Case Report: The value of contrast-enhanced ultrasound and contrast-enhanced computed tomography in the diagnosis of hepatic angiosarcoma.

Background: Enhanced imaging techniques have the overwhelming advantages of being noninvasive and sensitive enough to evaluate the microcirculation of lesions, thus making them accurate in the diagnosis of hepatic lesions. Unfortunately, there is very little research on and knowledge of the imaging features of a rare cancerous condition: hepatic angiosarcoma (HA). Case summary: In this study, we retrospectively collected the data of six patients who underwent both contrast-enhanced ultrasound (CEUS) and contrast-enhanced computed tomography (CECT), and subsequently obtained a definitive histopathologic diagnosis of HA. We described the imaging appearances of HA by comparing CEUS and CECT images. Furthermore, we analyzed these imaging characteristics from the perspective of histopathology and tumorigenesis. The study included the largest number (six) of histopathologically confirmed HA patients who had received CEUS examinations to date. Conclusion: By offering readers comprehensive knowledge of contrast imaging, especially CEUS, in the diagnosis of HA, our study may reduce misdiagnosis and further improve treatment options.

An Autopsy Case of Ruptured Hepatic Angiosarcoma Treated by Transcatheter Arterial Embolization.

An 80-year-old Japanese man presented to our hospital with intra-abdominal hemorrhage due to a ruptured liver tumor. Transcatheter arterial embolization (TAE) temporarily achieved hemostasis, but he died following re-rupture 4 days later. Based on autopsy findings, the liver tumor was diagnosed as hepatic angiosarcoma. Embolic agents used during embolization were identified within the hepatic small interlobular arteries. However, there were no findings of tumor cell necrosis or ischemic change in the angiosarcoma. In the present case, TAE alone did not induce ischemia-induced tumor necrosis, suggesting that TAE might be unsuitable to treat hepatic angiosarcoma. Treatment optimization for ruptured hepatic angiosarcoma is desired.

Primary Hepatic Angiosarcoma Presenting As Cryptogenic Cirrhosis.

Primary hepatic angiosarcoma (PHA) is an exceedingly rare and aggressive neoplasm of mesenchymal origin. PHA makes a very small portion of primary liver tumors and conveys a poor prognosis. Symptomatology can be vague and often mimics primary hepatocellular carcinoma upon presentation. Diagnosis requires careful immunohistopathologic confirmation. We present a case of PHA in a patient with abdominal pain and suspected underlying cryptogenic cirrhosis.

Mesenchymal Neoplasms of the Liver.

Mesenchymal neoplasms of the liver can be diagnostically challenging, particularly on core needle biopsies. Here, I discuss recent updates in neoplasms that are specific to the liver (mesenchymal hamartoma, undifferentiated embryonal sarcoma, calcifying nested stromal-epithelial tumor), vascular tumors of the liver (anastomosing hemangioma, hepatic small vessel neoplasm, epithelioid hemangioendothelioma, angiosarcoma), and other tumor types that can occur primarily in the liver (PEComa/angiomyolipoma, inflammatory pseudotumor-like follicular dendritic cell sarcoma, EBV-associated smooth muscle tumor, inflammatory myofibroblastic tumor, malignant rhabdoid tumor). Lastly, I discuss metastatic sarcomas to the liver, as well as pitfalls presented by metastatic melanoma and sarcomatoid carcinoma.

Advanced diffuse hepatic angiosarcoma treated successfully with TACE and targeted immunotherapy: A case report.

Primary hepatic angiosarcoma (PHA), a rare soft tissue tumor, accounts for only 2% of all liver malignancies. Pathologically challenging, PHA is difficult to be distinguished from other malignancies with ultrasound, Computed Tomography (CT), or Magnetic Resonance Imaging (MRI). Due to late diagnosis and resistance against traditional chemotherapy and/or radiotherapy, only 3% of PHA patients can survive up to two years after diagnosis. To our best knowledge, this case report presents the first case of an advanced diffuse PHA with ruptured hemorrhage that has been effectively treated with TACE and Anlotinib plus Camrelizumab. So far, the patient has received 10 cycles of treatment and is faring well. Latest MRI results show that the tumor has shrunk by 56% and can be assessed as a partial response (PR). This case report includes our experience in treating such a advanced malignancy, and we hope that larger studies on advanced PHA can better quantify the potential benefit.

A Typical Presentation of an Atypical Condition: Hepatic Angiosarcoma With Peritoneal Bleed.

Hepatic angiosarcoma (HA) is a rare primary malignancy of hepatic endothelial and fibroblastic vascular tissue origin. Patients typically present with vague constitutional symptoms of fatigue, weight loss, abdominal pain, and ascites. Hemoperitoneum is a frequent clinical manifestation of HA associated with higher mortality and is underrecognized. Here, we report the case of a patient with HA that was complicated by a peritoneal bleed, its management, and associated poor prognosis.

