High-Grade Left Atrial Pleomorphic Sarcoma: Case Report and Review

Laguado, Martin Ignacio Zapata; Rojas, Jonathan Orlando Palacios; Morales, Martha Lucía Velasco; Hoz, Carlos Eduardo Orozco de la

doi:10.1159/000485561

Cited by 2 publications

(1 citation statement)

References 19 publications

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“…:78-year-old woman with the UPS in left atrium underwent radical surgical resection and refused adjuvant chemotherapy. She survived 3 years and then presented with advanced metastatic disease and decompensated heart failure and died [14].…”

Section: Discussionmentioning

confidence: 99%

Multidisciplinary approach to rare primary cardiac sarcoma: a case report and review

et al. 2019

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Background Undifferentiated pleomorphic sarcoma is a very rare and aggressive type of primary cardiac tumors. Most cardiac sarcomas result in rapid growth and quick death. According to different sources the median survival is typically 6 to 12 months. We are presenting a case of primary cardiac sarcoma with 26 months disease free survival following cytoreductive surgery and chemotherapy. Case presentation A 48-year-old woman with progressing symptoms of dyspnea and palpitations for over 2 months was referred to a cardiologist. With the help of echocardiography and cardiovascular magnetic resonance cardiac sarcoma was suspected. Open biopsy and cytoreductive surgery were performed, complete resection of the tumor was not possible. Histology revealed undifferentiated pleomorphic sarcoma. Seven cycles of chemotherapy with Doxorubicine and Ifosfamide were completed. Cardiovascular magnetic resonance revealed a complete response – only signs of fibrosis without any signs of tumor were visible. Follow ups with echocardiography, cardiovascular magnetic resonance and chest, abdomen and pelvic computed tomography is performed every 3 months. Twenty-six months from initial diagnosis the patient is still free of recurrence of tumor with no compromises of the quality of life. Conclusion Standard chemotherapy together with cytoreductive surgery can have a complete response effect in undifferentiated pleomorphic sarcoma with unusual long-term survival.

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Section: Discussionmentioning

confidence: 99%

Multidisciplinary approach to rare primary cardiac sarcoma: a case report and review

et al. 2019

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The Heart of the Matter: A Unique Convergence of Cardiac Neoplasm, Hereditary Nonpolyposis Colorectal Cancer, and Spindle Cell Sarcoma

Bryer

Hartner

2019

EMJ

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Primary cardiac tumours are exceedingly unusual and aggressive; they often develop in younger patients and present with advanced disease. The rarity and heterogeneity of primary cardiac tumours challenge the standardisation of therapeutic guidelines. Undifferentiated primary cardiac spindle cell sarcomas, a distinct subset of primary cardiac sarcomas, are especially unique with <20 cases reported worldwide, the majority of which are of left atrial origin. This article presents a review of the aetiology, pathophysiology, and therapy of undifferentiated primary cardiac spindle cell sarcomas. In conjunction, the authors present a unique case of a woman with hereditary nonpolyposis colorectal cancer (Lynch syndrome) who presented with a primary cardiac spindle cell sarcoma of left ventricular origin; this is the first case of this type and location of cardiac tumour reported in a patient with Lynch syndrome.

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High-Grade Left Atrial Pleomorphic Sarcoma: Case Report and Review

Cited by 2 publications

References 19 publications

Multidisciplinary approach to rare primary cardiac sarcoma: a case report and review

Multidisciplinary approach to rare primary cardiac sarcoma: a case report and review

The Heart of the Matter: A Unique Convergence of Cardiac Neoplasm, Hereditary Nonpolyposis Colorectal Cancer, and Spindle Cell Sarcoma

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