Advanced and Metastatic Gastrointestinal Stromal Tumors Presenting With Surgical Emergencies Managed With Surgical Resection: A Case Series.

Advanced and metastatic gastrointestinal stromal tumors (GISTs) presenting with surgical emergencies are rare. Neoadjuvant imatinib being the treatment of choice for non-metastatic advanced disease with a proven role in downstaging the disease may not be feasible in patients presenting with bleeding and obstruction. We present a case series with retrospective analysis of a prospectively maintained database of patients with advanced and metastatic GISTs presenting with surgical emergencies. Clinical characteristics, imaging and endoscopic findings, surgical procedures, histological findings, and outcomes in these patients were studied. Four patients were included in this case series, with three males and one female (age range: 24-60 years). Two patients presented with melena; one with hemodynamic instability despite multiple blood transfusions underwent urgent exploratory laparotomy for bleeding gastric GIST, while the other underwent surgical exploration after careful evaluation given the recurrent, metastatic disease with a stable metabolic response on six months of imatinib. One patient with metastatic jejunal GIST who presented with an umbilical nodule and intestinal obstruction was given a trial of non-operative management for 72 hours, but due to non-resolution of obstruction, segmental jejunal en bloc resection with the dome of the urinary bladder with reconstruction and metastasectomy was needed. The patient with advanced gastric GIST who presented with gastric outlet obstruction was resuscitated, and an attempt of endoscopic naso-jejunal tube placement was tried, which failed, and exploration was needed. The mean length of hospital stay was 7.5 days. Histopathological examination confirmed GIST in all four patients with microscopic negative resection margins. All patients were started on imatinib with dose escalation to 800 mg in the patient with recurrent and metastatic disease; however, the patient with bleeding gastric GIST experienced severe adverse effects of imatinib and discontinued the drug shortly. All four patients are disease-free on follow-ups of 15 months, 48 months for the patient with advanced non-metastatic disease, and six and 24 months for the patients with metastatic disease. In the era of tyrosine kinase inhibitor (TKI) therapy for advanced and metastatic disease, upfront surgery is usually reserved for surgical emergencies only. Surgical resection, the cornerstone for the treatment of resectable GIST, may also be clinically relevant in metastatic settings, although it requires a careful and individualized approach.

Neuroendocrine tumour of the lesser omentum.

A man in his early 20s presented to us in the outpatient department with a history of diarrhoea for 4 months. Investigations revealed elevated serum chromogranin levels and an intensely avid lesion in the gastrohepatic ligament in Gallium DOTATATE positron emission tomography (PET). The tumour was excised laparoscopically, and no other lesions were seen. The patient improved clinically and had a normal serum chromogranin level postoperatively. He is currently much improved at the 1year follow-up. We did an extensive workup to look for a primary tumour. It was concluded that it was a de novo tumour arising from the lesser sac. The recommended investigations in case of neuroendocrine tumour (NET) with unknown primary include blood investigations to look for the functional status of the tumour, histopathological examination, including immunohistochemistry, and radiological imaging, which must include a Gallium DOTATATE PET. An isolated NET of the lesser sac has not been reported in the literature.

Small bowel obstruction: a rare presentation of systemic mastocytosis.

Mastocytosis (MC) is a heterogeneous group of disorders characterised by abnormal growth, neoplastic proliferation and accumulation of mast cells. Approximately, 80% of patients with MC have evidence of skin involvement, while the rest may involve the gastrointestinal tract, liver, spleen or bone. Isolated gastrointestinal presentation of aggressive MC without bone marrow involvement and cutaneous symptoms is rare. Isolated MC with tumour cells infiltrating the ileum presenting with mechanical obstruction has not been reported in the literature to date. Here, we present a case of a patient in his late 50s who presented with malignant MC of the small bowel with obstruction in the emergency surgical outpatient department. The patient underwent surgical resection of the affected bowel. Histopathological examination along with immunohistochemistry revealed malignant MC. The further evaluation consisted of bone marrow biopsies, PET CT and other mutation analyses. However, the patient succumbed to death during the further course of treatment. The differential of MC must be kept in mind when there is the presence of abnormal hematopoietic cells in gastrointestinal biopsies even in the absence of cutaneous manifestations and bone marrow abnormalities.

