Outcomes and Surgical Management of Malignant Rhabdoid Tumor of the Kidney: A Report From the Pediatric Surgical Oncology Research Collaborative. Pediatr Blood Cancer 2026 Jun;73(6):e70296
Date
03/27/2026Pubmed ID
41889149DOI
10.1002/1545-5017.70296Scopus ID
2-s2.0-105034093993 (requires institutional sign-in at Scopus site)Abstract
PURPOSE: Malignant rhabdoid tumor of the kidney (MRTK) is a rare, aggressive tumor seen in young children. The optimal timing of resection for locally advanced tumors is not well-defined. The purpose of this study is to evaluate modern oncologic outcomes and the impact of surgical timing.
METHODS: A multicenter retrospective review was performed by institutions participating in the Pediatric Surgical Oncology Research Collaborative. Children younger than 21 years old with MRTK diagnosed between 2000 and 2022 were included.
RESULTS: Sixty-nine patients were identified with MRTK and met the inclusion criteria. Median age of diagnosis was 10.1 months. Overall survival (OS) at 1, 5, and 10 years was 49%, 32%, and 19%, respectively. Patients with local Stage III disease who underwent upfront resection (n = 18) compared to those who had delayed resection after NAT (n = 15) had a similar OS, median OS greater than 60 months versus 14.6 months, respectively; p = 0.396. The surgical timing groups were balanced in terms of the presence of metastasis, length of follow-up, and tumor characteristics. There was one occurrence of primary intraoperative tumor spill and two occurrences of organ injury in the upfront resection group compared to none in the delayed resection group.
CONCLUSION: MRTK carries a poor prognosis despite multimodal treatment. Histologic diagnosis may not be confirmed at presentation, and MRTK cannot be reliably distinguished from Wilms tumor on imaging alone. As overall survival is similar, the decision regarding surgical timing in locally advanced tumors should be individualized based on perceived resectability, balancing the risk of intraoperative complications against the possibility of tumor progression during neoadjuvant therapy.
Author List
Rinehardt HN, Tracy ET, Paredes A, Beckhorn C, Leraas HJ, Fusco J, McKay KG, Kontchou NT, Kastenberg ZJ, Hoyt DW, Roach J, Myers EK, Cost NG, Mullapudi B, Marchese CR, Jensen AR, Lautz TB, Carter M, Dasgupta R, Lundstedt J, Brungardt JG, Talbot L, Davidoff AM, Murphy AJ, Aldrink JH, Mansfield S, Piche N, Le-Nguyen A, Lal DR, Craig BT, Schuh JM, Cromeens BP, Mannava S, Castle S, Lopez A, Mello K, Short J, Petroze RT, Rajavel S, Thompson GR, Mattei P, Rothstein DH, Fialkowski E, Fowler K, Martchenke N, Rich BS, Glick RD, Brown EG, Doyle K, Abril P, Seemann N, Davidson J, Wilson CA, Le HD, Joshi D, Stellon M, Ahmed T, Dimmer A, Ehrlich PF, Hammoud M, Williams K, Grant CN, Gorgy M, Polites SF, Debertin J, Cameron DB, Stetson A, Kim ES, Lee WG, Barkhordar A, Austin M, Coakley BA, Kahan A, Murphy JT, Pitonak M, Boehmer C, Malek MMAuthors
Brian T. Craig MD Assistant Professor in the Surgery department at Medical College of WisconsinDave Lal MPH, MD Chief, Professor in the Surgery department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
AdolescentChild
Child, Preschool
Female
Follow-Up Studies
Humans
Infant
Kidney Neoplasms
Male
Nephrectomy
Prognosis
Retrospective Studies
Rhabdoid Tumor
Survival Rate
Young Adult
