The diagnosis and treatment for primary cardiac angiosarcoma with N-ras gene mutation and MSI-L: A case report and review of the literature.

RATIONALE: Primary cardiac angiosarcomas (PCA) is a rare malignancy with a poor prognosis. Currently, there is no standard treatment protocol for the PCA. We report a case of PCA in a 51-year-old woman. PATIENT CONCERNS: A 51-year-old woman initially presented with unexplained palpitations and chest tightness accompanied by nausea and vomiting, which worsened after activity and improved after rest. After symptomatic treatment, the symptoms improved, and the above symptoms recurred 8 months later. DIAGNOSES: Positron emission tomography-computed tomography revealed multiple lung nodules of varying sizes, some of which exhibited increased glucose metabolism. Furthermore, a soft tissue mass protruding into the pericardial cavity and involving the adjacent right atrium was observed in the right pericardium. The mass exhibited increased glucose metabolism, suggestive of a pericardial tumor with multiple lung metastases. Finally, histopathologic diagnosis of metastatic angiosarcoma was done by computed tomography-guided percutaneous lung and mediastinal biopsy. INTERVENTIONS: The patient was treated with palliative chemotherapy for the primary cardiac angiosarcomas and hematogenous lung metastasis. One cycle later, the result of Next-Generation Sequencing showed that the microsatellite instability status was determined to be low-level. Based on this result, tislelizumab was added to the original chemotherapy regimen. OUTCOMES: Unfortunately, the patient with PCA passed away after only 2 cycles of chemotherapy, and the cause of death remained unknown. LESSONS: This case report well demonstrates typical imaging findings of a rare cardiac angiosarcomas and emphasizes importance of early investigation for accurate diagnosis and proper management of the cardiac angiosarcomas.

Malignant pleural effusion due to angiosarcoma: A rare entity.

ABSTRACT: Angiosarcoma is a rare and aggressive vascular malignancy that typically originates in the skin or soft tissue of the body. It is known to have a propensity for metastasis to the lung parenchyma in the form of pulmonary nodules and cavitary lesions; however, a less commonly described entity is in the form of a malignant pleural effusion. Management of a malignant pleural effusion due to angiosarcoma presents a unique challenge. This article describes the challenges faced during one patient's diagnostic and treatment course, and the anticipated future complications of his aggressive disease.

Epithelioid Angiosarcoma of the Popliteal Artery- Primary or Secondary? A Rare Disease Entity Difficult to Diagnose and Easily Overlooked-Literature Research and Case report.

INTRODUCTION: The popliteal artery is a common site of aneurysm formation, whereas sarcomas of the vascular system are very rare. The diagnosis is very difficult to establish. During our literature research we found only seven reports about angiosarcomas of the popliteal artery. Four of them were associated with aneurysms. Because of the poor prognosis early diagnosis is the key to successful treatment. REPORT: We present a well-documented case of an 83-year-old patient with an angiosarcoma of the popliteal artery diagnosed as a popliteal artery aneurysm at first. CONCLUSION: It is important to think of this rare, highly aggressive tumor entity. Especially the aneurysms that need revision surgery should cause suspicion- histological samples from the aneurysm wall should always be taken.

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.

Uncommon cause of cardiac tamponade in a young man.

We report a rare case of cardiac angiosarcoma in a young boy who presented with cardiac tamponade. His initial symptoms were non-specific. He was initially being managed in the line of fungal infection, with a possibility of malignancy. Cardiac imaging was also not conclusive and he worsened on antibiotics and antifungals and succumbed to the illness. After his death tissue biopsy from heart and lung was done and histopathological examination revealed the diagnosis of metastatic angiosarcoma. The case highlights the importance of considering the diagnosis of cardiac angiosarcoma in the patients presenting with haemorrhagic pericardial effusion and non-specific symptoms.

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.

Pediatric Metastatic Cardiac Angiosarcoma Successfully Treated With Multimodal Therapy: Case Report and Review of Literature.

