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← Wilms Tumor and Other Childhood Kidney Tumors

HEALTH PROFESSIONAL · SOURCE READING

Nephrectomy

Source: Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: April 15, 2025 · Captured 2026-09-09.

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Context: Congenital Mesoblastic Nephroma / Treatment of Congenital Mesoblastic Nephroma

Evidence (nephrectomy):

A SIOP/Gesellschaft für Pädiatrische Hämatologie und Onkologie (GPOH) nephroblastoma study evaluated 111 patients with congenital mesoblastic nephromas. Sixty-seven patients had classical congenital mesoblastic nephromas (60%), 29 patients had the cellular subtype (26%), and 15 patients had the mixed subtype (14%). Tumor stage consisted of stage I (35%), stage II (50%), and stage III (15%). Ninety-one patients were treated with surgery alone, and 19 patients were treated with chemotherapy in addition to surgery (12 preoperatively and 11 postoperatively).[8]

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A SIOP/Gesellschaft für Pädiatrische Hämatologie und Onkologie (GPOH) nephroblastoma study evaluated 111 patients with congenital mesoblastic nephromas. Sixty-seven patients had classical congenital mesoblastic nephromas (60%), 29 patients had the cellular subtype (26%), and 15 patients had the mixed subtype (14%). Tumor stage consisted of stage I (35%), stage II (50%), and stage III (15%). Ninety-one patients were treated with surgery alone, and 19 patients were treated with chemotherapy in addition to surgery (12 preoperatively and 11 postoperatively).[8]

Overall, the 5-year RFS rate was 93.2%, and the 5-year OS rate was 96.8%

A SIOP/Gesellschaft für Pädiatrische Hämatologie und Onkologie (GPOH) nephroblastoma study evaluated 111 patients with congenital mesoblastic nephromas. Sixty-seven patients had classical congenital mesoblastic nephromas (60%), 29 patients had the cellular subtype (26%), and 15 patients had the mixed subtype (14%). Tumor stage consisted of stage I (35%), stage II (50%), and stage III (15%). Ninety-one patients were treated with surgery alone, and 19 patients were treated with chemotherapy in addition to surgery (12 preoperatively and 11 postoperatively).[8]

The 5-year RFS rate was significantly superior for the classical type (98%) compared with the cellular type (89%, P = .039) or mixed type (80%, P = .002). There was no significant difference in OS by tumor types.

A SIOP/Gesellschaft für Pädiatrische Hämatologie und Onkologie (GPOH) nephroblastoma study evaluated 111 patients with congenital mesoblastic nephromas. Sixty-seven patients had classical congenital mesoblastic nephromas (60%), 29 patients had the cellular subtype (26%), and 15 patients had the mixed subtype (14%). Tumor stage consisted of stage I (35%), stage II (50%), and stage III (15%). Ninety-one patients were treated with surgery alone, and 19 patients were treated with chemotherapy in addition to surgery (12 preoperatively and 11 postoperatively).[8]

Seven patients relapsed (five local and two combined) and three patients died because of local relapse (one of each histological type).

A SIOP/Gesellschaft für Pädiatrische Hämatologie und Onkologie (GPOH) nephroblastoma study evaluated 111 patients with congenital mesoblastic nephromas. Sixty-seven patients had classical congenital mesoblastic nephromas (60%), 29 patients had the cellular subtype (26%), and 15 patients had the mixed subtype (14%). Tumor stage consisted of stage I (35%), stage II (50%), and stage III (15%). Ninety-one patients were treated with surgery alone, and 19 patients were treated with chemotherapy in addition to surgery (12 preoperatively and 11 postoperatively).[8]

Tissue was available for translocation analysis from 79 of the 111 tumors. Within the group of cellular congenital mesoblastic nephromas, patients who had translocation-positive tumors had a significantly improved RFS compared with the patients who had translocation-negative tumors (5-year RFS rate, 100% vs. 73%).

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Preserved source evidence · Not medical advice