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Lung Cancer |

Primary Pleural Lymphangiosarcoma - First Ever?

Pushan Jani*, MD; Kim Nguyen, MD; Ankit Upadhyay, MBBS; George Nassif, MD
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University of Texas Health Science Center, Houston, TX


Chest. 2012;142(4_MeetingAbstracts):648A. doi:10.1378/chest.1389144
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Abstract

SESSION TYPE: Cancer Cases II

PRESENTED ON: Tuesday, October 23, 2012 at 11:15 AM - 12:30 PM

INTRODUCTION: Angiosarcoma is a malignant soft tissue tumor originating from the vascular endothelial cells. Common sites of origin are skin, liver, spleen, breast, skeletal muscles, and subcutis. It rarely comes from the pleura or chest wall. Pleural angiosarcoma displaying lymphatic differentiation has never been described in literature. Prior to availability of tumor markers, hemangiosarcomas and lymphangiosarcomas were combined together and were referred as angiosarcomas due to the lack of histopathologic methods for differentiating the two. However, with the availability of markers like VEGFR-3 and D2-40, lymphangiosarcomas can be differentiated and mixed differentiation of angiosarcomas can also be identified.

CASE PRESENTATION: A 58 year old nonsmoker female of Chinese descent, with no past history, presented with one month history of dyspnea and weight loss. CT scan of the chest showed bilateral pleural thickening and effusion. Positron emission tomography demonstrated increased FDG uptake in the left basal pleura. Repeated thoracenteses showed bloody fluid with cytology negative for malignancy. Left video-assisted thoracoscopy (VATS) with pleural biopsies was performed. Pleural biopsies showed spindle cell neoplasm with cytologic atypia. Immunohistochemical staining was positive for vimentin, CD31, D2-40 and VEGF and negative for CD34, Calretinin and Factor 8. A unique aspect was diffuse positivity for D2-40, which suggests lymphatic differentiation of the angiosarcoma. Patient underwent pleurodesis with bleomycin. She tolerated four cycles of Paclitaxel. Despite chemotherapy, she continued to worsen clinically and died five months after diagnosis.

DISCUSSION: Pleural angiosarcoma is a rare and aggressive vascular tumor affecting the endothelial cells of blood/lymphatic vessels with 51 reported cases. Proposed theories of pathogenesis are tuberculosis producing a pyothorax in Japanese series, radiation and asbestos exposure in Western series, and de novo mutations. Manifestations include dyspnea, chest pain, weight loss and hemoptysis. PET scan only helps in determining the extent of the disease. Definitive diagnosis requires VATS with pleural biopsies.

CONCLUSIONS: Angiosarcoma cannot be distinguished from mesothelioma based on clinical, radiological, and histological traits. Immunohistochemistry can differentiate angiosarcoma from mesothelioma. Positive Vimentin and negative cytokeratin should prompt vascular marker testing (CD31, CD34 and Factor VIII). Testing for D2-40 should be performed for lymphatic variant of this tumor. Despite treatment, survival ranges from two to twelve months.

1) Mankey, Cohra. Can lymphangiosarcoma be resurrected? A clinicopathological and immunohistochemical study of lymphatic differentiation in 49 angiosarcomas. Histopathology 2010, 56, 364-371.

2) Dainese, Emanuele. Primary pleuralepithelioid angiosarcoma.A case report and review of the literature. Pathology -Research and Practice 206(2010) 415-419.

DISCLOSURE: The following authors have nothing to disclose: Pushan Jani, Kim Nguyen, Ankit Upadhyay, George Nassif

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University of Texas Health Science Center, Houston, TX

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