Primary Hepatic Angiosarcoma Research Articles

Imaging features of hepatic angiosarcoma: retrospective analysis of two centers

PurposeIdentifying primary hepatic angiosarcoma (PHA) preoperatively is challenging, often relying on postoperative pathology. Invasive biopsy increases bleeding risk, emphasizing the importance of early PHA diagnosis through imaging. However, comprehensive summaries of ultrasound, abdominal computed tomography (CT), magnetic resonance imaging (MRI), and whole- body positron emission tomography-CT (PET-CT) in this context are lacking. This study aimed to investigate the comprehensive imaging characteristics of PHA.Patients and methodsImaging data were collected from 7 patients diagnosed with PHA via pathology between January 2000 and December 2019 in two provincial grade III hospitals. All patients underwent routine color ultrasound examinations before surgery, with 3 patients receiving contrast-enhanced ultrasound (CEUS).CT scans, both plain and enhanced, were performed on 5 patients, and whole-body PET-CT examinations were conducted on 2 patients.ResultsAmong the 7 patients with PHA, 4 presented with a single solid intrahepatic mass (2 of which were large), 1 with a single exophytic macroblock type, 1 with a mixed type featuring multiple masses and nodules, and 1 with a multiple nodule type. Conventional ultrasound of PHA showed uneven echoes within the tumor, potentially accompanied by septal zone echoes, and a blood flow grade of 0-I. CEUS displayed early-stage circular high enhancement, a central non-enhancement area, and a "vascular sign" around the tumor. CT scans revealed low-density shadows in the plain scan stage, high peripheral ring enhancement, and punctate nodular enhancement in the arterial phase, with varying intensities and the presence of a "vascular sign." During the portal vein stage, the interior of the tumor was consistently unfilled and exhibited structural disorder. PET-CT showed low-density lesions in the liver and low fluorodeoxyglucose metabolism.ConclusionsImaging diagnosis plays a crucial role in PHA diagnosis. When liver tumor imaging matches the above characteristics, consider PHA.

Magnetic resonance imaging and clinicopathological findings of primary hepatic angiosarcoma.

To investigate the magnetic resonance imaging (MRI) and clinicopathological features of primary hepatic angiosarcoma (PHA) and enhance preoperative diagnosis. MRI and clinicopathological information of 12 cases proved PHA were reviewed. Summarize the MRI characteristics of PHA combined with literature reviews. Among 12 cases (6 males and 6 females; age range, 23-70 years; mean, 53.3 years), one presented as a large mass, two as a diffuse infiltrating tumor, and nine as a mixed pattern of large masses with multiple nodules, all involving both lobes of the liver and ranging from 0.1cm to 11cm in diameter. A total of 63 lesions were analyzed, including 21 masses and 42 nodules. 13 masses (61.9%) demonstrated intratumoral hemorrhage. 18 masses (85.7%) demonstrated heterogeneous patchy, ring-shaped, septate, or irregular shaped enhancing foci on late arterial phase (LAP). On dynamic contrast-enhanced MRI (DCE-MRI), 14 masses (66.7%) showed a centripetal or centrifugal pattern of incomplete progressive enhancement. 6 nodules (14.3%) appeared intratumoral hemorrhage. 31 nodules (73.8%) showed no enhancing foci on LAP images and 27 nodules (64.3%) showed enhancement pattern of complete filling, either centripetal or centrifugal pattern. Moreover, 12 cases (100%) exhibited prominent vessels within or adjacent to at least one lesion. PHA exhibits diverse appearances on MRI. Typical MRI signs include multifoci with intratumoral hemorrhage, prominent vessels within or adjacent to the foci, as well as varied degrees of progressive enhancement with incomplete filling in dominant masses of PHA.

Clinical characteristics, imaging features, and outcomes of primary hepatic angiosarcoma: A single‐center study and literature review

AbstractPrimary hepatic angiosarcoma (PHA) accounts for 0.5% to 2% of all primary hepatic malignancies, and histopathology findings are required to diagnose PHA accurately. This study investigated 12 patients with PHA at a single medical center in southern Taiwan. We analyzed the clinical characteristics, imaging features, histopathology findings, and survival outcomes of patients with PHA. Of the 12 patients, abdominal pain and fullness were the most common symptoms, and their liver biochemistry data and tumor markers were mostly within the normal limits. The liver tumors tended to present as multifocal or diffuse nodules with bilobar involvement. The median overall survival (OS) of the 12 patients after diagnosis was 9 months. Improved OS was observed in the surgery group compared with the nonsurgery group (15 vs. 2 months, p = .003). The median OS of patients in the surgery ± systemic therapy group was superior to that of patients in the systemic therapy group and in the no‐therapy groups (15 vs. 5 vs. 2 months, respectively; p = .012). Complete surgical resection remains the optimal treatment choice, and combined surgery and systemic therapy seem beneficial but require further investigation.

