Objective To discuss the surgical management of von Hippel Lindau(VHL) syn drome.Methods The clinical data of four VHL patients who were clinically diagnosed from March1999 to October 2006 were reviewed.The first patient was a 56 year old man with the chief complaintof hypodynamia and low serum glucose as 2.37 mmol/L.CT scan showed three masses in the the leftkidney.He had a history of cerebral haemangioblastoma ten years before.The second case was a 57 year old woman with the chief complaint of lumbago in the left side.CT scan showed masses in theleft kidney,adrenal gland and panerease.The third case was a 39 year old woman with the chief com plaint of an accident mass in the left adrenal gland.She had the history of cerebellar hemangioblastoma9 years before,spinal hemangioblastoma resection 7 years before.The fourth case was a 41-year oldwoman,she was found brain tumors and cured by gamma radiation abalation.Bilateral renal masseswere found by B ultrasound one month later,CT scan showed four masses in the both kidneys,leftadrenal gland and pancreas.Results All 4 cases underwent surgical approach.The first case under went radical nephrectomy which pathological report was PEComa of kidney.The blood glucose wasnormal one week later.The second case underwent resection of the left adrenal gland,kidney,pancre atic body and tail and spleen.Pathological report was clear cell carcinoma,islet cell tumor and adrenal cyst.Three months later she was found spinal hemangioblastoma and refused treatment.The thirdcase underwent adrenalectomy in the left side and pathologieal report was adrenal pheoehromocytoma.There was not tumor reeurrenee during 2 years' follow up.The nephrectomy and adrenalectomy wasperformed for the last ease whose pathological report was clear celt carcinoma and pheochromoeytoma.Three weeks later,tumor enueleating of the right kidney was undertaken; the result was clear cellcancer.During the follow up for one year there was no relapse of tumor.Conclusions For VHL ac companied with multiple organ tumors,surgery resection is the proper approaeh when tumors of centralnervous system is large.Different approaches could be taken to deal with multiple tumors of VHL such aswatchful waiting,nephron sparing surgery.

Objective To summarize the pathological and imaging features and treatment of retroperitoneal bronchogenic cyst.Methods The clinical data of 2 cases treated from October 2001 to November 2009 were summarized.The first patient was a 55-year-old woman with the chief complaint of lumbago in the left flank for 10 d.B-ultrasound showed mixed solid and cystic mass in spleen space with a diameter of 3.9 cm with thin wall and without rich blood supply.CT showed the lesion in the left adrenal gland region measured about 4 cm ×4 cm with low density with CT value of 10 HU,and enhanced scan was not obvious with CT value of 20 HU.It was diagnosed as left adrenal tumor and tumor resection was performed.The second case was a 17-year-old young man with the chief complaint of gross hematuria for 3 weeks after strenuous exercise.Ultrasonography found a 8.4 cm × 7.7 cm × 9.0 cm anechoic area surrounding the bladder.CT showed about 9.0 cm × 7.2 cm × 9.0 cm cystic lesion with thin wall,and the center density was uniformity in presacral space with CT value of8 HU.IVU showed visible semi-circular lower edge on the right edge of the bladder.The patient was diagnosed of presacral cyst and cystectomy was performed successfully.Results The pathology report of the first case:organizing wall with fibrous connective tissue,with most of the lining overlying pseudostratified ciliated columnar epithelium,goblet cells and subepithelial basement membrane.Pathological diagnosis was bronchogenic cyst,and the patient was followed up for 9 months without recurrence.The pathology report of the second case:pathological tissue fibers false wall tissue lining ciliated columnar epithelium,goblet cells seen in epithelium,fibrous tissue in the visible structure of mixed glands,a small amount of cartilage and muscle tissue.The diagnosis was bronchogenic cyst,and the patient was followed up for 2 years without recurrence.Conclusions Retroperitoneal bronchogenic cyst is rare and easily misdiagnosed.Radiology imaging can identify cystic features,while a few may be with high density without specificity.Surgical removal of retroperitoneal bronchogenic cyst with symptoms has good prognosis and may prevent malignant transformation and secondary infection.

