Objective To evaluate the role of adrenolectomy in ectopic ACTH syndrome. Methods Clinical data of 23 cases of ectopic ACTH syndrome were analysed.Of them,14 were males and 9 were female,the average age was 38.All of the 23 cases had clinical and biochemical and imaging evidences for the diagnosis of Cushing's syndrome.16 of the 23 cases were treated with total adrenalectomy and the other 7 were treated without surgical intervention. Results Sixteen cases,having no identifiable source of ectopic hormone production, treated with bilateral or unilateral total adrenalectomy,had resolution of their presenting signs and symptoms,1-year survival was 67%,2-year survival was 44% and 5-year survival was 19%.Seven of the patients who were managed without surgical intervention,1-year survival was 0%. Conclusions To most patients with no identifiable source of ectopic hormone production, bilateral adrenolectomy with hormonal replacement is effective.

Objective To investigate the expression o f telomerase activity in pheochromacytoma and to assess its utility as a prognos tic marker. Methods Telomerase activity was estimated in 23 samples of pheohromocytoma tissue and 6 samples of normal adrenal medullae by autoradiography-based telomeric repeat amplification protocol. The reaults w ere analized with follow-up findings. Results Of the 2 3 pheochromocytoma samples, 2 had telomerase activity and both the telomerase-p ositive pheochromocytomas apparently arose from extraadrenal tissues. All normal adrenal medullae samples were negative for telomerase activity. During follow- up, we found both of the telomerase positive patients developed mutiple metastat ic lesions after surgery. Only one of 21 telomerase negative patients clinically developed bilateral lung metastases and a local recurrence after surgery. Conclusions Telomerase activity may be used as an important i ndicator of potentially malignant pheochromocytoma and may serve as a prognosti c marker.

ObjectiveTo discuss the treatment of a rare case of Klippel-Trenaunay syndrome associated with bladder hemangioma and conduct a brief literature review. MethodsA 23-year-old female manifested episodes of painless hematuria for 1 month.A biopsy by cystoscopy diagnosed angiomatosis.The patient had a history of left lower limb hemangioma for 23 years,and underwent 3 surgical operations at 3 months old,3 years old,and 13 years old.The left leg was longer than the right leg.There was some soft tissue hypertrophy in the left lower extremity.Ultrasonography showed a bladder mass.Cystoscopy revealed a 3 cm x 5 cm sized hemangiomatous on the left lateral and anterior wall of the bladder. ResultsThe patient was diagnosed with bladder hemangioma and Klippel-Trenaunay syndrome.Partial cystectomy was performed.The pathological diagnosis was bladder intramuscular hemangioma. ConclusionKlippel-Trenaunay syndrome associated with bladder hemangioma can be treated by partial cystectomy.

Objective To retrospectively analyze the clinical data of 3 middle-aged and elderly cases with inflammatory myofibroblastic tumor (IMT) of the kidney,in order to improve its diagnosis and treatment.Methods The records of 3 patients who were diagnosed as IMT of the kidney by pathology in our hospital from Jan 2005 to Jun 2014 were reviewed to identify the characteristics including age,gender,clinical manifestation,tumor imaging and pathological features,treatment and prognosis.Results The mean age of the patients was 60 years.One patient underwent partial nephrectorny,1 patient underwent radical nephrectomy,and another patient received nephroureterectomy with bladder cuff excision.Patients were followed up for 8 months to 10 years,and no tumor recurrence was found.Conclusions IMT of the kidney is the tumor of low malignant potential with a good prognosis.Surgery is the first choice for the diagnosis and treatment.

Objective To review the diagnosis and treatment of adrenal myelolipoma(ML).Methods The medical records of 21 patients with adrenal myelolipoma were reviewed.All patients were examined by B-ultrasound,CT and endocrine tests while some were also examined by MRI and 131I-MIBG.Results All patients had undergone resection of adrenal ML.The mean diameter of tumors was 6.7 cm(4.5 cm～12 cm).Pathological examination of all tumors confirmed the diagnosis of adrenal ML.No recurrence was recorded during the follow-up period(1～15years).Conclusion Adrenal ML is a kind of rare,benign tumor and is often incidentally discovered.Its prognosis is generally good.

