Cardiac Sarcoma 14 Years after Treatment for Pleural Mesothelioma
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《新英格兰医药杂志》
To the Editor: The addition of chemoradiation therapy to cytoreductive surgery for malignant pleural mesothelioma can improve survival.1 Postradiation sarcoma has been reported in patients with breast, cervical, and head and neck cancers.2 Here, we describe a 56-year-old woman in whom unclassified spindle-cell sarcoma was diagnosed 14 years after pleurectomy and chemoradiation therapy for malignant pleural mesothelioma.
The patient originally underwent pleurectomy and adjuvant chemoradiation therapy for primary malignant pleural mesothelioma, receiving four cycles of chemotherapy and 45 Gy of radiation to the left chest. She presented 14 years later with intermittent dyspnea and chest pain. Computed tomography of the chest revealed a lobulated filling defect, 4.0x4.1 cm, in the left atrium, with no sign of recurrence of the chest tumor. Echocardiography revealed an echogenic mass with a highly vascular structure on the mitral valve; magnetic resonance imaging of the heart confirmed this finding. The patient underwent open-heart surgery with en bloc resection of the mass. Histopathological examination revealed a 5.5-cm gelatinous, variegated white-yellow mass with areas of focal fibrosis — features suggesting that it was an unclassified, high-grade spindle-cell sarcoma with positive margins (Figure 1).
Figure 1. Gross Appearance of Sectioned Specimen of Cardiac Sarcoma.
Three months after surgery, the patient returned with a recurrent atrial tumor and multiple bony metastases, for which she received chemoradiation therapy. Nine months after resection, she had progressive left-sided weakness from a metastasis to the right motor cortex. She underwent craniotomy and resection but was later found to have intracranial hemorrhage from a second tumor deposit in the left frontal lobe. She died shortly thereafter.
Burke and colleagues described the aggressive nature of cardiac sarcomas and their ability to spread hematogenously to other organs.3 Criteria for postradiation sarcoma, defined by Cahan et al.4 and later revised by Murray and associates,2 include a history of exposure to radiation, no prior sarcoma, and histologic features distinct from those of the primary tumor. Other relevant factors in the current case include the 14-year latency period between irradiation of the primary tumor and the development of a secondary, radiation-induced sarcoma, since similarly lengthy latency periods have previously been reported,2 and also, the dose of radiation (45 Gy), since radiation doses in the range of 25 to 72 Gy have been associated with the development of sarcoma.5 The appearance of this rare secondary cancer in a long-term survivor of malignant pleural mesothelioma underscores the importance of vigilant long-term follow-up after radiation therapy.
Siyamek Neragi-Miandoab, M.D.
Boston University Medical Center
Boston, MA 02118
Sidhu P. Gangadharan, M.D.
David J. Sugarbaker, M.D.
Brigham and Women's Hospital
Boston, MA 02115
dsugarbaker@partners.org
The patient originally underwent pleurectomy and adjuvant chemoradiation therapy for primary malignant pleural mesothelioma, receiving four cycles of chemotherapy and 45 Gy of radiation to the left chest. She presented 14 years later with intermittent dyspnea and chest pain. Computed tomography of the chest revealed a lobulated filling defect, 4.0x4.1 cm, in the left atrium, with no sign of recurrence of the chest tumor. Echocardiography revealed an echogenic mass with a highly vascular structure on the mitral valve; magnetic resonance imaging of the heart confirmed this finding. The patient underwent open-heart surgery with en bloc resection of the mass. Histopathological examination revealed a 5.5-cm gelatinous, variegated white-yellow mass with areas of focal fibrosis — features suggesting that it was an unclassified, high-grade spindle-cell sarcoma with positive margins (Figure 1).
Figure 1. Gross Appearance of Sectioned Specimen of Cardiac Sarcoma.
Three months after surgery, the patient returned with a recurrent atrial tumor and multiple bony metastases, for which she received chemoradiation therapy. Nine months after resection, she had progressive left-sided weakness from a metastasis to the right motor cortex. She underwent craniotomy and resection but was later found to have intracranial hemorrhage from a second tumor deposit in the left frontal lobe. She died shortly thereafter.
Burke and colleagues described the aggressive nature of cardiac sarcomas and their ability to spread hematogenously to other organs.3 Criteria for postradiation sarcoma, defined by Cahan et al.4 and later revised by Murray and associates,2 include a history of exposure to radiation, no prior sarcoma, and histologic features distinct from those of the primary tumor. Other relevant factors in the current case include the 14-year latency period between irradiation of the primary tumor and the development of a secondary, radiation-induced sarcoma, since similarly lengthy latency periods have previously been reported,2 and also, the dose of radiation (45 Gy), since radiation doses in the range of 25 to 72 Gy have been associated with the development of sarcoma.5 The appearance of this rare secondary cancer in a long-term survivor of malignant pleural mesothelioma underscores the importance of vigilant long-term follow-up after radiation therapy.
Siyamek Neragi-Miandoab, M.D.
Boston University Medical Center
Boston, MA 02118
Sidhu P. Gangadharan, M.D.
David J. Sugarbaker, M.D.
Brigham and Women's Hospital
Boston, MA 02115
dsugarbaker@partners.org