Clinical characteristics and surgical treatments of primary hepatic angiosarcoma

Purpose Primary hepatic angiosarcoma is a very rare and highly malignant tumor with poor prognosis. It is difficult to diagnose because of the lack of typical clinical features, and the treatment protocols for PHA are also not clear. Therefore, this study wants to find out the clinical characteristics and surgical treatments of primary hepatic angiosarcoma. Methods Among 8990 patients diagnosed with primary malignant tumor of the liver from January 2000 to December 2019 in our hospital, only four patients were diagnosed with primary hepatic angiosarcoma. The demographics, clinical manifestation, past history, serology test results, MRI features, pathology, treatment modality and prognosis of four patients were collected and analyzed. Results Three of four patients had no clinical symptoms, while one patient's symptom was abdominal pain. The levels of tumor markers of all four patients were within the normal reference range and serological tests were negative for hepatitis B and C virus. The MRI imaging findings of all four patients were mixed mass with highly disordered vascular characteristics. All four patients were misdiagnosed preoperatively. One patient who underwent hepatic lobectomy was still alive for about 18 months after surgery. One patient who underwent hepatic lobectomy has survived for only 6 months due to severe pneumonia. The other two patients who received transarterial chemoembolization survived 16 months and 11 months respectively. Conclusion The clinical symptoms of primary hepatic angiosarcoma are not typical, and primary hepatic angiosarcoma is easily misdiagnosed. The typical imaging manifestations are structural disorder and heterogeneous tumor. Hepatic lobectomy and transarterial chemoembolization may be important surgical treatments to improve the prognosis of patients.

A225 INFERIOR VENA CAVA STENOSIS AFTER HEPATIC LOBECTOMY: A RARE CAUSE OF PORTAL HYPERTENSION AND REFRACTORY ASCITES

Background Inferior vena cava (IVC) stenosis is a rare occurrence in post-liver transplant patients, affecting less than 3% of recipients. IVC stenosis is rarely described in non-transplant patients or considered in the differential of portal hypertension and refractory ascites. Patients with IVC stenosis may experience lower extremity edema, dyspnea, ascites and other signs of portal hypertension. Aims We describe a case of portal hypertension and refractory ascites secondary to IVC stenosis following hepatic lobectomy. Methods Case report. Results We report a 61-year-old woman with a history of diabetes, obesity status post sleeve gastrectomy and solitary neuroendocrine tumor of the right hepatic lobe, adjacent to the porta hepatis with no evidence of metastasis. She underwent a laparoscopic right hepatectomy with wedge resection (<5 cm) in segment 4 to remove the neuroendocrine tumor in 2016. Surgery also included an intraoperative cholangiogram and placement of intra-ductal stent using endoscopic retrograde cholangiopancreatography (ERCP) technology as well as significant adhesiolysis along the IVC, right hepatic vein and biliary tract. In February 2020, the patient was referred to Hepatology for evaluation of new ascites. Imaging did not demonstrate evidence of tumor recurrence or features of cirrhosis but identified a new findings of portal hypertension and moderate ascites. Common causes of chronic liver disease were excluded and hepatic synthetic function was normal. Paracentesis revealed a high serum albumin ascites gradient (SAAG; 20 g/L) and a high ascitic fluid protein (34 g/L). Although these findings were suggestive of cardiac ascites, a 2D echocardiogram was normal. A trans-jugular liver biopsy was attempted in July 2020. The right atrial pressure was 8 mmHg and inferior vena cava (IVC) pressure was 22 mmHg at the level of the liver. Loss of pulsation suggested hemodynamically significant stenosis of the hepatic IVC. The wedged hepatic venous pressure was 3 mmHg and the calculated portal systemic gradient was 4 mmHg. A trans jugular biopsy failed due to unfavourable anatomy. These findings, taken in conjunction with previous results, suggested non-cirrhotic post-hepatic portal hypertension. Subsequent computed tomography imaging confirmed stenosis of the hepatic portion of the IVC and this was felt to be the ultimate cause of the portal hypertension. She has since been referred back to interventional radiology (IR) for consideration of balloon angioplasty dilation of the IVC stenosis as well as additional testing to determine if there is any underlying hepatic fibrosis related to congestive hepatopathy. Conclusions IVC stenosis post hepatic lobectomy is a rare phenomenon described in the literature but warrants high suspicion in cases of refractory ascites. Funding Agencies None

Clinical characteristics and surgical treatments of primary hepatic angiosarcoma

Purpose Primary hepatic angiosarcoma is a very rare and highly malignant tumor with poor prognosis. It is difficult to diagnose because of the lack of typical clinical features, and the treatment protocols for PHA are also not clear. Therefore, this study wants to find out the clinical characteristics and surgical treatments of primary hepatic angiosarcoma. Methods Among 8990 patients diagnosed with primary malignant tumor of the liver from January 2000 to December 2019 in our hospital, only four patients were diagnosed with primary hepatic angiosarcoma. The demographics, clinical manifestation, past history, serology test results, MRI features, pathology, treatment modality and prognosis of four patients were collected and analyzed. Results Three of four patients had no clinical symptoms, while one patient's symptom was abdominal pain. The levels of tumor markers of all four patients were within the normal reference range and serological tests were negative for hepatitis B and C virus. The MRI imaging findings of all four patients were mixed mass with highly disordered vascular characteristics. All four patients were misdiagnosed preoperatively. One patient who underwent hepatic lobectomy was still alive for about 18 months after surgery. One patient who underwent hepatic lobectomy has survived for only 6 months due to severe pneumonia. The other two patients who received transarterial chemoembolization survived 16 months and 11 months respectively. Conclusion The clinical symptoms of primary hepatic angiosarcoma are not typical, and primary hepatic angiosarcoma is easily misdiagnosed. The typical imaging manifestations are structural disorder and heterogeneous tumor. Hepatic lobectomy and transarterial chemoembolization may be important surgical treatments to improve the prognosis of patients.

