Abstract
Epithelioid hemangioendothelioma is a malignant vascular neoplasm. The outdated term intravascular bronchioloalveolar tumor refers to a lung localization of epithelioid hemangioendothelioma.
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Notes
- 1.
EMA: epithelial membrane antigen.
- 2.
WWTR1-CAMTA1 fusion gene: WWTR1 (WW domain-containing transcription regulator 1) encodes a transcriptional coactivator which acts as a downstream regulatory target in the Hippo signaling pathway that plays a pivotal role in organ size control and tumor suppression by restricting proliferation and promoting apoptosis. CAMTA1 (calmodulin-binding transcription activator 1) encodes a protein that contains a CG1 DNA-binding domain, a transcription factor immunoglobulin domain, ankyrin repeats, and calmodulin-binding IQ motifs. The encoded protein is thought to be a transcription factor.
- 3.
YAP1-TFE3 fusion gene: YAP1 encodes Yes-associated protein 1, a downstream nuclear effector of the Hippo signaling pathway (which is involved in development, growth, repair, and homeostasis); YAP1 is known to play a role in the development and progression of multiple cancers as a transcriptional regulator of this signaling pathway. TFE3 belongs to the microphthalmia family of bHLH-LZ transcription factors (MiT/TFE) which is composed of four members: MITF, TFEB, TFE3, and TFEC; neoplasms with alterations in these genes are also called MiT family tumors.
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Mocellin, S. (2021). Epithelioid Hemangioendothelioma. In: Soft Tissue Tumors . Springer, Cham. https://doi.org/10.1007/978-3-030-58710-9_84
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