Cardiac angiosarcoma (AS) is an extremely rare, malignant vascular tumor with <10 cases reported in the pediatric literature. Prognosis is dismal with overall survival often <1 year from initial diagnosis. In this report, we present the case of a 10-year-old boy with metastatic cardiac AS who is currently alive and is the longest pediatric survivor of metastatic cardiac AS reported in the literature. This is the only published pediatric case to successfully use a combination of surgical resection, conventional chemotherapy, radiation and targeted therapies including bevacizumab and pazopanib for metastatic cardiac AS.

Rapidly progressive respiratory failure with multiple halo signs on computed tomography in a patient with primary cardiac angiosarcoma derived from the right atrium: a case report.

BACKGROUND: Primary cardiac neoplasms are extremely rare, with an autopsy incidence of 0.0001-0.003%. Primary cardiac sarcoma is usually derived from the right atrium and it manifests as chest pain, arrhythmia, hemoptysis, dyspnea, and fatigue. The most common target organ for metastasis of primary angiosarcoma is the lungs, but the radiological-pathological correlation has been rarely reported. CASE PRESENTATION: A 38-year-old healthy Japanese man was admitted to our hospital with persistent hemoptysis, exaggerated dyspnea, and two episodes of loss of consciousness in the past 3 months. Non-enhanced thoracic computed tomography (CT) revealed multiple scattered nodules with halo signs. Contrast-enhanced thoracic CT revealed a filling defect in the right atrium, which corresponded to the inhomogeneously enhancing tumor in the right atrium on enhanced electrocardiogram-gated cardiac CT. On day 2, acute respiratory failure occurred, and the patient was placed on mechanical ventilation. The patient was diagnosed with primary cardiac angiosarcoma based on the urgent transcatheter biopsied specimen of the right atrium mass and was treated with intravenous administration of doxorubicin. However, his respiratory status rapidly deteriorated, and he died on day 20. Postmortem biopsy showed that the multiple lung nodules with the halo signs corresponded to the intratumoral hemorrhagic necrosis and peripheral parenchymal hemorrhage in their background, suggesting the fragility of the lung tissue where the tumor had invaded, which caused hemoptysis. Furthermore, two episodes of loss of consciousness occurred probably due to a decreased cardiac output because of a massive tumor occupying the right atrium, recognized as an inhomogeneous centripetal enhancement on enhanced electrocardiogram-gated cardiac CT. CONCLUSIONS: This case clearly demonstrated that primary cardiac angiosarcoma could expand in the right atrial cavity, which led to a decreased cardiac output resulting in repeated syncope, together with the fragility of lung tissue by tumor invasion, thereby generating a halo sign on thoracic CT.

Angiosarcoma characterized by colonic polyps: A case report.

RATIONALE: Angiosarcoma is a highly invasive tumour with a low incidence rate but high rates of local recurrence and distant metastasis and a poor prognosis. Understanding the endoscopic characteristics of angiosarcoma will help with early diagnosis and treatment of this disease. PATIENT CONCERNS: The patient was a 77-year-old female who was admitted to the hospital due to recurring melena for 3 months. Outpatient gastroscopy showed that the patient had multiple gastric erosions. Colonoscopy revealed the presence of multiple protruding lesions in the colon and multiple rectal polyps. Pathological biopsy indicated that the patient had a tubular adenoma, which was removed by endoscopic resection. DIAGNOSES: Postsurgical pathologic assessment suggested that the histological subtype was epithelioid angiosarcoma. Positron emission tomography-computed tomography (PET-CT) revealed multiple metastases in the lymph nodes and bone. INTERVENTIONS: The patient underwent acid suppression to protect the stomach, fluid supplementation and red blood cell infusion, and subsequently, surgery, radiotherapy and chemotherapy were recommended. The patient's family refused further treatments for the patient and requested discharge. OUTCOMES: The patient refused further treatment and was not followed-up. LESSONS: Colorectal angiosarcoma is an extremely rare and highly malignant tumour, and understanding its endoscopic morphology will help aid in its diagnosis.

