Oesophageal GIST—A rare breed case report and review of the literature

INTRODUCTION: Gastrointestinal stromal tumours (GIST) account for only one percent of all gastrointestinal malignancies. PRESENTATION OF CASE: A 53 year old lady presented to the gastroenterology clinic with iron deficiency anaemia. Oesophagogastroduodenoscopy (OGD) and computed tomography (CT) demonstrated a non-obstructing 7 cm submucosal oesophageal lesion. An oesophagectomy was performed and subsequent histology sections showed a well-circumscribed spindle cell tumour without any cellular atypia. Immunohistochemistry on the tumour was negative for S100 and positive for smooth muscle actin and desmin. C-kit (CD117) showed focal positivity in some of the tumour cells and the overall features were of a gastro-intestinal stromal tumour (GIST). The patient has been fully compliant with the follow-up arrangements and is disease-free six years after her original operation. DISCUSSION: GISTs are mesenchymal tumours of the gastrointestinal (GI) tract that express the KIT protein. The incidence rate for GIST is 15 cases to one million in the USA. GISTs in the oesophagus are rare (5%) and their management is surrounded by some debate. Once the diagnosis has been secured the patient should be evaluated for suitability for surgical resection. Optimal surgical therapy should aim to achieve complete resection, if possible, without injury to the pseudocapsule surrounding the tumour. Meticulous atraumatic surgical technique is necessary and preoperative biopsies are best to be avoided as they might propagate seeding. CONCLUSIONS: Oesophagectomy with clear resection margins should be the preferred method for surgical treatment combined with the use of novel TKI strategies.