Primary hepatic angiosarcoma: a systematic review.

Hepatic angiosarcoma is a rare type of tumour. In adults, the diagnosis remains challenging as the clinical manifestations are generally nonspecific and are usually made too late when complications or metastases are already present, associated with a poor prognosis. Due to the lack of agreement regarding the optimal treatment approach, a comprehensive analysis of the evidence in the literature on the surgical and survival outcomes in terms of disease-free survival time (DFS) and overall survival (OS) for patients treated for primary hepatic angiosarcoma is needed. A systematic review of articles published in Pubmed, EMBASE, and Scopus, from 2000 to 2023 with the search terms hepatic angiosarcoma and liver resection or hepatectomy. Pooled individual data concerning the prognosis following various therapies was analyzed. A total of 15 articles involving 886 patients were eligible for inclusion. The male population represents 66.2% (n=586) of the population, with a median age of 57 years (41-66). The median survival rate was 7 months. The median OS after surgical resection was 17.18 months (SD 12.6) vs. 3.72 months (SD 4.6) for patients treated without a surgical approach. The median DFS was 15.2 months (SD 11). Primary liver angiosarcoma remains a surgical challenge with a poor prognosis even with complete surgical resection and adjuvant therapy. Surgical management shows increased overall survival in comparison with non-surgical management. Early diagnosis could change the natural history of the disease. The literature available is scarce, and further studies are required to create standardized diagnostic and treatment protocols.

Primary hepatic angiosarcoma: a clinicopathological analysis of nine cases

Objective: To investigate the clinical manifestations, histomorphology, and differential diagnosis of primary hepatic angiosarcoma. Methods: Nine cases of primary hepatic angiosarcoma diagnosed in the Department of Pathology, the First Affiliated Hospital of Nanjing Medical University from January 2014 to December 2021 were collected, including biopsy and surgical specimens. The histomorphology, clinical, and radiologic findings were analyzed. The relevant literature was also reviewed. Results: There were six males and three females, aged 30 to 73 years (mean 57 years). Grossly, the growth pattern of the tumor was classified as either mass formation or non-mass formation (sinusoidal). Microscopically, the mass-forming primary hepatic angiosarcoma were further subdivided into vasoformative or non-vasoformative growth patterns; and those non-vasoformative tumors had either epithelioid, spindled, or undifferentiated sarcomatoid features. Sinusoidal primary hepatic angiosarcoma on the other hand presented with markedly dilated and congested blood vessels of varying sizes, with mild to moderately atypical endothelial cells. Follow-up in all nine cases revealed 8 mortality ranging from 1 to 18 months (mean 5 months) from initial diagnosis. One patient was alive with disease within a period of 48 months. Conclusions: Primary hepatic angiosarcoma is a rare entity with a wide spectrum of histomorphology, and often misdiagnosed. It should be considered when there are dilated and congested sinusoids, with overt nuclear atypia. The overall biological behavior is aggressive, and the prognosis is worse.

S3602 A Rare and Aggressive Liver Cancer With Atypical Presentation: Primary Hepatic Angiosarcoma Presenting With Hypercalcemia

S3602 A Rare and Aggressive Liver Cancer With Atypical Presentation: Primary Hepatic Angiosarcoma Presenting With Hypercalcemia

Management Strategies and Outcomes in Primary Liver Angiosarcoma.

Primary hepatic angiosarcoma is a rare tumor of the liver that originates from endothelial and fibroblastic tissue, with poor prognosis and lack of standardized treatment. We retrospectively analyzed the clinical characteristics and treatment outcomes of 23 patients with primary liver angiosarcoma treated at an academic sarcoma center. We screened all patients with primary liver angiosarcoma treated at Stanford between 2000 and 2022. Data was collected from EPIC electronic medical records and included patient demographics, tumor characteristics, treatment modalities, and patient outcomes. Statistical analysis was completed using Python 3.0, while survival curves were generated using the Kaplan-Meier method and Lifelines Packages. There were nearly equal numbers of males (11) and females (12) in our study, with most patients aged 70 to 79 at diagnosis. The median overall survival (OS) was 6 months (range 0.07 to 222.6 mo). The 2- and 5-year OS were both 38.6%. 71% of patients received systemic treatment with chemotherapy, while 29% received immunotherapy. Local treatment with surgery or radioembolization was performed in 14% of patients. Three patients in our study displayed particularly improved OS and received various treatments, which ranged from hepatic resection to ipilimumab/nivolumab. Our study demonstrated that primary liver angiosarcoma has poor outcomes despite treatment. Surgical resection with negative margins is the only curative modality. However, most patients present with advanced disease and are not surgical candidates. Further research is needed to identify more effective systemic therapy options for this devastating disease.

