Inflammatory Myofibroblastic Tumors in Children: A Clinical Retrospective Study on 19 Cases

Abstract

Background: Inflammatory myofibroblastic tumor (IMFT) is a rare neoplasm mainly affecting children and young adults. We conducted a retrospective study to evaluate the clinical features and treatment alternatives of childhood inflammatory myofibroblastic tumors.Methods: A total of 19 patients who were pathologically diagnosed with IMT between December 2008 and October 2018 were included. Collected data were demographic information, main complaints, tumor characteristics, treatment, pathological results, immunohistochemical analysis, and prognosis.Results: The male/female ratio was 13:6. The mean age at disease onset was 44.9 ± 33.9 months (range 4 to 111 months). The mean tumor size was 6.5 ± 4.0 cm (range 1.2 to 17.0 cm). The most common site was the abdomen (13/19). The most commonly used detection tool was CT. Eleven patients (57.9%) had aggressive tumor growth, including eight receiving extensive resection and three receiving palliative resection due to high local invasiveness and postoperative chemotherapy. Eight cases whose tumors were completely enveloped received complete resection. Immunohistochemistry was performed for 17 patients and ALK positivity was found in 11 patients. Despite three children lost to follow-up, sixteen patients were followed up for 6 to 132 months (average 63.9 months, median 66 months). Of which, twelve children survived with no evidence of IMT, and four cases (21%) showed local recurrences (two of them died). No distant metastasis was detected.Conclusions: IMT is rare in children with various locations, mostly appearing in the abdomen. Whether the tumor could be completely removed, the location and the invasiveness of surrounding tissues might be highly prognosis-related.

An extremely rare case of malignant jejunal mesenteric inflammatory myofibroblastic tumor in a 61-year-old male patient: A case report and literature review

“…IMT imitates malignant neoplasm, with a local recurrence rate of up to 25% ( 8 , 13 ). Based on demographic data, IMT often affects children and young adults ( 14 ). While some reported female predominance in IMT cases (female:male = 1.67:1) ( 15 ), others reported the opposite sex ratio ( 16 ).…”

Child inflammatory myofibroblastic tumor of the kidney misdiagnosed as Wilms’ tumor : Case report

“…Inflammatory myofibroblastic tumor (IMT) is also known as inflammatory pseudotumor, plasma cell granuloma, fibrous xanthogranuloma and myofibroblastoma. 5 Histologically, it is composed of spindle cells with an inflammatory infiltrate of plasma cells, lymphocytes, and/or eosinophils. Microscopically, three histologic patterns have been previously described: Type I was characterized as loosely organized spindle cells admixed with small blood vessels and inflammatory cells in a myxoid background, namely mucous/vascular intensive; Type II is featured with dense clumps of spindle cells, scattered in a small number of inflammatory cells, called spindle cell rich type; Type III is the hypocellular fibrous pattern, usually occurs in soft tissue of limbs, showing large collagen fibers mixed with a few tumor cells and local calcification or ossification.…”

Update of Diagnosis and Targeted Therapy for ALK+ Inflammation Myofibroblastic Tumor

“…The most common sites of IMT occurrence vary among studies; the lungs [ 1 , 28 ], abdomen [ 13 , 29 ], and soft tissues of the limbs or hips [ 16 ] have been reported. However, other anatomical sites [ 29 ], including the meninges of lobes, spinal cord, orbit, mandible, throat, thorax, heart, liver, duodenum, small intestine, colon, and uterus, have also been reported [ 16 ].…”

“…In terms of demographics, despite IMT can be diagnosed at any age, it seems to have a predilection for children and young adults [ 30 – 32 ]. Prevalence according to sex has been inconsistent among studies [ 13 , 16 , 28 , 29 ]. Overall, the general prevalence of IMT ranges from 0.04 to 0.7%, irrespective of sex or race [ 33 , 34 ].…”

“…In summary, pathological and immunohistochemical tests are considered the gold standard for IMT diagnosis [ 29 ]; however, NGS can provide genetic information for more appropriate treatments.…”