Inflammatory myofibroblastic tumor: A multi-institutional study from the Pediatric Surgical Oncology Research Collaborative

Barrie S. Rich, Joanna Fishbein, Timothy Lautz, Nathan S. Rubalcava, Tanvi Kartal, Erika Newman, Pei En Wok, Rodrigo L.P. Romao, Richard Whitlock, Bindi Naik-Mathuria, Stephanie F. Polites, Katrine Løfberg, Danny Lascano, Eugene Kim, Jacob Davidson, Andreana Bütter, Zachary J. Kastenberg, Scott S. Short, Rebecka L. Meyers, Rosemarie MastropoloMarcus M. Malek, Jennine Weller, Ahmer Irfan, Daniel S. Rhee, Alan F. Utria, David H. Rothstein, Kimberly Riehle, Sarah Jane Commander, Elisabeth Tracy, Kerri Becktell, Brian Hallis, Dave Lal, Orville Li, Dorothé B. Dal-Soglio, Nelson Piché, Oswaldo Gomez Quevedo, Andrew J. Murphy, Andrew M. Davidoff, Jo Cooke Barber, Erin Watters, Roshni Dasgupta, Richard D. Glick

Research output: Contribution to journalArticlepeer-review

7 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1059-1067
Number of pages9
JournalInternational Journal of Cancer
Volume151
Issue number7
DOIs
StatePublished - Oct 1 2022
Externally publishedYes

Keywords

  • inflammatory myofibroblastic tumor
  • pediatric

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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