Primitive Neuroectodermal Tumor
Associated Genetic Biomarkers
NCI Definition: A malignant neoplasm that originates in the neuroectoderm. The neuroectoderm constitutes the portion of the ectoderm of the early embryo that gives rise to the central and peripheral nervous systems and includes some glial cell precursors. 
Primitive neuroectodermal tumors most frequently harbor alterations in TP53, SQSTM1, SETBP1, CDKN2B, and XPO1 .
TP53 c.217-c.1178 Missense, TP53 c.142-c.212 Missense, TP53 c.1-c.137 Missense, TP53 Mutation, and TP53 Missense are the most common alterations in primitive neuroectodermal tumor .
There are 3 clinical trials for primitive neuroectodermal tumor, of which 2 are open and 1 is completed or closed. Of the trials that contain primitive neuroectodermal tumor as an inclusion criterion, 3 are phase 1 (2 open).
Iodine i 131 monoclonal antibody 8h9, non-myeloablative tcr alpha/beta-depleted haploidentical hematopoietic stem cell transplantation, and zoledronic acid are the most common interventions in primitive neuroectodermal tumor clinical trials.
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 4. This dataset does not represent the totality of the genetic landscape; see paper for more information.