Paediatric Gastrointestinal, Hepatic and Pancreatic Inflammatory Myofibroblastic Tumours, A Single Centre Experience

Abstract

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.

Development and validation of a nomogram for predicting survival in patients with malignant myofibroblastic tumor

“…Curative resection remains the primary treatment modality for myofibroblastic tumors. 29 According to Meng et al grade 1 MS should be managed with wide excision and long‐term follow‐up, and grade 2 MS should be managed by excision with a wide margin of normal tissue and adjuvant radiation therapy or systemic chemotherapy. 3 Casanova et al reported that IMT patients treated with full surgical resection had excellent outcomes and required no adjunctive therapy.…”

“…31 Postoperative radiotherapy has been reported to be potentially effective in MS. 32 For locally advanced or metastatic myofibroblastic tumors, chemotherapy and radiotherapy can be considered. 29 Notably, alterations of the ALK gene are the predominant molecular characteristics of IMTs. 33 The ALK inhibitor crizotinib is, therefore, a recommended targeted agent for the treatment of IMT.…”

“…Because the incidence of myofibroblastic tumors is extremely rare, the standardization of its treatments is highly warranted. Curative resection remains the primary treatment modality for myofibroblastic tumors 29 . According to Meng et al grade 1 MS should be managed with wide excision and long‐term follow‐up, and grade 2 MS should be managed by excision with a wide margin of normal tissue and adjuvant radiation therapy or systemic chemotherapy 3 .…”