In our most recent publication in Genes, Chromosomes and Cancer, Alexandra and Waihay have characterized an interesting biphenotypic sinonasal sarcoma with a PAX3-FOXO1 fusion gene. We found the PAX3-FOXO1 genetic fusion characteristic of a highly aggressive, lethal sarcoma (alveolar rhabdomyosarcoma) driving a locally-aggressive, but essentially non-metastatic tumor; how’s that for cellular/genetic context and tumor microenvironment modulating oncogenic events? Our modest case report contributes to an increasing understanding of the genetics of biphenotypic sinonasal sarcoma, and raises interesting biological questions that are also being explored by colleagues from Mayo Clinic and MSKCC in larger case series.
Our DFSP work, characterizing CDK4 as a therapeutic target in imatinib-resistant DFSP and introducing the first patient-derived representative model of FS-DFSP, has been published in the AACR journal Molecular Cancer Therapeutics. Check it out here.
This nice study led by Leona Doyle and Jason Hornick demonstrates that epithelioid FH is yet another ALK-rearranged neoplasm (!), providing interesting insights regarding epithelioid FH biology, nosology and diagnostics. Read the full story in Modern Pathology (Epub on April 10).
Cher-wei Liang and JC Lee, friends and colleagues from Taipei, led this study that identifies recurrent FN1-FGFR1 gene fusions in phosphaturic mesenchymal tumor, a rare neoplastic process that induces osteomalacia by secreting factors such as FGF23. Published in March 2015 in The Journal of Pathology.
Food for thought! A little commentary on the potential of studying the frequently overlooked but interesting biology of benign tumors. Take a look here.
Timely review on Biologically important refinements in subclassification of round cell sarcomas. Check it out here.
A fantastic new publication from Jonathan Fletcher’s lab just came out in Nature Genetics. Dystrophin is a Tumor Supressor in Myogenic Sarcomas. Check it out!
An interesting new publication from our clinical colleagues in endocrinology just came out in The New England Journal. D3 activity in GIST, leading to hypothyroidism. Check it out here.