INTRODUCTION: Angiosarcomas are rare malignant epithelial neoplasms - with very few primary pulmonary angiosarcomas described in the literature.
CASE PRESENTATION: A 49 year old woman presented with a two month history of productive cough, fevers and shortness of breath. She had quit smoking one month earlier, with a history of 15 cigarettes per day for 20-25 years. She worked as a sales consultant. An isolated wheeze was audible in the right infrascapular region, with no other findings on examination. Her chest radiograph revealed an opacity involving the right basal segments and middle lobe, along with widened superior mediastinum. A chest CT scan identified a 3.5 x 4.4 x 4.5cm endobronchial mass in the right middle lobe, with peripheral airspace opacification and nodularity. Flexible bronchoscopy revealed a mass in the take off to the right middle lobe and tissue biopsies was suggestive but not diagnostic of squamous cell carcinoma. A rigid bronchoscopy and biopsy then revealed an angiosarcoma. A PET-CT scan was performed - this confirmed the presence of a SUV avid large right middle lobe mass which merged with right hilar lymphadenopathy with no metastatic lesions. A right thoracotomy and pneumonectomy was performed - a large tumour was found in the middle lobe, with extension around the superior pulmonary vein (see Figure 1.) Approximately 40-50% of the tumour was soft, necrotic tissue. The pathologist confirmed findings were that of an epithelioid Angiosarcoma. In light of the patient’s PET-CT scan results and tissue pathology, a diagnosis of Primary pulmonary angiosarcoma was made. The patient recovered well post-operatively and was discharged home from hospital eleven days later. She was progressing well when reviewed one month post-operatively and has now returned to work and normal daily activities.
DISCUSSION: Angiosarcomas are very rare malignant epithelial neoplasms, comprising 2% of all sarcomas. The primary tumours are most commonly found in the head/neck region. Although these frequently metastasise to the lung, only a handful of primary lung tumours have been reported worldwide, with only six cases identified using an Ovid Medline search. Of the few cases documented, the presentation has varied from massive pulmonary haemorrhage or haemoptysis, spontaneous haemothorax, multiple bilateral nodules on chest radiograph, pleuritic chest pain, non-productive cough or dyspnoea. Macroscopically, angiosarcomas are characterised by a large mass of tan coloured fleshy tissue, often with central necrosis and haemorrhage. Microscopically there are clusters of round to oval/polygonal epithelial cells with erythrophagocytosis.. Immunohistochemistry is required to confirm the diagnosis. Following tissue diagnosis, further imaging is required to evaluate the disease burden and determine appropriate treatment options. A PET scan is recommended to exclude distant metastases if surgery is proposed. With localised disease, radical surgery with wide resection margins is required. This may be followed with adjuvant radiation therapy to reduce the high rates of local recurrence. There is currently no data supporting use of chemotherapy, except for widespread metastatic disease . Studies are currently underway to investigate the use of targeted therapy with biological molecules, such as the VEGF-A monoclonal antibody, bevacizumab. Angiosarcomas generally have a poor prognosis, with an overall 5 year survival of approximately 35%- with up to 45% of patients having metastatic disease at presentation. Large tumours (>5cm) and advanced patient age are poor prognostic indicators. Due to the infrequency of primary pulmonary angiosarcomas, there is very little information regarding accurate incidence, pre-disposing risk factors, effective treatment and prognosis.
CONCLUSIONS: We present a rare Lung malignancy.
Reference #1 Kumar: Robbins and Cotran Pathologic Basis of Disease, Professional Edition, 8th ed.
Reference #2 Young RJ, Brown NJ, Reed MW, et al. Angiosarcoma (Review). Lancet Oncol. 2010;11:983-91.
DISCLOSURE: The following authors have nothing to disclose: Vinu Abraham
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