NCI Definition: A rare malignant neoplasm characterized by the presence of large epithelioid cells with abundant cytoplasm forming nests and pseudoalveolar structures. The groups of the epithelioid cells are separated by thin-walled sinusoidal spaces. It occurs most often in adolescents and young adults. In adults the most common sites of involvement are the extremities, and in infants and children, the head and neck. It usually presents as a slowly growing mass and it frequently metastasizes to other anatomic sites. The most common sites of metastasis are the lungs, bone, and brain. [1]

Alveolar soft part sarcomas most frequently harbor alterations in TFE3, ASPSCR1, KMT2D, TP53, and NTRK3 [2].

Most Commonly Altered Genes in Alveolar Soft Part Sarcoma

TFE3 Fusion, ASPSCR1-TFE3 Fusion, TFE3-ASPSCR1 Fusion, KMT2D Mutation, and NTRK3 Q586K are the most common alterations in alveolar soft part sarcoma [2].

Top Alterations in Alveolar Soft Part Sarcoma

Significant Genes in Alveolar Soft Part Sarcoma




Disease Details

Soft Tissue Sarcoma
Cervical Alveolar Soft Part Sarcoma and Vulvar Alveolar Soft Part Sarcoma
OncoTree Name
Alveolar Soft Part Sarcoma
OncoTree Code


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 8. 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.