Intra-abdominal Inflammatory Myofibroblastic Tumour (IMFT)-Uncommon Entity

Abstract

IMFT (inflammatory myofibroblastic tumour) is an uncommon tumour predominantly affecting the lungs and mediastinum. Most of the published literature supports that it affects children and young individuals. IMFT involving the gastrointestinal tract is rare. We report a case of multifocal IMFT affecting the GI tract which was managed with gross total excision followed by chemotherapy. Surgical resection remains the treatment of choice. The role of chemotherapy and radiation therapy remains limited. The aetiology of these tumours remains unclear and is mostly ALK-positive that could be targeted. Local recurrences are common and hence require close follow-up. The risk of recurrences and metastasis is increased in cases with TP53 positivity, aneuploidy and recurrent lesions.

Keywords: ALK Rearrangement; GI tract; IMFT; Recurrence.

Fig. 1

An approx. 8.1 × 19.2 cm (AP × TR) hypodense cystic lesion (avg. HU 10–20) with an imperceptible wall is seen occupying the abdomen and pelvic region, insinuating between bowel loops. The lesion exerts locoregional mass effect with the displacement of adjacent bowel loops. The lesion is also seen to indent on the right ureter with a prominence of the pelvicalyceal system. The extent of the lesion is as follows: superiorly reaching till hepato-renal pouch. Inferiorly, till pre-rectal space, right laterally and anteriorly abutting the abdominal wall, medially the lesion is seen to cross the midline

Fig. 2

a, b Predominantly cystic lesions of various sizes (ranging from 6 cm to 1 mm) involving omentum, and mesentery with deposits over the serosa of the small and large bowel, stomach, liver and spleen

Fig. 3

a, b The resected specimen (gross total excision along with omentectomy and appendectomy). Appendix not visualised

Fig. 4

a, b Haemotoxylin and eosin (H&E) stain depicts a hypocellular area with a myxoid background and extensive inflammatory infiltrate with plasma cells seen