¿Es útil el trasplante cardíaco como tratamiento del sarcoma cardíaco primario?

Mazuecos, Jesús M. Jiménez; Manso, Raquel Fuentes; Cubero, Javier Segovia; Ramos, Jorge Toquero; Domínguez, Juan Francisco Oteo; Rivera, Luís E.

doi:10.1016/s0300-8932(03)76886-9

Cited by 22 publications

(26 citation statements)

References 12 publications

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“…Despite the high incidence of unresectable cases and other risk factors, similar to sarcomas at other sites, wide surgical resection remains the cornerstone for treatment of cardiac sarcomas with variable outcomes in the literature [10,34]. Heart transplantation has recently been used to treat cardiac sarcoma, but its role is still controversial and has not been widely accepted as a main surgical mean for treating cardiac sarcoma [12,35,36]. In our series, 3 patients were treated with heart transplant, but only 1 was primarily for resecting the tumor and the other 2 were for ischemic heart disease either related or unrelated to the tumor.…”

Section: Discussionmentioning

confidence: 99%

Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival

Zhang¹,

Brooks²,

Goldblum³

et al. 2008

Human Pathology

114

100

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Summary Although cardiac sarcomas are rare in comparison to their soft tissue counterparts, they are the second most common type of primary cardiac neoplasm. Of the few hundred cases reported, most has been based on autopsy series. A series of 27 cardiac sarcomas removed at surgery for curative and diagnostic intent were reviewed for clinicopathologic features with correlation to available postoperative follow-up data in 17 patients. There were 6 angiosarcomas, 6 myxofibrosarcomas, 3 malignant peripheral nerve sheath tumors, 3 leiomyosarcomas, 2 synovial sarcomas, 1 epithelioid hemangioendothelioma, 1 chondrosarcoma, 1 osteosarcoma, and 4 poorly differentiated sarcomas. There was a wide age and size range with slight female predilection. There were 20 cases that arose in the atria/pulmonary vessels, 4 in the ventricles, 1 in mitral valve, and 2 in epi/pericardium. There was a slight left predilection. The histologic grade was low in 4, moderate in 3, and high in 20 cases. Six high-grade and 1 low-grade tumors were also treated with adjuvant chemotherapy and/or radiation. In 17 patients with follow-up data, 6 of 12 patients with high-grade tumor died (4 within 5 days of the initial surgery, 1 in 21 months, and 1 in 131 months), and 1 patient with moderate-grade tumor and all 4 patients with low-grade tumor were alive without evidence of disease at the end of follow-up. Tumor grade appeared to be prognostically important in cardiac sarcoma. Long survival was achieved in patients who survived the initial surgery well.

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Section: Discussionmentioning

confidence: 99%

Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival

Zhang¹,

Brooks²,

Goldblum³

et al. 2008

Human Pathology

114

100

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“…Additionally, metastases have been reported in liver, lymph nodes, bone, adrenal glands, and to a lesser extent the central nervous system (CNS) and spleen [4, 9]. Due to the late stage at presentation, prognosis for patients with primary angiosarcoma of the heart is poor; median overall survival varies from 6 months or less for untreated patients to 12 months for patients with incomplete tumor resection [6, 10–12]. Tumor often invades into the adjacent tissues, making complete resection challenging, if not impossible.…”

Section: Introductionmentioning

confidence: 99%

Case report: whole exome sequencing of primary cardiac angiosarcoma highlights potential for targeted therapies

et al. 2017

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BackgroundPrimary cardiac angiosarcomas are rare, but they are the most aggressive type of primary cardiac neoplasms. When patients do present, it is with advanced pulmonary and/or cardiac symptoms. Therefore, many times the correct diagnosis is not made at the time of initial presentation. These patients have metastatic disease and the vast majority of these patients die within a few months after diagnosis. Currently the treatment choices are limited and there are no targeted therapies available.Case presentationA 56-year-old male presented with shortness of breath, night sweats, and productive cough for a month. Workup revealed pericardial effusion and multiple bilateral pulmonary nodules suspicious for metastatic disease. Transthoracic echocardiogram showed a large pericardial effusion and a large mass in the base of the right atrium. Results of biopsy of bilateral lung nodules established a diagnosis of primary cardiac angiosarcoma. Aggressive pulmonary disease caused rapid deterioration; the patient went on hospice and subsequently died. Whole exome sequencing of the patient’s postmortem tumor revealed a novel KDR (G681R) mutation, and focal high-level amplification at chromosome 1q encompassing MDM4, a negative regulator of TP53.ConclusionMutations in KDR have been reported previously in angiosarcomas. Previous studies also demonstrated that KDR mutants with constitutive KDR activation could be inhibited with specific KDR inhibitors in vitro. Thus, patients harboring activating KDR mutations could be candidates for treatment with KDR-specific inhibitors.Electronic supplementary materialThe online version of this article (doi:10.1186/s12885-016-3000-z) contains supplementary material, which is available to authorized users.

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“…According to previous reports [ 5 – 27 ], HTx for PCS produced a broad range of survivals from only several months to almost a decade. Some reports denied the benefit of HTx [ 12 , 23 ], whereas a few reports supported it [ 8 , 16 ]. Actually, PCS are a heterogenous group of soft tissue sarcomas with a wide spectrum of clinical behaviors.…”

Section: Introductionmentioning

confidence: 99%