Primary cardiac sarcoma presenting as acute left-sided heart failure

Pathak, Ranjan; Nepal, Santosh; Giri, Smith; Ghimire, Sushil; Aryal, Madan Raj

doi:10.3402/jchimp.v4.23057

Cited by 7 publications

(6 citation statements)

References 24 publications

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“…The clinical presentation depends on tumor size and anatomical location rather than on histological type. It varies from symptoms of congestive heart failure to thromboembolism and arrhythmias[2].…”

Section: Introductionmentioning

confidence: 99%

Successful multidisciplinary clinical approach and molecular characterization by whole transcriptome sequencing of a cardiac myxofibrosarcoma: A case report

Saponara

Indio

Pizzi

et al. 2019

WJCC

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BACKGROUNDCardiac tumors are rare and complex entities. Surgery represents the cornerstone of therapy, while the role of adjuvant treatment remains unclear and, in case of relapse or metastatic disease, the prognosis is very poor. Lack of prospective, randomized clinical trials hinders the generation of high level evidence for the optimal diagnostic workup and multimodal treatment of cardiac sarcomas. Herein, we describe the multidisciplinary clinical management and molecular characterization of a rare case of cardiac myxofibrosarcoma in an elderly woman.CASE SUMMARYA 73-year-old woman presented signs and symptoms of acute left-sided heart failure. Imaging examination revealed a large, left atrial mass. With suspicion of a myxoma, she underwent surgery, and symptoms were promptly relieved. Histology showed a cardiac myxofibrosarcoma, a rare histotype of cardiac sarcoma. Eight months later, disease unfortunately relapsed, and after a multidisciplinary discussion, a chemotherapy with doxorubicin and then gemcitabine was started, achieving partial radiologic and complete metabolic response, which was maintained up to 2 years and is still present. This report is focused on the entire clinical path of our patient from diagnosis to follow-up, through surgery and strategies adopted at relapse. Moreover, due to their rarity, very little is known about the molecular landscape of myxofibrosarcomas. Thus, we also performed and described preliminary genome analysis of the tumor tissue to get further insight on mechanisms involved in tumor growth, and to possibly unveil new clinically actionable targets.CONCLUSIONWe report a case of cardiac myxofibrosarcoma that achieved a very good prognosis due to an integrated surgical, cardiac and oncologic treatment strategy.

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Section: Introductionmentioning

confidence: 99%

Successful multidisciplinary clinical approach and molecular characterization by whole transcriptome sequencing of a cardiac myxofibrosarcoma: A case report

Saponara

Indio

Pizzi

et al. 2019

WJCC

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“…Complete resection of the tumor is ideal. Life expectancy is nearly twice as long in patients with complete tumor resection compared to incomplete excision [9]. Although surgery is still the first-line treatment for MFS, other treatment modalities such as adjuvant chemotherapy, radiotherapy, or combination of both are also helpful.…”

Section: Discussionmentioning

confidence: 99%

Primary cardiac myxofibrosarcoma with osteoid differentiation mimicking a left atrial myxoma: A rare entity

Reddy

Kumar

Sanzgiri

et al. 2020

Journal of Cardiology Cases

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Primary cardiac tumors are rare and their incidence varies from 0.3% to 0.7% [1]. They are usually benign and only 25% are malignant. Primary cardiac sarcomas are even rarer with an incidence of 0.0001% in various autopsy series [2]. Angiosarcomas are the commonest accounting for about 33%-40%. Myxofibrosarcoma (MFS), pleomorphic sarcoma, and osteosarcoma are exceptionally rare primary cardiac tumors.MFS is an aggressive soft tissue neoplasm. It is often asymptomatic with extensive local invasion or distant metastases at detection. Although, a total of 31 cases have been reported, one with bone/osteoid differentiation has not been described [3]. To the best of our knowledge this is one of few reported cases of high grade primary cardiac MFS with areas of woven bone or osteoid matrix formation in the medical literature. Here, we share our case experience and problems encountered during diagnosis and management of this extremely rare histological subtype of MFS. Case reportA 62-year-old woman presented with the complaint of dyspnea on exertion (New York Heart Association class II) for the previous month. No significant past history was elicited. On examination, pulse was regular and blood pressure was 130/80 mmHg. On auscultation of chest, S1 was soft and S2 was normally split. There was an early diastolic plop sound after the S2 with a low pitched, mid-diastolic rumbling murmur (Grade II) over apex (mimicking mitral stenosis) without presystolic accentuation. The intensity of murmur changed in relation to patient's body position.Electrocardiogram (ECG) was normal. X-ray of chest showed cardiomegaly. N-terminal (NT)-proB-type natriuretic peptide (NT-proBNP) level was 120 pg/mL. Two-dimensional echocardiography (2D-ECHO) and transesophageal echocardiography (TEE) showed a large mass measuring 4.8 Â 3.5 cm almost filling the entire left atrium (LA). This mass was lobulated, sessile, having heterogenous echogenicity with areas of dense calcification and cystic degener-

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“…The most important factor dictating prognosis is the ability to achieve a complete surgical resection of the tumor. It has been observed that the median survival increases to 27 months with complete resection compared to 10 months where complete resection is not possible [ 4 ]. Surgery not only alleviates symptoms but also confirms diagnosis and avoids future embolic and hemodynamic complications [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

Malignant Perivascular Epithelioid Cardiac Sarcomas: A Case Report and a Review of the Literature

Baldeo

Hritani

Ali

et al. 2015

Case Reports in Cardiology

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Cardiac tumors, either benign or malignant, are difficult to diagnose due to their rarity, variety, and nonspecific presentation. Since primary cardiac sarcoma remains an unusual diagnosis, the literature on its presentation, diagnosis, and optimal management remains scarce. To our knowledge the following case of cardiac perivascular epithelioid cell tumor is the fourth reported case found in the literature. Although complete surgical resection remains the gold standard for cardiac sarcomas, our case demonstrates that not all of them can be completely resected.

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Primary cardiac sarcoma presenting as acute left-sided heart failure

Cited by 7 publications

References 24 publications

Successful multidisciplinary clinical approach and molecular characterization by whole transcriptome sequencing of a cardiac myxofibrosarcoma: A case report

Successful multidisciplinary clinical approach and molecular characterization by whole transcriptome sequencing of a cardiac myxofibrosarcoma: A case report

Primary cardiac myxofibrosarcoma with osteoid differentiation mimicking a left atrial myxoma: A rare entity

Malignant Perivascular Epithelioid Cardiac Sarcomas: A Case Report and a Review of the Literature

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