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      Angiosarcoma cardíaco: una causa rara de insuficiencia cardíaca Translated title: Cardiac angiosarcoma: a rare cause of heart failure

      case-report

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          Abstract

          RESUMEN Se presenta el caso de un hombre, de 76 años, con un angiosarcoma cardíaco que debutó con insuficiencia cardíaca y taponamiento pericárdico. Se trata de un tumor muy raro, esto hace que el diagnóstico sea difícil y en ocasiones tardío. Es más frecuente en hombres de mediana edad y suele debutar con clínica de insuficiencia cardíaca. Se debe diagnosticar con pruebas de imagen y el tratamiento de elección es el quirúrgico, siempre que sea posible, aunque las recidivas locales y las metástasis son frecuentes, lo que lo convierte en un tipo de tumor con mal pronóstico.

          Translated abstract

          ABSTRACT We report the case of a 76-year-old man with a cardiac angiosarcoma that commenced with heart failure and pericardial tamponade. This is an extremely rare tumour and often overlooked as an initial diagnosis. It is more common in middle-aged men and usually presents with clinical symptoms of heart failure. It must be diagnosed with imaging tests and the treatment of choice is surgery whenever possible, although local recurrence and metastases are common. This makes it a tumour with a poor prognosis.

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          Most cited references7

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          Primary cardiac angiosarcoma – a review

          Primary cardiac neoplasms are extremely rare. Angiosarcoma is the most commonly seen histological subtype and is characterized by its permeating and destructive nature. Unfortunately, primary cardiac angiosarcoma is often overlooked as an initial diagnosis because of its rarity. Since the time it was first identified in 1934, little progress has been made in improving survival outcome. Complete or partial surgical resection is still the best option for palliation, with little hope for cure. Improvements have been made in the ability to view and distinguish tumors. Echocardiography is one of the most useful diagnostic tools because of its high sensitivity; therefore, CT and MR images are often used to detect sites of metastatic disease. Immunohistochemistry staining can also be employed as an adjunctive diagnostic tool. CD31, CD34, FLI-1, and von Willebrand factor are the most commonly used markers in detecting tumors of endothelial origin. However, due to the vast heterogeneity within a tumor, immunohistochemistry staining can be quite variable. Surgical resection remains the standard modality of treatment. Primary cardiac angiosarcoma is largely resistant to chemotherapy and/or radiation. However, the exact benefit and its place in a multimodality treatment regimen are still under investigation.
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            Cardiac tumors--diagnosis and surgical treatment.

            Primary tumors of the heart are rare even in major cardiac surgery centers. Because of the low case numbers, there is an insufficient evidence base to determine the optimal treatment, particularly for malignant tumors.
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              Primary sarcoma of the heart: case report and literature review

              Background Primary cardiac tumors are extremely rare. Most primary cardiac tumors are benign and around one quarter is malign. Sarcomas are accounting for 95% of these malign tumors and they show different histologies. The prognosis is poor with a mean survival of 3 months to 1 year, even with complete radical resection. We report the cases of two patients with primary cardiac sarcoma treated with surgery and radiation and/or chemotherapy. In addition we retrospectively collected data of patients with primary cardiac sarcoma treated between 2005 and 2019 with minimum follow-up of 12 months. Clinical characteristics, treatment modalities and outcomes were collected and analyzed. Finally a literature review was done. Case presentation The first patient presented with cerebellar infarction. When she developed a recurrence analysis showed a suspicious myocardial lesion for which irradical surgery (R2) was performed. Histopathology showed an intimal sarcoma of the left atrium. Postoperative radiotherapy was applied without complications. Three months after treatment multiple metastases were diagnosed and she died 13 months after initial diagnosis. The second patient presented with pericardial effusion. A tumor was found located in the right atrium and radical surgery was performed. Histopathology showed an angiosarcoma, without signs of metastases. Adjuvant radiotherapy was added because of close margins and based on high risk of recurrence and metastases it was decided to add chemotherapy. One year after finishing treatment, evaluation showed local recurrence together with pulmonary metastases. Conclusions Surgery combined with postoperative radiotherapy is feasible in patients with resectable cardiac sarcoma. Distant metastases occur frequently. In patients with an irresectable sarcoma of the heart primary radiotherapy should be considered.
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                Author and article information

                Journal
                albacete
                Revista Clínica de Medicina de Familia
                Rev Clin Med Fam
                Sociedad Española de Medicina de Familia y Comunitaria (Barcelona, Cataluña, Spain )
                1699-695X
                2386-8201
                2022
                : 15
                : 2
                : 119-121
                Affiliations
                [2] Madrid orgnameCS de Manzanares El Real orgdiv1Consultorio de El Boalo España
                [3] Madrid orgnameHospital Universitario de la Paz España
                [1] Madrid orgnameHospital Universitario de la Princesa España
                Article
                S1699-695X2022000200119 S1699-695X(22)01500200119
                5af68422-8672-414b-92a9-52144245c8d0

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 21 June 2021
                : 26 May 2021
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 7, Pages: 3
                Product

                SciELO Spain

                Categories
                Un paciente con...

                cardiomegalia,heart failure,cardiomegaly,cardiac tamponade,taponamiento cardíaco,insuficiencia cardíaca,angiosarcoma,hemangiosarcoma

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