NCI Definition: A malignant neoplasm arising from mesothelial cells in the pleura. It is characterized by the presence of neoplastic cells with an epithelioid appearance. In the majority of cases, the neoplastic epithelioid cells lack significant cytologic atypia; mitotic figures are infrequently seen. In a minority of cases, the neoplastic cells are poorly differentiated and there is evidence of nuclear atypia and increased mitotic activity. [1]

Epithelioid mesotheliomas most frequently harbor alterations in BAP1, CDKN2A, NF2, CDKN2B, and SETD2 [2].

Most Commonly Altered Genes in Epithelioid Mesothelioma

BAP1 Mutation, CDKN2A Loss, CDKN2B Loss, NF2 Mutation, and SETD2 Mutation are the most common alterations in epithelioid mesothelioma [2].

Top Alterations in Epithelioid Mesothelioma

Disease Details

Epithelial Mesothelioma, Malignant Epithelial Mesothelioma
Malignant Mesothelioma
Pleural Epithelioid Mesothelioma


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.