Osteosarcoma with Cardiac Metastasis in a 22-year-old Man: A Case Report and Review of Cardiac Tumors

Pinder, Marco; Charaf-Eddine, Ahmad; Parnell, Aimee S.; DiBardino, Daniel J.; Knudson, Jarrod D.

doi:10.1111/chd.12113

Cited by 3 publications

(1 citation statement)

References 28 publications

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“…1 The estimated incidence of metastasis to the heart is 10% to 15.4%; lung tumors, lymphomas, and carcinomas of the breast are the typical metastatic sources. 2,3 Ewing sarcoma is the second most frequent primary malignant bone tumor (after osteosarcoma) but accounts for only a small proportion of cardiac metastases. [4][5][6] Its metastatic incidence in the right ventricle (RV) is not precisely known.…”

Section: Ewing Sarcoma In the Right Ventriclementioning

confidence: 99%

Ewing Sarcoma in the Right Ventricle

Petrović

Zhao

Thangam

et al. 2016

Texas Heart Institute Journal

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Ewing sarcoma is the second most prevalent malignant primary bone tumor but constitutes only a small proportion of cardiac metastases. We present a case of asymptomatic Ewing sarcoma metastatic to the right ventricle.A 1 Approximately 25% of primary cardiac tumors are malignant.1 Metastases to the heart are 20 to 40 times more prevalent than are primary cardiac malignancies.1 The estimated incidence of metastasis to the heart is 10% to 15.4%; lung tumors, lymphomas, and carcinomas of the breast are the typical metastatic sources. 2,3Ewing sarcoma is the second most frequent primary malignant bone tumor (after osteosarcoma) but accounts for only a small proportion of cardiac metastases. 4-6 Its metastatic incidence in the right ventricle (RV) is not precisely known. We present the case of a patient who had asymptomatic Ewing sarcoma metastatic to the RV. Case ReportIn December 2013, a 36-year-old asymptomatic man presented for evaluation of a mass in his RV. Three years earlier, because of translocation-negative Ewing sarcoma, he had undergone below-the-knee amputation of the left leg and consequent chemotherapy. Two years later, he was found to have a lung metastasis. Genetic analysis of the resected lung tissue identified mechanistic target of rapamycin (mTOR) E1799K, Ewing sarcoma breakpoint region 1 (EWSR1) gene fusion with nuclear factor of activated Tcell cytoplasmic 2 gene (NFATc2), and topoisomerase 1 (TOP1) amplification lesions. These were consistent with Ewing sarcoma and prompted further chemotherapy.Three months after the lung resection, magnetic resonance images (MRI) of the lungs revealed a 3.5 × 2.6-cm enhancing mass in the RV (Fig. 1), and the patient presented at our institution for possible resection of the mass. He was exceptionally physically fit and had no history of cardiac diseases. Results of positron emission tomography (PET) confirmed the presence of a metabolically active tumor (Fig. 2). The patient underwent cardiac evaluation and was cleared for surgery.An intraoperative transesophageal echocardiogram showed a preserved left ventricular (LV) ejection fraction. Other than a pedunculated mass attached to the RV, there were no structural abnormalities of the heart.Under direct vision, with a normothermic beating heart, the tumor was resected with negative margins and no residual mass. Right atrial biopsies were also performed (with 2-mm negative margins on a frozen section). The total cardiopulmonary bypass time was 22 min. Postoperative transthoracic echocardiograms (TTEs) showed normal LV and RV size and function.The morphology and immunohistochemical stains of the resected mass were consistent with a metastatic Ewing sarcoma tumor deposit. It was composed of highly malignant cells with moderate nuclear pleomorphism, a high nuclear-to-cytoplasmic ratio, frequent mitoses (16 per 10 high-power fields), and clear-to-finely vacuolated

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Section: Ewing Sarcoma In the Right Ventriclementioning

confidence: 99%

Ewing Sarcoma in the Right Ventricle

Petrović

Zhao

Thangam

et al. 2016

Texas Heart Institute Journal

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A Rare Complication of Metastatic Osteosarcoma

Pagel

Davidovich

Steck

et al. 2017

Journal of Cardiothoracic and Vascular Anesthesia

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Osteosarcoma Metastases With Direct Cardiac Invasion: A Case Report and Review of the Pediatric Literature

Hartemayer

Kuo²,

Kent³

2017

Journal of Pediatric Hematology/Oncology

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Metastatic osteosarcoma with direct cardiac involvement is an exceptionally rare finding, with only 63 total reported cases in the English literature over the past 123 years. Although the precise incidence is unknown, we estimate that direct cardiac involvement currently occurs in <2% of metastatic osteosarcoma cases. We also find that before the adoption of adjuvant chemotherapy as a standard of care therapy for osteosarcoma, metastatic osteosarcoma to the heart was much more common than it is today, as cardiac involvement occurred in ∼20% of cases of metastatic osteosarcoma before the 1980s. This suggests that adjuvant chemotherapy has not only improved the overall prognosis of osteosarcoma, but also altered the metastatic pattern of disease. In this paper we present the case of an 11-year-old boy with metastatic osteosarcoma to the cardiac interventricular septum, as well as review 20 other previously reported pediatric cases of metastatic osteosarcoma to the heart. We also analyzed the cardiac surgical outcomes for 11 pediatric patients with metastatic osteosarcoma to the heart. The median disease-free survival time was 12 months, demonstrating that metastatic osteosarcoma to the heart is currently a rare occurrence with a poor prognosis.

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Osteosarcoma with Cardiac Metastasis in a 22-year-old Man: A Case Report and Review of Cardiac Tumors

Cited by 3 publications

References 28 publications

Ewing Sarcoma in the Right Ventricle

Ewing Sarcoma in the Right Ventricle

A Rare Complication of Metastatic Osteosarcoma

Osteosarcoma Metastases With Direct Cardiac Invasion: A Case Report and Review of the Pediatric Literature

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