Angiosarcoma Of Liver Research Articles

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.

Association of IL-8 and CXCR2 with AST/ALT Ratio in Liver Abnormalities Screening during Oxidative Stress Injury Caused by VCM.

Liver impairment caused by VCM has been linked to irreversible damage such as fibrosis, necrosis, hepatocellular carcinoma, and liver angiosarcoma. However, the ability to detect abnormalities during initial phase have not been achieved so far. Thus, this study aimed to investigate the effect of interleukin 8 (IL-8) and C-X-C chemokines 2 (CXCR2) on screening for a VCM-exposed group (n = 227) from a PVC manufacturing factory compared to a control group (n = 110) in Tianjin City in 2020 with influence factors evaluation. Ambient concentrations of VCM and health archives from 2012 to 2018 were collected for establishing the dose-effect trend. A cross-sectional survey in 2020 was performed to measure TDGA, IL-8, CXCR2, 8-OHdG, SOD, GPX, CAT, MDA, and ROS levels. Results indicated a continuous increased incidence on liver abnormalities despite a fluctuated downward trend in cumulative time-weighted average (CTWA) VCM concentrations over the years. ALT, AST, and AST/ALT ratio all contributed to liver abnormalities that contained fatty liver, liver calcification, and liver cysts, IL-8 and CXCR2 correlated with each other strongly and showed significant associations with oxidative stress markers, even AST/ALT ratio. IL-8 (>1547 µg/m3) or CXCR2 (<139 µg/m3) influenced the AST/ALT ratio through reciprocal interactions under oxidative stress injury, CXCR2 (>222 µg/m3), working years of 21 to 30 (a) and 11 to 20 (a), TDGA (>1.52 mg/L), alcohol consumption, smoking habit, and a less sleeping duration of <4 h per day would also be potential factors affecting the AST/ALT ratio. In conclusion (1) even with decreased VCM concentrations in PVC manufacturing factories liver abnormalities that contained fatty liver, liver calcification, and liver cysts could still occur due to oxidative stress injury with involvement of IL-8 and CXCR2. The status of protective measure and appropriate mask types also play a role; (2) the AST/ALT ratio could be a specific indicator for detecting abnormalities when combined with liver B ultrasonography results before impairment altered from bad to worse; and (3) factors such as definite medication history, fully broken protective facilities, alcohol consumption, less sleeping duration, inappropriate mask types, and longer working years could also influence AST/ALT ratio alterations through complex interactions.

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.

Aristolochic acid (AA) exposure and telomere maintenance mechanism in liver angiosarcoma (AS).

11551 Background: Liver AS has a grave prognosis and our previous study (Chen TW et al. Cancer 2022) showed that the incidence of liver AS is higher in Asia. We aimed to investigate the etiology of liver AS and its molecular characteristics. Methods: We performed whole exome (WES) and transcriptome sequencing (RNA-seq) of AS (liver 10, scalp 5, breast 2, trunk 1, heart 1) and hepatocellular carcinoma (HCC, 9) from National Taiwan University Hospital (NTUH) and re-analyzed WES &amp; RNA-seq of AS from the Count Me In Project (CMI, 46). Somatic mutations and copy number alternation were called by Mutect2 and Sequenza from tumor-normal matched WES. Non-negative matrix factorization was used to determine mutational signature profiles and the contributions of each signature to each sample. Telomere length was estimated using TelSeq. Results: We identified four single-base-substitution (SBS) signatures using somatic mutations from 74 (AS/HCC 65/9) WES data. The dominated signature in liver AS had exceptionally high cosine similarity (93.08%) with COSMIC SBS22 (ver 3.2), which is related to AA exposure. The AA-mutation signature contributed significantly (adjusted p &lt; 0.01) to the DNA mutations in 70% (7/10), 0% (0/9), 7% (3/46), and 56% (5/9) of liver AS (NTUH), nonliver AS (NTUH), CMI AS, and HCC (NTUH), respectively. TP53 (60% vs 22% vs 32%) and ATRX (40% vs 0% vs 11%) mutations were more common in liver than nonliver AS (NTUH) or CMI, while KDR mutation rates were similar (30% vs 44% vs 22%). Among TP53 mutations from liver AS with AA signature, 86% (6/7) showed the typical A:T &gt; T:A mutation caused explicitly by AA-induced DNA damage. In liver vs nonliver AS, the median number of mutations and neoantigen burdens were 6.55 vs 0.98 (p = 0.0076) and 1572 vs 544 (p = 0.095), respectively. Although liver AS and HCC were highly associated with AA-mutation signature, liver AS had significantly longer telomeres in terms of tumor/normal telomere ratio (median 1.27 vs 1.16, p = 0.0009) but a significantly lower telomerase enzyme activity (p = 0.016). We did not find an association between ATRX mutation, or its mRNA expression level, with the extended telomere length in liver AS. Compared to nonliver AS, the activity of the telomere replication molecule BUB1 was significantly higher in liver AS (p = 0.0029). Conclusions: Our study is the first to connect AA exposure to the etiology of liver AS. The high mutation rate and distinct telomere maintenance mechanism of liver AS provides insights into future treatments.

