Objective To investigate the surgical treatment of severe ectopic adrenocorticotrophic hormone(ACTH) syndrome.Methods The clinical data of 12 cases of severe ecotopic ACTH syndrome from January 1996 to December 2016 were retrospectively analyzed.The cases include 7 males and 5 females with a mean age 38 years(range 11 to 64 years).12 cases demonstrated typical Cushing syndrome(CS),accompanied by hypertension, diabetes, hypokalemia and severe osteoporosis.9 cases were complicated with thoracolumbar compression fractures.8 cases presented with pulmonary infection and fever during operation.7 cases had cardiac dysfunction.Laboratory tests showed elevated serum cortisol(695.0 ～ 1 661.5 nmol/L,mean 1 055.7 nmol/L), high urinary free cortisol excretion (807.3 ～ 28 240.0 nmol/24 h, mean 5 270.5 nmol/24h)and high ACTH plasma levels(16.5 ～ 273.9 pmol/L, mean 80.4 pmol/L).Source of ectopic ATCH were not identified and CT showed bilateral enlargement of adrenal in 12 patients.Results We performed emergency bilateral or unilateral adrenalectomy in 12 cases.Among them, one-stage bilateral adrenalectomy were given to 6 cases, staged bilateral adrenalectomy was performed in 3 cases, and unilateral adrenalectomy were given to 3 cases.The patients were treated with hormone replacement therapy after bilateral adrenalectomy.The excised adrenal gland showed diffuse thickening and multiple nodular.Pathological diagnosis were adrenal cortical hyperplasia.They have been followed up for 1 to 8years(median 2.5 years), 9 cases survived, 2 cases died of diabetes and severe pulmonary infection, and 1 case was lost to follow-up.Conclusions Severe ectopic ACTH syndrome is difficult to treat.Emergency adrenalectomy is effective for the management of severe ectopic ACTH syndrome especially for those patients with severe Cushing syndrome but primary tumor can not be located.

Objective: To investigation the diagnosis and treatment of ectopic adrenocorticotrophic hormone (ACTH) syndrome.Methods: The clinical characters of 57 cases of ecotopic ACTH syndrome from Jan.1996 to Dec.2016 were collected and analyzed.The 57 cases included 32 males and 25 females.The age ranged from 11 to 68 years (average 32 years).ACTH levels significantly increased from 16.5 to 365.6 pmol/L, with average 77.6 pmol/L (normal range <10.1 pmol/L).The pituitary MRI did not found lesions.The CT showed that their bilateral adrenal glands diffused small nodular changes or nodular hyperplasia.The 57 cases were divided into 3 groups according to different treatment options.In the study, 25 ectopic ACTH syndrome cases (44%) were group A, without identified source of ectopic hormone, were treated with bilateral or unilateral adrenalectomy due to the severity of the disease and difficulty of operation.Group B was composed of 16 cases (28%) diagnosed as ectopic ACTH syndrome by finding ectopic ACTH tumors and surgical resection.Group C included 16 cases (28%) with nonsurgical therapy.Different treatment results and prognosis were analyzed.Results: In the study, 40 cases of the 57 had been followed up for 6 months to 10 years.In group A, of the 25 cases with bilateral or unilateral adrenalectomy, 4 died of diabetes and severe pulmonary infection, 18 survived, and 3 were lost to the follow-up, and the survival rate was 81% (18/22).In group B, of the 16 cases with radical tumor resection, 5 died of tumor recurrence 0.5-6.0 years after operation, 3 survived, and 8 were lost to the follow-up, and the survival rate was 37.5% (3/8).In group C, of the 16 non-operation patients, 4 with radiotherapy and chemotherapy died of metastases, diabetes or pulmonary infection, 6 with chemotherapy died of pulmonary infection within 1 year and the others were lost to the follow-up, and the survival rate was 0.Conclusion: Ectopic ACTH syndrome is difficult to treat.Adrenalectomy is effective for the management of ectopic ACTH syndrome, especially for those patients with severe Cushing''s syndrome, but the primary tumor can not be located.