Clinical Profile and Surgical Management of Primary Hepatic Angiosarcoma: A Case Report and Review of Current Approaches

Clinical Profile and Surgical Management of Primary Hepatic Angiosarcoma: A Case Report and Review of Current Approaches

Cystic presentation of primary hepatic angiosarcoma

Cystic presentation of primary hepatic angiosarcoma

Primary and secondary angiosarcomas of the liver: a multi-institutional study of 32 cases

Angiosarcomas involving the liver can be hepatic primary or metastasis from another anatomic site, which have not been systematically compared. We analyzed a series of liver biopsy or resection specimens carrying a diagnosis of angiosarcoma collected between 2005 and 2022 at 3 tertiary medical centers. The cohort included 32 patients (20M and 12F) with a median age of 64 years. Nineteen were primary hepatic angiosarcoma (PHA) and 13 metastatic angiosarcoma to liver (MA). Males were predominant in PHA (15/19, 78%) compared to MA (5/13, 38%, P=.025). There was no age difference between the 2 groups. Five cases had background hepatic cirrhosis, which more likely harbored PHA (4/5, 80%). Multifocality and multiorgan involvement were common in both groups. Tumor size was significantly larger in PHA than in MA (10.4 versus 4.7cm, P<.01). Histologically, there were no differences in terms of tumor morphology (spindled versus epithelioid) and growth patterns (vasoformative versus solid) between the 2 groups. Immunohistochemically, all tumor cells were positive for CD31 (28/28, 100%) and ERG (18/18, 100%). Molecular analysis in 5 cases demonstrated different mutation profiles involving different genes, including MTOR, PIK3CA, ARID1A, CDKN2A, PTEN, TP53, ATRX, KDR/VEGFR2, and so forth. On follow-up, 30patients (93%) died of disease, with a median survival of 114 days. Univariate and multivariate analyses revealed PHA and epithelioid morphology to be associated with worse survival (P<.05), while treatment was associated with better survival (P<.001). Our results confirmed that angiosarcoma, particularly PHA, is extremely aggressive. Epithelioid morphology is an adverse prognosticator and may be used for tumor subclassification.

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-oldman 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.

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-oldman 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. Key Words:Liver, Hepatic angiosarcoma, Magnetic resonance imaging.

Liver transplantation for primary hepatic angiosarcoma: A report of two cases

Sung-Min Kim, Chul-Soo Ahn, Deok-Bog Moon, Tae-Yong Ha, Gi-Won Song, Dong-Hwan Jung, Gil-Chun Park, Woo-Hyoung Kang, Young-In Yoon, Shin Hwang. Ann Liver Transplant 2022;2:151-6. https://doi.org/10.52604/alt.22.0020

S3138 Primary Hepatic Angiosarcoma: A Rare Cause of Decompensated Pseudo-Cirrhosis and Acute Liver Failure

Introduction: Primary hepatic angiosarcoma (PHA) is a rare aggressive endothelial cell tumor which is seen in patients in their 60s and 70s with 3:1 male predominance. Industrial exposure to vinyl chloride, radium, chronic arsenic ingestion, anabolic steroid use, and iatrogenic exposure to thorotrast radiocontrast are some of the known etiologies for the development of PHA. Whereas, in most cases, attributable risk factors are rarely identified. Case Description/Methods: A 68-year-old male patient with NIDDM presented with chest pain and abdominal distension. Examination revealed marked ascites, abdominal tenderness, and bilateral leg edema. Labs were significant for thrombocytopenia and cholestatic pattern of liver enzymes with AST 94, ALT 47, ALP 413, T bilirubin 5.7. Paracentesis was negative for SBP or malignant cytology and was indicative of portal hypertensive ascites. Tumor marker workup was positive for CA 19-9 106.1, whereas negative for CEA 1.82, and AFP 2.7. CT A/P showed multifocal large heterogeneous enhancing lesions in the liver which were biopsied showing diffuse proliferation of abnormal vascular endothelial cells staining positive for CD34 and a diagnosis of PHA was made. With multifocal lesions, the patient was started on paclitaxel chemotherapy, which was eventually stopped with worsening liver function. The hospital course was complicated by hepatic encephalopathy and coagulopathy. The patient and the family later chose to transition to palliative therapy for comfort (Figure 1). Discussion: PHA is a rare and aggressive tumor with poor outcomes and an average survival rate of less than a year. Early diagnosis is challenging as it presents with nonspecific abdominal symptoms. With progression, PHA can present as decompensated pseudocirrhosis due to compression of liver parenchyma leading to portal hypertension. Contrast-enhanced US and CT can help in diagnosis by showing lesions characteristic of central non-enhancement and peripheral irregular enhancement in the arterial and portal phase, and complete wash-out in the late phase. A definitive diagnosis of PHA is established via histopathological analysis with immunohistochemistry staining but can make diagnosis more challenging due to the potential of associated bleeding. Surgical excision with negative surgical margins is the standard treatment for PHA which is localized and resectable. Multifocal and metastatic PHA is a radio-resistant tumor with a paucity of treatment options, however, TACE and/or salvage chemotherapy can be potentially attempted.Figure 1.: CT Abdomen pelvis showing multiple mass lesions occupying most of liver parenchyma.

