Journal article
Inflammatory myofibroblastic tumor: A multi-institutional study from the Pediatric Surgical Oncology Research Collaborative
International journal of cancer, Vol.151(7), pp.1059-1067
10/01/2022
DOI: 10.1002/ijc.34132
PMID: 35604778
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.
Details
- Title: Subtitle
- Inflammatory myofibroblastic tumor: A multi-institutional study from the Pediatric Surgical Oncology Research Collaborative
- Creators
- Barrie S. Rich - Cohen Children's Medical CenterJoanna Fishbein - Feinstein Institute for Medical ResearchTimothy Lautz - Northwestern UniversityNathan S. Rubalcava - C. S. Mott Children's HospitalTanvi Kartal - C. S. Mott Children's HospitalErika Newman - C. S. Mott Children's HospitalPei En Wok - Institut für Wissenschaft und KunstRodrigo L. P. Romao - Institut für Wissenschaft und KunstRichard Whitlock - Texas Children's HospitalBindi Naik-Mathuria - Texas Children's HospitalStephanie F. Polites - Mayo ClinicKatrine Lofberg - OHSU Doernbecher Childrens Hosp, Div Pediat Surg, Portland, OR USADanny Lascano - Children's Hospital of Los AngelesEugene Kim - Children's Hospital of Los AngelesJacob Davidson - London Health Sciences CentreAndreana Butter - London Health Sciences CentreZachary J. Kastenberg - Primary Children's HospitalScott S. Short - Primary Children's HospitalRebecka L. Meyers - Primary Children's HospitalRosemarie Mastropolo - Children's Hospital of PittsburghMarcus M. Malek - Children's Hospital of PittsburghJennine Weller - Johns Hopkins UniversityAhmer Irfan - Johns Hopkins UniversityDaniel S. Rhee - Johns Hopkins UniversityAlan F. Utria - Seattle Children's HospitalDavid H. Rothstein - Seattle Children's HospitalKimberly Riehle - Seattle Children's HospitalSarah Jane Commander - Duke Medical CenterElisabeth Tracy - Duke Medical CenterKerri Becktell - Medical College of WisconsinBrian Hallis - Medical College of WisconsinDave Lal - Medical College of WisconsinOrville Li - Université de MontréalDorothe B. Dal-Soglio - Centre Hospitalier Universitaire Sainte-JustineNelson Piche - Centre Hospitalier Universitaire Sainte-JustineOswaldo Gomez Quevedo - St. Jude Children's Research HospitalAndrew J. Murphy - St. Jude Children's Research HospitalAndrew M. Davidoff - St. Jude Children's Research HospitalJo Cooke Barber - Cincinnati Children's Hospital Medical CenterErin Watters - Cincinnati Children's Hospital Medical CenterRoshni Dasgupta - Cincinnati Children's Hospital Medical CenterRichard D. Glick - Cohen Children's Medical Center
- Resource Type
- Journal article
- Publication Details
- International journal of cancer, Vol.151(7), pp.1059-1067
- DOI
- 10.1002/ijc.34132
- PMID
- 35604778
- NLM abbreviation
- Int J Cancer
- ISSN
- 0020-7136
- eISSN
- 1097-0215
- Publisher
- Wiley
- Number of pages
- 9
- Language
- English
- Date published
- 10/01/2022
- Academic Unit
- Surgery
- Record Identifier
- 9985014847002771
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