Delayed diagnosis of pulmonary angiosarcoma in a patient presenting with recurrent hemoptysis and widespread pulmonary nodules: a case report.

INTRODUCTION: Hemoptysis is a common clinical symptom, which can be caused by a wide spectrum of local, systemic, benign and malignant pathologies. Hemoptysis in conjunction with multiple pulmonary nodules raises suspicion for granulomatous diseases (such as granulomatosis with polyangiitis) or pulmonary metastases from a distant malignancy. Here, we report an unusual case of a patient who presented with subjective fever, hemoptysis and scattered pulmonary nodules, and was subsequently diagnosed with pulmonary angiosarcoma (probable primary pulmonary origin).

CASE PRESENTATION: A 67-year-old North American White gentleman of Greek ancestry initially presented with fever, cough, and dyspnea, and was found to have reticulonodular pulmonary infiltrates. Over the next 5 months, he developed persistent constitutional symptoms and new hemoptysis. During this interval, he underwent serial outpatient evaluations and was treated empirically for presumed pneumonia and heart failure, followed by unrevealing infectious, interstitial lung disease, and rheumatologic workup. At 6 months, CT demonstrated multiple bilateral pulmonary nodules and masses, and bronchoscopy with transbronchial needle biopsy showed only hemosiderin-laden macrophages, consistent with chronic alveolar hemorrhage. One month later, during a third hospitalization for worsening hemoptysis and hypoxemia, repeat imaging showed progression of innumerable pulmonary nodules and new liver lesions. Ultrasound-guided percutaneous liver biopsy was nondiagnostic, revealing fibrosis, bile ductular proliferation, and granulomatous inflammation without malignancy. During the same hospitalization, thoracoscopic wedge biopsy of the lung was performed. Final surgical pathology, available 1 week later, demonstrated CD34 and factor-VIII-positive malignant cells, establishing the diagnosis of pulmonary angiosarcoma (probable primary pulmonary origin). Thus, definitive diagnosis was made > 7 months after initial presentation. By this time, the patient was profoundly deconditioned and no longer a candidate for systemic chemotherapy; he died approximately 1 month after diagnosis and 8 months after presentation.

CONCLUSIONS: Recurrent hemoptysis along with widespread pulmonary nodules is a non-specific presentation, which can be caused by pulmonary angiosarcoma in rare cases. A surgical wedge biopsy is necessary for accurate diagnosis as percutaneous biopsies provide insufficient tissue to delineate the histopathological characteristics pathognomonic of this malignancy. A high index of suspicion and early surgical consultation is necessary to establish an accurate diagnosis and initiate timely treatment.