Proposed diagnostic and prognostic markers of primary malignant hepatic vascular neoplasms.

INTRODUCTION: Primary malignant hepatic vascular tumors with various malignant potentials include epithelioid hemangioendothelioma (EHE) and angiosarcoma (AS), which may overlap pathologically. This study aimed to compare the pathological findings of hepatic EHE with those of AS, in association with patient outcomes. METHODS: Fifty-nine histologically confirmed patients with 34 EHE and 25 AS were admitted to a tertiary hospital from 2003 to 2020. Their EHE and AS pathological features were compared. Immunohistochemistry for CD31, ERG, CAMTA-1, TFE3, P53, and Ki-67 labeling was performed on paraffin-embedded blocks. Markers, along with histological findings, were analyzed for the purposes of diagnostic and prognostic significance by multivariate analysis. RESULTS: CAMTA-1 was 91.2% positive in EHE, but negative in AS (p = < 0.001). AS was significantly correlated to an aberrant p53 expression, high Ki-67 labeling, and high mitotic activity, compared to EHE (all, p = < 0.001). EHE can be classified as low grade (LG) and high grade (HG) using the prognostic values of mitotic activity and ki-67 labeling (sensitivity = 1, specificity = 1). Low grade-EHE showed significantly better overall survival than high grade-EHE (p = 0.020). CONCLUSIONS: Immunohistochemistry for CAMTA-1, P53, and Ki-67 labeling may help distinguish EHE and AS in histologically ambiguous cases, especially small biopsied tissue. Moreover, the combination of mitotic activity and Ki-67 labeling can be a prognostic factor for EHE with various clinical features.

Characteristics and outcomes of primary pleural angiosarcoma: A retrospective study of 43 published cases.

ABSTRACT: Primary pleural angiosarcoma (PPA) is an extremely rare malignancy for which there is no consensus on treatment. The clinical course of PPA is usually quickly fatal, regardless of the treatment used.We summarized and evaluated a relatively large population of published PPA cases to assess prognostic factors, diagnostic approaches, treatment methods and clinical outcomes. Using the CNKI, Embase, and PubMed databases, literature published in English and Chinese from 1988 through 2020 was searched using the terms "primary pleural angiosarcoma," "pleural angiosarcoma," and "pleuropulmonary angiosarcoma."A total of 43 patients with PPA were identified in retrospective case series and case reports. The median age at diagnosis was 64 years (range 24-87 years), and the median overall survival was 4 months (range 0.1-180 months). Approximately 80% of patients died from PPA within 10 months of diagnosis, and the 2-year survival rate was approximately 4.4%. In univariate analyses, the presence of pleural effusion and hemothorax were significant predictors of decreased survival, with hazard ratios (HRs) of 2.7 (P = .04) and 3.3 (P = .006), respectively. Sixteen patients received no therapy, and their prognosis was worse than patients who did receive therapy (P = .019). Radiation therapy improved survival more than no radiation therapy (P = .007). Patients appeared to derive clinical benefit from chemotherapy (P = .048). However, tumor resection did not seem to provide a survival benefit (P = .051). In multivariate analysis, tumor resection, and radiation were independent, statistically significant, positive predictors of better survival, with HRs of 0.3 (P = .017) and 0.1 (P = .006), respectively. The presence of hemothorax was an independent predictor of worse prognosis (P = .006).Primary angiosarcoma of the pleura is a rare, poorly understood malignancy with a poor prognosis; hence, the clinical spectrum of PPA is not completely defined. By multivariate analysis, this retrospective study showed a survival benefit of tumor resection or radiation therapy, and the presence of hemothorax was a significant prognostic factor for poor outcomes.

Characteristics and survival outcomes of primary splenic cancers: A SEER population-based study.

