Resectable hepatoblastoma with tumor thrombus extending into the right atrium after chemotherapy: A case report

AbstractHepatoblastoma with intraatrial tumor thrombus is relatively rare. We report a case of hepatoblastoma with tumor thrombus extending into the right atrium, which responded well to chemotherapy and was resected using extracorporeal circulation. A 4-year-old girl was referred to our hospital because of abdominal distention and tenderness. A computed tomography (CT) scan showed a large tumor occupying the left 3 segments of the liver with tumor thrombus extending into the right atrium. There was also a small intrahepatic metastasis in the right lobe of the liver. She was diagnosed with hepatoblastoma on the basis of the results of open biopsy. Neoadjuvant chemotherapy with an intense CDDP-based regimen was performed. The tumor responded well to chemotherapy, and intrahepatic metastasis became undetectable on CT scan, although the tumor thrombus remained in the right atrium. After 7 courses of chemotherapy, we performed resection using extracorporeal circulation. The postoperative course was uneventful, and adjuvant chemotherapy was started 10 days after the operation. Her serum alpha-fetoprotein (AFP) level decreased to the normal range, and she was free of disease for 1 year after the operation. Tumor resection using extracorporeal circulation can be performed safely and is justified in patients with intraatrial tumor thrombus

Hemorrhagic shock due to rupture of a nephroblastoma in an infant: A case report

Introduction: Nephroblastoma is the most common type of malignant renal tumor in children, but hemorrhage in ruptured nephroblastoma is rare. Case presentation: A three-month-old boy was admitted to our hospital with a large right renal tumor and hemorrhagic shock. Laparotomy showed that the tumor had invaded the right lobe of the liver; therefore, surgeons decided to perform only hemostasis and a tumor biopsy. The following day, intratumoral bleeding persisted, necessitating continuous blood infusion, resulting in abdominal compartment syndrome. Therefore, tumor resection was performed. Intra-tumoral hemorrhage continued during the operation, resulting in bradycardia and chest compressions. Histopathological examination confirmed a diagnosis of nephroblastoma. The postoperative course was uneventful. Conclusion: This report underlines the need to choose treatment based on both extratumoral and intratumoral bleeding, keeping in mind the risks of each treatment option

Data_Sheet_1_Sirolimus treatment for intractable lymphatic anomalies: an open-label, single-arm, multicenter, prospective trial.PDF

IntroductionIntractable lymphatic anomalies (LAs) include cystic lymphatic malformation (LM; macrocystic, microcystic, or mixed), generalized lymphatic anomaly, and Gorham–Stout disease. LAs can present with severe symptoms and poor prognosis. Thus, prospective studies for treatments are warranted. We conducted a prospective clinical trial of sirolimus for intractable LAs.MethodsThis was an open-label, single-arm, multicenter, prospective trial involving five institutions in Japan. All patients with LAs received oral sirolimus once daily, and the dose was adjusted to ensure that the trough concentration remained within 5–15 ng/mL. We prospectively assessed the drug response (response rate for radiological volumetric change in target lesion), performance state, change in respiratory function, visceral impairment (pleural effusion, ascites, bleeding, pain), laboratory examination data, quality of life (QOL), and safety at 12, 24, and 52 weeks of administration.ResultsEleven patients with LAs (9 generalized lymphatic anomaly, 1 cystic LM, 1 Gorham–Stout disease) were treated with sirolimus, of whom 6 (54.5%; 95% confidence interval: 23.4–83.3%) demonstrated a partial response on radiological examination at 52 weeks of administration. No patients achieved a complete response. At 12 and 24 weeks of administration, 8 patients (72.7%) already showed a partial response. However, patients with stable disease showed minor or no reduction after 12 weeks. Adverse events, such as stomatitis, acneiform dermatitis, diarrhea, and fever, were common with sirolimus. Sirolimus was safe and tolerable.ConclusionSirolimus can reduce the lymphatic tissue volume in LAs and may lead to improvements in clinical symptoms and QOL.</p