BACKGROUND: Inflammatory myofibroblastic tumor (IMFT) is an uncommon neoplasm in children. METHODS: Retrospective review from 1993 to 2014 of patients ≤18years of age with a histopathologic diagnosis of IMFT treated at two tertiary centers. RESULTS: Thirty-two patients were diagnosed with IMFT. Mean (±SD) age was 9.3±5.7years at diagnosis. Tumor location was variable: abdomen/pelvis (28%), head/neck region (22%), intrathoracic (22%), genitourinary (9%), bowel (6%) liver (6%), and musculoskeletal (6%). Median follow-up was 2.6±4.6years, with 3 recurrences and 2 deaths, which occurred only after recurrence. Positive microscopic margin after resection was associated with recurrence, compared to those that had a negative margin (40% vs. 0%, p=0.04). Recurrence was associated with increased mortality (67% vs 0%, p=0.01). Time from first symptoms to resection was shorter in those with recurrence (25.8±22 vs. 179±275days, p=0.01) and in nonsurvivors (44.0±8.0 vs. 194.3±53.4days, p=0.02). Adjuvant chemotherapy, not including steroid monotherapy, either given before or after resection, was administered more often to nonsurvivors (100% vs 4%, p=0.009), and use of corticosteroids was also higher in the nonsurvivors (100% vs. 15%, p=0.04). CONCLUSIONS: IMFT is a rare pediatric neoplasm with variable locations. Complete excision is critical for cure. Proposed guidelines for diagnosis, treatment and surveillance of theses tumors in children are reported.
BACKGROUND: Inflammatory myofibroblastic tumor (IMFT) is an uncommon neoplasm in children. METHODS: Retrospective review from 1993 to 2014 of patients ≤18years of age with a histopathologic diagnosis of IMFT treated at two tertiary centers. RESULTS: Thirty-two patients were diagnosed with IMFT. Mean (±SD) age was 9.3±5.7years at diagnosis. Tumor location was variable: abdomen/pelvis (28%), head/neck region (22%), intrathoracic (22%), genitourinary (9%), bowel (6%) liver (6%), and musculoskeletal (6%). Median follow-up was 2.6±4.6years, with 3 recurrences and 2 deaths, which occurred only after recurrence. Positive microscopic margin after resection was associated with recurrence, compared to those that had a negative margin (40% vs. 0%, p=0.04). Recurrence was associated with increased mortality (67% vs 0%, p=0.01). Time from first symptoms to resection was shorter in those with recurrence (25.8±22 vs. 179±275days, p=0.01) and in nonsurvivors (44.0±8.0 vs. 194.3±53.4days, p=0.02). Adjuvant chemotherapy, not including steroid monotherapy, either given before or after resection, was administered more often to nonsurvivors (100% vs 4%, p=0.009), and use of corticosteroids was also higher in the nonsurvivors (100% vs. 15%, p=0.04). CONCLUSIONS: IMFT is a rare pediatric neoplasm with variable locations. Complete excision is critical for cure. Proposed guidelines for diagnosis, treatment and surveillance of theses tumors in children are reported.
Authors: Giacomo Giulio Baldi; Mehdi Brahmi; Salvatore Lo Vullo; Elena Cojocaru; Olivier Mir; Michela Casanova; Bruno Vincenzi; Tommaso Martino De Pas; Giovanni Grignani; Maria Abbondanza Pantaleo; Jean Yves Blay; Robin Lewis Jones; Axel Le Cesne; Anna Maria Frezza; Alessandro Gronchi; Paola Collini; Angelo Paolo Dei Tos; Carlo Morosi; Luigi Mariani; Paolo Giovanni Casali; Silvia Stacchiotti Journal: Oncologist Date: 2020-07-12
Authors: Rachel V Brady; Robert B Rebhun; Katherine A Skorupski; Jenna H Burton; Sami Al-Nadaf; Eunju Choi; Jennifer L Willcox Journal: J Vet Diagn Invest Date: 2022-03-15 Impact factor: 1.569