A rare cause of recurrent hemopneumothorax.

Background: Angiosarcoma is a rare and aggressive tumor of vascular endothelial origin. Pulmonary metastasis can lead to potential life-threatening complications, such as bleeding and pneumothorax.Methods: We report on a 82-year-old male with major hemoptysis, recurrent hemopneumothorax and cystic lung lesions.Results: Although initial diagnostic findings were misleading, thoracoscopic exploration revealed pulmonary and pleural metastasized angiosarcoma.Conclusion: The presence of hemoptysis or (recurrent) hemopneumothorax associated with cystic lung lesions should alert the clinician of possible pulmonary metastasized angiosarcoma.

Arteriovenous graft in kidney transplant patients: Lookout for the rare but fearsome angiosarcoma.

INTRODUCTION: Angiosarcomas are rare tumors, comprising less than 1% of all sarcomas. However, they portend a poor prognosis, as they tend to metastasize early, being of uttermost importance a prompt diagnosis and treatment. CASE DESCRIPTION: We present the case of a 55-year-old female with history of kidney transplantation, immunosuppressed with tacrolimus, prednisolone, and mofetil mycophenolate. Fifteen years after the transplant, she developed an ulcerated lesion on the site of a nonfunctioning arteriovenous graft, which was excised. Histology was compatible with a high grade angiosarcoma that invaded the margins, and immunosuppression was switched to everolimus. Staging imaging exams revealed lymph node, muscle, and lung metastases. Shortly after, nodular lesions appeared compatible with local recurrence of the disease, and the patient showed severe deterioration of her clinical condition, being proposed for palliative chemotherapy. However, the disease showed an explosive progression and the patient died before starting the treatment. CONCLUSION: This case emphasizes the importance of including inspection of the vascular access (functioning or not) in regular post-transplant consultation and value any alterations in the attempt of a timely diagnosis. Although rare, angiosarcoma is an important entity that should be considered in the differential diagnosis of soft tissue masses arising from a vascular access, especially in immunocompromised patients. Aggressive treatment should be offered whenever possible.

A radiological and clinical description of metastatic angiosarcoma simulating a hydatid cyst / Descripción clínico-radiológica de un angiosarcoma cardiaco con metástasis cerebral que simula un quiste hidatídico

Angiosarcoma is the most malignant sarcoma originating in endothelial vascular cells. It has a wide differential diagnosis due to its similarities with other entities, such as parasitic diseases. More often, angiosarcoma is diagnosed by exclusion. Neurocysticercosis and hydatid disease, or echinococcosis, are parasitic infections that may involve the central nervous system and their incidence is higher in South American countries. Diagnosis is established based on the epidemiological profile, the parasitological examination, the radiological appearance of the lesions, and the histopathology analysis of specimens. We present the case of a female adolescent with parasitosis risk factors whose neuroimages suggested cerebral hydatid cysts and who was finally diagnosed with cardiac metastatic angiosarcoma.

Los angiosarcomas son sarcomas malignos que se originan en las células endoteliales vasculares. Su diagnóstico diferencial es muy amplio debido a su parecido con otras enfermedades, como las parasitarias, y usualmente es un diagnóstico por exclusión. La neurocisticercosis y la hidatidosis cerebral son parasitosis intestinales que pueden comprometer el sistema nervioso central y tienen mayor incidencia en los países suramericanos. El diagnóstico se establece a partir del perfil epidemiológico, el estudio parasitológico, la apariencia radiológica de las lesiones y el estudio de histopatología del espécimen. Se presenta el caso de una adolescente con factores de riesgo para parasitosis y neuroimágenes sugestivas de hidatidosis cerebral, cuyo diagnóstico definitivo fue angiosarcoma cardiaco metastásico.

