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• W4220668200 abstract "A fit and healthy 47-year-old male presented with abdominal distension, an altered bowel habit and a palpable epigastric mass on examination. Outpatient computed tomography (CT) demonstrated a heterogeneously enhancing left abdominal lesion extending from the level of the gastro-oesophageal junction, interposed between the stomach anteriorly, the liver on the right and the left kidney, spleen, and pancreas on the left, to the level of the umbilicus inferiorly (See Fig. 1). No locoregional lymphadenopathy or distant metastatic disease was identified on CT. The patient was admitted under a specialist Upper GI surgery unit at a tertiary hospital for further staging investigations, with the mass demonstrating FDG-PET avidity. Based on these findings the initial impression was that of a retroperitoneal sarcoma. Following discussion at an Upper GI oncological MDT, the location, mass effect and rapidly increasing size of the tumour was thought more in keeping with a diagnosis of gastrointestinal stromal tumour (GIST). As such the decision was made to proceed to open surgical excision with curative intent. A midline laparotomy was performed and after mobilization of the omentum from the colon the tumour was visualized arising from the posterior stomach. Extensive left-sided mobilization revealed the mass to be inseparable from the tail of the pancreas and the spleen and as such a partial gastrectomy, distal pancreatectomy and splenectomy were performed. The patient had an uneventful recovery from surgery, was administered routine post-splenectomy vaccinations and referred to the local splenectomy registry. The resected specimen measured 240 mm × 230 mm × 170 mm and weighed 3585 g (see Fig. 2) and on histopathological examination was consistent with a GIST, AJCC stage p3A. The margins were clear, with no nodal involvement. Based on the modified Fletcher et al. histopathological criteria the tumour was deemed ‘High Risk’.1 The patient was commenced on adjuvant imatinib under the direction of medical oncology. This 24 cm × 23 cm × 17 cm, roughly 3.5 kg GIST represents one of the largest tumours of its kind ever removed in Australia. Although accounting for only 1–2% of all gastrointestinal neoplasms,2 GISTs, arising from precursor cells of the Interstitial cells of Cajal, are the most common type of sub-epithelial, mesenchymal tumour of the gastrointestinal tract (GIT) and occur at an estimated incidence of 6.8–14.5 cases per million individuals per year.3 GISTs can arise anywhere along the GIT, most commonly the stomach (60%) and small intestine (30%).2 GISTs remain asymptomatic in approximately 20% of patients, with incidental micro-GISTs (<1 cm) frequently discovered at endoscopy or on abdominal imaging.3 Symptomatic lesions typically declare themselves in the sixth or seventh decades of life and depending on their size may present as abdominal pain, fullness, early satiety or gastric outlet obstruction due to mass effect.3 Although originating in the submucosal, stromal elements of the GIT, GISTs can invade into either the serosal and/or mucosal layers of the GIT and can therefore also present with ulceration, bleeding, melaena and if sustained; anaemia and its sequalae.3 Most widely used risk stratification methods for GISTs require surgical excision or biopsy of the mass and histopathological analysis of tumour size, mitotic count and specific genetic mutations. The present case provides a useful demonstration of some of the morphological CT imaging findings identified by Cannella et al. in their recent study as being independently associated with high risk GISTs, including size >5 cm, heterogeneous enhancement and enlarged feeding vessels.4 Utilizing pre-operative CT to assist in risk stratification allows for better prognostication and treatment planning for patients newly diagnosed with GISTs.4 Despite demonstrable survival benefits from targeted tyrosine kinase inhibitors such as imatinib, surgical excision remains the gold-standard treatment of GISTs.2, 3, 5 Broadly, this involves (where possible) ruling out metastatic disease with staging imaging followed by en-bloc, occasionally multi-visceral resection, taking meticulous care to avoid tumour rupture and spillage so as to avoid peritoneal seeding of disease. Interestingly, a recent meta-analysis demonstrated similar short and long-term surgical and oncological outcomes for laparoscopic and open approaches to large gastric GISTs greater than 5 cm, thus suggesting the former approach should be used when able for larger tumours.5 However, there exists a relative paucity of evidence around the best management of giant GISTs and most of the literature pertaining to such large tumours consists of case reports describing en-bloc resection, as undertaken here6-10 (see Table 1). The present case contributes to this small body of literature and along with Table 1 demonstrates that these massive tumours can indeed be removed, en-bloc, with a high degree of safety. Mohamed et al.8 42 cm × 31 cm × 23 cm Weight – 18.5 kg Significant blood loss requiring transfusion of 12 U PRBCs +4 U FFP + 1 U of platelets +6 L of colloid IV fluids. Division of transverse colon mesentery resulting in colonic ischemia and end colostomy formation Zhang et al.10 30 cm × 30 cm × 25 cm Weight – 11.0 kg 37 cm × 24 cm × 13 cm Weight – 8.5 kg 25 cm × 20 cm × 14 cm Weight – 7.3 kg 39 cm × 27 cm × 14 cm Weight – 6.1 kg 24 cm × 23 cm × 17 cm Weight– 3.5 kg The first author is not a surgeon in training. All authors are in agreement with the content of the manuscript. Informed consent was obtained from the patient. The manuscript has not been published previously, nor is it under consideration elsewhere. Open access publishing facilitated by The University of Newcastle, as part of the Wiley – The University of Newcastle agreement via the Council of Australian University Librarians. Luca Borruso: Conceptualization; writing – original draft; writing – review and editing. Krishna Kotecha: Conceptualization; data curation; supervision; writing – review and editing. Aleksey D'Jamirze: Conceptualization; data curation. Varsha Sharma: Conceptualization; data curation; supervision; writing – review and editing. Anubhav Mittal: Conceptualization; data curation; supervision; writing – review and editing. Jaswinder Samra: Conceptualization; data curation; supervision; writing – review and editing." @default.
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• W4220668200 date "2022-03-28" @default.
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• W4220668200 title "Do you get the <scp>GIST</scp>? Successful resection of a giant 3.5 kg gastrointestinal stromal tumour" @default.
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