Visceral Obesity as a Predictor of Postoperative Complications After Pancreaticoduodenectomy.

INTRODUCTION: Obesity is associated with increased morbidity and mortality post surgery. The measurement of visceral obesity can predict postoperative outcomes after pancreaticoduodenectomy. METHODS: This is a prospective observational study. Visceral obesity was calculated by measuring the fat thickness in the retro-renal area by using a computed tomography scan. Visceral obesity was defined as retro-renal fat thickness (RRFT) of ≥ 2 cm. Patients were divided into two groups: Group-A (RRFT < 2 cm, non-obese) and Group-B (RRFT > 2 cm, obese). Demographic, clinical, and intraoperative variables were correlated with postoperative outcomes. RESULTS: Fifty-six patients were included in the study. Thirty-two patients were included in Group-A, and 24 patients were included in Group-B. The two groups had comparable outcomes. A total of 21 patients in Group-A (65.62%) and 17 patients in Group-B (70.83%) had comorbidities, including diabetes mellitus, hypertension, and coronary disease (p=0.680). American Society of Anesthesiologists (ASA) grading was comparable (p=0.927). BMI was also comparable (p=0.354). Type of pancreaticoduodenectomy, pancreatic texture, pancreatic duct diameter, and technique of pancreaticojejunostomy anastomosis were comparable. The mean operative time was longer in Group-B (362 ± 36.2 min vs. 298 ± 45.2 min) (p=0.001). Intraoperative blood loss was more in Group-B (312 ± 36.8 ml vs. 267 ± 23.7 ml) (p=0.001). The rates of postoperative pancreatic fistula and delayed gastric emptying were comparable (p=0.402 and p=0.134, respectively). The length of hospital stay was longer in patients in Group-B (p=0.004). There was one death in Group-B (obese group). CONCLUSION: Visceral obesity is a risk factor for postoperative complications after a pancreaticoduodenectomy.

Correlation of Multi-Detector Computed Tomography and Intraoperative Variations of the Celiac Trunk and Hepatic Artery in Resectable Hepatobiliary Pancreatic Cancers.

Introduction Knowledge of celiac artery variations is imperative to perform complex hepato-biliary pancreatic surgical procedures to avoid inadvertent complications. Multi-detector computed tomographic (MDCT) angiography aids in detecting these variations preoperatively. Surgical confirmation is considered the gold standard. Aims and objectives Preoperative assessment of celiac artery variations by MDCT angiography and surgical confirmation intraoperatively in resectable hepato-biliary pancreatic cancers. Patients and methods MDCT angiography was performed in 40 patients with clinical evidence of resectable hepato-biliary-pancreatic cancers. Three dimensional (3D) reconstructions were performed to confirm the celiac artery variations. Surgery was performed as per the institute's protocol in all these patients for resection of tumor and confirmation of celiac artery anatomy. Variations were confirmed surgically that were identified through imaging. Results MDCT angiography identified normal trifurcated celiac artery anatomy in 33 (82.5%) patients and variant anatomy in seven (17.5%) patients. The most common variation was a replaced right hepatic artery (r-RHA) from the superior mesenteric artery (SMA) in four (10%) of patients. A replaced left hepatic artery (r-LHA) from the celiac trunk, a common hepatic artery (CHA) from the abdominal aorta, and an accessory right hepatic artery (ac-RHA) from the proper hepatic artery itself were identified in one (2.5%) patient each, respectively. All these findings were confirmed intraoperatively. There was a 100% statistical correlation between imaging and surgical findings. Conclusion Surgical confirmation of radiological data of celiac artery variations is the gold standard to avoid disastrous complications such as inadvertent vascular bleeds, biliary injuries, and hepatic necrosis. Since the presence of variations warrants the preservation or excision of the arterial system without oncological compromise and minimizing surgical complications.