INTRODUCTION: Primary cardiac tumors are a rare entity and only one-fourth of these tumors, usually sarcomas, exhibit malignant characteristics. These tumors can metastasize to the lungs, mediastinum, thoracic lymph nodes, and vertebral column. We report a case of a woman with a history of pre-B ALL who presents with an RV mass, pericardial mass, and multiple pulmonary nodules. Biopsy of all three masses reveal metastatic spindle cell type, high grade rhabdomyosarcoma.
CASE PRESENTATION: A 75 year old Caucasian female presented to the Emergency Department with a 2 week history of dyspnea on exertion and an abnormal EKG. She had a history of pre-B ALL treated with chemotherapy 1 year prior to presentation. A chest radiograph was performed, showing a small to moderate right pleural effusion and multiple irregular patchy opacifications in both lung fields. At the time of presentation, a ventilation perfusion scan was performed showing several bilateral moderate mismatches. A left and right heart catheterization showed an ejection fraction of 70% and an abnormal contour at the apex of the right ventricle. Subsequently, the patient had a CT Angiogram which revealed multiple pulmonary nodules ranging from 5 mm to 2 cm, a left pericardial mass measuring 2.6 cm, and an RV mass measuring 2.9 x 6 x 5.6 cm. For further diagnostic evaluation, the patient underwent CT guided biopsy of a lung nodule, thoracentesis, and RV mass biopsy. Cytologic examination of all samples were performed as well as special staining. The final diagnosis was determined to be rhabdomyosarcoma, spindle cell type, high grade. Pt was started on vincristine and ironotecan with documented shrinking of her pulmonary nodules. However, the patient had persistent diarrhea and neutropenia requiring a change in her chemotherapy to vinscristine, actinomycin-D, and cyclophosphamide (VAC). Five months after her initial diagnosis the patient had worsening of her heart failure and a new onset pneumonia. She went into respiratory failure and succumbed to her disease.
DISCUSSIONS: Metastatic sarcoma is 30 times more common than primary cardiac tumors. In adults, one quarter of primary cardiac tumors are malignant. Rhabdomyosarcomas are extremely uncommon. This subtype is associated with an infiltrative growth pattern and an aggressive clinical course. Most patients with cardiac involvement present with progressive dyspnea and unexplained heart failure as well as precordial pain and pericardial effusions. Cardiac findings are determined by the extent of invasion and the location of the tumor. For initial screening, transthoracic echocardiography can be performed. MRI or CT can show the degree of tumor infiltration. Final diagnosis should include a tissue biopsy of the primary tumor or an accessible metastasis. Although possible in less than 50% of patients, complete excision of the tumor allows patients a better overall prognosis. Anthracycline plus ifosfamide based chemotherapy has shown benefit in some case series. If the tumor is primarily intramural, arrhythmias, conduction abnormalities, and sudden death can occur. This tumor usually leads to a rapid downhill course over weeks to months due to hemodynamic compromise, local invasion, or distant metastasis. Orthotopic heart transplantation may be an option for those patients who have unresectable primary tumors without distant metastasis.
CONCLUSION: Primary cardiac rhabdomyosarcoma is a very rare tumor with a poor prognosis usually because it is diagnosed late in the disease course. Echocardiography can be used as a first diagnostic step. Surgery and chemotherapy does alter the disease course, but survival rates continue to be dismal.
DISCLOSURE: Jessica Freyer, No Financial Disclosure Information; No Product/Research Disclosure Information