Clinical and imaging characteristics of patients with primary cardiac angiosarcoma / 中华心血管病杂志

Objective: To investigate the clinical, cardiac imaging characteristics and prognosis of patients with primary cardiac angiosarcoma. Methods: The clinical data of 14 patients hospitalized with primary cardiac angiosarcoma from January 2001 to December 2017 in Peking Union Medical College Hospital were collected and analyzed. Metastatic cardiac angiosarcoma was not included in this study. Patients were followed up post discharge per telephone call or clinical visit. Results: Of the 14 patients, 8 were males and 6 were females, average age was 48 years. The main clinical symptoms were shortness of breath (8/14), hemoptysis (6/14), fever (5/14), chest pain (4/14) and cough (3/14). Imaging examinations showed that the tumors of 8 patients were located in the right heart and 6 in the pericardial cavity. Tumors in the right heart often infiltrate the atrial wall and cause pericardial effusion (7/8). Tumors in the pericardium were characterized by recurrent bloody pericardial effusion (6/6), prone to progressive constrictive pericarditis (3/6), pericardial fluid cytology was often negative (6/6). MRI showed heterogeneous high signal intensity (cauliflower aspect) on T2-weighted image and heterogeneous enhancement with a"sunray" aspect at the perfusion study. At the time of diagnosis, 8 patients developed lung or adrenal metastasis (8/14). The median survival was only 305 days. Conclusions: Primary cardiac angiosarcoma is a rare disease with non-specific clinical manifestation and poor prognosis. Imaging examinations may help diagnosis. The high invasiveness and the easy-to-metastasis feature of the tumor contribute to the poor prognosis of cardiac angiosarcoma.

Intra-Axial Metastatic Angiosarcoma of the Central Nervous System Associated with Anemia, Pulmonary Tuberculosis and Short Survival

Introduction Angiosarcoma (AG) is a malignant mesenchymal neoplasm that predominantly affects the soft tissues and, to variable degrees, expresses themorphological and functional characteristics of the endothelium. The incidence of sarcomas of the central nervous system(CNS) is low (0.5% to 2.7%), and AGs involving the brain are even rarer. Case Description A 45-year-old male patient presented with complaints of headache, nausea, and vomiting. An examination showed bilateral papilledema and a right lung pleurotomy. The patient's previous history included drug addiction, pulmonary tuberculosis, lung abscess, pleural empyema, and pulmonary artery embolization for severe hemoptysis. Computed tomography/magnetic resonance imaging scans revealed a large intra-axial lesion extending into the right parietal and temporal lobes, with hemorrhagic zones. The patient underwent surgical resection of the lesion. Microscopy showed a poorly-differentiated, high-grade malignant tumor composed of plump/epithelioid cells forming small vascular spaces and solid nests, compatible with AG.In the postoperative period, the patient developed recurrent hemoptysis. A biopsy of the tissues adjacent to the pleurotomy determined the diagnosis of pulmonary AG. At 30 days after the resection, the patient died from hemoptysis, hemothorax, lung atelectasis, and intracranial hypertension related to the recurrence of the brain tumor. Conclusion Angiosarcoma is a rare neoplasia related to short survival due to the high proliferative index, which must be considered in patients presenting hemorrhagic tumors. No specific genetic abnormalities have been described for this neoplasia.

Radiation-induced angiosarcoma: case report / Angiossarcoma induzido por radiação: relato de caso

ABSTRACT Angiosarcoma of the breast accounts for less than 1% of breast tumors. This tumor may be primary or secondary to previous radiation therapy and it is also named "radiogenic angiosarcoma of the breast", which is still a rare entity with a poor prognosis. So far, there are only 307 cases reported about these tumors in the literature. We present a case of a 73-year-old woman with a prior history of breast-conserving treatment of right breast cancer, exhibiting mild pinkish skin changes in the ipsilateral breast. Her mammography was consistent with benign alterations (BI-RADS 2). On incisional biopsy specimens, hematoxylin-eosin showed atypical vascular lesion and suggested immunohistochemisty for diagnostic elucidation. Resection of the lesions was performed and histology showed radiogenic angiosarcoma. The patient underwent simple mastectomy. Immunohistochemistry was positive for antigens related to CD31 and CD34, and C-MYC oncogene amplification, confirming the diagnosis of angiosarcoma induced by breast irradiation. A delayed diagnosis is an important concern. Initial skin changes in radiogenic angiosarcoma are subtle, therefore, these alterations may be confused with other benign skin conditions such as telangiectasia. We highlight this case clinical aspects with the intention of alerting to the possibility of angiosarcoma of the breast in patients with a previous history of adjuvant radiation therapy for breast cancer treatment. Sixteen months after the surgery the patient remains asymptomatic.