Single-center study of different treatment for advanced or unresectable angiosarcoma patients

Objective: To evaluate the efficacy and safety of different medical treatment in advanced or unresectable angiosarcoma. Methods: This study was a single-center retrospective clinical study. Fifty-five advanced or unresectable angiosarcoma patients treated in Sun-Yat Sen University Cancer Center from January 2005 to August 2020 were enrolled. There were 34 patients who received first-line doxorubicin-based chemotherapy (doxorubicin group), 12 patients received first-line doxorubicin or liposome doxorubicin plus paclitaxel or albumin bound paclitaxel chemotherapy (combination therapy group), and 4 patients received first-line paclitaxel-based treatment (paclitaxel group). There were 6 patients who received anti-angiogenesis targeted therapy, another 2 patients received anti-PD-1 antibody plus anti-angiogenesis targeted therapy. Targeted therapy and immunotherapy plus targeted therapy included 5 cases of first-line therapy and 3 cases of second-line therapy. The therapeutic effect was evaluated by RECIST 1.1 standard. The adverse reactions were evaluated by CTCAE4.0 standard. Kaplan-Meier survival analysis was evaluated with Log rank test. Cox proportional hazard model was used to analyze the influencing factors. Results: There were 18 patients achieved partial response (PR) in 34 patients in the doxorubicin group, median progression-free survival (mPFS) was 4.5 months, and median overall survival (mOS) was 15 months. Four patients achieved PR in 12 patients in the combination therapy group, mPFS and mOS were 4 months and 19 months. Two patients achieved PR in 4 patients in the paclitaxel group, mPFS and mOS were 3 months and 9 months. However, only 1 in 6 patients achieved PR for anti-angiogenesis targeted therapy, mPFS and mOS were 3 months and 16 months. Two patients who received anti-PD-1 immunotherapy combined with anti-angiogenesis targeted therapy acquired PR for 17 months and more than 16 months. Median PFS (7.5 months) were longer in those with primary liver, lung and spleen angiosarcoma than in those with other primary site (3.0 months, P=0.028). The mOS (20 months) was longer in females than that in males (12 months, P=0.045). Primary tumor site, sex, age and treatment were not independent prognostic factors for angiosarcoma patients (P>0.05). Grade 3-4 cardiac toxicity was found in 2 patients in the combination therapy group. Conclusions: Doxorubicin-based and paclitaxel-based chemotherapy are the most important treatment for advanced angiosarcoma. Potential efficacy for targeted therapy combined with anti-PD-1 immunotherapy are showed in some patients with long duration of response and moderate adverse event.

Improvements in protective measures in factories with acetylene hydrochlorination and ethylene oxychlorination techniques declined risk assessment levels and affected liver health status.