ABSTRACT: Primary splenic cancers represent a small number of cancer cases and studies on its clinicopathological features and outcomes are limited. Splenic lymphomas and primary splenic angiosarcoma (PSA) are the 2 most common histological types of splenic cancers. This population-based study aimed to investigate the clinical characteristics and survival outcomes of patients with splenic lymphomas or PSA.Patients diagnosed with splenic lymphomas or PSA between 2000 and 2015 were identified from the Surveillance Epidemiology and End Results database of the National Cancer Institutes. Overall survival (OS) and cancer-specific survival (CSS) rates were calculated using the Kaplan-Meier method. A Cox proportional hazard models were used to identify independent predictors of cancer-specific mortality.A total of 700 patients with splenic lymphoma and 48 patients with PSA were included in this study. The median age of patients with splenic lymphoma was 65 years and 57 years for patients with PSA. For patients with splenic lymphoma, the most prevalent histological subtypes were splenic marginal zone lymphoma and diffuse large B-cell lymphoma. A total of 52.6% of the cases had stage IV disease based on the Ann Arbor staging system. Five-year OS and CSS were 76.9% and 83.4%, respectively. Multivariate analysis revealed that independent predictors of splenic lymphoma CSS included race, stage, chemotherapy, and histological subtype. However, a much shorter OS time was seen in the PSA cohort which had a 5-year OS of 11.8%, a median OS of 10.0 months and the 5-year CSS of 12.4%. Chemotherapy was correlated with better outcomes in patients with PSA. However, the survival benefits of surgery for splenic cancer were not statistically significant in our study.The current study is the largest cohort of primary splenic cancer presented in literature based on the Surveillance Epidemiology and End Results database and our large series describe the characteristics and survival outcomes of such rare diseases which may provide reliable information for further studies and clinicians.

Neoadjuvant radiation for cutaneous and soft tissue angiosarcoma.

BACKGROUND AND OBJECTIVES: Neoadjuvant radiation (NRT) is frequently utilized in soft tissue sarcomas to increase local control. Its utility in cutaneous and soft tissue angiosarcoma remains poorly defined. METHODS: This retrospective cohort study was performed using the National Cancer Database (2004-2016) evaluating patients with clinically localized, surgically resected angiosarcomas. Factors associated with receipt of NRT in the overall cohort and margin positivity in treatment naïve patients were identified by univariate and multivariable logistic regression analyses. Survival was assessed using Kaplan-Meier analysis. RESULTS: Of 597 patients, 27 (4.5%) received NRT. Increasing age (odds ratio [OR] 0.95, p = 0.025), tumor size more than or equal to 5 cm (OR 3.16, p = 0.02), and extremity tumor location (OR 3.99, p = 0.04) were associated with receipt of NRT. All patients who received NRT achieved an R0 resection (p = 0.03) compared with 17.9% of patients without NRT. Factors associated with risk of margin positivity included tumor size more than or equal to 5 cm (OR 1.85, p = 0.01), and head/neck location (OR 2.24, p = 0.006). NRT was not significantly associated with improved survival (p = 0.21). CONCLUSIONS: NRT improves rates of R0 resection but is infrequently utilized in cutaneous and soft tissue angiosarcoma. Increased usage of NRT, particularly for patients with lesions more than or equal to 5 cm, or head and neck location, may help achieve complete resections.

New nomograms to predict overall and cancer-specific survival of angiosarcoma.

OBJECTIVE: This study was designed to establish and validate promising and reliable nomograms for predicting the survival of angiosarcoma (AS) patients. METHODS: The Surveillance, Epidemiology, and End Results database was queried to collect the clinical information of 785 AS patients between 2004 and 2015. Data were split into a training cohort (n = 549) and a validation cohort (n = 236) without any preference. Univariate Cox and multivariate Cox regression analyses were performed to analyze the clinical parameters. Independent prognostic factors were then identified. Two nomograms were constructed to predict overall survival (OS) and cancer-specific survival (CSS) at 3 and 5 years. Finally, the models were evaluated using concordance indices (C-indices), calibration plots, and decision curve analysis (DCA). RESULTS: Based on the inclusion and exclusion criteria, 785 individuals were included in this analysis. Univariate and multivariate Cox regression analyses revealed that age, tumor size, and stage were prognostic factors independently associated with the OS of AS. Tumor site, tumor size, and stage were associated with the CSS of AS. Based on the statistical results and clinical significance of variables, nomograms were built. The nomograms for OS and CSS had C-indices of 0.666 and 0.654, respectively. The calibration curves showed good agreement between the predictive values and the actual values. DCA also indicated that the nomograms were clinically useful. CONCLUSION: We established nomograms with good predictive ability that could provide clinicians with better predictions about the clinical outcomes of AS patients.

An open-label single-arm phase II study of regorafenib for the treatment of angiosarcoma.

