Surgical management of yon HippeI-Lindau disease(report of 4 cases) / 中华泌尿外科杂志

ABSTRACT

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.