Acetylene hydrochlorination and ethylene oxychlorination are the two most common methods of producing vinyl chloride monomer (VCM), which has been linked to liver impairment, hepatocellular carcinoma (HCC), and angiosarcoma of the liver (ASL) in occupational settings. However, whether and how these impairments could be effectively improved from workplace root causes has yet to be discovered. This study aimed to evaluate whether improvements in protective measures in groups Y (408 subjects) and Z (349 subjects) could have an influential impact on the alleviation of liver impairment by comparing risk assessment levels under several semi-quantitative models and results from liver ultrasound detection and liver function tests before and after the improvement. Importantly, significant differences in constituent ratio involved in parameters among age, length of employment, weekly exposure time, smoking status, alcohol consumption, and sleeping quality were found between Y and Z before improvement took place in 2020 (P < 0.05 or P < 0.001), and population distribution by gender between Y and Z was in a large homogeneity with differences in age and length of employment. CSTE involves ore breaking, acetylene generation, steam stripping, outward processing, and welding maintenance, was disqualified in 2020 compared to OEL, and was said to have declined to meet OEL requirements by 2021. Further, a negative correction of fresh air requirement and ventilation air changing rate with ambient concentration toward hazards in Y was stronger in 2021 than in 2020. Significant differences in risk levels in Y between 2020 and 2021 were found as ore breaking, acetylene generation, steam stripping, outward processing, VCM polymerization, welding, and repairing, decreasing to relatively lower risk levels in 2021 from the original ones in 2020 only under the semi-quantitative comprehensive index model. Abnormal rates toward other hepatic symptoms decreased in the majority of positions after the improvement, as referred to by alterations such as ALT, AST, and GGT. Overall, the effect of improvements on protective measures effectively reduced positions' risk assessment levels through ventilation enhancement and airtight strengthening, which further affected abnormal rates toward other hepatic symptoms, and alterations such as ALT, AST, and GGT were much more significant in Y than effect in Z.

S3327 Angiosarcoma Presenting as Hemorrhagic Shock: A Rare Diagnosis of Liver Lesions in a Young Patient

Introduction: Angiosarcoma is an aggressive tumor and rare in young healthy patients. Angiosarcoma is associated with exposure to arsenic, vinyl chloride, and oral contraceptives1. This is an unusual case of angiosarcoma in a young patient presenting with an acute abdomen from hemorrhagic shock. Angiosarcoma has poor prognosis and even removal and transplantation of the Liver is no sufficient 1. It is important to include angiosarcoma in the differential for patients presenting with acute abdomen and explore aggressive medical therapy along with surgical resection. Case Description/Methods: A 19-year-old male with no past medical history presented with sudden severe diffuse abdominal pain, difficulty breathing, nausea, vomiting, and diarrhea. FAST exam showed large volume of free fluid in the abdomen. Initial labs showed elevated liver associated enzymes, Hgb 5.4, lactic acidosis, fibrinogen 171. CT of abdomen and pelvis showed hepatomegaly with multiple small hypervascular lesions in liver [Figure 1]. Massive transfusion protocol was initiated, and he subsequently required emergent exploratory laparotomy with 3L of old-appearing blood drained. Intraoperatively, the liver was found to be multinodular, woody, enlarged, and cirrhotic with fresh heme. Final surgical pathology showed angiosarcoma with atypia over epithelioid hemangioendothelioma. Patient continued losing blood and required a hepatic angiogram with embolization. He continued to worsen, developing acute liver failure. Patient continued to decompensate and eventually required palliative care. (Figure) Discussion: Angiosarcoma often presents in elderly patients with nonspecific symptoms such as abdominal distention, abdominal discomfort, weight loss, and fatigue2,3,4. Differentiating liver tumors such as hepatoma, adenoma, or vascular malformations on imaging is challenging and surgical resection is essential. Average survival of patients with untreated liver angiosarcoma is approximately 6 months and it increases by 2 years with treatment2,3,4. The standard treatment for liver angiosarcoma is surgical resection. Liver transplant is not indicated because of the high recurrence rate and poor prognosis2,3,4. There are no standardized treatments for patients presenting with hemorrhagic shock besides embolization and resection. More treatments need to be explored for aggressive treatment for hepatic angiosarcoma, as this case highlights a healthy 19-year-old patient dying one month after initial diagnosis with limited options for therapeutic intervention.Figure 1.: CT Abdomen and Pelvis with multiple hypervascular lesions.