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.

Treatment Outcomes for Primary Hepatic Angiosarcoma: National Cancer Database Analysis 2004-2014.

Simple SummaryPrimary hepatic angiosarcoma is a rare tumor of the liver. The prognosis and treatment of this rare tumor remains an enigma. Despite being the most common liver tumor of mesenchymal origin, the prognosis of patients diagnosed with primary hepatic angiosarcoma has never been compared with that of the most common liver tumor which is hepatocellular carcinoma. In this manuscript, we have analyzed all the recorded cases in the National Cancer Center Database and determine the best approach to treat this rare tumor. We have also conducted a brief review of the literature to guide the reader toward the finer nuances of managing patients diagnosed with primary hepatic angiosarcoma, especially in the context of a liver transplant.Background: To determine the risk of mortality and factors associated with survival amongst patients diagnosed with primary hepatic angiosarcoma (PHA). Methods: All patients diagnosed with hepatocellular carcinoma (HCC) or PHA from 2004 to 2014 were identified from the National Cancer Database (NCDB). Further analysis was performed within the cohort of patients with PHA to assess the impact of surgery, chemotherapy, radiation, and facility type on overall survival (OS). A multivariable analysis using the Cox proportional methods and a survival analysis using the Kaplan–Meier method were used. Results: A total of 117,633 patients with HCC were identified, out of whom 346 patients had PHA. Patients with PHA had a mean age of 62.9 years (SD 13.7), the majority were men (64.7%), white (85.8%), and had a Charlson comorbidity index (CCI) of zero (66.2%). A third of the patients with PHA (35.7%) received chemotherapy, and 14.6% underwent a surgical resection. The median survival was 1.9 months (1.8–2.4 months) compared to patients with HCC (10.4 months, 10.2–10.5) (aHR-2.41, 95% CI: 2.10–2.77, p < 0.0001). Surgical resection was associated with a higher median survival (7.7 versus 1.8 months, aHR-0.23, 95% CI: 0.15–0.37, p < 0.0001). A receipt of chemotherapy was associated with a higher median survival than no chemotherapy (5.1 versus 1.2 months, aHR-0.44, 95% CI: 0.32–0.60, p < 0.0001), although the survival benefit did not persist long term. Conclusion: PHA is associated with poor outcomes. A surgical resection and chemotherapy are associated with improved survival outcomes; however, the long-term benefits of chemotherapy are limited.

Primary hepatic angiosarcoma in an elderly patient.

