Citations 2
Outcomes and Surgical Management of Malignant Rhabdoid Tumor of the Kidney: A Report From the Pediatric Surgical Oncology Research Collaborative
Paredes, Anghela Z. , Beckhorn, Catherine B , Fusco, Joseph , McKay, Katlyn G. , Kontchou, Nelly‐Ange T. , Kastenberg, Zachary J. , Roach, Jonathan P. , Myers, Emily K. , Cost, Nicholas G. , Mullapudi, Bhargava , Jensen, Amanda , Dasgupta, Roshni, Lundstedt, John, Brungardt, Joseph G. , Davidoff, Andrew M., Murphy, Andrew J., Piché, Nelson , Le‐Nguyen, Annie, Craig, Brian T. , Schuh, Jennifer M , Cromeens, Barrett P., Lopez, A. Javier, Mello, Kelsey , Short, Joshua , Petroze, Robin T. , Rajavel, Shay, Thompson, Grace R., Fialkowski, Elizabeth , Fowler, Kathryn , Martchenke, Nathan, Rich, Barrie S. , Glick, Richard D. , Doyle, Kathleen , Abril, Paige, Seemann, Natashia M. , Davidson, Jacob B., Wilson, Claire A. , Joshi, Devashish , Stellon, Michael , Hammoud, Maya M. , Williams, Keyonna M , Grant, Christa N. , Gorgy, Merit, Debertin, Julia , Cameron, Danielle B. , Stetson, Alyssa , Lee, William G., Barkhordar, Aaron, Austin, Mary, Coakley, Brian A. , Murphy, Joseph T., Pitonak, M., Boehmer, Chloé , Beckhorn, Catherine , Kastenberg, Zachary J , Lautz, Timothy , Brungardt, Joseph G , Murphy, Andrew J , Thompson, Grace R , Glick, Richard D , Seemann, Natashia M , Joshi, Devashish , Stellon, Michael , Dimmer, Alexandra M , Williams, Keyonna , Polites, Stephanie F. , Stetson, Alyssa , Lee, William G , Barkhordar, Aaron , Malek, Marcus M , Rinehardt, Hannah , Tracy, Elisabeth T. , Leraas, Harold J. , Hoyt, David , Marchese, Charles R. , Lautz, Timothy B. , Carter, Michela M. , Dasgupta, Roshni , Talbot, Lindsay J , Davidoff, Andrew M. , Aldrink, Jennifer H. , Mansfield, Sara A. , Lal, Dave R. , Mannava, Sindhu V , Castle, Shannon L. , Lopez, Adriana, Rajavel, Shay , Mattei, Peter , Rothstein, David H. , Fowler, Kathryn, Brown, Erin G. , Le, Hau D. , Ahmed, Tamer, Ehrlich, Peter F. , Gorgy, Merit , Kim, Eugene S. , Kahan, Anastasia M , Murphy, Joseph T., Malek, Marcus M.
Pediatric Blood & Cancer | March 27, 2026 | Volume: 73 | Issue: 6 | Pages: No page available
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.