1521P Comprehensive genomic profiling (CGP) of epithelioid hemangioendothelioma (EHE) and liver angiosarcomas (LAS)

Epithelioid hemangioendothelioma (EHE) and liver angiosarcomas (LAS) are rare, malignant vascular sarcomas of the liver. LAS is frequently progressive and fatal as it is often detected in advanced stages. The clinical course of EHE exhibits a dual nature as it is often indolent but can grow rapidly and metastasize unpredictably. There is a dearth of effective targeted therapies in this disease space.

Soft tissue sarcoma incidences and clinical characteristics are significantly different in France and Taiwan.

The incidence of different soft tissue sarcoma (STS) histotypes among ethnic and geographic populations has not been comprehensively investigated. Data from 2013 to 2016 were obtained from national cancer registry databases in France and Taiwan. Liposarcoma (LPS), leiomyosarcoma (LMS), angiosarcoma (AS), synovial sarcoma (SS), and malignant peripheral nerve sheath tumor (MPNST) were selected as index STSs to estimate the age-standardized incidence rates (ASRs) and other clinical features between patients. In total, 9398 patients (7148 from France and 2250 from Taiwan) were included. The ASRs of AS (5.4 vs. 2.8) and MPNST (2.0 vs. 1.0) were significantly higher in Taiwan; France had significantly higher ASRs for LPS (12.0 vs. 10.0), LMS (9.7 vs. 7.6), and SS (1.7 vs. 1.2). Patients in Taiwan with LMS or LPS were younger than their French counterparts. With regard to the distribution according to primary anatomic site, French patients had higher odds for extremity and truncal LMS (odds ratio [OR], 2.84; p < .001), AS (OR, 2.67; p < .001), MPNST (OR, 1.55; p=.027), and LPS (OR, 1.38; p < .001) and for breast AS (OR, 10.58; p < .001). Taiwanese patients had higher odds for liver AS (OR, 10.72; p < .001) and uterine LMS (OR, 3.21; p < .001). SS age and distribution according to primary anatomic site did not differ significantly between the French and Taiwanese populations. Significant differences in the incidence and clinical characteristics of index STS suggested that geographic (environmental) and ethnicity factors likely play a vital role in the pathogenesis of STS.

Hepatic Angiosarcoma Post-Renal Transplantation: A Case Report

DNA damage and oncogenic viruses increase the risk of cancer post-kidney transplantation, including skin cancer, Kaposi's sarcoma, oral cancer, and non-Hodgkin lymphoma. Here we report an uncommon case of liver angiosarcoma that occurred 8 years after kidney transplantation. This study strictly complied with the Helsinki Congress and the Istanbul Declaration regarding donor source. A 57-year-old female patient received a cadaver kidney transplantation 8 years ago. She followed a long-term regimen of tacrolimus, mycophenolate sodium, and everolimus, with good renal function. She received annual regular abdominal ultrasound examinations after kidney transplantation, which showed no findings. The patient suffered from several symptoms for approximately 2 weeks before a scheduled abdominal ultrasound: diarrhea, epigastric pain, abdominal fullness, tea-colored urine, and little stool passage. The abdominal computerized tomography showed multiple hepatic tumors in both the hepatic lobes with engorged vasculatures and mild hemoperitoneum. A liver biopsy revealed the histopathology of angiosarcoma. The patient suffered multiple organ failure within one month of treatment. Various post-transplant malignancies are not uncommon after transplantation, warranting periodic screenings for any symptoms in these patients.

A Novel Entity Among Vascular Liver Tumors: The First Reported Liver Transplantation. Is It Feasible?

