ABSTRACT
Gastrointestinal stromal tumours (GISTs) are neoplasms that arise either from the mesenchymal tissue of the gastrointestinal tract (GIT) or, rarely, from other intra-abdominal soft tissues.
They represent the most common (85%) mesenchymal tumors that affect the GIT, and form 0.1% ̶ 3% of GI malignancies. More than 80% occurs in individuals older than 50 years, with no sex predilection.
GISTs may develop anywhere along the tubular GIT from the oesophagus to the anorectum; most commonly they affect the stomach and small intestine. They vary greatly in size from a few mm to more than 30 cm. The malignant potential of GISTs ranges from small lesions with a benign behavior to aggressive sarcomas.
The clinical manifestations of GISTs are usually related to tumor location, biological features, and disease spread. The most common presentation is GI bleeding or bleeding into the abdominal cavity with acute anaemia and abdominal pain. The initial diagnosis of GIST may be delayed due to non-specific presentation of the disease. Contrast-enhanced CT of the abdomen and pelvis is the imaging modality of choice.
Surgical removal is the primary treatment of choice in localized GISTs. The main aim of surgical treatment is to achieve complete (R0) resection of GISTs with their pseudocapsule intact. The innovative specific, molecularly-targeted therapies such as imatinib mesylate which is a receptor tyrosine kinase inhibitor is the only adjuvant or neoadjuvant treatment for patients with GISTs who are at intermediate or high risk of recurrence. Imatinib prevents recurrent or metastatic GIST progression and improves the progression-free survival and the overall survival.
In spite of all these data, many surgeons don’t know much about these aggressive tumours.
The aim of this work is to address some important data about these rare fatal tumours and to focus on different updates in their management options including the new highly effective targeted therapies.