Primary lung angiosarcoma is a rare entity with poor prognosis. We present a patient with pulmonary epithelioid angiosarcoma misdiagnosed as granulomatous lung disease on pathology and try to iterate the importance of following expected symptom resolution with appropriate therapy.
36 year old non smoking man presented to the emergency room with progressively increasing hemoptysis over a 4 week period. He was coughing about 150 to 200 ml of fresh blood a day. No history of hematuria, weight loss, rheumatologic or vasculitic symptoms. CT chest revealed a 2.5 × 2.0 cm left hilar mass. CT guided fine needle aspiration and core biopsy of this mass was consistent with vasculitides, most probably wegener’s granulomatosis. Pulse steroids were administered with methyl prednisolone at a dose of 1 gram a day after confirming no fungal elements in the mass. One week into steroid therapy patient did not have a significant symptomatic or radiologic improvement. ANCA (anti-neutrophil cytoplasmic antibody) screen was negative. This prompted us to rebiopsy the mass under CT guidance. Fine needle biopsy revealed an invasive malignancy with tumor cells staining positive with CD31, cytokeratin AE1/3 and factor 8 consistent with epithelioid angiosarcoma. Both the initial and later pathology specimens were reconfirmed by a pulmonary pathologist. Subsequent staging revealed the tumor was confined to thorax suggesting primary pulmonary epithelioid angiosarcoma.
Hemoptysis in a young patient with lung mass is concerning for primary pulmonary neoplasm versus non malignant etilogies like infection or inflammatory vasculitis. 5% of patients can have ANCA negative wegener’s granulomatosis. Although a combination of cyclophosphomide and steroids for 6 months could be necessary for complete remission of wegener’s, majorityof the patients with pulmonary vasculitis as the cause of hemoptysis respond to high doses of steroids alone. Angiosarcoma constitutes less than 1% of all sarcomas. The sites most frequently involved are the skin and subcutaneous tissue, liver, breast, and heart. Angiosarcoma occurring in the lung usually represents metastasis from the heart, the pulmonary arterial trunk, or an extrathoracic organ. Angiosarcoma of pulmonary origin is very rare, being reported only in a handful of cases. It usually presents with intractable hemoptysis or massive intraparenchymal or intrapleural hemorrhage. Differential diagnosis between pulmonary angiosarcomas and other masses, especially lung carcinoma and a variety of vascular lesions with epithelioid endothelial cells, is not possible without biopsy. The distinction between benign and malignant vascular lesions can be quite challenging, even on histopathological studies, and requires immunohistochemical analysis in most patients. The epithelioid form of angiosarcoma has been fully characterized only in the past decade. Among the various immunohistochemical markers used for classification, factor VIII-related antigen and CD31 are considered specific for tumors derived from the endothelium. The prognosis is poor with mortality approaching 100% within months of initial presentation. Therapeutic modalities such as radiation therapy, chemotherapy, and surgical intervention have all been attempted, but none of them have been shown to be dramatically effective.
We present this case in attempt to bring forward the importance of expected results of any therapy and clinical followup which could be the only clues when pathologic diagnosis is in question and not agreeing with our clinical diagnosis. A second look at the tissue in such circumstances may steer us in the right direction.
Akhil Vallabhaneni, No Financial Disclosure Information; No Product/Research Disclosure Information