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- Title
SINGLE-SESSION NEPHRON-SPARING SURGERY FOR MULTIPLE SYNCHRONOUS BILATERAL RENAL TUMORS IN VON HIPPEL LINDAU DISEASE: A SURGICAL CHALLENGE.
- Authors
I. A., Nechifor-Boila; Veronica, Ghirca; S., Tataru; Corina, Golovei; Orsolya, Martha; C., Chibelean
- Abstract
Background: Von Hippel-Lindau (VHL) disease is a relatively rare autosomal dominant disorder (1:36000) responsible for the early onset of multiple renal clear cell carcinomas (RCC) as well as other neoplasias. As RCCs in VHL tend to be more aggressive, resection of all solid and cystic lesions using nephron-sparing surgery (NSS) should be deployed whenever possible. Single-session NSS can be a good option providing that the number of tumors and their topography is not an obstacle. We aim to present a complex case of VHL successfully treated in our department using single-session NSS. Material and methods: We present the case of a 43-year old female VHL patient referred to our department with gross hematuria. CT scan revealed a globally enlarged right kindney, with a 50 mm upper pole mass of and two midrenal and lower pole masses of 80 and 56 mm respectively, suggestive for malignancy. In the right kidney a 59 mm upper pole tumor was found, together with several cortical tumors, the largest having 16 mm in diameter. No signs of venous or lymph node involvement were present. Radical nephrectomy was performed on the left side. On the right, partial nephrectomy was used for the upper pole mass and enucleation was successfully performed for the others. In the same operative session, a large abdominal hernioplasty was performed. Recovery from surgery was uneventful. Results: The final pathology report revealed one T3a Fuhrman 4 and two T1b RCCs in the left kidney. For the right kidney, all four resected masses were Fuhrman 2 T1a RCCs. Negative surgical margins were obtained for all tumors. At 6 months no recurrence was found on echography. The patient had normal creatinine levels. Conclusions: NSS is the treatment of choice in familial RCC diseases like VHL. Still, single-session surgeries can be particularly challenging, especially in multiple tumors with poor surgical access.
- Subjects
RENAL cancer treatment; VON Hippel-Lindau disease; NEPHRONS; SURGICAL excision; LYMPH nodes; SURGERY
- Publication
Acta Medica Marisiensis, 2016, Vol 62, p95
- ISSN
2068-3324
- Publication type
Case Study