Pleura
Other tumors
Angiosarcoma

Author: Vaidehi Avadhani, M.D. (see Authors page)

Revised: 23 March 2018, last major update February 2014

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Pleura angiosarcoma

Cite this page: Avadhani, V. Angiosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/pleuraangiosarcoma.html. Accessed September 22nd, 2018.
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
Epidemiology
Etiology
  • 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
Treatment
  • 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

Vimetin, 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