A 35-year-old Native American without significant medical history was transferred from another facility for the evaluation of worsening shortness of breath, cough, and hemoptysis of over 2 months. He also had a history of on and off fever, and weight loss of about 20 pounds. He was a known smoker with a history of occasional alcohol intake. Family history was unremarkable. There was no history of recent travel or contact with tuberculosis patients. On examination, vital signs were normal. Mild pallor was noticed with trace pedal edema. A few crepitations at the right lung base were noted. Cardiac examination was unremarkable.
Laboratory examination revealed hemoglobin of 7.5 g/dL and white blood cell count of 7,900/mm3 (neutrophils 93%). Antinuclear cytoplasmic antibody (ANCA) panel and antinuclear antibody (ANA) were negative. A computerized tomography (CT) of the chest showed right-sided pleural effusion with mediastinal lymphadenopathy measuring 3.2x2.2 cm, and a ground glass density with coarsening of interstitial markings in bilateral lower lobes (Fig. 1). Pleurocentesis revealed pinkish fluid with 1,2750/mm3 red blood cells, 400/mm3 white blood cells (neutrophils 17%, monocytes 31%, and lymphocytes 45%), 1.3 g/dL protein, 80 IU/L lactate dehydrogenase, 115 mg/dL glucose, and pH of 7.49 without any malignant cells. Serum protein and glucose were 6.6 g/dL and 180 g/dL, respectively. Bronchoscopic evaluation was also done which was unremarkable.
Transthoracic echocardiogram (TTE) was done which revealed a large left atrial mass measuring 8.15x2.2 cm attached to interatrial septum on the left, causing pulmonary venous hypertension with an elevated right ventricular systolic pressure (RVSP). This was followed by a transesophageal echocardiogram (TEE) that revealed a mass completely occupying the left atrium and protruding across the mitral valve into the left ventricle with a mild mitral regurgitation. There was also invasion of the pulmonary veins. The RVSP was elevated at 120 mmHg with a severe tricuspid regurgitation (Fig. 2).
At the time of initial presentation with shortness of breath and hemoptysis, our differential diagnosis was broad and included connective tissue diseases, tuberculosis, lung malignancy, Wegener's granulomatosis, and heart failure. Bronchoscopy and CT of the chest ruled out lung malignancy. ANA, ANCA, and quantiferon tests were negative, ruling out SLE, Wegener's granulomatosis, and tuberculosis, respectively.
Cardiothoracic surgery was consulted for surgical excision of the mass, and the patient underwent a limited excision of the mass along with mitral valve repair. A post procedure TEE showed an improvement in the RVSP of 47 mmHg. Biopsy of the mass revealed high grade undifferentiated pleomorphic neoplasm composed of atypical cells with hyperchromatic nuclei and scattered atypical mitotic figures (Fig. 3). An immunochemical stain was positive for smooth muscle actin and desmin. Stain was negative for S-100, cytokeratin AE1/AE3, myogenin, and Myo D1.
A follow-up positron emission tomography (PET) scan showed residual disease activity in the pulmonary veins. Patient was started on chemotherapy with ifosfamide and doxorubicin and was referred to a higher center for additional management. Patient has received four cycles of chemotherapy so far and one dose of radiation treatment. At 9-month follow up, the patient is doing well without any complications.
CT scan without contrast showing right pleural effusion and right hilar adenopathy with suggestion of node or mass with size of 3.2x2.2 cm.