Anesthesia in hepatobiliary disorders

Multiple factors have led to an increased incidence of hepatobiliary disease across the continents, and with it we witness an increased number of patients, who report for surgery for a hepatobiliary disease, e.g. hepatic lobectomy, cholecystectomy, corrective surgery on bile ducts and even liver transplantation, or they report for surgery for an unrelated disease but with a concurrent hepatobiliary disease. These patients require special considerations during perioperative period. Please select the one best option in each of the following questions.

Congenital Absence of the Portal Vein

A 59-year-old woman presented with abdominal bloating, elevated alkaline phosphatase and transaminases, and computed tomography abdomen/pelvis demonstrating large right-sided hepatic masses. A percutaneous fine needle aspiration demonstrated hepatocellular neoplasm concerning for hepatocellular carcinoma. Preoperative imaging demonstrated possible porto-caval shunt. She underwent uneventful right hepatic lobectomy with confirmation of porto-systemic shunt. Congenital porto-systemic shunt, or Abernethy malformation, is rare and is associated with congenital cardiac and gastrointestinal abnormalities. Additionally, congenital porto-systemic shunt is associated with increased risk of hepatic neoplasms including hepatocellular carcinoma. Recommended surveillance for these patients is not well defined.

Primary hepatic gastrinoma being diagnosed preoperatively: a case report and literature review

Background A majority of gastrinomas causing Zollinger–Ellison syndrome are located in the duodenum or pancreas. Primary hepatic gastrinomas are rare and difficult to diagnose. We report a rare case of primary hepatic gastrinoma, which could be diagnosed preoperatively. Case presentation A 29-year-old man with a 55-mm tumor in segments 5 and 6 (S 5/6) of the liver was admitted to our hospital. After thorough investigations, he was treated for a suspected inflammatory pseudotumor and advised to undergo routine follow-up. Two years later, he revisited our hospital with a complaint of abdominal pain, vomiting, and diarrhea. Upper gastrointestinal endoscopy revealed multiple duodenal ulcers. His serum gastrin level was 2350 pg/mL (normal: 37–172 pg/mL), suggesting Zollinger–Ellison syndrome. Abdominal computed tomography showed a 78-mm hypervascular tumor with cystic degeneration in the S 5/6 region of the liver, with a potential to increase over time. The tumor showed hypointensity on T2-weighted and hyperintensity on diffusion-weighted abdominal contrast-enhanced magnetic resonance imaging. Somatostatin receptor scintigraphy (SRS) only detected a hepatic tumor. No tumors in the gastrinoma triangle were detected by endoscopic ultrasonography. Hence, selective arterial calcium injection (SACI) test was performed to determine the location of the gastrinoma. The serum gastrin concentration increased from 4620 pg/mL to 23,600 pg/mL at 20 s after calcium gluconate injection into the proper hepatic artery. Conversely, no effect on serum gastrin level was observed after the injection into any other arteries. Extended right hepatic lobectomy and cholecystectomy were performed after percutaneous transhepatic portal vein embolization. A histopathological examination of the liver tumor revealed a gastrinoma. The patient’s serum gastrin concentration on postoperative day 1 decreased to 65 pg/mL. Conclusion We report a surgical case of primary hepatic gastrinoma correctly diagnosed preoperatively. The patient underwent extended right hepatic lobectomy, resulting in a histological definitive diagnosis of primary hepatic gastrinoma.

MANIFESTATIONS OF GLUCAGONOMA SYNDROME

Objective: Glucagonoma is a rare neuroendocrine tumor of the pancreas. We present the case of a young female patient who presented with the major clinical manifestations of glucagonoma syndrome. Methods: The major clinical manifestations of glucagonoma syndrome are described in a 44-year-old, female patient. Beyond glucagonoma, the patient also displayed deep venous thrombosis, depression, diabetes, and necrolytic migratory erythema. We discuss the difficulty of treatment of patients with glucagonoma due to the low prevalence of the disorder, scarcity of medical evidence, lateness of diagnosis with liver metastases in most cases, and poor response to chemotherapy with high rates of relapse after surgery. In this case, pancreatectomy and hepatic lobectomy followed by somatostatin analogue therapy was the chosen treatment strategy. Results: The clinical findings were pancreatic and hepatic masses, proximal deep venous thrombosis, depression, diabetes, and necrolytic migratory erythema. The patient also had elevated levels of glucagon. Pancreatectomy and right hepatic lobectomy were performed and confirmed the glucagonoma. Conclusion: Our case adds new knowledge about glucagonoma which is important due to the low incidence of the disease and the particular characteristics of the syndrome.