Sudden death in an adolescent due to primary angiosarcoma of the right atrium

Toit-Prinsloo, Lorraine Du; Niekerk, E.; Cronjé, Christien M.; Dinkel, Jurgen Erich

doi:10.1007/s12024-016-9779-8

Cited by 2 publications

(4 citation statements)

References 14 publications

(20 reference statements)

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“…The most frequent symptoms relate to right heart failure, as the right atrium is the most common location [9,10]. Dyspnea is present in 59%-88% of patients with such a diagnosis [2,7].…”

Section: Discussionmentioning

confidence: 99%

“…Dyspnea is present in 59%-88% of patients with such a diagnosis [2,7]. It is usually multifactorial: vulnerability of the tricuspid valve due to the location in the right atrium, pericardial effusion with or without cardiac tamponade, right ventricle obstruction due to tumor's growth, pleural effusion, and lung metastasis [4,9,11,12]. Less frequently, patients may present hemopericardium, hemoptysis, anemia, pulmonary embolism, and superior vena cava syndrome [10,12].…”

Section: Discussionmentioning

confidence: 99%

“…The signs and symptoms usually begin gradually and can be absent for a long time [ 4 , 5 ]. They may be nonspecific or related to the tumor’s location, the affected area’s size, or the presence or absence of metastasis [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…In the presence of disseminated disease, surgical resection is not curative, but it may be advantageous to reduce the associated symptoms [ 5 ]. As a result, early diagnosis improves the possibility of complete surgical removal of the tumor and its prognosis [ 9 ]. Additional treatment is not established due to the rarity, but multidiscipline therapies, including radiotherapy and chemotherapy, are most commonly used [ 17 ].…”

Section: Discussionmentioning

confidence: 99%

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Primary Cardiac Angiosarcoma: A Rare and Fatal Diagnosis

Gonçalves¹,

Nunes²,

Vieira³

et al. 2021

Cureus

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Primary cardiac angiosarcoma is a rare malignant neoplasm and occurs most frequently in middle-aged males. It has an aggressive nature, with highly variable clinical features, which results in delayed diagnosis and high mortality.We report a 19-year-old man presented to the ED with a three-month history of hemoptysis and one-week history of anterior chest pain. Additionally, an aortic diastolic murmur grade II/VI was found on physical examination. Thoracic CT scan revealed bilateral dispersed hypodense pulmonary nodes with peripheral halo, alveolar densification, and pericardial effusion. The transthoracic echocardiogram confirmed sizeable pericardial effusion and bicuspid aortic valve, without other significant findings. A pericardiocentesis removed 1300 mL of hemorrhagic fluid, consistent with an exudate without malignant cells. Both cardiac magnetic resonance and transesophageal echocardiogram revealed a large mass on the right atrium's anterior wall. Mass biopsy was performed, revealing malignant cardiac angiosarcoma. The biopsy of the lung lesions was compatible with lung metastasis of primary cardiac angiosarcoma. The patient was submitted to palliative chemotherapy but died 12 months after the diagnosis.

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“…The most frequent symptoms relate to right heart failure, as the right atrium is the most common location [9,10]. Dyspnea is present in 59%-88% of patients with such a diagnosis [2,7].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Primary Cardiac Angiosarcoma: A Rare and Fatal Diagnosis

Gonçalves¹,

Nunes²,

Vieira³

et al. 2021

Cureus

View full text Add to dashboard Cite

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Clinical characteristics associated with primary cardiac angiosarcoma outcomes: a surveillance, epidemiology and end result analysis

et al. 2019

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Background Primary cardiac angiosarcoma (PCAS) is a rare type of tumour. Furthermore, descriptions of the demographic features and prognostic factors of PCAS patients have been poorly reported. Methods A population cohort study was conducted using retrospectively extracted data from the SEER (Surveillance, Epidemiology and End Results) database for patients with histological diagnoses of PCAS; the extracted information included demographic, treatment and outcome data. Results A total of 168 cases of PCAS from 1973 to 2013 were included. The mean age at diagnosis was 44.4 ± 15.5 years. PCAS was more prevalent in men than in women. The majority of PCAS patients were white (67.3%), while the incidence of PCAS in black individuals was relatively infrequent (19.0%). In addition, 87 cases were classified as distant stage, 44 as regional stage, and 33 as localized stage. The median disease-specific survival (DSS) was 7.22 months, and the 1-, 2- and 5-year DSS rate for PCAS patients was 34.7%, 14.3% and 10.2%, respectively. Further multivariate analyses showed that an age at (greater than or equal to) 45 years (HR 2.165), no radiotherapy (HR 1.629), tumour size > 5 cm (HR 3.182), and the summary stage was associated with worse PCAS-related survival. Cancer-directed surgery and radiotherapy significantly improved the DSS for patients with PCAS ( P < 0.05). The C-index of the nomograms was 0.706 (95% CI 0.654–0.758), and the calibration curves showed good agreement between the nomogram prediction and actual observation. Conclusion PCAS is a rare cancer that is prone to have poor prognoses. To understand PCAS more thoroughly, more cases with adequate information are needed.

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Sudden death in an adolescent due to primary angiosarcoma of the right atrium

Cited by 2 publications

References 14 publications

Primary Cardiac Angiosarcoma: A Rare and Fatal Diagnosis

Primary Cardiac Angiosarcoma: A Rare and Fatal Diagnosis

Clinical characteristics associated with primary cardiac angiosarcoma outcomes: a surveillance, epidemiology and end result analysis

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