Citations 12
Inflammatory myofibroblastic tumor: A multi‐institutional study from the Pediatric Surgical Oncology Research Collaborative
Rich, Barrie S. , Fishbein, Joanna , Lautz, Timothy B. , Rubalcava, Nathan S. , Kartal, Tanvi , Newman, Erika, Wok, Pei En, Romao, Rodrigo , Polites, Stephanie F. , Løfberg, Katrine , Lascano, Danny , Davidson, Jacob , Bütter, Andreana , Short, Scott S. , Meyers, Rebecka L. , Mastropolo, Rosemarie, Weller, Jennine , Irfan, Ahmer , Rhee, Daniel S. , Riehle, Kimberly J. , Tracy, Elisabeth T. , Becktell, Kerri, Hallis, Brian, Lal, Dave R. , Li, Orville, Dal‐Soglio, Dorothé B., Piché, Nelson , Quevedo, Oswaldo Gomez, Davidoff, Andrew M. , Barber, Jo Cooke, Watters, Erin, Dasgupta, Roshni , Glick, Richard D. , Lautz, Timothy , Tracy, Elisabeth T , Dasgupta, Roshni , Whitlock, Richard S. , Naik‐Mathuria, Bindi , Kim, Eugene S. , Kastenberg, Zachary J. , Malek, Marcus M. , Utria, Alan Fremder , Rothstein, David H. , Commander, Sarah Jane , Li, Orville, Dal-Soglio, Dorothée, Murphy, Andrew J. , Watters, Erin
International Journal of Cancer | May 23, 2022 | Volume: 151 | Issue: 7 | Pages: 1059-1067
Abstract Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate malignancy. We describe the largest cohort of IMT patients to date, aiming to further characterize this rare, poorly understood tumor. This is a multi‐institutional review of IMT patients ≤39 years, from 2000 to 2018, at 18 hospitals in the Pediatric Surgical Oncology Research Collaborative. One hundred and eighty‐two patients were identified with median age of 11 years. Thirty‐three percent of tumors were thoracic in origin. Presenting signs/symptoms included pain (29%), respiratory symptoms (25%) and constitutional symptoms (20%). Median tumor size was 3.9 cm. Anaplastic lymphoma kinase (ALK) overexpression was identified in 53% of patients. Seven percent of patients had distant disease at diagnosis. Ninety‐one percent of patients underwent resection: 14% received neoadjuvant treatment and 22% adjuvant treatment. Twelve percent of patients received an ALK inhibitor. Sixty‐six percent of surgical patients had complete resection, with 20% positive microscopic margins and 14% gross residual disease. Approximately 40% had en bloc resection of involved organs. Median follow‐up time was 36 months. Overall 5‐year survival was 95% and 5‐year event‐free survival was 80%. Predictors of recurrence included respiratory symptoms, tumor size and distant disease. Gross or microscopic margins were not associated with recurrence, suggesting that aggressive attempts at resection may not be warranted.
Wilms Tumor (Nephroblastoma), Version 2.2021, NCCN Clinical Practice Guidelines in Oncology
Anderson, Clarke, Cook, Shelly, Gow, Kenneth W. , Hiniker, Susan M. , Jasty‐Rao, Rama, Lin, Chi , Lovvorn, Harold N. , MacEwan, Iain, Martínez-Agosto, Julián A. , Mullen, Elizabeth A. , Murphy, Erin S. , Ranalli, Mark , Rhee, Daniel S. , Tracy, Elisabeth T. , Vern-Gross, T.Z., Walsh, Michael F. , Wickiser, Jonathan E., Zapala, Matthew A. , Berardi, Ryan A., Hughes, Miranda, Vern-Gross, Tamara, Tracy, Elisabeth T , Balis, Frank M. , Green, Daniel M. , Dhillon, Jasreman , Rokitka, Denise A. , Walz, Amy L.
Journal of the National Comprehensive Cancer Network | August 20, 2021 | Volume: 19 | Issue: 8 | Pages: 945-977
The NCCN Guidelines for Wilms Tumor focus on the screening, diagnosis, staging, treatment, and management of Wilms tumor (WT, also known as nephroblastoma). WT is the most common primary renal tumor in children. Five-year survival is more than 90% for children with all stages of favorable histology WT who receive appropriate treatment. All patients with WT should be managed by a multidisciplinary team with experience in managing renal tumors; consulting a pediatric oncologist is strongly encouraged. Treatment of WT includes surgery, neoadjuvant or adjuvant chemotherapy, and radiation therapy (RT) if needed. Careful use of available therapies is necessary to maximize cure and minimize long-term toxicities. This article discusses the NCCN Guidelines recommendations for favorable histology WT.