PURPOSE: Despite aggressive screening patients with hereditary renal cancers can present with large multifocal tumors. We present oncological outcomes in patients with hereditary renal cell carcinoma treated with partial nephrectomy for multifocal solid tumors with the largest lesion greater than 4 cm. MATERIALS AND METHODS: Between 1995 and 2008 we identified 58 patients with hereditary renal cell carcinoma treated at our institution with partial nephrectomy for solid tumors greater than 4 cm. Data collected included demographic parameters, tumor size, pathological findings and laterality. Overall and metastasis-free survival was calculated based on information from the most recent followup evaluation and imaging. RESULTS: The cohort included 41 patients (71%) with von Hippel-Lindau disease, 10 (17%) with Birt-Hogg-Dubé syndrome and 7 (11%) with hereditary papillary renal carcinoma. Mean age was 43.7 years (range 18 to 63) and mean largest tumor size was 5.3 cm (range 4 to 13). A mean of 6.4 kidney tumors (range 1 to 44) was resected. There was a predominance of nuclear grade 2 tumors (51 cases or 85%) and clear cell histology (44 or 73%), followed by papillary type I histology (7 or 11.7%). Overall and metastasis-free survival rates were 93% and 96.5%, respectively, at a median followup of 45 months (range 2 to 163). CONCLUSIONS: Metastasis-free and overall survival of our patients is similar to that in the literature of those who undergo partial nephrectomy for T1B tumors in the sporadic population. Multifocality does not affect oncological outcomes at intermediate followup. Partial nephrectomy can be offered to patients with hereditary disease who present with multifocal tumors greater than 4 cm. Copyright (c) 2010 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.
PURPOSE: Despite aggressive screening patients with hereditary renal cancers can present with large multifocal tumors. We present oncological outcomes in patients with hereditary renal cell carcinoma treated with partial nephrectomy for multifocal solid tumors with the largest lesion greater than 4 cm. MATERIALS AND METHODS: Between 1995 and 2008 we identified 58 patients with hereditary renal cell carcinoma treated at our institution with partial nephrectomy for solid tumors greater than 4 cm. Data collected included demographic parameters, tumor size, pathological findings and laterality. Overall and metastasis-free survival was calculated based on information from the most recent followup evaluation and imaging. RESULTS: The cohort included 41 patients (71%) with von Hippel-Lindau disease, 10 (17%) with Birt-Hogg-Dubé syndrome and 7 (11%) with hereditary papillary renal carcinoma. Mean age was 43.7 years (range 18 to 63) and mean largest tumor size was 5.3 cm (range 4 to 13). A mean of 6.4 kidney tumors (range 1 to 44) was resected. There was a predominance of nuclear grade 2 tumors (51 cases or 85%) and clear cell histology (44 or 73%), followed by papillary type I histology (7 or 11.7%). Overall and metastasis-free survival rates were 93% and 96.5%, respectively, at a median followup of 45 months (range 2 to 163). CONCLUSIONS:Metastasis-free and overall survival of our patients is similar to that in the literature of those who undergo partial nephrectomy for T1B tumors in the sporadic population. Multifocality does not affect oncological outcomes at intermediate followup. Partial nephrectomy can be offered to patients with hereditary disease who present with multifocal tumors greater than 4 cm. Copyright (c) 2010 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.
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