We present a female patient, 13 years old, with diagnosis of hepatocellular carcinoma of fibrolamellar type, which was rapidly evolving. The fibrolamellar hepatocellular carcinoma invaded more than 80% of the hepatic parenchyma without surgical possibility or liver transplantation. Measures applied corresponded to chemotherapy of 1 cycle of cisplatin 40 mg/s/5 days + vincristine 1.5 mg/m2/day, 5-fluorouracil, doxorubicin, and dexrazoxane. The case presented aggressive evolution of hepatocellular carcinoma, which led to acute liver failure, with hyperammonemia, sepsis, pulmonary focus plus septic shock, grade III-IV encephalopathy, portal hypertension, and ascites with intra-abdominal hypertension. Death occurred due to multiple organ failure, which involved respiratory failure type KDIGO 1 and 2, acute liver failure, severe pneumonia, pericardial effusion, AKIN 2 acute kidney injury, carcinoma, and pulmonary metastasis. This type of ailment is infrequent in children and adolescents, and the first symptoms are crucial to achieve treatment possibilities.
In the digestive system, mesenchymal origin of tumors is quite rare; in general, they are recognized as gastrointestinal stromal tumors (GISTs). The incidence of GISTs is very low (2 in 100,000), while jejunal GISTs are extremely rare, accounting for 0.1-3% of all gastrointestinal (GI) tumors. Small intestinal GISTs are the second most common (25%) site in the GI tract, usually occurring in the duodenum. We present the case of a 62-year-old Bangladeshi female with a history of GI bleeding 3 years earlier; the cause of the bleeding had not been found despite extensive investigations. In the meantime, the patient had developed occasional abdominal pain and lumpy feelings in the right side of the abdomen without any GI bleeding. Exploratory laparotomy was carried out in view of a small intestinal mesenteric mass in a computed tomography scan. On midline incision there was a 6 × 6 cm mass in the antimesenteric border of the jejunum approximately 30 cm from the duodenojejunal flexure, which was resected followed by anastomosis. The presentation of GISTs ranges from asymptomatic to mild abdominal pain and mass (5-50%) and mechanical obstruction (5%) as well as hemorrhage - perforation having rarely been reported (0.8%) - making the diagnosis difficult. Exophytic growth of these tumors has been noted in 18-30% of cases. In view of intermediate risk of malignancy, the patient was started with adjuvant imatinib 400 mg once daily due to probability of disease recurrence (24%).
京公网安备 11010802042870号
京ICP备2023020795号-1
扫码关注我们
求助内容：
应助结果提醒方式：