Liver transplant could be considered for certain tumors even if there is still dubious indication, as in the case of hepatic small vessels neoplasms that pose a difficult differential diagnosis with liver angiosarcoma. Liver transplant could be the best choice for patients with stable or slow-progressing tumors, for young patients with impaired quality of life, and when it would use organs that would be otherwise discarded but are capable of affording a good function. Vascular tumors are very heterogeneous cancers and our case represents the first description of a new histologic lesion that cannot be included in any of the pre-existing diagnostic categories. In our paper we want to present our decision to transplant a patient in whom the diagnosis was hepatic small vessels neoplasms (but was still in doubt for angiosarcoma as suggested by pathologists form other institutions). Furthermore, we want to highlight that after liver transplant, a new lesion never described before resulted from the specimen analysis that does not fit any of the pathologic diagnostic categories. This finding could open a scenario for additional improvement in molecular analysis in order to differentiate liver vascular tumors or even single lesion histotypes. This article emphasizes how little we know about the real behavior of liver vascular tumors and their clinical and therapeutic outcomes; we want to raise a question about the possibility for liver transplant in the setting of tumors not yet fully known and in which, in cases of diagnostic doubts, this option has been traditionally excluded, possibly bypassing ethical implications by using marginal donors.

Chemical Risk Factors of Primary Liver Cancer: An Update.

Primary liver cancer has the sixth highest incidence and fourth highest cancer mortality worldwide. Hepatitis B is the leading cause of liver cancer, though its incidence is decreasing with vaccination. Alcohol is the leading cause of liver transplant, cirrhosis, and cancer in the developed world, and is projected to surpass hepatitis B as the leading hepatic cancer etiology worldwide. Tobacco smoking has shown a positive association with liver cancer in a majority of studies, though not all. Aflatoxin, a mycotoxin produced by Aspergillus, is estimated to account for 3–20% of global liver cancer cases, 40% of which occur in sub-Saharan Africa. These statistics are confounded by the prevalence of hepatitis B, which may have a synergistic effect on hepatic carcinogenesis. Aflatoxin is ingested and likely inhaled from agricultural products, placing farmers, food processors, and textile workers in developing nations at risk. Vinyl-chloride is used in the production of PVC plastics and causes rare liver angiosarcoma, hepatocellular carcinoma, and other neoplasms. Arsenic and cadmium are naturally-occurring, hepatocarcinogenic metals with high occupational exposure in industries involving coal, metals, plastics, and batteries. Millions of laborers in waste-disposal and manufacturing are exposed to organic solvents and N-nitrosamines, which vary from carcinogenic (group 1) to possibly carcinogenic (group 2B) in their IARC designation. Insecticide DDT is possibly hepatocarcinogenic (group 2B), though continues to be used for malaria control in the developing world. While suggested by case reports, anabolic steroids and oral contraceptives have not been shown to increase liver cancer risk in large studies.

Impact of tumor histology and socioeconomic factors on survival of patients suffering from malignant vascular tumors of liver and hepatocellular carcinomas: A SEER database analysis.

e16612 Background: Primary malignant vascular tumors of the liver are rare, aggressive and poorly understood subtypes of liver cancers. In this analysis, we aim to determine the impact of tumor histology and other socio economic factors on survival of these tumors and hepatocellular carcinomas. Methods: Patients with malignant histopathological diagnoses of hepatocellular carcinoma not otherwise specified (HCC NOS), hepatocellular carcinoma with spindle cell (HCC SP), fibrolamellar (HCC F), clear cell (HCC CL), scirrhous (HCC SC) and pleomorphic variants ( HCC PL), combined hepatocellular carcinoma and cholangiocarcinoma (Mixed), hepatic angiosarcoma (AS), hemangioendothelioma (HE), its epithelioid variant (EHE) and hemangiopericytoma (HP) were identified using the SEER (version 2018) database. Overall survival was studied with Kaplan–Meier with the log rank method. Multivariable analysis was performed to assess the impact of race, ethnicity, marital and insurance status on survival of these patients. Results: We analyzed de-identified data of 104502 patients from the year 1973 to 2016 with hepatocellular carcinomas and malignant vascular tumors of liver (including 101851 patients with HCCNOS, 70 with HCC SP, 378 with HCC F, 104 with HCC SC, 593 with HCC CL, 23 with HCC PL, 950 with Mixed, 367 with AS, 36 with HE, 120 with EHE and 10 with HP ) respectively. Median overall survival (OS) of HCC NOS was calculated to be 7 months. Among hepatocellular carcinoma patients HCC F had the longest median OS of 29 months and HCC SP had the shortest median OS of 3months (P &lt; 0.001). Additionally, among patients with malignant vascular tumors, AS had the shortest median OS (1 month) while patients with EHE had the longest median OS of 81 months (P &lt; 0.001). Overall, married and insured patients had a better overall survival than unmarried and uninsured patients (P &lt; 0.04). Conclusions: Malignant vascular tumors of liver are rare in comparison to hepatocellular carcinomas. Spindle cell variant of HCC and liver angiosarcomas carry the worst prognosis and fibrolamellar variant of HCC, hemangioendothelioma (HE) and its epithelioid variant (EHE) have the best prognosis. Insurance and marital status has a positive impact on overall survival of liver cancer patients.

