Abstract
BackgroundThe management of Von Hippel–Lindau syndrome (VHLS) is multidisciplinary. The urologist is involved in treating the renal, epididymal tumors and often adrenal pheochromocytoma. Preservation of renal and adrenal function is a challenge. We present 17 cases of VHLS in a tertiary care center in South India.MethodsA retrospective review of the patients who underwent surgical treatment under urology for VHLS from January 2009 to November 2018 was conducted. The demographic data, the spectrum of manifestation, treatment, change in glomerular filtration rate, adrenal insufficiency, and recurrence-free survival were analyzed.ResultsThere were 17 patients diagnosed with VHLS. The median age of diagnosis was 39 years (range 23–41). The spectrum of clinical manifestation was: multifocal RCC (88%), pancreatic cysts/tumors (70%), cerebellar hemangioblastoma (59%), retinal angiomas (47%), epididymal cysts/tumors (47%), pheochromocytomas (41%), and spinal hemangiomas (30%). There were seven patients with ten pheochromocytoma lesions. Three underwent cortical sparing and seven total adrenalectomies; 13 patients underwent nephron sparing surgery (NSS), of which seven patients had bilateral tumors. The median duration of follow-up was 6.5 years (range 2–12 years). Following NSS, seven patients had a local recurrence, and one developed pancreatic metastasis. Two patients (11%) were lost to follow-up. Renal function was preserved in all patients at the last follow-up, and there was no postoperative adrenal crisis or mortality.ConclusionNephron sparing surgery and cortical sparing adrenalectomy are the treatment of choice for multifocal RCC and pheochromocytomas in patients with VHLS providing good oncological outcomes and preservation of renal and adrenal function.
Highlights
The management of Von Hippel–Lindau syndrome (VHLS) is multidisciplinary
The urologist is involved in managing renal cell carcinoma, pheochromocytoma, epididymal cysts, and cystadenomas
We present a review of patients with VHLS, emphasizing achieving oncological clearance and preservation of renal and adrenal function
Summary
The management of Von Hippel–Lindau syndrome (VHLS) is multidisciplinary. The urologist is involved in treating the renal, epididymal tumors and often adrenal pheochromocytoma. Preservation of renal and adrenal function is a challenge. We present 17 cases of VHLS in a tertiary care center in South India. Von Hippel–Lindau syndrome (VHLS) is a rare genetic disorder with multisystem involvement due to the mutation in the VHL gene (tumor suppressor) on chromosome 3p25. The urologist is involved in managing renal cell carcinoma, pheochromocytoma, epididymal cysts, and cystadenomas. The disease is often multiple and bilateral, Jayanth et al Afr J Urol (2021) 27:150 so organ preservation (nephron and adrenal sparing surgery) is paramount. There is a lack of data from India. We present a review of patients with VHLS, emphasizing achieving oncological clearance and preservation of renal and adrenal function
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