Management of Renal Malignancies in Von Hippel-Lindau Syndrome: Lessons Learned from a Series of Six Patients from Sri Lanka

Management of renal malignancies in Von Hippel-Lindau (VHL) is challenging. We present six patients [mean age = 35.1 years (range: 24-54), males = 5] with VHL syndrome with multiple bilateral renal malignancies and the lessons learned during their management. The number of tumors at the time of pres...

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Bibliographic Details
Published in:Saudi journal of kidney diseases and transplantation 2023-03, Vol.34 (2), p.187-190
Main Authors: Jayarajah, Umesh, Balagobi, Balasingam, Thanusan, Vimalakanthan, Gunasekara, Densil, Abeygunasekera, Anuruddha M
Format: Article
Language:English
Subjects:
Adult
Carcinoma, Renal Cell - complications
Carcinoma, Renal Cell - surgery
Humans
Kidney Neoplasms - complications
Kidney Neoplasms - surgery
Male
Neoplasm Recurrence, Local - complications
Prostatic Neoplasms - complications
Sri Lanka - epidemiology
von Hippel-Lindau Disease - complications
von Hippel-Lindau Disease - diagnosis
von Hippel-Lindau Disease - surgery
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Summary:Management of renal malignancies in Von Hippel-Lindau (VHL) is challenging. We present six patients [mean age = 35.1 years (range: 24-54), males = 5] with VHL syndrome with multiple bilateral renal malignancies and the lessons learned during their management. The number of tumors at the time of presentation ranged from 1 to 6, while the number of new lesions varied from 1 to 3. Different combinations of radical nephrectomy (n = 2), partial nephrectomy (n = 7), and focal therapy (n = 6) were used appropriately. Median follow-up was 36 months (range: 12-72). Two patients developed new lesions which were managed with focal therapy. Nephron-sparing approaches are successful even in bilateral, multifocal, large, and recurring renal tumors associated with VHL. Awareness about the availability of efficacious surgical and minimally invasive measures would reduce psychosocial problems faced by patients and their families due to the social stigma associated with malignancies running in a family and burden of renal replacement therapy.
ISSN:1319-2442
2320-3838
DOI:10.4103/1319-2442.391898