Objective To investigate the operative indication,therapeutic effects and feasibility of laparoscopic bilateral cortical-sparing adrenalectomy for pheochromocytoma.Methods The clinical data of 9 cases of bilateral pheochromocytoma from Jan.2011 to Dec.2016 were retrospectively analyzed.The cases included 7 males and 2 females with a mean age of 40 years old,ranging from 22 to 58.CT showed all the 9 cases had bilateral adrenal tumors.Results All patients accepted laparoscopic bilateral cortical-sparing adrenalectomy for pheochromocytoma.All survived and had been followed up for 4 months to 6 years (median 3.2 years).All cases kept normal adrenal function and avoid long-term corticosteroid dependence.Conclusions Bilateral pheochromocytoma is rare and difficult to treat.Adrenal function protection under laparoscopic bilateral cortical-sparing adrenalectomy for pheochromocytoma is safe and effective for patients.This opertation can both completely resect bilateral adrenal tumors and avoid long-term corticosteroid dependence.

Objective To explore the clinicopathologic features,diagnosis,treatment and prognosis of ACTH-independent subclinical Cushing syndrome caused by cortisol-secreting and hypo-androgen black adrenal cortical adenomas with lipomatous metaplasis.Methods The clinical and pathologic features of 1 patient presented with hypo-androgen and atypical signs and symptoms of Cushing syndrome were reported.The patient's 24 h UFC (235.62 μg) was higher than normal range,his plasma ACTH concentrations (＜1.1 pmol/L,was lower than normal range and his serum cortisol concentrations lacked diurnal rhythm.There was absence of serum cortisol suppression in overnight dexamethasone suppression tests.Serum levels of androgens (1.2 nmol/L) were lower than normal range.24 h urine catecholamine was normal,NE was 160.5 nmol/24 h (5-591 nmol/24 h),E was 23.1 nmol/24 h (0-82 nmol/24 h),DA was 1496nmol/24 h (424-2612 nmol/24 h).Aldosterone lying and standing test:ALD1 138-415 pmol/L,AT-Ⅱ1 64.54 ng/L,PRA1 0.80 μg · L-1 · h-1 Abdomen CT showed right adrenal mass (3.1 cm).131Ⅰ-MIBG scanning was normal.Adrenocortical carcinoma was considered as a possible diagnosis based on cortisol hypersecretion,hypo-androgen and the imaging characteristics.This patient accepted laparoscopic adrenal adenoma resection.Results The adrenal tumor size was 3.5 cm.Immunohistochemical (IHC) staining showed Melan-A,CgA,α-inhibin and Syn positive and Ki-67 ＜ 1％.Histopathologic results showed black adrenal cortical adenomas with myelolipomatous change.After surgery,patient's serum androgen was normal,and the signs and symptoms of Cushing syndrome and oligo-androgen disappeared.There was recurrence after 1.5 years follow-up.Conclusions Although most patients with corticotropin-independent Cushing syndrome associated with abnormal androgen proved to have adrenocortical carcinoma,the clinician should be aware of the possibility of benign,black adrenal adenoma.

Objective: To review and discuss the experience of diagnosis and treatment of adrenal ganglioneuroma. Methods: Clinical data of 80 cases of adrenal ganglioneuroma undergoing surgery during January 1982 to May 2017 at Peking Union Medical College Hospital were retrospectively analyzed. There were 36 male and 44 female patients. Age ranged from 8 to 69 years old (mean 37.7 years old). The tumor diameter were 1.5 to 18.0 cm (mean 4.3 cm). There were 61 cases of adrenal ganglioneuroma diagnosed by imaging examination. All patients underwent adrenalectomy. Results: All procedures were performed successfully without serious complication. Including 15 open surgery and 64 retroperitoneal laparoscopic surgery. One laparoscopic case conversion to open surgery. The pathological diagnosis was adrenal ganglioneuroma. Fifteen cases were mixed with other tumor components. No recurrence or metastasis was observed during the follow-up of 2 months to 35 years. Conclusions The diagnostic techniques for adrenal ganglioneuroma are helpful to judge the tumor property before operation. The laparoscopic surgery has become the current method for resecting tumors. It shows advantages of small trauma and speedy recovery. The relationship between tumor and surrounding tissues should be understand clearly before operation. Full preparation must be made before operation if adrenal ganglioneuroma mixed with other tumor components.

