Correct!
5. All of the above

There are only about 50 published case reports of pleural epithelial angiosarcoma in the English literature with an incidence of approximately 1% of all soft tissue tumors (3,4).
The mean age of diagnosis is 58 years and the male to female ratio is 6:1. Risk factors can include occupational exposure to asbestos, prior radiation therapy, cigarette smoking and prior history of tuberculous complications. The most common presenting symptoms can include chest pain, dyspnea, hemoptysis, cough and weight loss. Radiological findings on chest x-ray can include pleural thickening in early stages or diffuse opacification of the hemithorax. On chest CT, a lobulated mass with irregular margins can be seen. Positron emission tomographic (PET) scan shows non-specific diffuse and homogeneous fluorodeoxyglucose (FDG) uptake indistinguishable from malignant mesothelioma.

Our case is unique because our patient had no identifiable risk factors and imaging did not show typical radiographic findings of angiosarcoma. In addition, inspection of the pleural at the time of surgery was grossly unremarkable.

Although localized angiosarcomas may have a relatively good prognosis after resection. diffuse disease has a poor prognosis. Chemotherapy and radiotherapy are often used. Bevaciziumab and paclitaxel may have some beneficial effect (5,6). There is an ongoing trial of regorafenib, an oral multi-kinase inhibitor. 

References

  1. Hooper C, Lee YC, Maskell N; BTS Pleural Guideline Group. Investigation of a unilateral pleural effusion in adults: British Thoracic Society Pleural Disease Guideline 2010. Thorax. 2010 Aug;65 Suppl 2:ii4-17. [CrossRef] [PubMed]
  2. Merchant M, Romero AO, Libke RD, Joseph J. Pleural effusion in hospitalized patients with Coccidioidomycosis. Respir Med. 2008 Apr;102(4):537-40. [CrossRef] [PubMed]
  3. Dainese E, Pozzi B, Milani M, Rossi G, Pezzotta MG, Vertemati G, Tricomi P, Sessa F. Primary pleural epithelioid angiosarcoma. A case report and review of the literature. Pathol Res Pract. 2010 Jun 15;206(6):415-9. [CrossRef] [PubMed]
  4. Kao YC, Chow JM, Wang KM, Fang CL, Chu JS, Chen CL. Primary pleural angiosarcoma as a mimicker of mesothelioma: a case report **VS**. Diagn Pathol. 2011 Dec 30;6:130. [CrossRef] [PubMed]
  5. Agulnik M, Yarber JL, Okuno SH, von Mehren M, Jovanovic BD, Brockstein BE, Evens AM, Benjamin RS. An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas. Ann Oncol. 2013 Jan;24(1):257-63. [CrossRef] [PubMed]
  6. Penel N, Bui BN, Bay JO, et al. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study. J Clin Oncol. 2008 Nov 10;26(32):5269-74. [CrossRef] [PubMed]

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