PURPOSE: Angiosarcomas represents a diverse group of aggressive high-grade vascular tumours with limited therapeutic options. We sought to determine the safety and efficacy of regorafenib, a small-molecule multikinase inhibitor, in the treatment of metastatic or locally advanced unresectable angiosarcoma. PATIENTS AND METHODS: In this single-arm multicentre, open-label phase II clinical trial, 31 patients were enrolled and received regorafenib 160 mg PO daily for 21 days of a 28-day cycle. The primary endpoint for the study was progression-free survival at 4 months. Secondary endpoints included overall survival, response rate, and safety. Patients (≥18 years) with an Eastern Cooperative Oncology Group (ECOG) score of 0-1, a life expectancy of at least 4 months who had progressed on at least one but no more than 4 prior lines of therapy were eligible. RESULTS: Of the 23 patients evaluable for efficacy, 2 had a complete response (8.7%), and 2 had a partial response (8.7%), for a total overall response rate of 17.4%. Median PFS was 5.5 months, and 12/23 patients (52.2%) had a PFS of greater than 4 months. 10/31 (32.3%) patients evaluable for toxicity had a grade 3 or higher adverse events. CONCLUSIONS: Regorafenib is a safe and active treatment for refractory metastatic and unresectable angiosarcoma. Rates of adverse events were comparable to prior studies of regorafenib for other tumour types. Regorafenib, the single agent, could be considered as therapy for patients with metastatic or unresectable AS.

Current Management of Angiosarcoma: Recent Advances and Lessons From the Past.

OPINION STATEMENT: Despite their rarity, angiosarcomas are one of the most aggressive soft tissue sarcomas. Management can often be challenging due to their location and infiltrative nature. A multidisciplinary treatment approach is always warranted, but the recurrence remains high even for localized tumors despite multimodality treatment. In the metastatic setting, cytotoxic chemotherapies, targeted therapies, and, more recently, immunotherapy are used. The sequence of systemic therapies remains currently a topic of active investigation. Over the last couple of years, there have been significant advances in understanding angiosarcoma biology, most notably via patient-driven initiatives like the Angiosarcoma Project. The knowledge derived from such translational work has led to identifying potential biomarkers of response to treatments and exploring new therapeutic avenues. More clinical trials are underway to expand treatment options and improve patient outcomes.

Tumor Biology Impacts Survival in Surgically Managed Primary Hepatic Vascular Malignancies.

BACKGROUND: Hepatic angiosarcoma (AS) and hepatic epithelioid hemangioendothelioma (HEHE) are rare primary hepatic vascular malignancies (PHVM) that remain poorly understood. To guide management, we sought to identify factors and trends predicting survival after surgical intervention using a national database. MATERIALS AND METHODS: In a retrospective analysis of the National Cancer Database patients with a diagnosis of PHVM were identified. Clinicopathologic factors were extracted and compared. Overall survival (OS) was estimated and predictors of survival were identified. RESULTS: Three hundred ninty patients with AS and 216 with HEHE were identified. Only 16% of AS and 36% of HEHE patients underwent surgery. The median OS for patients who underwent surgical intervention was 97 months, with 5-year OS of 30% for AS versus 69% for HEHE patients (P< 0.001). Tumor biology strongly impacted OS, with AS histology (Hazard Ratio [HR] of 3.61 [1.55-8.42]), moderate/poor tumor differentiation (HR = 3.86 [1.03-14.46]) and tumor size (HR = 1.01 [1.00-1.01]) conferring worse prognosis. The presence of metastatic disease in the surgically managed cohort (HR = 5.22 [2.01-13.57]) and involved surgical margins (HR = 3.87 [1.59-9.42]), were independently associated with worse survival. CONCLUSIONS: In this national cohort of PHVM, tumor biology, in the form of angiosarcoma histology, tumor differentiation and tumor size, was strongly associated with worse survival after surgery. Additionally, residual tumor burden after resection, in the form of positive surgical margins or the presence of metastasis, was also negatively associated with survival. Long-term clinical outcomes remain poor for patients with the above high-risk features, emphasizing the need to develop effective forms of adjuvant systemic therapies for this group of malignancies.

Breast Angiosarcoma Surveillance Study: UK national audit of management and outcomes of angiosarcoma of the breast and chest wall.

