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.

Hepatic artery vasospasm masquerading as early hepatic artery thrombosis in progressive familial intrahepatic cholestasis 3: a case report.

Post-liver transplant (LT) hepatic artery vasospasm is a vascular complication that is not well recognized and its incidence is not known. As a possible sequela to vasospasm, hepatic artery thrombosis is the second major cause of allograft failure after primary nonfunction and its reported incidence is 2.9% in adults and 8.3% in pediatric LT. Lacuna in knowledge regarding early hepatic artery vasospasm post-LT makes it a difficult condition to diagnose and treat, as the initial ischemic insult on graft can have devastating consequences. We report a case of pediatric progressive familial intrahepatic cholestasis type 3-related chronic liver disease who underwent cadaveric orthotopic LT and postoperatively developed fever, worsening hypotension, and elevated liver enzymes with an absence of arterial flow in intrahepatic branches on Doppler ultrasound. Suspecting early hepatic artery thrombosis, the patient was re-explored and the graft hepatic artery was found to be in a state of vasospasm. Following the infusion of intra-arterial papaverine, urokinase, and intravenous nicorandil, there was an improvement in blood flow. The patient responded well and was discharged on postoperative day 23 with normal liver enzymes.

Comparison of Efficacy of Vacuum Dressing Versus Conventional Dressing Over Autologous Split Skin Grafts in Burn Wounds: A Single-Center Prospective Cohort Study.

This study was carried out to compare the efficacy of vacuum dressing and conventional dressing over split skin grafts in burn patients. It was a single-center prospective cohort study on patients, with less than 40% TBSA burn, undergoing skin grafting at 2 different sites simultaneously over the burn wounds. One site underwent conventional dressing following grafting, and the other underwent vacuum dressing. The primary objective was to compare the percentages of skin graft uptake on Day 5 and Day 14. The secondary objectives were to compare the duration of dressings, time for complete epithelization, need for regrafting, and wound swab culture positivity rates. Three independent examiners assessed the efficacy of dressings in terms of epithelization percentage, need for continued dressing, graft loss, wound swab culture positivity rate, and qualitative assessment of grafts. A total of 16 patients were included in the study. The graft loss, number of days of dressings, and complete epithelization time were statistically lower in the negative-pressure wound therapy (NPWT) group compared with the conventional group (P values .007, .006, and 0009, respectively). The percentage of epithelization was also found to be higher in the NPWT group (P = .006). The incidence of positive cultures and clinically significant graft loss was found to be lower in the NPWT group. However, this was not found to be statistically significant. NPWT dressings can be used in burn wounds following skin grafting and have been found to reduce the time for epithelization compared to conventional dressing.

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.