A complementary comment on primary hepatic angiosarcoma: A case report.

BACKGROUND: This article examines primary hepatic angiosarcoma (PHA) and fat-poor angiomyolipoma (AML), two uncommon vascular cancers. Clinical decisions in these situations are frequently aided by pathology reports and imaging techniques. Uncommon malignant tumors of the vascular endothelium include PHA. Another diagnosis that should not be overlooked when employing contrast-enhanced MR and contrast-enhanced computed tomography (CT) imaging techniques is fat-poor AML, one of the uncommon vascular tumors of the liver. In both conditions, biopsy is the primary means of diagnosis. CASE SUMMARY: In our article, besides the diagnosis of PHA, fat-poor AML, one of the other rare vascular tumors of the liver, is mentioned. In the case, a 50-year-old female patient with VHL Syndrome was admitted to our hospital with nonspecific lesions such as right upper quadrant pain, weight loss, and nausea. Abdominal ultrasonography (US) revealed a hypoechoic heterogeneous lesion with occasional faint contours. In computed tomography, it was observed as a hyperdense nodular lesion in segment 4. Magnetic resonance imaging (MRI) revealed that the lesion did not contain fat. In connection with the known history of VHL Syndrome, we first evaluated the possibility of AML. Thereupon, a histopathological sample was taken and the diagnosis was made as fat-poor AML with 5% fat content. CONCLUSION: In conclusion, PHA in our case report and fat-poor AML in our clinic are two uncommon liver vascular malignancies with comparable incidences. Important imaging techniques like contrast-enhanced US (CEUS), CECT, and CEMRI give us substantial advantages in both cases. However, a biopsy is used to provide the final diagnosis.

Double cancer of primary hepatic angiosarcoma and hepatocellular carcinoma treated with atezolizumab plus bevacizumab.

AIM: Primary hepatic angiosarcoma (PHA) is extremely rare, and its imaging findings are similar to those of other liver tumors including hepatocellular carcinoma (HCC). Here, we report a case of hepatitis C virus (HCV)-related HCC followed by PHA that showed remarkable clinical response to atezolizumab plus bevacizumab (Atezo/Bev) therapy. CASE PRESENTATION: A 78-year-old man with recurrent HCC had a liver tumor with lymphadenopathy. Although considered as HCC recurrence, microscopic examination of the resected liver and lymph node showed PHA. Three months later, a solitary lung nodule was newly detected and subsequently resected. The pathological diagnosis was poorly differentiated HCC. Therefore, the patient was finally diagnosed with double cancer of PHA and HCC. Thereafter, he developed a new liver tumor with lymphadenopathy and received Atezo/Bev therapy. Liver tumor biopsy was carried out before the treatment. The pathological diagnosis was angiosarcoma. The patient showed a partial response after two courses of Atezo/Bev therapy. CONCLUSION: To our best knowledge, this report is the first case to present HCV-related HCC followed by PHA and to show that Atezo/Bev therapy is beneficial for PHA.

Images in Immunotherapy and Precision Oncology: Angiosarcoma of the Spleen and Liver.

Primary splenic or hepatic angiosarcomas are ultra-rare and aggressive malignancies associated with poor prognosis. The mainstay treatments are surgical resection and chemotherapy. We report a case of angiosarcoma in a 50-year-old woman who presented with bruising, fatigue, ecchymosis, and hepatosplenomegaly. She was treated with the multi-kinase inhibitor sunitinib for 4 weeks before developing a splenic hemorrhage and succumbing. Recent studies have demonstrated the clinical benefit of immunotherapies in angiosarcomas. Additionally, sequencing techniques have showcased the diverse molecular aberrations involved in angiosarcomas, which offer opportunities for precision-matched targeted therapies such as inhibitors of the VEGF/VEGFR axis and PI3K/Akt/mTor pathway.

Primary hepatic angiosarcoma with noncirrhotic portal hypertension: A case report.

Background: Primary hepatic angiosarcoma (PHA) is a rare malignant tumor of mesothelial tissue origin in the liver. The diagnosis of PHA relies on pathology, and it is frequently misdiagnosed as multiple hepatic hemangioma. Noncirrhotic portal hypertension is a relatively rare pathological manifestation, and there are few reports of PHA as an uncommon cause of noncirrhotic portal hypertension. Case summary: A 36-year-old male was admitted with abnormal liver function and suspected drug-induced liver injury (DILI), initially manifesting as multifocal hepatic hemangioma. The liver biopsy revealed features of noncirrhotic portal hypertension (NCPH), and the patient was eventually diagnosed with multifocal hepatic angiosarcoma. Conclusion: Patients with PHA may present with NCPH in the liver due to injury to hepatic sinusoids; therefore, it is necessary to consider the possibility of unsampled vascular malignancy when hepatic masses are identified, and the histology is consistent with PHA.