RESUMO Os angiossarcomas de mama representam menos de 1% dos tumores da mama e podem ser primários ou secundários à radioterapia prévia. Tais tumores são chamados de "angiossarcomas radiogênicos da mama" e representam uma entidade mais rara ainda e de prognóstico ruim. Atualmente, na literatura, são encontrados apenas 307 casos desses tumores. Relatamos o caso de uma mulher de 73 anos, com história prévia de tratamento conservador de câncer de mama direita, apresentando alteração rósea discreta em pele da mama homolateral. A mamografia demostrou resultado compatível com alterações benignas (BI-RADS 2). No material de biópsia incisional, a hematoxilina-eosina demonstrou lesão vascular atípica e sugeriu imuno-histoquímica para elucidação diagnóstica. A paciente foi submetida à ressecção de lesões, e a histologia demonstrou angiossarcoma radiogênico. Em seguida, mastectomia simples foi realizada. A imuno-histoquímica demonstrou positividade para os antígenos relacionados a CD31 e CD34, e a amplificação do oncogene C-MYC confirmou o diagnóstico de angiossarcoma induzido por radiação mamária. O atraso no diagnóstico constitui questão importante. Considerando que as alterações iniciais da pele do angiossarcoma radiogênico são sutis e podem ser confundidas com outras condições benignas da pele, como telangiectasias, neste relato destacamos os aspectos clínicos, no intuito de alertar sobre a possibilidade de angiossarcoma de mama em pacientes com história prévia de radioterapia adjuvante para tratamento de câncer de mama. A paciente permaneceu assintomática 16 meses após a cirurgia.

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.

Primary renal angiosarcoma with extensive hemorrhage: CT and MRI findings

ABSTRACT Primary angiosarcomas of the kidney are very rare, but highly aggressive tumors showing poor prognosis. Patients frequently complain of flank pain, hematuria, or a palpable mass. We present a case of primary renal angiosarcoma occurring in a 61-year-old man. CT images depicted a huge exophytic mass (16 cm in diameter) in the right kidney, exhibiting extensive hemorrhage. The mass showed centripetal peripheral nodular enhancement on dynamic contrast-enhanced images. Furthermore, MR imaging revealed a tangled mesh of tumor vessels in the periphery of the mass. We suggest its inclusion in the differential diagnosis of cases of hemorrhagic renal tumors with prominent vasculature.

Primary cardiac angiosarcoma with rare presenting feature and successful surgical treatment

Abstract Primary angiosarcoma is a rare clinical entity, it's typically located within the right atrium and known to be rapidly fatal. A 37-year-old female was presented with a history of recurrent facial paralysis and left hemiparesis. A cranial mass was identified at cranial magnetic resonance imaging and she underwent neurosurgery operation. The immunohistochemical examination was determined as metastatic cardiac angiosarcoma. The tumor, as well as part of the right pericardium, were resected. A piece of bovine pericardium was used to reconstruct the right atrial wall. Tricuspid valve was reconstructed with ring annuloplasty. Due to resection of right coronary artery with the tumor, coronary bypass surgery was performed successfully. The patient is currently healthful without any recurrence and complaint 12 months after the diagnosis as followed up.

A rare cause of abdominal pain in childhood: cardiac angiosarcoma

Abstract Cardiac angiosarcomas are extremely rare in childhood, they are rapidly progressive tumours that often present themselves as diagnostic dilemmas, resulting in delayed diagnosis. Also, extracardiac manifestations, including abdominal pain, are extremely rare in patients with intracardiac tumors. We herein present the case of a 15-year-old girl who presented with abdominal pain. Echocardiography and thoracic computed tomography showed right atrial mass. The patient underwent surgery, chemotherapy, and radiotherapy. Eight months after treatment, abdominal recurrence was detected. The abdominal mass was resected, and radiotherapy and new chemotherapy protocol were given. The present case illustrates a rare case of primary cardiac angiosarcoma posing a diagnostic dilemma in an adolescent girl.

Intra-cardiac angiosarcoma.

Intra-cardiac angiosarcoma is a challenging disease – difficult to diagnose early and an aggressive tumour to treat. Transoesophageal echocardiography (TEE) and cardiac computed tomography are the two most sensitive diagnostic tools. Though there are various multimodality treatment options available, the results are not impressive. We present a case of intra-cardiac angiosarcoma in a 62-year-old gentleman.