Objective To investigate the clinicopathologic features of epithelioid angiomyolipoma of renal(REAML). Methods Six cases of REAML were analyzed by histopathological and clinical characteristics.Of the 6 cases,4 cases were solitary tumors and 2 cases were multiple lesions.The diameter of tumors was about 9 cm in average.One case had a family history of nodular sclerosis.Three cases were found adipose tissue in CT scan and diagnosed for RAML,the other 3 cases were diagnosed for renal cancer.All cases were undergone surgical approach,3 cases were undergone resection of tumors only and the other 3 were performed nephrectomy. Results Pathological characters:tumor was mainly composed of epithelioid cells presented with invasive hyperplasia of atypical pleomorphism,hyperchromatic nuclei with frequent mitotic figures,giant neoplasic cells and extensive hemorrhagic necrosis immunohistochemcial staining showing a positive HMB45 and negative of EMA,CK in most neoplasic cells.All cases were followed up for 10 to 44 months,5 cases did not recurrence and metastases;the other had lung metastasis after operation 18 months later,without any treatment the patient died 10 months later. Conclusions Most of REAMLs are benign and often misdiagnosed for renal cancer by CT scan.HMB45 is positive in immunohistochemcial staining available for diagnose.The minority of REMAL is malignant potentially and should be followed up closely.Operation is major method.

Objectlve To discuss the characters and management of renal sinus tumors. Methods The clinical data of 3 tumors occurring in tenal sinus were reviewed.The first patient was a 33-year-old man with the chief complaint of lumbago in the right side for 6 months.B-ultrasound showed low echo in the right pelvis and CT scan showed that a mass measured about 3.5 cm in diameter in the right kidney collective system.Intravenous urography showed the upper collective system was tompressed.He was diagnosed for renal sinus tumor.The second case was a 34-year-old woman with the chief complaint of sudden lumbago in the right for 3 d.Color ultrasonography found hyperechoic in the right kidney.CT scan showed a mass measured 8 cm×6 cm × 8 cm in the right renal sinus,its CT value was about-70 HU.Intravenous urography showed the lower collective system was compressed.She was diagnosed for renal angiomyolipoma of right kidney.The third case was a 55+year-old woman with the chief complaint of lumbago for 1 year.B ultrasound showed hyperechoic in the leftrenal hilum.CT scan showed a nlass of 8 cm×5 cm×5 am in the left renal sinus with CT value of 50 HU.Intravenous urography showed the collective system of the left kidney was compressed.Mag-netic resonance imaging showed high signal on T1 WI and T2WI but low signal in fat suppression ima-ging.She was diagnosed for lipoma of the left renal sinus. Results All the 3 cases had undergone surgical approaches.The first case underwent surgical exploration of renal sinus tumor.Fast pathologic diagnosis was benign tumor,only the tumor was resected.Postoperative pathology confirmed the diagnosis of angioleiomyoma.There was no recurrence during follow-up of 3 years.The second case was scheduled for tumor enucleating,but nephrectomy was perform because of serious bleeding and damaged renal pelvis.Pathological report was angiomyolipoma.The third case was scheduled for lipoma enucleating but nephrectomy was performed because of tumor encapsulated renal pedicle vascular.Pathological report was lipoma.During the follow-up for 4 years there was no relapse. ConclusionsTumors occurred in renal sinus are rare, most of which are benign.CT scan, MRI and intravenous urography are the best imaging examination methods for differential diagnosis.Surgical operation is the major approach, while for the tumor radical nephrectomy less than 4 cm watchful waiting could be a choice.Tumor resection with nephron sparing is feasible while tumor is larger than 4 cm.Radical nephrectomy should performed for the malignant tumor.