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  Incidence: 3000 new cases per year
Median age: 60 years
Male to female ratio: 3:1

Peto J et al. Lancet 1995;345:535–539

Vogelzang NJ et al. Cancer 1984;53:377–383


H & E staining

  1. Epithelioid: 60% of cases = tubopapillary, granular, solid (occasional 5-year survival)

  2. Sarcomatoid/mixed: 40% of cases (0% 5-year survival)

Immunohistochemical staining: Keratin positive, CEA negative, Leu M negative, calretinin positive

Cytogenetics: Deletion of short arm of chromosome 1 and 3 and long arm of chromosome 22


Chaihinan AP et al. In: Holland JC, Frei E, eds. Cancer Medicine. 5th ed. Hamilton, ON: BC Decker; 2000:1293–1312

Corson JM. Semin Thorac Cardiovasc Surg 1997;9:347–355


No defined universal staging system


Rusch VW. Chest 1995;108:1122–1128


Additional information

  1. Thoracic lymph node involvement: 20% at presentation/70% at autopsy

  2. Hematogenous metastases: Liver, lung, and bone: usually late in disease course


Curran D et al. J Clin Oncol 1998;16:145–152

Herndon JE et al. Chest 1998;113:723–731

Symanowski JT et al. Proc Am Soc Clin Oncol 2003;22:647 [Abstract 2602]


  1. Chest x-ray: Initial tool in diagnosing pleural plaques and effusion

  2. CT scan or MRI: CT scan or MRI can be used to assess extent of disease. Calcifications are not generally visible on CT scan. Furthermore, the CT scan is less sensitive than the MRI in depicting diaphragmatic, pericardial, and chest wall involvement

  3. 3. PET scan: Has shown benefit in assessing lymph node involvement but is useful in only 50–70% of cases

  4. Thoracentesis: Used when there is a pleural effusion (30% diagnostic yield)

  5. CT-guided biopsy: Depends on CT findings. If tumor is thick and easily biopsied, do a CT-directed biopsy

  6. Video-assisted thorascopic (VAT) surgery: Do if on CT scan disease is thin or minimal or in a difficult location and thoracentesis is negative (90% diagnostic yield)


Flores RM et al. Proc Am Soc Clin Oncol 2003;22:620 [Abstract 2495]

Patz EF Jr et al. AJR Am J Roentgenol 1992;159: 961–966

Schneider DB et al. J Thorac Cardiovasc Surg 2000;120: 128–133

Steele JPC. Semin Oncol 2002;29:36–40


Overall survival: 6–18 months

Expert Opinion

Surgical management

Data regarding the choice of surgical procedure are derived mostly from observational studies involving selected patient populations treated with a variety of surgical techniques and adjuvant chemotherapies

  • Pleurectomy and decortication (lung sparing surgery): Indicated for minimal bulky disease associated with massive or recurrent pleural effusions

  • Extrapleural pneumonectomy (en bloc resection of ipsilateral lung, pleura [parietal and visceral], pericardium, and hemidiaphragm): Indicated for highly selected patients with early stage epithelioid type disease with extensive involvement of the diaphragm and visceral pleural surfaces, no nodal metastases, good performance status, and no comorbidities


Radiation therapy

Because of the large volume of lung in the ...

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