Pulmonary epithelioid hemangioendothelioma: an unusual case and a review of the literature

Chest. 2004 Feb;125(2):789-93. doi: 10.1378/chest.125.2.789.


We describe a case of pulmonary epithelioid hemangioendothelioma, previously known as intravascular bronchoalveolar tumor, in a 35-year-old woman with an initial diagnosis made by transbronchial biopsy. This is a rare disease, with approximately 50 cases described in the literature. All previous cases have been diagnosed by surgical lung biopsy. Although our patient underwent thoracoscopic lung biopsy, the diagnosis was initially made on transbronchial biopsy; to our knowledge, this has not been previously described in the English-language literature. We also described findings on high-resolution CT, both typical and atypical relative to previously published reports. This tumor can affect multiple organs. The prognosis is very unpredictable, with life expectancy ranging from 1 to 15 years. There is no single effective treatment, though spontaneous regression and response to chemotherapy and interferon are reported.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Biopsy, Needle
  • Disease Progression
  • Fatal Outcome
  • Female
  • Hemangioendothelioma, Epithelioid / pathology*
  • Hemangioendothelioma, Epithelioid / therapy
  • Humans
  • Immunohistochemistry
  • Lung Neoplasms / pathology*
  • Lung Neoplasms / therapy
  • Neoplasm Invasiveness / pathology*
  • Neoplasm Staging
  • Palliative Care / methods
  • Rare Diseases
  • Risk Assessment