Pleura & peritoneum
Pleura other tumors

Topic Completed: 1 February 2014

Minor changes: 3 September 2020

Copyright: 2003-2020,, Inc.

PubMed Search: Pleura angiosarcoma

Vaidehi Avadhani, M.D.
Page views in 2019: 474
Page views in 2020 to date: 488
Cite this page: Avadhani V. Angiosarcoma. website. Accessed September 29th, 2020.
Definition / general
  • Angiosarcomas are malignant tumors that recapitulate functional and morphological features of normal endothelium
  • Primary pleural angiosarcomas are morphologically very similar to their counterpart in soft tissue
  • Unknown
  • History of tuberculous pyothorax in cases from Japan (Hum Pathol 2000;31:29)
  • History of asbestos in cases from Western countries
Clinical features
  • Most common presentations are chest pain (47.5%), dyspnea (35%), hemoptysis (27.5%), cough (15%), weight loss (10%)
Radiology description
  • CXR: may only show pleural thickening in early cases or diffuse opacification of the hemithorax in advanced lesions
  • CT: may show a lobulated mass with irregular margins and heterogeneous contrast enhancement; mimics mesothelioma
  • Positron emission tomographic (PET) scan: nonspecific and may show diffuse and homogeneous fluorodeoxyglucose (FDG) uptake indistinguishable from malignant mesothelioma
Radiology images

Images hosted on other servers:

Right side loculated pleural effusion

FDG uptake at the dorsal parietal pleura

FDG uptake all over the parietal pleura

Case reports
  • In general, have poor prognosis
  • Localized angiosarcomas may have a relatively good prognosis after resection
  • Chemotherapy and radiotherapy are often used
Gross description
  • Tumors may grow along the serosal surface and encase the lung
Microscopic (histologic) description
  • Tumor may be composed of epithelioid or spindle cells arranged in sheets, cords or strands, demonstrating an infiltrating growth pattern, in a collagenous or hyalinized stroma
  • Extensive hemorrhagic background may be seen
  • Tumor cells have moderate eosinophilic cytoplasm with round nuclei and small nucleoli
  • Tumor grade can vary from well to poorly differentiated
  • Poorly differentiated tumors have pleomorphic nuclei with marked nuclear atypia and mitotic figures may be readily seen
Microscopic (histologic) images

Images hosted on other servers:

Diaphragmatic pleural masses

Scattered tumor cells in hemorrhagic background

CD31, cytokeratin

Vimentin, CD31, CD68, FLI1

Positive stains
  • CD31 is a more sensitive and specific for endothelial differentiation than CD34
  • Newer markers:
    • FLI1: a nuclear transcription factor, highly sensitive, relatively highly specific
      • Also present in some adenocarcinomas, melanomas
    • ERG: an ETS family transcription factor
    • Claudin5: present in endothelial and some epithelial cells
  • Positive cytokeratin staining has been reported in 60% of primary pleural angiosarcoma, especially epithelioid variant (Hum Pathol 2000;31:29)
Negative stains
Electron microscopy description
  • Well differentiated angiosarcomas show features of normal endothelium - partial investiture of basal lamina along the antiluminal borders, tight junctions between cells, pinocytotic vesicles, occasional cytofilaments
  • For poorly differentiated tumors, EM is not very useful
Differential diagnosis
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