BACKGROUND: Breast angiosarcomas are rare tumours of vascular origin. Secondary angiosarcoma occurs following radiotherapy for breast cancer. Angiosarcomas have high recurrence and poor survival rates. This is concerning owing to the increasing use of adjuvant radiotherapy for the treatment of invasive breast cancer and ductal cancer in situ (DCIS), which could explain the rising incidence of angiosarcoma. Outcome data are limited and provide a poor evidence base for treatment. This paper presents a national, trainee-led, retrospective, multicentre study of a large angiosarcoma cohort. METHODS: Data for patients with a diagnosis of breast/chest wall angiosarcoma between 2000 and 2015 were collected retrospectively from 15 centres. RESULTS: The cohort included 183 patients with 34 primary and 149 secondary angiosarcomas. Median latency from breast cancer to secondary angiosarcoma was 6 years. Only 78.9 per cent of patients were discussed at a sarcoma multidisciplinary team meeting. Rates of recurrence were high with 14 of 28 (50 per cent ) recurrences in patients with primary and 80 of 124 (64.5 per cent ) in those with secondary angiosarcoma at 5 years. Many patients had multiple recurrences: total of 94 recurrences in 162 patients (58.0 per cent). Median survival was 5 (range 0-16) years for patients with primary and 5 (0-15) years for those with secondary angiosarcoma. Development of secondary angiosarcoma had a negative impact on predicted breast cancer survival, with a median 10-year PREDICT prognostic rate of 69.6 per cent, compared with 54.0 per cent in the observed cohort. CONCLUSION: A detrimental impact of secondary angiosarcoma on breast cancer survival has been demonstrated. Although not statistically significant, almost all excess deaths were attributable to angiosarcoma. The increased use of adjuvant radiotherapy to treat low-risk breast cancer and DCIS is a cause for concern and warrants further study.

Hepatic vascular malignancies in children are associated with increased rates of surgical resection and improved overall survival compared with adults.

BACKGROUND: Hepatic vascular malignancies (HVMs) are rare malignancies, with no standardized treatment regimens. The most common HVMs, angiosarcoma and malignant epithelioid hemangioendothelioma (EHE), are often grouped together in the literature complicating our ability to achieve reliable survival data and treatment strategies. OBJECTIVE: To compare the disease characteristics of HVMs, with a subanalysis on pediatric patients. METHODS: The 2016 National Cancer Database was queried for patients with HVMs using international classification of diseases-oncology-3 (ICD-O-3) codes yielding 699 patients. Descriptive statistics, chi-square, Kaplan-Meier, and log-rank analyses were performed. RESULTS: We found 478 patients (68%) with angiosarcoma and 221 (32%) with EHE. The median (Q1, Q3) age for angiosarcoma patients was 65 years (56, 75) versus 54 years (37, 65) in EHE patients (P < .001). The rate of resection was lower in patients with angiosarcoma than EHE (13% vs 32%, P < .001). The mean 1-, 3-, and 5-year overall survival for angiosarcoma patients was 17%, 8%, and 6%, respectively, versus 80%, 65%, and 62% in EHE patients (P < .0001). A subgroup analysis was performed on pediatric patients demonstrating six with angiosarcoma and 10 with EHE. The mean 1-, 3-, and 5-year overall survival for pediatric angiosarcoma patients was 67%, 50%, and 50%, respectively, and 90%, 90%, and 90% for pediatric EHE patients. CONCLUSION: In the largest study of HVMs to date, we found angiosarcoma has significantly worse overall survival than EHE. Pediatric patients appear to have improved survival and higher rates of resection. Larger studies of HVMs are needed to clearly differentiate tumor types, standardize care, and improve survivorship.

Primary Angiosarcomas Of The Bone: An Evaluation Of 4 Cases.

Angiosarcoma is a rare mesenchymal neoplasm that may arise from vascular or lymphatic tissue. Angiosarcoma of bone is a rare high-grade malignant vascular tumour, representing less than 1% of all angiosarcomas. The most common locations of unifocal tumour are the long and short tubular bones, followed by the pelvis, and trunk. The literature regarding treatment and outcome of patients with this tumour is limited. We performed a retrospective study to analyse treatment and survival of four patients with angiosarcoma of bone.

Real-World Clinical Outcomes and Prognostic Factors for Patients with Advanced Angiosarcoma who Received Systemic Treatment.

