1. Biallelic Deletion of PALB2 Occurs Across Multiple Tumor Types and Suggests Responsiveness to Poly (ADP-ribose) Polymerase Inhibition.
- Author
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Bhangoo, Munveer S., Costantini, Carrie, Clifford, Brian T., Chung, Jon H., Schrock, Alexa B., Ali, Siraj M., and Klempner, Samuel J.
- Subjects
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MULTIPLE tumors , *SINGLE-strand DNA breaks , *HEREDITARY cancer syndromes , *BRCA genes , *CHILDHOOD cancer - Abstract
DNA repair pathways are disrupted in PALB2-deficient cells, thereby accounting for the tumor suppressor gene properties of I PALB2 i .[1],[2] Durable benefit from olaparib in a patient with metastatic prostate cancer and heterozygous I PALB2 i gene deletion[30] and a case of PALB2-mutant pancreatic cancer treated with mitomycin C have been reported.[31] Because it is not yet known, further study is warranted to determine how closely the PALB2 alterations described thus far share traits with cancers defined by BRCAness. Pseudopapillary neoplasm of the pancreas is a rare epithelial tumor comprising 1% to 2% of exocrine pancreatic neoplasms.[32] These tumors generally present as large encapsulated masses, although metastatic disease has been reported. Although several local therapies (eg, surgical resection, tumor embolization, and radiotherapy) have been described, no standard of care exists.[33]-[35] Our patient highlights the potential of CGP in identifying tumor-specific and actionable drug targets for uncommon neoplasms. [Extracted from the article]
- Published
- 2017
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