Electrochemotherapy for advanced cutaneous angiosarcoma: A European register-based cohort study from the International Network for Sharing Practices of electrochemotherapy (InspECT).

BACKGROUND: Cutaneous angiosarcoma (cAS) is a highly aggressive malignancy that challenges the radicality of surgical treatment. Electrochemotherapy (ECT), a skin-directed treatment based on cytotoxic chemotherapy combined with local electric pulses, may be an intraoperative adjunct and a new opportunity in the therapeutic strategy. This cohort study reports the experience with ECT as an option. METHODS: Data on patients with locally-advanced/metastatic cAS who underwent ECT between October 2013 and October 2018 at eight European centres were prospectively submitted to the InspECT (International network for sharing practices of ECT) register. Patients received therapy according to the European Standard Operating Procedures of ECT (ESOPE). Treatment feasibility was assessed based on tumour coverage with electrodes and recorded tissue current; treatment toxicity and tumour response were graded according to CTCAE v5.0 and RECIST v1.1 criteria, respectively; patient-reported outcomes (PRO) were evaluated using a visual analogue score (VAS) for pain, acceptance of retreatment and the EQ-5D questionnaire. RESULTS: We enrolled 20 patients with advanced cAS in the scalp/face (n = 7), breast/trunk (n = 10) or limbs (n = 3). Target tumours (n = 51) had a median size of 2.3 cm (range, 1-20). We administered 24 ECT courses using 1-4 cm treatment safety margin around tumours. In five patients, ECT was combined/sequenced with surgery. Median tissue current was 3 A (range, 1.5-10), tumour margins coverage rate was 75% (15/20 patients). The objective response rate (ORR) was 80% (complete, 40%). Grade-3 toxicity included skin ulceration (15%) and pain (10%), with no significant change of PRO scores. Bleeding control was achieved in 13/14 patients with ulcerated tumours. With a median overall survival of 12.5 months, the local progression-free survival (LPFS) was 10.9 months. CONCLUSION: ECT produces sustained response rate with minimal side effects and should be considered an option for advanced cAS. Palliative benefits include patient tolerability, local haemostasis and durable local control. Definition of optimal timing, treatment safety margins and combination with surgery need further investigation.

Descripción clínico-radiológica de un angiosarcoma cardiaco con metástasis cerebral que simula un quiste hidatídico / A radiological and clinical description of metastatic angiosarcoma simulating a hydatid cyst

RESUMEN Los angiosarcomas son sarcomas malignos que se originan en las células endoteliales vasculares. Su diagnóstico diferencial es muy amplio debido a su parecido con otras enfermedades, como las parasitarias, y usualmente es un diagnóstico por exclusión. La neurocisticercosis y la hidatidosis cerebral son parasitosis intestinales que pueden comprometer el sistema nervioso central y tienen mayor incidencia en los países suramericanos. El diagnóstico se establece a partir del perfil epidemiológico, el estudio parasitológico, la apariencia radiológica de las lesiones y el estudio de histopatología del espécimen. Se presenta el caso de una adolescente con factores de riesgo para parasitosis y neuroimágenes sugestivas de hidatidosis cerebral, cuyo diagnóstico definitivo fue angiosarcoma cardiaco metastásico.

ABSTRACT Angiosarcoma is the most malignant sarcoma originating in endothelial vascular cells. It has a wide differential diagnosis due to its similarities with other entities, such as parasitic diseases. More often, angiosarcoma is diagnosed by exclusion. Neurocysticercosis and hydatid disease, or echinococcosis, are parasitic infections that may involve the central nervous system and their incidence is higher in South American countries. Diagnosis is established based on the epidemiological profile, the parasitological examination, the radiological appearance of the lesions, and the histopathology analysis of specimens. We present the case of a female adolescent with parasitosis risk factors whose neuroimages suggested cerebral hydatid cysts and who was finally diagnosed with cardiac metastatic angiosarcoma.