PURPOSE: Angiosarcoma is a highly aggressive mesenchymal tumor. Although systemic chemotherapy is often considered for the inoperable or metastatic angiosarcoma, the outcome of such treatment is unsatisfactory and poorly delineated. MATERIALS AND METHODS: We reviewed electronic medical records of 75 patients with angiosarcoma who were treated with systemic chemotherapy for inoperable or metastatic disease. Patients were classified as having liver involvement if they had either primary or metastatic hepatic lesions. RESULTS: Among the patients evaluated, 51 patients (68%) were male and 24 patients (32%) had primary cutaneous angiosarcoma. Liver involvement was present in 28 patients (37.3%). A total of 59 patients received first-line weekly paclitaxel (wPac) and showed an objective response rate (ORR) of 23.7% (n=14), a median progression-free survival (mPFS) of 4.0 months (95% confidence interval [CI], 3.0 to 6.1), and a median overall survival (mOS) of 10.2 months (95% CI, 7.0 to 14.6). Among patients without liver involvement, patients receiving wPac (n=35) had significantly prolonged mPFS (5.8 months vs. 3.2 months, respectively; p=0.014) with a tendency for prolonged mOS (13.8 months vs. 11.6 months, respectively; p=0.13) than those receiving other regimens (n=12). A total of 24 patients received second- or later-line pazopanib monotherapy and showed an ORR of 16.7% (n=4), a mPFS of 2.4 months (95% CI, 1.8 to 4.3) and a mOS of 5.4 months (95% CI, 3.5 to not available). CONCLUSION: Treatment with first-line wPac and later-line pazopanib seems to provide survival benefit, especially for patients with advanced angiosarcoma without liver involvement.

The treatment landscape of advanced angiosarcoma in Asia-A multi-national collaboration from the Asian Sarcoma Consortium.

Angiosarcoma (AS) is a rare disease with a dismal prognosis. The treatment landscape and prognostic factors for advanced AS, including locally advanced, unresectable, and metastatic disease remain elusive. The Asian Sarcoma Consortium is an international collaborative effort to understand the sarcoma treatment landscape in Asia. We undertook a retrospective chart review of AS patients seen in 8 sarcoma academic centers across Asia. Patients with complete clinical, treatment, and follow-up data were enrolled. Overall, 276 advanced AS patients were included into this study; 84 (30%) of the patients had metachronous metastatic AS. The median age was 67 y; primary sites of AS was cutaneous in 55% and visceral in 45% of patients. In total, 143 (52%) patients received at least 1 line of systemic chemotherapy. The most common first-line chemotherapy regimen used was paclitaxel (47.6%) followed by liposomal doxorubicin (19.6%). The median overall survival (OS) was 7.8 mo. Significant prognostic factors for OS included age > 65 (hazard ratio (HR) 1.54, P = .006), male gender (HR 1.39, P = .02), and a cutaneous primary AS site (HR 0.63, P = .004). The median progression-free survival (PFS) for first-line chemotherapy was 3.4 mo. PFS for single vs combination or paclitaxel vs liposomal doxorubicin chemotherapy regimens were comparable. This study provides an insight into the treatment patterns and prognostic factors of advanced AS patients in Asia. Prognosis of advanced AS remains poor. Data from this study serve as a benchmark for future clinical study design.

Angiosarcoma associated with radiation therapy after treatment of breast cancer. Retrospective study on ten years.

PURPOSE: The breast sarcoma induced by radiation therapy is rare but increasing, given the increased long-term survival of patients receiving radiation therapy. Fibrosarcoma, histiocytofibroma and angiosarcoma are the most common breast sarcoma. Angiosarcoma is the most common after breast cancer treated by radiation therapy, often diagnosed too late, with a severe prognosis and a high rate of recurrence. However, because of the low incidence of angiosarcoma associated with radiation therapy (AAR), the benefit of radiation therapy in breast cancer treatment outweighs the risk to develop angiosarcoma. The aim of this study is to evaluate these rare cases of AAR diagnosed in eastern Belgium in comparison to the data from the literature. PATIENTS AND METHODS: Nine cases of AAR after radiation for breast ductal carcinoma were included in this retrospective study. AAR was diagnosed according to Cahan criteria between January 2007 and December 2016. Latency, incidence, management and prognosis are comparable to the literature. RESULTS, CONCLUSION: The median latency was 10 (4-24) years, the incidence of AAR in the East Belgian area was 0.09% of the patients irradiated on the same period. Patients were treated by surgery with wide local excision with or without reconstructive surgery, without radiotherapy and chemotherapy treatment. Kaplan-Meier analysis showed median overall survival of 61.8 months, patient survival of 55.6% at one year and 29.6% at five years. With the constant progress of medicine and its technologies, it would be possible to limit the occurrence of AAR or to diagnose it at an earlier stage.

Multidisciplinary Management of Angiosarcoma - A Review.

