What is the clinical behavior of vascular sarcomas of the pleura?

Updated: Dec 25, 2019
  • Author: Joseph F Tomashefski, Jr, MD; more...
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The clinical behavior of EHE varies based on its site of origin. It is generally regarded as a low grade or indolent malignancy in the soft tissue, with a mortality rate of about 20%, whereas hepatic or osseous EHE proves lethal in 30%-40% of cases. In the pleura, however, EHE behaves in a highly aggressive manner and is invariably fatal, causing death within several years. In this respect, EHE resembles the aggressive behavior of malignant mesothelioma or pseudomesotheliomatous carcinoma, with extensive spread within the pleural space.

Metastatic spread to lung and mediastinal lymph nodes is often seen at autopsy. Extrathoracic spread to abdominal viscera or retroperitoneum has also been documented. [51] Angiosarcomas, including epithelioid angiosarcomas, of the pleura are highly malignant neoplasms and behave in fashion similar to that of malignant mesothelioma. [40]

Curative surgery is the treatment of choice for vascular sarcomas, but this modality is often prohibited in primary tumors of the pleura owing to the diffuse and advanced nature of the disease at the time of clinical presentation. Various chemotherapeutic agents and radiation therapy regimens have been used, without demonstrable therapeutic benefit. [51]

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