Objective To investigate the clinical features, diagnosis and treatment of cardiac pheochromocytoma. Methods The clinical data of 3 cases (2 males and 1 female) who were 17,40 and 35 years old,respectively,were analyzed.They all presented with headache, sweating, palpitations and hypertension.Before operation 24-hour urinary catecholamine excretion was examined for the 17-year-old man and the woman twice with the results as follows.In the 17-year-old man,epinephrine (E) was 1.84,150.28 ?g/24h (normal range,4.08?2.34 ?g/24h);norepinephrine (NE):734.77,508.00 ?g/24h (normal range,28.67?11.98 ?g/24h);dopamine (DA):823.00,620.31 ?g/24h (normal range,225.76?104.83 ?g/24h).In the woman,E was 12.40,16.03 ?g/24h;NE:1778.92,1243.96 ?g/24h;DA:1517.47,926.89 ?g/24h.All the 3 patients had positive result with Tc-99m-Otretide scintigraphy,and all underwent orthotopic excision of tumors under cardiopulmonary bypass. Results During operation,the tumors of 2-5 cm in size were seen to lie at the base of the aorta,left atrium and interauricular septa respectively,and grew invasively like carpet,thus were difficult to remove. Postoperative pathology of 3 cases confirmed the diagnosis of cardiac pheochromocytoma.After follow-up for 8-24 months,3 cases had a mean fall of 30 mmHg in systolic pressure and 20 mmHg in diastolic pressure,respectively.The 17-year-old man had decline in cardiac function that was proved by Tc-99m-Otretide scintigraphy test;another male patient suffered from arrhythmias 1 month after operation,and was found to have tumor remains by PET test.Myocardial infarction occurred in the female patient. Conclusions Cardiac pheochromocytoma is a rare tumor, and its diagnosis is rather difficult and complex.Surgical excision is very difficult with the possibility of complications.

Objective To discuss the treatment of renal or adrenal tumor with cancer thrombus in the inferior vena cava. Methods From Jan 1984 to Apr 2008,29 cases of renal or adrenal malignancy with thrombosis involving the inferior vena cava underwent treatment.The diagnoses were confirmed by Doppler uhrasonography,CT and MRI.In the 29 surgical patiens the tumor thrombus was level I in 7,level Ⅱ in 10,level Ⅲ in 8 and levelⅣin 4.According to TNM classification,23 cases were classified to T2N.M.,1 case was T2Nl Mo,1 case was TzNlMl,1 case was T3NoMo,2 case were T3NlMl and 1 case was T3N2Mo.The mean tumor size was 8.7(4.O-16.O)cm in diameter.The mean tumor thrombosis length was 3.2(2.5-4.0)cm in level I,5.3(4.5-6.0)cm in level Ⅱ,8.2(6.5-9.O)cm in levelⅢand 15.1(12.0-18.5)cm in level IV. Results The operation was performed succesgfullv in 29 patients Patholocieal examination showed that 18 cases of clear cellcarcinoma,3 cases sarcomatoid carcinoma,2 cases renal papillary adenocarcinoma,1 case renal cell carcinoma (undifferentiated),1 case granule carcinoma,3 cases adrenocortical carcinoma and 1 case metastatic malignant melanoma of adrenal gland.Of 29 patients,3 were out of contact.Twenty-six patients were followed up for 35(0-62)months after treatment,3-and 5-year survival rates were 15/26 and 11/26.Three-year survival rates for stage T2 and T3 were 14/22 and 1/4.Five-year survival rates for stage T2 and T3 were 10/22 and 1/4.Three-year survival rates for level I、Ⅱ、Ⅲ andⅣ were 4/6,5/8,5/8 and 1/4.Five-year survival rates for level I,Ⅱ、Ⅲ andⅣ were 3/6,4/8,3/8 and 1/4.Three-year survival rates for a tumor thrombus in the below or above diaphragm were 14/22 versus 1/4,5-year survival rates were 10/22 versus 1/4.Three-year and 5-year survival rates for the patients without distant metastases and lymph node involvement were 12/18 and 9/18.Three-year and 5-year surviral rates for the patients with distant metastases and lymph node involvement were 3/8 and 2/8.The 3 surgical patients with metastatic disease died at 6,10,22 months. Conclusions Surgical treatment could be the preferred approach for the patients of renal or adrenal tumor with cancer thrombus in the inferior vena cava without distant metastases and lymph node involvement.It could improve the quality of life and may prolong survival.