Cytomorphologic and immunocytochemical diagnosis of primary hepatic angiosarcoma in a young adult: Challenging diagnosis of a rare, aggressive malignancy.

Primary hepatic angiosarcoma (PHA) is a rare, rapidly growing, highly aggressive hepatic malignancy, primarily seen in elderly males. Establishing an accurate clinical diagnosis is challenging owing to its rarity and nonspecific presentation. Rendering a cytologic diagnosis of PHA is extremely difficult, and immunocytochemistry(ICC) on cell block(CB) sections is essential to confirm the diagnosis. The characteristic cytomorphologic features of PHA have rarely been documented, further augmenting the diagnostic challenge. A 32-year-old male presented with abdominal pain, abdominal distension, and significant weight loss over the past 9 months. On examination, the abdomen was distended, with multiple spider angiomas and a large mass in the right hypochondrium. His renal function tests, liver function tests, and serum tumor markers were within normal limits. An abdominal triphasic computerized tomography revealed a large lobulated mass in the right lobe with central necrosis. An ultrasound-guided FNA was performed from the liver lesion. The cytologic smears showed singly scattered large, markedly pleomorphic, epithelioid-elongated tumor cells having oval-elongated nuclei, irregular nuclear contours, coarse chromatin, prominent macronucleoli, and abundant finely vacuolated cytoplasm in a background of blood. ICC on the CB demonstrated diffuse positivity for vimentin, CD31, and nuclear positivity for FLI-1, confirming a vascular origin. Hence, a final cytologic diagnosis of primary hepatic angiosarcoma was rendered. The index report describes the characteristic cytomorphologic and immunocytochemical features of a rare hepatic malignancy in a young male and reiterates the usefulness of FNAC coupled with CB-ICC in the quick and precise diagnosis of such challenging cases.

Primary hepatic angiosarcoma: A case report.

BACKGROUND: Primary hepatic angiosarcoma (PHA) is a rare malignant tumor of the vascular endothelium. Clinical manifestations and laboratory and imaging examinations often lack specificity for PHA. We report a case of PHA, and describe the ultrasound characteristics and characteristic changes in laboratory values associated with PHA. CASE SUMMARY: A 75-year-old woman presented with right upper quadrant abdominal pain for half a month. Magnetic resonance imaging (MRI) at a local hospital revealed multiple liver space-occupying lesions, and she was admitted to our hospital for further diagnosis. Contrast-enhanced ultrasound (CEUS) revealed multiple slightly hyperechoic nodules in the liver, which were suspected to be of malignant vascular origin. Contrast-enhanced computed tomography revealed multiple low-density nodules in the liver, considered to be metastatic hematopoietic malignancies. Contrast-enhanced MRI showed that the multiple liver nodules shared features with infectious lesions. Laboratory examination revealed normal alpha-fetoprotein levels, slightly increased other liver enzymes, decreased platelets, and significantly increased D-dimer levels. Liver biopsy and histopathology confirmed the presence of PHA. CONCLUSION: CEUS can provide valuable clues for the diagnosis of PHA and greatly improve the success rate of puncture biopsy.

Case of Primary Hepatic Angiosarcoma Presenting With Jaundice and Infiltrative Liver Masses.

Primary hepatic angiosarcoma (PHA) is a rare and aggressive mesenchymal liver tumor with a poor prognosis and high mortality. Treatment options are limited to palliative chemotherapy with surgical resection reserved for the few cases that present early. We present a case of a patient who presented with jaundice and elevated liver enzymes. Imaging identified a diffusely heterogeneous liver consistent with cirrhosis, findings of portal hypertension, and 2 ill-defined liver lesions. Biopsy results confirmed PHA. Primary hepatic angiosarcoma does not have a typical presentation but should be considered for any patient presenting with an infiltrative liver mass.

Primary hepatic angiosarcoma manifesting as hepatic sinusoidal obstruction syndrome: A case report.

BACKGROUND: Primary hepatic angiosarcoma (PHA) is a rare malignancy with a poor prognosis. It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers, and it rapidly progresses and has a high mortality. To our knowledge, PHA has not been reported to mimic hepatic sinusoidal obstruction syndrome. Herein, we present a case of PHA manifesting as hepatic sinusoidal obstruction syndrome, diagnosed using transjugular liver biopsy, that resulted in the death of the patient. CASE SUMMARY: A 71-year-old man was admitted with the primary complaint of abdominal distension, decreased appetite, fatigue in the previous month, and loss of 10 kg of weight in the past 2 years. Both the liver and spleen were enlarged, and the liver had a medium-hard texture on percussion. Laboratory examinations were performed, and abdominal plain computed tomography (CT) and contrast-enhanced CT showed hepatomegaly and splenomegaly, as well as diffuse low-density shadows distributed in the liver and spleen. Contrast-enhanced CT revealed diffuse, hypodense, nodular or flake shadows in the liver and heterogeneous enhancement in the spleen. A transjugular liver biopsy was performed. Based on the pathology results, the patient was diagnosed with hepatic sinusoidal obstruction syndrome secondary to PHA. The patient's status further deteriorated and he developed serious hepatic failure. The patient was discharged, and died 3 d later. CONCLUSION: PHA is rare and has a poor prognosis; however, transjugular liver biopsy can be safely performed to aid in diagnosis.