Objective To discuss the diagnosis,perioperative treatment,cardiac function changing of pheochromocytoma/paraganglioma (PHEO/PGL) patients with catecholamine cardiomyopathy.Methods Fifteen PHEO/PGL patients with catecholamine cardiomyopathy were included in our hospital from Jan 2008 to Mar 2018.There were 8 males and 7 females with an average age of 32.8 years,ranging 13-64 years old.4 cases were found left PHEO.4 cases were found right PHEO and 3 cases were bilateral PHEO,including 2 cases of VHL.One case was jugular PGL.2 cases were lift PGL and 1 case was right PGL.The tumors diameter ranged from 2.3 to 7.2 cm.14 patients were diagnosed as PHEO/PGL with catecholamine cardiomyopathy with typical clinical manifestations of PHEO/PGL,such as headache,palpitation and perspiration.Their 24 hours of urine catecholamines showed an average of 24.87 μg/24 h in epinephrine and 551.70 μg/24 h in norepinephrine.Their average value of dopamine was 395.41 μg/24 h.Among 7 cases,the octreotide scan and Iodine-131-meta-iodobenzylguanidine (131 I-MIBG) s scan were positive in 6 and 1 case,respectively.Enhanced CT showed significantly heterogeneous enhancement tumors in adrenal or retroperitoneal area.Ischemia and necrosis area were found inside tumors.The blood flow of some tumors were extremely rich and irregular vascular network with a racemose distribution around the tumors could be observed.Echocardiography showed that the left ventricular ejection fraction (LVEF) ranged from 32％ to 54％,mean (42.0 ± 7.1) ％ during catecholamine cardiomyopathy.5 cases were mild abnormal,which the LVEF ranged from 45％ to 54％.9 cases were moderate abnormal,which the LVEF ranged from 30％ to 44％.Their catecholamine cardiomyopathy performance including left ventricular hypertrophy,myocardial echo enhancement and left ventricular enlargement.9 cases combined with acute left ventricular failure manifested as pulmonary edema and pink foamy phlegm.Sensitive antibiotics were given to suspected pulmonary infection patients.14 cases accepted regular alpha blockers,beta blockers and calcium antagonists treatment for 1 to 3 months and underwent surgery after a significant improvement in cardiac function.1 non-functional PHEO patient was lack of typical clinical and imaging changing.Both of her 24 hours of urine catecholamines and octreotide scan were negative.Echocardiography showed that LVEF was 73％ before operation.She was misdiagnosed as an adrenal non-functional adenoma.All patients underwent surgical treatment,including 13 laparoscopic surgery.One case switched to open surgery.2 PGL patients all accepted open surgery.Rusults All cases accepted complete resection of the tumors.1 case of nonfunctional PHEO had severe fluctuations in blood pressure during operation.Her BP were 190/130 to 80/50 mmHg (1 mmHg =0.133 kPa) and heart rate raised to 150 bpm.Catecholamine cardiomyopathy appeared in ICU ward after operation.Echocardiography showed that LVEF was 37％.The left ventricular enlargement and myocardial systolic function decreased.Fibrous bronchoscopy showed pink foamy phlegm in both sides of lungs.Chest X ray showed bilateral pulmonary edema which predominant in left side.The diagnosis was catecholamine cardiomyopathy of this patient.The LVEF was (55.9 ± 7.6)％ after treatment in 14 PHEO/ PGL patients with catecholamine cardiomyopathy,and there was statistically significant difference between before and after treatment (P =0.041).The LVEF was (66.1 ± 8.5) ％ postoperation,and there was statistically significant difference between postoperation and after treatment (P =0.013).The non-functional PHEO case occurred severe catecholamin cardiomyopathy after operation and cardiac function recovered after treatment.15 cases were followed up from 1 months to 10 years without recurrence.Conclusions PHEO/PGL patients with catecholamine cardiomyopathy should have adequate medication.The abnormal cardiac function of patients would be reversed after surgical treatment.Cardiac function failure would be recovered after active treatment.Non-functional pheochromocytoma patients were extremely dangerous.Adequate medication should be given to any suspected non-functional pheochromocytoma patients.