Primary liver angiosarcoma and factors associated with improved outcomes: An analysis of the National Cancer Database.

498 Background: Primary liver angiosarcoma (LAS) is a rare and aggressive tumor of the liver. In this analysis of the national cancer database (NCDB) we sought the risk of mortality and factors associated with survival amongst patient diagnosed with LAS. Methods: Patients diagnosed with hepatocellular carcinoma (HCC) or LAS from 2004 – 2014 were identified in the NCDB. The Kaplan-Meier method with the log-rank test was used to calculate survival for HCC and LAS patients. Additional analyses were performed on the cohort with LAS to assess the impact of surgery, chemotherapy, radiation therapy (RT) and facility type on overall survival (OS). Multivariable analyses using cox proportional methods, adjusted for age, sex, Charlson/Deyo score, race, ethnicity, insurance status, facility location and type, surgery status, and chemotherapy status were performed to obtain adjusted hazard ratio (aHR). Results: Total of 118,066 patients with HCC and 346 patients with LAS were identified in the database. Median survival for HCC patients was 11.9 months (95% CI: 11.7-12.2) and 2.0 months for LAS patients (95% CI: 1.8 – 2.4). Risk of mortality was higher for patients with LAS compared to those with HCC (aHR (95% CI): 2.23 (1.97 - 2.53), p &lt; .0001). Among the LAS patients, those who received surgery had a median survival of 8.6 months (95% CI: 5.6 - 17.3), and 1.8 months for those who did not (95% CI: 1.48 - 1.94). Risk of mortality was lower in patients who received surgery compared to those who did not (aHR (95% CI): 0.23 (0.15 - 0.37), p &lt; .0001). Patients treated at and academic center had a higher median survival (3.3 months, 95% CI: 2.2 - 4.1) then those treated at a non-academic center (1.5 months, 95% CI: 1.2 - 1.8). Though, there was no significant difference in OS (aHR (95% CI): 0.48 (0.21 - 1.10), p = 0.082). A very small number of patients received chemotherapy or RT to conduct a meaningful analysis. Conclusions: Patients diagnosed with primary LAS have a worse OS compared to those with HCC. Amongst patients with primary LAS, surgical resection is associated with best survival outcomes. Treatment at an academic center is associated with better median survival, although OS did not reach statistical significance in our analysis.

Epitheloid Angiosarcoma of Liver: A Hanging Tumour

Primary Epitheloid Angiosarcoma (EA) of liver is an aggressive malignant tumour with poor prognosis irrespective of aggressive surgical resection with or without adjuvant therapy. It constitutes for 0.1-2% of primary malignant tumours of liver and is seen commonly in sixth or seventh decade of life, with male preponderance. It can be asymptomatic or may present with nonspecific symptoms. Lack of pathognomonic features on serological, biochemical and radiological tests, makes it even difficult for an early diagnosis. Despite early surgical removal, EA has very high recurrence rate. Chemotherapy agents (paclitaxel, bevacizumab) and immunomodulators (mTOR inhibitors) have shown some survival benefit and reduction in recurrence rates in recent years as adjuvant therapy. To the best of our knowledge, a hanging morphological variant of EA of liver has not been reported yet in literature. Author here by report a case of 37-years-old male incidentally diagnosed with aggressive, hanging variant of EA of liver. Despite early surgical resection and adjuvant, after chemotherapy patient had survival of less than eight months since first presentation.

