Women's and Children's Hospital, Adelaide, Australia.
J Pediatr Gastroenterol Nutr. 2022 Feb 1;74(2):253-257. doi: 10.1097/MPG.0000000000003311.
Inflammatory myofibroblastic tumours (IMTs) are rare soft tissue tumours. Reports of gastrointestinal tract, liver and pancreas tumours are limited. The objective of this study is to identify presenting features, contributing prognostic / etiological factors and any variability in outcomes in the context of different historical treatments. We retrospectively reviewed the records of seven children treated at our hospital between 2006 and 2019 and assessed the demographic, presentation, treatment, immunohistochemistry, and outcomes of their tumours. Age range at presentation was 4 months-15 years with a male predominance. Presentations were typically due to local mass effect or incidental discovery. Systemic symptoms were rare. Outcomes were good with six out of seven stable or in remission irrespective of treatment. Surgical resection where possible is the treatment of choice. Medical therapy had good outcomes with chemotherapy acting as first line treatment when required. The only negative prognostic factor identified was local spread at the time of presentation.
炎性肌纤维母细胞瘤（IMTs）是一种罕见的软组织肿瘤。关于胃肠道、肝脏和胰腺肿瘤的报道有限。本研究的目的是确定不同历史治疗背景下的表现特征、有助于预后/病因的因素以及结果的任何差异。我们回顾性地审查了 2006 年至 2019 年在我院治疗的 7 名儿童的记录，并评估了他们肿瘤的人口统计学、表现、治疗、免疫组织化学和结果。就诊时的年龄范围为 4 个月至 15 岁，男性居多。表现通常是由于局部肿块效应或偶然发现。全身症状罕见。结果良好，7 例中有 6 例稳定或缓解，无论治疗与否。尽可能进行手术切除是首选治疗方法。化疗作为一线治疗药物的医学治疗有良好的效果。唯一确定的负预后因素是就诊时的局部扩散。