Contemporary Biology, Management, and Outcomes of Renal Medullary Carcinoma in Children and Adults: A Pediatric Surgical Oncology Research Collaborative Study
Beckhorn, Catherine B , Malek, Marcus M. , Lovvorn, Harold N. , McKay, Katlyn G. , Kontchou, Nelly‐Ange T. , Kastenberg, Zachary J. , Hoyt, David , Roach, Jonathan P. , Myers, Emily K. , Cost, Nicholas G. , Mullapudi, Bhargava , Marchese, Charles R. , Jensen, Amanda , Lundstedt, John, Brungardt, Joseph G. , Davidoff, Andrew M. , Piché, Nelson , Craig, Brian T. , Schuh, Jennifer M , Cromeens, Barrett P., Castle, Shannon L. , Mello, Kathryn Bandeira de, Short, Joshua , Petroze, Robin T. , Rajaval, Shay, Thompson, Grace R., Fialkowski, Elizabeth , Martchenke, Nathan, Rich, Barrie S. , Glick, Richard D. , Doyle, Kathleen , Abril, Paige, Seemann, Natashia M. , Davidson, Jacob B., Wilson, Claire A. , Joshi, Devashish , Stellon, Michael , Newman, Erika A. , Hammoud, Maya M. , Williams, Keyonna M , Grant, Christa N. , Gorgy, Merit , Polites, Stephanie F. , Debertin, Julia , Cameron, Danielle B. , Stetson, Alyssa , Lee, William G. , Barkhordar, Aaron, Austin, Mary T. , Coakley, Brian A. , Kahan, Anastasia M , Murphy, Joseph T., Pitonak, Michael, Boehmer, Chloé , Tracy, Elisabeth T. , Kastenberg, Zachary J , Hoyt, David W , Cost, Nicholas G , Marchese, Charles R , Lautz, Timothy , Lundstedt, John , Brungardt, Joseph G , Talbot, Lindsay J , Murphy, Andrew J , Aldrink, Jennifer H , Piche, Nelson , Mannava, Sindhu V , Fialkowski, Elizabeth A , Rich, Barrie S , Seemann, Natashia M , Stellon, Michael , Dimmer, Alexandra M , Williams, Keyonna , Barkhordar, Aaron , Beckhorn, Catherine , Tracy, Elisabeth T , Malek, Marcus M , Kahan, Anastasia Morgan , Leraas, Harold J. , Lautz, Timothy B. , Carter, Michela M. , Dasgupta, Roshni , Murphy, Andrew J. , Aldrink, Jennifer H. , Mansfield, Sara A. , Lal, Dave R. , López, Adriana , Mattei, Peter , Rothstein, David H. , Fowler, Kathryn L. , Brown, Erin G. , Abril, Paige, Rinehardt, Hannah , Le, Hau D. , Ahmed, Tamer, Kim, Eugene S. , Pitonak, M.
Pediatric Blood & Cancer | May 21, 2025 | Volume: 72 | Issue: 8 | Pages: No page available
ABSTRACTBackgroundRenal medullary carcinoma (RMC) is an aggressive tumor associated with sickle cell trait. Despite treatment advances for other rare renal tumors, RMC survival remains poor. We aimed to describe the contemporary management and survival of children and adults with RMC.ProcedureIn this multicenter retrospective cohort study, Pediatric Surgical Oncology Research Collaborative sites searched their databases for patients diagnosed with RMC (2000–2022). Descriptive statistics were calculated and survival analyses performed using Kaplan–Meier and Cox regression.ResultsThirty‐four patients with RMC were identified. Median age was 19 years (IQR: 15–28; range: 7–52). Most were male (24/34; 71%), Black (27/32; 84%), had sickle cell trait or disease (30/33; 91%), presented with metastatic disease (27/34; 79%), and were symptomatic at presentation (32/34; 94%). Median overall survival (OS) was 24 months from diagnosis (16 months for children, 28 months for adults, p = 0.6). Receipt of platinum‐based chemotherapy (23/34; 68%) was associated with significantly higher OS than other regimens (35 vs. 5 months, p < 0.001). Nephrectomy (24/34; 71%) was associated with significantly improved OS compared with non‐operative management (34 vs. 7 months, p = 0.001). Immunotherapy, targeted therapy, or radiation therapy were not associated with significant differences in OS, nor were age, sex, race, sickle cell status, SMARCB1/INI‐1, stage, nephrectomy approach, retroperitoneal lymph node dissection, gross residual disease, margins, or tumor size.ConclusionsRMC survival remains poor despite newer therapies. Nephrectomy and platinum‐based chemotherapy should be considered in locally advanced and metastatic disease. Coordinated international cooperative group studies are needed to meaningfully improve RMC survival.