Arsenic and disinfection by-products (DBPs) in drinking water in Canada

OPS 25: Drinking water contamination and adult health, Room 411, Floor 4, August 26, 2019, 10:30 AM - 12:00 PM Introduction: People are exposed to Arsenic and DBPs (Trihalomethanes (THMs) and Haloacetic acids (HAAs) by drinking contaminated water. Arsenic is a naturally occurring element and known as a strong carcinogen. DBPs are formed by chlorination of water containing organic substances and considered possible human carcinogen. Water quality reports demonstrate the presence of high level of arsenic and DBPs in some areas of Newfoundland and Labrador (NL). However, there is no population level data on any association between the exposure to arsenic and DBPs and related cancer. Aim: To explore any association between Arsenic and DBPs present in drinking water and relevant cancers at the community level. Methods: High and low risk communities were identified based on water quality reports of provincial surveys. Cancer data were extracted from the NL Cancer Registry for cases diagnosed between 2007 and 2016. Certain disease information such as histology and topography and demographic information like sex, year of birth and residence at time of diagnosis were extracted. An ecological analysis was then conducted comparing cancer incidence rates between high risk and low risk communities. Results: Relative risk (RR) of arsenic induced cancers (a combination of renal cell, squamous cell of lung, skin, basal cell and Merkel cell of skin, adenocarcinoma of colon and bile ducts, hepatocellular and angiosarcoma of liver, cholangiocarcinoma, transitional cell carcinoma of urinary bladder) was 3.8 (95% CI, 1.09, 1.58). For DBPs, RR of THMs and HAAs induced cancer (a combination of urothelial cell carcinoma of urinary bladder, adenocarcinoma of rectum, colon and esophagus, malignant mesothelioma of lungs, and acute and chronic myeloid leukemia) was 2.1 (95% CI 0.67, 0.82). Discussion: Population level data showed that arsenic in drinking water was significantly associated in cancer incidences. However, despite the high relative risks, DBPs were not significantly associated with relevant cancer.

Mortality from liver angiosarcoma, hepatocellular carcinoma, and cirrhosis among vinyl chloride workers.

Occupational exposure to vinyl chloride monomer (VCM) has been established as a cause of hepatocellular carcinoma (HCC) and liver angiosarcoma (ASL). However, some controversy remains due to conflicting results on liver cirrhosis, and to evidence on HCC based on few confirmed cases. The aim of the study is to clarify the association between VCM exposure and mortality from liver diseases. In a cohort of 1658 workers involved in VCM production and polymerization, Poisson regression was adopted to estimate rate ratios (RR) across categories of VCM exposure for mortality due to ASL (n = 9), HCC (n = 31) confirmed by histological/clinical records, and the combination of deaths from liver cirrhosis and from liver cancer with clinical/histological evidence of cirrhosis (n = 63). Cumulative VCM exposure was associated with study outcomes; RRs in the highest compared to the lowest exposure category were: ASL 91.1 (95%Confidence Interval 16.8-497), HCC 5.52 (2.03-15.0), liver cirrhosis 2.60 (1.19-5.67). The risk of death from liver cirrhosis, as well as from HCC in the largest available series of confirmed cases, increased with VCM exposure.

A pooled analysis of treatment and prognosis of hepatic angiosarcoma in adults

BackgroundHepatic angiosarcoma is a rare malignant vascular tumor presenting unique treatment challenges. The aim of the present study was to determine the treatment and prognosis of this entity. Data sourcesA systematic literature search was conducted using PubMed, Embase and Chinese Biomedical Literature database, to identify articles published from January 1980 to July 2017. Search terms were “hepatic angiosarcoma” and “liver angiosarcoma”. Additional articles were retrieved through manual search of bibliographies of the relevant articles. Pooled individual data concerning the prognosis following various therapeutic modalities were analyzed. ResultsA total of 75 articles involving 186 patients were eligible for inclusion. The median overall survival (OS) was 8 months, with 1-, 3-, and 5-year OS rates of 36.6%, 22.3%, and 12.0%, respectively. The median OS after partial hepatectomy (n = 86), chemotherapy (n = 36), liver transplantation (n = 17), and supportive care (n = 46) were 15, 10, 5 and 1.3 months, respectively. Small tumor size (<10 cm) was the only significant favorable factor for OS after partial hepatectomy (P = 0.012). ConclusionsDespite the dismal prognosis, partial hepatectomy could prolong the survival of hepatic angiosarcoma patients, particularly those with tumors <10 cm. Chemotherapy could be an option for unresectable disease. Liver transplantation is not a recommendable option for the management of this malignancy.

