Pediatric non-Wilms renal tumors: Subtypes, survival, and prognostic indicators

Ying Zhuge, Michael C. Cheung, Relin Yang, Eduardo A. Perez, Leonidas G. Koniaris, Juan E. Sola

Research output: Contribution to journalArticle

31 Citations (Scopus)

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 languageEnglish (US)
Pages (from-to)257-263
Number of pages7
JournalJournal of Surgical Research
Volume163
Issue number2
DOIs
StatePublished - Oct 1 2010

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Rhabdoid Tumor
Clear Cell Sarcoma
Renal Cell Carcinoma
Pediatrics
Kidney
Survival
Neoplasms
Age Distribution
Radiotherapy
Multivariate Analysis
Databases

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 A.; Koniaris, Leonidas; Sola, Juan E.

In: Journal of Surgical Research, Vol. 163, No. 2, 10.2010, p. 257-263.

Research output: Contribution to journalArticle

Zhuge, Ying ; Cheung, Michael C. ; Yang, Relin ; Perez, Eduardo A. ; Koniaris, Leonidas G. ; Sola, Juan E. / Pediatric non-Wilms renal tumors : Subtypes, survival, and prognostic indicators. In: Journal of Surgical Research. 2010 ; Vol. 163, No. 2. pp. 257-263.
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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.",
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AU - Koniaris, Leonidas G.

AU - Sola, Juan E.

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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.

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