Objective To analyze the clinical characteristics for hypertensive attack during operation and clinical experience of preoperative evaluation and preparation in patients with pheochromocytoma and paraganglioma(PHEO/PGL).Methods A total 219 PHEO/PGL cases from September 2016 to September 2018 were retrospectively reviewed.It included 99 males and 120 females,aged 13 to 76 (average 47) years old.The mean diameter of tumor was 5.3 cm (1.5-18.0 cm).140 cases were unilateral PHEO,6 cases were bilateral PHEO,68 cases were PGL(jugular,mediaphragm,heart,retroperitoneum,pelvic and bladder) and 5 cases were PHEO combined with PGL.Preoperative highest systolic blood pressure (SBP)was 240 mmHg(1 mmHg-0.133 kPa) and highest diastolic blood pressure (DBP) was 160 mmHg.20 cases were occult PHEO without hypertension.217 cases accepted preoperative preparation of alpha-blocker [phenoxy-benzamine,dosage ranging from 5 mg Q12h to 40 mg Q8h,maximum dosage not exceeding 1 mg/(kg· 24 h)].2 cases did not accept preoperative preparation.All cases accepted open or endoscope surgery.The patients were divided into 2 groups depending on the presence or absence of hypertensive attack at the time of surgery.Patient demographic characteristics and preoperative evaluations were assessed for their prognostic relevance with respect to hypertensive attack.Results Histopathological results showed that all cases were PHEO or PGL,while 205 cases were benign,14 cases were malignant.Hypertensive attack were recorded in 112 cases(51％).The diameter of tumors in the hypertensive attack group were larger than that in the non-hypertensive attack group[(6.70 ± 2.95)cm vs.(3.95 ± 1.70) cm,P =0.005].There was no significant difference between the two groups among age [(51.0 ± 10.8) years vs.(38.5 ± 17.6) years,P =0.105],preoperative catecholamine level [norepinephrine (111.20 ± 41.49) μg/24 h vs.(419.15 ± 154.81) μg/24 h,P =0.075],time of use of alpha blockers [(53.0 ± 7.5) d vs.(38.0 ± 6.4) d,P =0.139],daily dosage of alpha blocker [(40.0 ±7.2)mg vs.(27.1 ± 1.8) mg,P =0.111] and blood pressure at diagnosis[(173.75 ± 26.69) mmHg vs.(155.0 ± 20.75) mmHg,P =0.139].Among 219 cases,2 case had emergency hemostasis after operation,1 case had catecholamine cardiomyopathy after operation for occult pheochromocytoma,and no perioperative death occurred.Conclusions Patients with large tumor tend to have hypertensive attack during operation so that should be better prepared.

Objective:To investigate the etiological spectrum, clinical features, and surgical treatment of patients with Cushing's syndrome (CS) who underwent adrenal surgery.Methods:From August 2002 to August 2022, the clinical data of 985 patients with Cushing's syndrome who underwent surgical treatment in the department of urology, Peking Union Medical College Hospital were retrospectively analyzed. There were 210 males and 775 females. The average age was 43.33±13.49 years old. The age of males was older than that of females (45.53±14.39 vs. 42.68±13.16 years, P=0.016). The principle of preoperative surgical method selection for patients in this group was described as follow. For adrenocorticotropin (ACTH) independent CS, adrenal tumor resection was considered for unilateral solitary lesions and unilateral adrenalectomy was considered for unilateral multiple lesions. For bilateral lesions, the larger tumor was removed first, and the contralateral operation was decided according to the follow-up results. Patients with suspicion of cortical cancer are subjected to R0 resection, and open surgery was performed if the tumor diameter is≥6 cm. The clinical characteristics of CS patients were summarized. The clinical symptom characters, etiology spectrum and the corresponding selection principles of surgical methods were analyzed. Results:Among the 985 cases, ACTH-independent CS accounted for 92.8% (914/985), and ACTH-dependent CS accounted for 7.2% (71/985). According to the postoperative pathological results, ACTH-independent CS was the most common, among which adrenal adenoma was the most common 75.94% (748/985), PBMAH 11.37% (112/985), Cushing's disease 4.26% (42/985), PPNAD 3.25% (32/985), EAS 2.94 (29/985), cortical carcinoma 2.23% (22/985). In terms of clinical manifestations, full moon face, bloody face, buffalo back, central obesity and weight gain were more common. The incidence of the above single symptoms was >30%. In terms of hormone secretion, the cortisol level of ACTH-dependent CS patients was significantly higher than that of ACTH-independent CS ( P<0.001). Cortisol [(16.61±6.78) μg/dl] and 24h-UFC [103.65 (59.83, 175.70) μg/24h] in patients with subclinical cortical adenoma were lower than those in other types of patients ( P<0.001). Among the patients with bilateral adenomas, 4 patients underwent simultaneous resection of bilateral adrenal tumors due to severe CS symptoms, and the remaining 74 patients underwent two-stage simple tumor resection, total adrenalectomy or subtotal adrenalectomy according to the size and number of tumors. Of the 22 patients with cortical carcinoma, 2 patients underwent R0 resection after neoadjuvant therapy with mitotane after being diagnosed by needle biopsy due to local infiltration of the tumor. In patients with EAS and Cushing's disease, 62.07% (18/29) and 23.81% (10/42) were treated with simultaneous bilateral target gland resection due to severe CS symptoms, respectively. In 112 cases of PBMAH and 32 cases of PPNAD, the initial treatment was unilateral adrenalectomy or subtotal adrenalectomy, and the follow-up was continued to decide whether to perform contralateral adrenalectomy or subtotal adrenalectomy. Conclusions:The etiological spectrum of CS patients admitted to the urology department is different from that of the overall CS, and is dominated by adrenal disease, and Cushing's disease is rare. Due to the different etiological spectrum, the patient's symptom spectrum is different, and difficulty in squatting and fracture are rare. The main treatment for unilateral adrenal disease is tumor resection or unilateral adrenalectomy. For ACTH-dependent CS in bilateral lesions, bilateral adrenalectomy is recommended, and hormone supplementation is recommended after surgery. For ACTH-independent CS, unilateral adrenalectomy is recommended first, and individualized treatment plans are formulated based on the results of follow-up.