The lymphocyte cytokinesis block micronucleus test in human populations occupationally exposed to vinyl chloride: A systematic review and meta-analysis.

Vinyl chloride (VC) is widely used in industry in the production of polyvinyl chloride (PVC), which is used to manufacture a large variety of materials. VC was classified as a known (Group 1) human carcinogen by IARC on the basis of increased risk for liver angiosarcoma and hepatocellular cancer, and the carcinogenicity of VC was shown to be mediated by a genotoxic mechanism. Following inhalation, the compound is rapidly absorbed and metabolized in the liver to the electrophilic metabolites chloroethylene-oxide and chloroacetaldehyde, which form DNA adducts that can be processed into point mutations in cancer-related genes detected in humans and rats exposed to VC. A number of genotoxicity biomarkers were applied in workers exposed to VC to detect early biological responses associated with the carcinogenesis process. The present systematic review analyzed the published studies in which the cytokinesis-block micronucleus assay in peripheral lymphocytes (L-CBMN) was applied in VC-exposed subjects. Thirteen out of fifteen retrieved studies performed in China showed increased MN frequencies (FR 1.92-3.98) associated with increased cumulative exposure or employment time. Twofold and more than threefold increases were detected in PVC-exposed workers exposed to a mean of 50ppm of VC in the former Yugoslavia and in South India, respectively. The meta-analysis of MN frequency from six eligible studies confirmed this tendency (pooled MR 2.32 - 95% CI 1.64-3.27). The benchmark dose lower limit for 10% excess risk (BMDL 10) calculated from three studies resulted in an estimated exposure limit of 0.03-0.07mg/m3. Overall the results of this review showed the need for further studies, especially because PVC products from China may contain high levels of uncoupled VCM that could represent a source of exposure to workers and consumers. Moreover, the results underline the importance of re-evaluating the recommended exposure limits using new biomonitoring methods in addition to MN.

Unusual Indications for a Liver Transplant: A Single-Center Experience.

This study sought to evaluate the efficacy of liver transplant for unusual liver diseases. The results of 476 patients who underwent liver transplant from 1988 to January 2015 were retrospectively analyzed. Two hundred forty-five of them were adult patients and 231 of them were pediatric. Thirty-one patients had unusual liver disease. Of the 31 patients with unusual liver disease, 9 (29%) were adult and 22 (71%) were pediatric patients. In the pediatric group, indications for liver transplant were Alagille syndrome (n = 5), Crigler-Najjar syndrome type 1 (n = 5), glycogen storage disease (n = 3), oxalosis (n = 3), familial hypercholesterolemia (n = 2), alpha-1-antitrypsin deficiency (n = 2), Caroli disease (n = 1), and cystic neuroblastoma metastasis (n = 1). Six patients (27.2%) had acute rejection episodes and were successfully treated with pulse steroids. In 2 patients, retransplant was performed for chronic rejection. Three patients died during follow-up (13.6%): 2 because of sepsis and 1 because of cranial hemorrhage. In the adult group, indications for liver transplant were neuroendocrine tumor metastasis (n = 1), liver angiosarcoma (n = 1), familial hypercholesterolemia (n = 2), alveolar hydatid disease (n = 2), cystic fibrosis (n = 1), congenital hepatic fibrosis (n = 1), and oxalosis (n = 1). Four patients (44.4%) had acute rejection episodes and were successfully treated with pulse steroid therapy. One patient died due to the recurrence of primary disease (liver angiosarcoma) during follow-up (11.1%). Advances in liver transplant and our understanding about unusual liver disease have led to significant improvements in managing these diseases. Liver transplant effectively treats the underlying defect and the complications of portal hypertension, or risk of malignancy for those disorders, in which the liver is affected.