Objective To compare the efficacy and safety of 1.9 μm thulium laser with transurethral resection of bladder tumor(TURBT) for the treatment of superficial bladder cancer.Methods We reviewed 53 patients with superficial bladder cancer,who were divided into 1.9 μm laser (n =25) and TURBT groups (n =28) from January 2013 to December 2015.The operation time,blood loss volume in operation,catheter indwelling time,hospital stay time,and complications,cumulative recurrence rate were compared between the two groups.Results Compared to TURBT group,1.9 μm laser group showed significantly lower rate of blood loss volume in operation (21.6 ± 4.6) min,catheter indwelling time (22.4 ± 6.4) h,hospital stay time (2.2 ± 0.7) d,less complications (12％)and recurrence(16％) (P ＜ 0.05).Conclusions 1.9 μm thulium laser is safe and effective for the treatment of patients with superficial bladder cancer.The approach has less complications than TURBT.

Objective To summarize the clinical characteristics of primary pigmented nodular adrenocortical disease (PPNAD).Methods Eight patients with PPNAD from 2001 to 2009 in PUMC Hospital were reviewed,their clinical data were collected.Results PPNAD often occurred in adolescents.62.5% of patients with PPNAD were complicated with Carney complex(CNC).In addition to general features of Cushing's syndrome,amenorrhea and growth retardation in stature were very frequent in clinical manifestations of PPNAD.Plasma ACTH was undetectable,circadian rhythm of plasma cortisol was disappeared,glucocorticoid excretion was increased paradoxically during the dexamethasone suppression test in 50% patients with PPNAD.Adrenal imaging from 75% patients revealed normal-sized adrenal glands or suspectable micronodular changes.Adrenal pathologic analysis revealed numerous brown cortical nodules containing lipofuscin pigmentation.Unilateral adrenalectomy may relieve symptoms of Cushing's syndrome,but plasma ACTH and circadian rhythm of plasma cortisol were difficult to recovere.Hypercorticoidism might re-occure after unilateral adrenalectomy.Conclusion PPNAD should be bewared in ACTH independent Cushing's syndrome patients without apparent adrenal mass,and CNC should be screened and followed up.

Objective To evaluate the feasibility of laparoscopic nephroureterectomy with bladder-cuff resection by TUR for upper urinary tract carcinoma. Methods Eighty-two patients with upper urinary tract transitional cell carcinoma(69 cases in renal pelvis and 13 in ureter)underwent retroperitoneal laparoscopic nephroureterectomy and bladder-cuff resection by TUR. This group of cases was retrospectively summarized including operative time, blood loss, drainage mounting days, catheterizing days, post-operative complications and hospital stays. Results All 82 operation procedures were successfully performed without severe complication. The mean operative time was 135 minutes.The mean length of hospital stay was 7 days postoperatively. The mean time with drainage and Foley catheter were 3 days and 6 days respectively. Follow-up time ranged from 6 to 76 months for 74 patients. The 3-year carcinoma recurrence was 10.6% (5/47). Only 1 patient was found incision tumor recurrence in 8 month after the procedure. Conclusion Retroperitoneoscopic nephroureterectomy with bladder cuff resection by TUR could be a feasible procedure to treat upper urinary tract transitional cell carcinoma.