Total mesorectal excision (TME) is the gold standard for the treatment of middle and low rectal tumors. Since the first patient who underwent transanal total mesorectal excision (taTME) was reported in 2010, taTME has attracted wide attention from scholars at home and abroad. Fecal incontinence is one of the common complications after taTME, which severely affects the postoperative quality of life of patients. This paper systematically summarizes the commonly used assessment methods, current situation, influencing factors, treatment and nursing progress of postoperative fecal incontinence patients with taTME, in order to provide evidence for improving the quality of life of postoperative defecation incontinence patients with taTME.

Objective:To explore the feasibility of deep learning technology for renal artery recognition in retroperitoneal laparoscopic renal surgery videos.Methods:From January 2020 to July 2021, the video data of 87 cases of laparoscopic retroperitoneal nephrectomy, including radical nephrectomy, partial nephrectomy, and hemiurorectomy, were retrospectively analyzed. Two urological surgeons screened video clips containing renal arteries. After frame extraction, annotation, review, and proofreading, the labeled targets were divided into training set and test set by the random number table in a ratio of 4∶1. The training set was used to train the neural network model. The test set was used to test the ability of the neural network to identify the renal artery in scenes with different difficulties, which was uniformly transmitted to the YOLOv3 convolutional neural network model for training. According to the opinion of two senior doctors, the test set was divided into high, medium, and low discrimination of renal artery and surrounding tissue. High identification means a clean renal artery and a large exposed area. For middle recognition degree, the renal artery had a certain degree of blood immersion, and the exposed area was medium. Low identification means that the exposed area of the renal artery was small, often located at the edge of the lens, and the blood immersion was severe, which may lead to lens blurring. In the surgical video, the annotator annotated the renal artery truth box frame by frame. After normalization and preprocessing, all images were input into the neural network model for training. The neural network output the renal artery prediction box, and if the overlap ratio (IOU) with the true value box was higher than the set threshold, it was judged that the prediction was correct. The neural network test results of the test set were recorded, and the sensitivity and accuracy were calculated according to IOU.Results:In the training set, 1 149 targets of 13 videos had high recognition degree, 1 891 targets of 17 videos had medium recognition degree, and 349 targets of 18 videos had low recognition degree. In the test set, 267 targets in 9 videos had high recognition degree, 519 targets in 11 videos had medium recognition degree, and 349 targets in 18 videos had low recognition degree. When the IOU threshold was 0.1, the sensitivity and accuracy were 52.78% and 82.50%, respectively. When the IOU threshold was 0.5, the sensitivity and accuracy were 37.80% and 59.10%, respectively. When the IOU threshold was 0.1, the sensitivity and accuracy of high, medium and low recognition groups were 89.14% and 87.82%, 45.86% and 78.03%, 32.95%, and 76.67%, respectively. The frame rate of the YOLOv3 algorithm in real-time surgery video was ≥15 frames/second. The false detection rate and missed detection rate of neural network for renal artery identification in laparoscopic renal surgery video were 47.22% and 17.49%, respectively (IOU=0.1). The leading causes of false detection were similar tissue and reflective light. The main reasons for missed detection were image blurring, blood dipping, dark light, fascia interference, or instrument occlusion, etc.Conclusions:Deep learning-based renal artery recognition technology is feasible. It may assist the surgeon in quickly identifying and protecting the renal artery during the operation and improving the safety of surgery.