to misdiagnosis or missed.Adrenal pheochromocytoma is a rare tumor originating from adrenal medulla pheochromocyte,which is essentially a syndrome characterized by high blood pressure caused by the secretion of catecholamines.Ganglioneuroma is considered to be a benign tumor originating from neural crest cells,including sympathetic ganglia and adrenal medulla,involvement of the adrenal is rare.The clinical data of pheochromocytoma and adrenal ganglioneuroma were analyzed in order to improve the understanding of them and provide a reference for the perioperative treatment safety in this study.Methods:Retrospectively analyzed demographic,clinical manifestations,biochemical examination,imaging examination,surgical treatment,pathology and postoperative follow-up data and other relevant information of patients,those with adrenalectomy and pathologically confirmed adrenal pheochromocytoma or adrenal ganglioneuroma at Shandong Provincial Hospital Affiliated to Shandong University from January 2013 to January 2018.All data were analyzed using SPSS 22.0 software.Paired t test was used to compare the endocrine indexes of pheochromocytoma and adrenal ganglioneuroma as well as the preoperative and postoperative blood pressure of each group.P<0.05 was considered statistically significant.Results:1.A total of 93 patients were included in this study,including 73 patients with pheochromocytoma(42 males and 31 females)and 20 patients with adrenal ganglioneuroma(11 males and 9 females).Pheochromocytoma accounted for 4.72%of the patients admitted to our hospital due to adrenal diseases(1545 cases),adrenal ganglioneuroma accounted for 1.29%.The clinical manifestations of pheochromocytoma varied greatly,mainly including hypertension(51 cases,69.86%),headache/dizziness(47 cases,64.38%),palpitation(24 cases,32.88%),and no performance in 14 cases(19.18%).Most of the patients with adrenal gangliocytoma had no clinical manifestations(10 cases,50%),except for hypertension(5 cases,25%)and waist and abdominal discomfort(4 cases,20%).2.Among the 73 patients with pheochromocytoma,52 cases were tested for plasma Adrenaline(AD)and Noradrenaline(NAD).The sensitivity of plasma AD and NAD was respectively 51.92%and 82.69%,and the overall sensitivity of plasmacatecholamine(AD or NAD)was 86.54%.38 cases were tested for Metanephrine(MN)and Normetanephrine(NMN).The sensitivity of plasma MN and NMN was respectively 94.73%and 89.47%,and the overall sensitivity of plasma MNs(MN or NMS)was 97.37%.59 patients underwent Vanillylmandelic Acid(VMA)detection with a sensitivity of 62.71%.Among the 20 patients with adrenal ganglioneuroma,16 patients underwent plasma AD and NAD detection.The plasma AD and NAD test results were higher than the upper limit in 2 patients(12.50%)and 2 patients(12.50%),and plasma catecholamine(AD or NAD)overall results were higher than the upper limit in 3 cases(18.75%).7 cases were tested plasma MN and NMN,and the test results were all within the normal range.Urine VMA test results were higher than the normal upper limit in 2 cases(15.38%).The test results of the two groups were expressed as mean± standard deviation,and the t-test showed that the difference was statistically significant(p<0.01).The adrenal medulla hormone in patients with pheochromocytoma was significantly higher than that in patients with adrenal ganglioneuroma.3.According to all preoperative imaging examinations,among the 73 patients with pheochromocytoma,63 patients’(86.30%)preoperative imaging examinations were consistent with postoperative pathology.Among the 20 cases of adrenal ganglioneuroma,16 cases’,(80.00%)preoperative imaging examination were consistent with postoperative pathology.4.All patients with pheochromocytoma before surgery were treated with strict preoperative preparation.Preoperatively considered patients with adrenal ganglioneuroma,if the preoperative examination meets the following one of three points:1)hypertensive;2)can secrete catecholamine;3)preoperative imaging examination found that the tumor diameter was larger than 4cm and pheochromocytoma could not be excluded,strict preoperative preparation was required according to pheochromocytoma.All patients underwent transabdominal laparoscopic approach,and none of them was transferred to open surgery.5.Immunohistochemical analysis was performed in 61 of 73 patients with pheochromocytoma.The positive rates of immunohistochemistry were CgA(57 cases,93.44%),CD56(57 cases,93.44%),ki-67<1(55 cases,90.16%),Syn(53 cases,86.88%),s-100(52 cases,85.25%),NSE(50 cases,81.96%)and Vimentin(2 cases,3.28%).Immunohistochemical analysis was performed in 14 of 20 patients with adrenal ganglioma.The positive rates of immunohistochemistry were s-100(14 cases,100.00%),NSE(10 cases,71.43%),Vimentin(4 cases,28.57%),CgA(4 cases,28.57%),Syn(2 cases,1429%),CD56(2 cases,14.29%)and ki-67<1(0 cases,0%).6.51 patients with pheochromocytoma had preoperative hypertension.After a median follow-up period of 40 months,the difference in preoperative and postoperative blood pressure was statistically significant(p<0.01),and postoperative blood pressure was lower than that before surgery.The number and proportion of cured,relieved and ineffective patients were 31 cases(60.78%),19 cases(37.25%)and 1 case(1.96%),respectively.During the follow-up,1 patient experienced recurrence at 23 months after surgery.No patients developed metastasis.5 patients with preoperative hypertension in patients with adrenal ganglioneuroma.Preoperative and postoperative blood pressure showed statistically significant difference(p<0.01),and postoperative blood pressure was lower than preoperative blood pressure.All 5 patients with hypertension were cured or relieved.No patients had recurrence or metastasis during follow-up.Conclusion:1.Adrenal medullary tumor has a low incidence rate,which is prone to misdiagnosis or missed diagnosis.2.Imaging examination is of great significance in the localization and qualitative diagnosis of adrenal medulla tumors,among which CT and MRI are the preferred means of examination.The level of adrenal medulla hormone in patients with pheochromocytoma was significantly higher than that in patients with adrenal ganglioneuroma.Among them,plasma MNs is a sensitive index for screening pheochromocytoma.3.Preoperative preparation for adrenal medullary tumors varies.All patients with pheochromocytoma required adequate preoperative antihypertensive dilatation.If the preoperative examination meets the following one of three points:(1)hypertension;(2)can secrete catecholamine;(3)preoperative imaging examination showed that the tumor diameter was larger than 4cm and pheochromocytoma could not be excluded,strict preoperative preparation was required according to pheochromocytoma.4.CgA,Syn and NSE were highly expressed in pheochromocytoma.S-100 and NSE were highly expressed in adrenal ganglioneuroma.5.Laparoscopic adrenalectomy has become the standard treatment for adrenal medullary tumors,and tumor size is no longer a contraindication for experienced surgeons. |
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