Pleuropulmonary Angiosarcoma Presenting as Bilateral Hemothoraces and Hemoptysis

Abstract

INTRODUCTION: Pleuropulmonary angiosarcoma (PAS) is a very rare highly malignant tumor. Tumors of the pleura, either primary or secondary in origin can be differentiated by immunohistochemistry. Accurate diagnosis is important for treatment and prognosis. CASE PRESENTATION: A 67 year old African American nonsmoker female with history of goiter and childhood asbestos exposure presented with right upper abdominal pain, neck swelling and shortness of breath for 1 week duration. CT chest showed calcified mass in left lobe of thyroid, right upper lobe pleural based mass, bilateral upper lobe pulmonary nodules, calcified lymph nodes and bilateral large pleural effusions. Ultrasound of the thyroid showed multinodular goiter, negative for color Doppler. She was intubated for hypoxic respiratory failure. Bilateral chest tubes drained 1500ml of blood. Videoassisted thoracoscopy (VATS) showed multiple pleural nodules and dark blood stained pleura. Immunomarkers were negative for thyroid, lung, breast, colon, kidney and ovary. Preliminary biopsy report suggested either mesothelioma or adenocarcinoma of occult primary (AOP). Clinical course was complicated by heparin induced thrombocytopenia. Unfortunately patient passed away 2 weeks after presentation. Final pathology report showed poorly differentiated epithelioid angiosarcoma with no hemolymphoid differentiation. DISCUSSION: PAS is a very rare sarcoma, (<0.1/100000/year). It presents as pleural thickening, pulmonary mass and hemorrhagic effusion. It is prevalent in Caucasians (65-85%) and men between the ages of 34-85. Risk factors are asbestos exposure, prior radiation and smoking. Mesothelioma, AOP and PAS look alike clinically, radiologically and histologically. The key feature is poorly differentiated cells lining irregular sized vascular channels. Immunomarkers are diagnostic. PAS is positive for vascular markers, Factor 8, CD31 and vimentin. Presence of benign mesothelial cells positive for calretinin, WT1, mesothelin and D2-40, as in our case confounds the diagnosis. PAS is a very difficult tumor to treat. For a solitary mass, surgery and radiation is preferred. Review of the literature showed, for metastatic disease, either monotherapy with Adriamycin, Paclitaxel, Doxorubicin or combination therapy with Mesna, Adriamycin and Ifosfamide can be tried. In PAS maximum survival is 6 months. In mesothelioma patients who complete trimodality therapy, median survival is 29 months. Based on the molecular studies and chemotherapy sensitivity, survival rate varies in AOP. CONCLUSIONS: Pulmonary angiosarcoma is a rare tumor of vascular origin. In our case, the patient presented with bilateral hemorrhagic pleural effusions, pulmonary nodules and pleural mass, which rapidly proved fatal. Finding the origin of the tumor is facilitated by Immunomarkers. We have only few chemotherapy options and prognosis is dismal.