Lung Cancer |

A Middled-aged Male With Daily Hemoptysis: Primary Pulmonary Angiosarcoma FREE TO VIEW

Angel Brown, MD
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Pulmonary, Allergy, and Critical Care Medicine, University of Alabama at Birmingham, Birmingham, AL

Chest. 2014;146(4_MeetingAbstracts):631A. doi:10.1378/chest.1993892
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SESSION TYPE: Affiliate Case Report Slide

PRESENTED ON: Wednesday, October 29, 2014 at 11:00 AM - 12:15 PM

INTRODUCTION: Angiosarcoma is an uncommon malignant neoplasms characterized by anaplastic cells derived from blood vessels. Angiosarcomas are aggressive and have a high rate of lymph node and systemic metastases. Primary pulmonary angiosarcoma is extremely rare. To date there have been only 20 cases identified.

CASE PRESENTATION: 59-year-old Gulf War veteran presented with cough and hemoptysis. Additional complaints included dyspnea, weight loss and chest pain. Past medical history was significant for DM, PTSD, HTN and hyperlipidemia. He was a former cigarette smoker(40-pack-year history). He worked as a military pipe fitter for 23 years. He denied herbals, NSAIDS and anticoagulants. Vital signs were normal. Pertinent positives on exam included bibasilar crackles and lymphadenopathy. Routine labs revealed microcytic anemia. Contrast chest computed tomography revealed pulmonary nodules with surrounding ground glass. Bronchoscopy was performed. Blood was noted throughout the airway with no identifiable source; mucosa was edematous. The bronchoscopy was negative for malignancy and infection. Autoimmune work up was negative. He was treated with broad spectrum antibiotics, anti-fungals and stress-dose glucocorticoids. He developed bilateral hemothoraces. He was deteriorating rapidly. High-dose steroids and plasma exchange were initiated. Video-assisted thoracic surgery was performed. The final pathology report confirmed angiosarcoma. No other neoplasm was identified.

DISCUSSION: Angiosarcomas represent less than 2% of all sarcomas. They are aggressive. Average survival post diagnosis is 1-9 months. It has been observed in individuals exposed to vinyl chloride monomer gas or arsenic containing insecticides. They occur most often in skin and soft tissues of the head and neck. Diagnosis requires biopsy. Three positive vascular markers, and a negative epithelial marker, by immunohistochemistry support the diagnosis. Treatment options include glucocorticoids, radiotherapy, surgical resection, immunotherapy and chemotherapy. Unfortunately, these therapies have done little to alter the disease course.

CONCLUSIONS: There have been very few cases of primary pulmonary angiosarcoma reported in the literature. A diagnosis of pulmonary angiosarcoma is considered metastatic and warrants further work up to locate a primary neoplasm. As the above case illustrates, symptoms are non-specific making diagnosis difficult.

Reference #1: Judy BF et al. Metastic Primary Pulmonary Angiosarcoma. Surgical Science 2011;2:130-133.

DISCLOSURE: The following authors have nothing to disclose: Angel Brown

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