Citations 2
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.
Incidence and management of pleural effusions in patients with Wilms tumor: A Pediatric Surgical Oncology Research Collaborative study
Al‐Hadidi, Ameer , Rinehardt, Hannah , Sutthatarn, Pattamon , Condon, Sienna, Chen, Stephanie , Wong‐Michalak, Shannon , Short, Scott S. , Meyers, Rebecka L. , Johnston, Michael E. , Zens, Tiffany , Dasgupta, Roshni , Calabro, Kristen , Piché, Nelson , Callas, Hannah, McKay, Katlyn G. , Lovvorn, Harold N. , Tracy, Elisabeth T. , Lund, Sarah , Polites, Stephanie F. , Davidson, Jacob , Dhooma, Janel, Seemann, Natashia M. , Marquart, John , Gainer, Haley , Lal, Dave R. , Rich, Barrie S. , Glick, Richard D. , Maloney, Lauren , Radu, Stephani, Fialkowski, Elizabeth , Kwok, Pei En , Romao, Rodrigo , Newman, Erika A. , Diehl, Thomas M. , Polcz, Valerie E., Petroze, Robin T. , Aldrink, Jennifer H. , Lautz, Timothy , Lund, Sarah B, Tracy, Elisabeth T , Aldrink, Jennifer H , Dasgupta, Roshni , Talbot, Lindsay J , Murphy, Andrew J. , Whitlock, Richard S. , Naik‐Mathuria, Bindi , Utria, Alan Fremder , Rothstein, David H. , Kim, Eugene S. , Kastenberg, Zachary J. , Malek, Marcus M. , Lautz, Timothy B. , Commander, Sarah Jane , Radu, Stephanie , Rubalcava, Nathan S. , Ehrlich, Peter F. , Le, Hau D. , Stanek, Joseph
International Journal of Cancer | June 24, 2022 | Volume: 151 | Issue: 10 | Pages: 1696-1702
AbstractWilms tumor (WT) is the most common renal malignancy in children. Children with favorable histology WT achieve survival rates of over 90%. Twelve percent of patients present with metastatic disease, most commonly to the lungs. The presence of a pleural effusion at the time of diagnosis of WT may be noted on staging imaging; however, minimal data exist regarding the significance and prognostic importance of this finding. The objectives of our study are to identify the incidence of pleural effusions in patients with WT, and to determine the potential impact on oncologic outcomes. A multi‐institutional retrospective review was performed from January 2009 to December 2019, including children with WT and a pleural effusion on diagnostic imaging treated at Pediatric Surgical Oncology Research Collaborative (PSORC) participating institutions. Of 1259 children with a new WT diagnosis, 94 (7.5%) had a pleural effusion. Patients with a pleural effusion were older than those without (median 4.3 vs 3.5 years; P = .004), and advanced stages were more common (local stage III 85.9% vs 51.9%; P < .0001). Only 14 patients underwent a thoracentesis for fluid evaluation; 3 had cytopathologic evidence of malignant cells. Event‐free and overall survival of all children with WT and pleural effusions was 86.2% and 91.5%, respectively. The rate and significance of malignant cells present in pleural fluid is unknown due to low incidence of cytopathologic analysis in our cohort; therefore, the presence of an effusion does not appear to necessitate a change in therapy. Excellent survival can be expected with current stage‐specific treatment regimens.