Primary hepatic angiosarcoma is a high-grade mesenchymal tumor with very aggressive behavior, and accounts for 0.1-2% of all liver malignancies.1 Most of the time, patients present with abdominal pain, fatigue, weight loss, hepatosplenomegaly, ascites, jaundice, and anemia. This neoplasm is known for intraperitoneal rupture. Poor prognostic factors include older age, large tumor size, and a high Ki-67 index. It occurs in association with exposure to known chemical carcinogens such as vinyl chloride monomer, thorotrast, anabolic steroids, and arsenic. However, in 75% of cases, the etiology is not known. The current molecular data revealed KRAS mutations in sporadic cases and TP53 mutations in vinyl chloride-related cases.2 Primary hepatic angiosarcoma may have different growth patterns on imaging. It includes 1) Multiple nodules, 2) Large dominant mass, 3) Mixed pattern of dominant mass with nodules, and 4) Diffuse infiltrating micronodular mass.3 The liver biopsy is the gold standard for confirming the diagnosis of primary hepatic angiosarcoma. Microscopically, the lesion is heterogeneous, ranging from well-defined anastomotic vessels (Vaso formative) to solid sheets of epithelioid or spindled cells; some tumors may show mixed patterns. Immunohistochemically, ERG (erythroblast transformation specific-related gene) and endothelial markers such as CD31 and CD34 show expression, indicating the endothelial origin of the tumor. D2-40 and CD10 may also show positivity. Of note, the epithelioid variant may show keratin expression. Surgical resection seems the best therapeutic option, leading to a median overall survival of between 17 and 19 months.4 We describe gross examination and supportive microscopic findings of primary hepatic angiosarcoma in a 66-year-old gentleman with no predisposing environmental risk factors. He presented with fatigue, weight loss, jaundice, abdominal pain, and swelling in the right hypochondrium. On investigation, he was found to have raised serum levels of ammonia (183.8 µmol/L; RR: 11-32 µmol/L), AST (89.5 U/L; RR: 5-40 U/L), ALT (77.6 U/L; RR: 7-56 U/L), ALP (410 IU/L; RR: 44-147 IU/L). Viral markers were negative, and the coagulation profile was within the normal limit. Hepatocellular carcinoma and Cholangiocarcinoma involving the right lobe were differential diagnoses based on clinical and imaging studies. Right hepatectomy was done and submitted for histopathological examination. Gross examination showed a dominant mass, measuring 18x10x5cm, and nodules of variable size. The liver capsule was smooth, shiny and there was no breach by the tumor. The cut surface of the tumor appeared variegated and showed pale white to yellowish firm areas with intervening areas of hemorrhage and necrosis (Figure 1 A). The adjacent native liver parenchyma was non-cirrhotic. Microscopically, the tumor was ill-defined, and the cells were arranged along with abortive vascular spaces and as clusters (Figure 1B). The tumor cells displayed moderate to marked degrees of nuclear pleomorphism with oval to plump to spindle-shaped vesicular nuclei, prominent nucleoli, and moderate eosinophilic cytoplasm (Figure 1C). Mitotic activity was brisk (>8 per 10 high power fields). Lymphovascular emboli were seen. Areas of necrosis and hemorrhage were present. The tumor cells exhibited membranous positivity for CD31 (Figure 1D) and CD34 immunomarkers. HepPar1, Arginase, and Keratin 7 were negative. Based on the light microscopy and immunohistochemistry findings, the diagnosis of primary hepatic angiosarcoma was offered. Figure 1 A – Gross view of the surgical specimen. Slices of right hepatectomy specimen showing the mixed growth pattern with dominant mass, measuring 18x10x5cm and nodules of variable size. Cut surface of the tumor showing pale white to yellowish firm areas with areas of hemorrhage and necrosis (scale bar = 15 cm); B – Microscopy showing an infiltrating tumor with areas of hemorrhage and fibrosis. (H&E,40 x); C – Higher magnification depicting the large, pleomorphic tumor cells lining the abortive sinusoids. (H&E, 200 x); D – Immunohistochemistry for CD31 showing membranous positivity in the tumor cells (400x).:

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.

Clinical characteristics and surgical treatments of primary hepatic angiosarcoma

PurposePrimary 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.MethodsAmong 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.ResultsThree 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.ConclusionThe 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.

Clinical characteristics of primary hepatic angiosarcoma outcomes: a SEER database analysis.

BackgroundPrimary hepatic angiosarcoma (PHA) is a rare malignant tumor. We explored the demographic features and prognostic factors of PHA.MethodsWe used the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database to extract patients diagnosed with PHA from 1975 to 2016. We used the Kaplan-Meier method and Cox proportional hazards regression to evaluate the risk factors for overall survival (OS) and disease-specific survival (DSS). The nomograms were constructed and validated using the concordance index (C-index) and calibration plots.ResultsIn total, 366 patients were included in this study. The disease onset was hidden, and most patients already had advanced disease when diagnosed. The prognosis of PHA was very poor, and the overall 6-month, 1-year and 2-year survival rates were 20.3%, 12.8% and 9.3%, respectively. Sex, age and surgery were all predictors of both OS and DSS in multivariate analysis. Women had better survival rates than men, and patients aged <60 years benefited from surgery in the multivariate models. The nomograms presented good accuracy, with C-index values of 0.679 and 0.665 for the OS and DSS prognostic models, respectively. The calibration plots showed good agreement between the nomogram predictions and actual observations.ConclusionsPHA has a poor prognosis. Regular physical examinations are essential for the elderly. Patients aged <60 years could benefit from surgery. We constructed accurate nomograms to predict survival that can greatly benefit clinicians.