Abstract
Background: To determine the outcomes and predictors of survival for pediatric non-Wilms renal tumors (NWRT). Methods: The SEER database (1973-2005) was queried for all patients < 20 y of age. Results: Overall, 349 cases of NWRT were identified. The major histologic groups included renal cell carcinoma (RCC) (44%), clear cell sarcoma of the kidney (CCSK) (17%), and malignant rhabdoid tumor (MRT) (12%). A bimodal age distribution was observed, with tumors commonly presenting in patients ≤4 y of age and ≥15 y of age. More than 50% of RCC presented at ≥15 y of age, whereas ≥80% of CCSK or MRT patients were ≤4 y of age. Most RCC (57%) and CCSK (53%) were locally staged while most MRT presented with distant disease (51%, P < 0.001). Overall 10-y survival was 63% with improved survival observed in patients with CCSK (79%) and RCC (70%) versus MRT (29%, P < 0.001). By univariate analysis, surgical resection was associated with improved overall 10-y survival (68% versus 30%, P < 0.001), while no benefit was observed for radiotherapy (60% versus 63%, P = 0.8). By multivariate analysis, worse overall survival was observed for patients ≥ 10 y old (HR 4.01, P = 0.013) and those with advanced disease (HR = 12.78, P < 0.001). Patients with MRT (HR = 11.61, P < 0.001) and CCSK (HR = 3.68, P = 0.038) had significantly worse prognosis compared with those with RCC. Surgical resection improved overall survival (HR = 0.36, P = 0.001). Conclusion: For pediatric NWRT, younger patients and those with RCC have improved survival, while a diagnosis of MRT portends a worse prognosis. Surgical extirpation significantly improves survival for all patients.
| Original language | English |
|---|---|
| Pages (from-to) | 257-263 |
| Number of pages | 7 |
| Journal | Journal of Surgical Research |
| Volume | 163 |
| Issue number | 2 |
| DOIs | |
| State | Published - Oct 1 2010 |
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Keywords
- clear cell sarcoma of the kidney
- malignant rhabdoid tumor
- non-Wilms renal tumors
- outcomes
- renal cell carcinoma
ASJC Scopus subject areas
- Surgery
Cite this
Pediatric non-Wilms renal tumors : Subtypes, survival, and prognostic indicators. / Zhuge, Ying; Cheung, Michael C.; Yang, Relin; Perez, Eduardo; Koniaris, Leonidas G.; Sola, Juan E.
In: Journal of Surgical Research, Vol. 163, No. 2, 01.10.2010, p. 257-263.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Pediatric non-Wilms renal tumors
T2 - Subtypes, survival, and prognostic indicators
AU - Zhuge, Ying
AU - Cheung, Michael C.
AU - Yang, Relin
AU - Perez, Eduardo
AU - Koniaris, Leonidas G.
AU - Sola, Juan E
PY - 2010/10/1
Y1 - 2010/10/1
N2 - Background: To determine the outcomes and predictors of survival for pediatric non-Wilms renal tumors (NWRT). Methods: The SEER database (1973-2005) was queried for all patients < 20 y of age. Results: Overall, 349 cases of NWRT were identified. The major histologic groups included renal cell carcinoma (RCC) (44%), clear cell sarcoma of the kidney (CCSK) (17%), and malignant rhabdoid tumor (MRT) (12%). A bimodal age distribution was observed, with tumors commonly presenting in patients ≤4 y of age and ≥15 y of age. More than 50% of RCC presented at ≥15 y of age, whereas ≥80% of CCSK or MRT patients were ≤4 y of age. Most RCC (57%) and CCSK (53%) were locally staged while most MRT presented with distant disease (51%, P < 0.001). Overall 10-y survival was 63% with improved survival observed in patients with CCSK (79%) and RCC (70%) versus MRT (29%, P < 0.001). By univariate analysis, surgical resection was associated with improved overall 10-y survival (68% versus 30%, P < 0.001), while no benefit was observed for radiotherapy (60% versus 63%, P = 0.8). By multivariate analysis, worse overall survival was observed for patients ≥ 10 y old (HR 4.01, P = 0.013) and those with advanced disease (HR = 12.78, P < 0.001). Patients with MRT (HR = 11.61, P < 0.001) and CCSK (HR = 3.68, P = 0.038) had significantly worse prognosis compared with those with RCC. Surgical resection improved overall survival (HR = 0.36, P = 0.001). Conclusion: For pediatric NWRT, younger patients and those with RCC have improved survival, while a diagnosis of MRT portends a worse prognosis. Surgical extirpation significantly improves survival for all patients.
AB - Background: To determine the outcomes and predictors of survival for pediatric non-Wilms renal tumors (NWRT). Methods: The SEER database (1973-2005) was queried for all patients < 20 y of age. Results: Overall, 349 cases of NWRT were identified. The major histologic groups included renal cell carcinoma (RCC) (44%), clear cell sarcoma of the kidney (CCSK) (17%), and malignant rhabdoid tumor (MRT) (12%). A bimodal age distribution was observed, with tumors commonly presenting in patients ≤4 y of age and ≥15 y of age. More than 50% of RCC presented at ≥15 y of age, whereas ≥80% of CCSK or MRT patients were ≤4 y of age. Most RCC (57%) and CCSK (53%) were locally staged while most MRT presented with distant disease (51%, P < 0.001). Overall 10-y survival was 63% with improved survival observed in patients with CCSK (79%) and RCC (70%) versus MRT (29%, P < 0.001). By univariate analysis, surgical resection was associated with improved overall 10-y survival (68% versus 30%, P < 0.001), while no benefit was observed for radiotherapy (60% versus 63%, P = 0.8). By multivariate analysis, worse overall survival was observed for patients ≥ 10 y old (HR 4.01, P = 0.013) and those with advanced disease (HR = 12.78, P < 0.001). Patients with MRT (HR = 11.61, P < 0.001) and CCSK (HR = 3.68, P = 0.038) had significantly worse prognosis compared with those with RCC. Surgical resection improved overall survival (HR = 0.36, P = 0.001). Conclusion: For pediatric NWRT, younger patients and those with RCC have improved survival, while a diagnosis of MRT portends a worse prognosis. Surgical extirpation significantly improves survival for all patients.
KW - clear cell sarcoma of the kidney
KW - malignant rhabdoid tumor
KW - non-Wilms renal tumors
KW - outcomes
KW - renal cell carcinoma
UR - http://www.scopus.com/inward/record.url?scp=77956939572&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77956939572&partnerID=8YFLogxK
U2 - 10.1016/j.jss.2010.03.061
DO - 10.1016/j.jss.2010.03.061
M3 - Article
C2 - 20538287
AN - SCOPUS:77956939572
VL - 163
SP - 257
EP - 263
JO - Journal of Surgical Research
JF - Journal of Surgical Research
SN - 0022-4804
IS - 2
ER -