Angiosarcomas (AS) are a diverse group of soft tissue sarcomas, arising from blood and lymphatic vessels. They frequently present in the elderly, and in patients with previous radiation or lymphedema. A wide range of genetic derangements contribute to their development, and AS histology is often high-grade in keeping with aggressive disease biology. The clinical presentation, while often innocuous, is marked by its infiltrative and aggressive nature, with a proclivity for metastatic spread, and outcomes are often poor. Surgery is performed for localized, resectable cases. A multidisciplinary approach, appropriately employing surgery, radiation, chemotherapy, or potentially recently approved immune-oncology agents, can result in positive outcomes.

MYC gene amplification by fluorescence in situ hybridization and MYC protein expression by immunohistochemistry in the diagnosis of cutaneous angiosarcoma: Systematic review and appropriate use criteria.

BACKGROUND: Secondary angiosarcoma (AS) most commonly follows breast cancer and includes postirradiation AS (PRAS) and lymphedema-associated AS. The frequent amplification of MYC (8q24.21) in secondary AS and the rising incidence of PRAS and atypical vascular lesions (AVLs) have prompted interest in the diagnostic and prognostic utility of MYC in AS. METHODS: Retrospective series with ≥2 cases of cutaneous AS and describing the use of fluorescence in situ hybridization (FISH) for MYC amplification or immunohistochemistry (IHC) for MYC overexpression were included. RESULTS: Sixteen studies met inclusion criteria. Overall, 93% of cases evaluated by FISH and IHC were concordant. The sensitivity of FISH in primary AS was only 6.8%, and protein overexpression occurred without amplification in sun-damaged skin. FISH and IHC were over 78% sensitive in secondary AS but negative in over 98% of AVLs. MYC amplification and FLT4 coamplification were associated with shorter overall survival in secondary AS. CONCLUSION: FISH for MYC amplification and IHC for MYC overexpression are useful in distinguishing PRAS from AVLs and may also have prognostic value in secondary AS. In contrast, these methods have little diagnostic or prognostic value in primary AS and should not be used to distinguish primary AS from benign vascular neoplasms.

Angiosarcoma of the Breast: Management and Outcomes.

OBJECTIVE: Angiosarcoma of the breast is rare and has a poor prognosis. We reviewed our institution's experience with this disease to characterize presentation, identify management patterns, and report outcomes. METHODS: Fifty-eight patients with nonmetastatic angiosarcoma were identified from 1998 to 2019 and retrospectively reviewed. Overall survival (OS) and recurrence-free survival (RFS) were calculated using the Kaplan-Meier analysis and log-rank test. RESULTS: The median follow-up was 43.4 months (range: 1.8 to 203.3 mo). Twenty-four patients had primary angiosarcoma (PAS) and 34 patients had secondary angiosarcoma (SAS). Patients with PAS were significantly younger than those with SAS (P<0.0001). Mastectomy was the main surgical treatment in our cohort (n=47) and 3 underwent a lumpectomy. The multifocal disease was found in 5/23 patients with PAS and 11/35 patients with SAS. Twenty-eight patients received chemotherapy. Radiation was administered to 13 patients with PAS and 3 patients with SAS. Five-year OS was 73.7% for PAS and 63.5% for SAS. Local recurrence occurred in a greater proportion of patients with margins <5 mm than those with margins ≥5 mm. Chemotherapy did not impact RFS and was not associated with OS in PAS (P=0.35). Those with SAS treated with chemotherapy had significantly greater OS than those who did not receive chemotherapy (P=0.043). Radiation did not significantly influence RFS or OS. CONCLUSIONS: Five-year OS was higher than anticipated. Margins >5 mm appear important for local control. Patients with SAS, but not PAS, may achieve improved survival with chemotherapy. National trials using prespecified agents may be needed to identify an optimal chemotherapy regimen for women with SAS.

[Diagnostic difficulties and legal analysis of potential claims to occupational medicine specialists based on a case of unrecognizable cardiac angiosarcoma]. / Trudnosci diagnostyczne i analiza prawna potencjalnych roszczen wobec lekarza medycyny pracy na przykladzie nierozpoznanego naczyniakomiesaka serca.

The article presents a case of sudden death of a 56-year-old woman at the workplace, caused by a very rare primary cardiac tumor. The patient's family reported a crime to the prosecutor's office suggesting participation of third parties in causing the death or malpractice in physical examinations before the death. A review of clinical data concerning cardiac angiosarcoma, available in electronic databases (e.g., Web of Science, PubMed), was presented, which could be useful in the practice of occupational medicine specialists. A legal analysis of potential claims to occupational medicine specialist in the case of failure to recognize primary cardiac tumors was also included in the article. Med Pr. 2020;71(6):757-64.