Liver Resection for Primary Hepatic Angiosarcoma: Bicentric Analysis of a Challenging Entity.

Primary hepatic angiosarcoma (PHA) is a rare malignant tumor of the liver, and data on patient outcome after surgical treatment are scarce. The aim of this study was to evaluate postoperative morbidity and overall survival (OS) of patients who underwent hepatectomy for PHA. This is a bicentric retrospective analysis of all consecutive patients who underwent liver resection in curative intent for PHA between 2012 and 2019 at the University Hospital of Muenster and the University Hospital of Bern. Nine patients (five female, four male) were included from both centers. Median age was 72 years (44-82). Most lesions (77.8%) were larger than 5 cm, and mean size of the biggest lesion was 9.4 ± 4.5 cm. Major hepatectomy was performed in four (44.4%), and radical resection (R0) was achieved in six (66.7%) patients. Postoperative complication rate was 88.8%, including 44.4% higher than 3a in the Clavien-Dindo classification. OS survival rates at 1, 2, and 3 years were 44.4%, 22.2%, and 12.5%, respectively, and median OS was 5 months. OS was significantly better after radical resection (R0: 15 months vs. R1: 0 months, p = 0.04), whereas presentation with tumor rupture at diagnosis was associated with the worst OS (0 months vs. 15 months, p = 0.005). Disease recurrence occurred in three patients (33.3%) between three and seven months after surgery. Radical resection remains the only potentially curative treatment option for PHA. However, postoperative morbidity is high, and the overall prognosis remains poor. Multimodal therapy options and management strategies are urgently needed and could improve the prognosis of patients suffering from PHA in the future.

Conversion surgery for recurrent hepatic angiosarcoma after systemic chemotherapy with paclitaxel.

A 67-year-old man presented with hemorrhagic shock due to the rupture of hepatic tumor and underwent emergency partial resection of the right liver. Pathological examination revealed hepatic angiosarcoma with involvement in its surgical margin. Six months after the operation, disease recurrence was detected, and he was referred to our hospital for second opinion. CT revealed tumors at the liver cut surface and left lateral segment. The tumor at the liver cut surface abutted to the common bile duct and the portal vein. The tumor was deemed unresectable, and systemic chemotherapy with 4 courses of weekly paclitaxel was given with excellent response. Then, we performed partial liver resection of S4 and S1 with remnant right liver and middle hepatic vein, and wedge resection for the metastatic lesion of segment 3 as a conversion surgery. He developed a grade B bile leakage postoperatively and was discharged on postoperative day 28. He remained disease free for 8 months after the operation.

Hepatic Angiosarcoma-Uncommon Presentation of a Rare Tumor and its Management by Interventional Radiology.

Hepatic angiosarcoma is an uncommon primary malignancy of the liver. It carries a poor prognosis because of very aggressive nature of the tumor. Clinical presentation of hepatic angiosarcoma is variable, most common being hepatomegaly or abdominal mass. Here we report a case of primary hepatic angiosarcoma presented with spontaneous rupture and hemoperitoneum, which was managed successfully by transarterial embolization.

Primary Hepatic Angiosarcoma: A Rare and Very Aggressive Liver Tumour.

Primary hepatic angiosarcoma (PHA) is a sporadic and aggressive tumour of the liver that originates from mesenchymal cells and represents less than 2% of all primary liver tumours. It is known to be associated with several environmental and industrial carcinogens; however, in 75% of cases, aetiology remains unclear. Patients generally present with nonspecific symptoms and laboratory findings. Imaging has a limited role in the diagnosis.  We herein present a case of a 52-year-old man with a history of hepatitis B-related cirrhosis who was referred to our hospital for liver transplantation assessment. Magnetic resonance imaging (MRI) revealed two small nodular lesions of 5 and 6 mm in segment IV of the liver, categorised as Liver Imaging Reporting and Data System (LI-RADS) category 3. The patient was discussed at a multidisciplinary tumour meeting, and an MRI follow-up in three months was planned. Three months later, MRI depicted a substantial increase in the lesion size measured 8.5 cm. An ultrasound-guided tru-cut biopsy was performed, and the diagnosis of PHA was confirmed by pathology. In this report, we aim to highlight PHA's MRI features and underline this rare entity's rapid and fatal progression.