Sinonasal renal cell-like adenocarcinoma arising in von Hippel Lindau (VHL) syndrome. Maharaj, S., Seegobin, K., Wakeman, K., Chang, S., Potts, K., Williams, B., & Redman, R. Oral Oncology, 125:105705, February, 2022. doi abstract bibtex Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare and relatively novel diagnosis. Hereditary and somatic genomic signatures are not well defined in this disease. We report the case of a 35-year-old African-American male with von Hippel Lindau (VHL) syndrome who developed SNRCLA. He underwent surgical resection followed by adjuvant radiation and has no recurrence one year from diagnosis. A review of the literature yielded two similar cases in the setting of VHL. In our case with associated VHL syndrome, next generation sequencing detected MST1R mutation, a possible driver. SNRCLA is an emerging tumor associated with VHL syndrome and it is hoped that future studies shed light on the underlying biology of this unique tumor.
@article{maharaj_sinonasal_2022,
title = {Sinonasal renal cell-like adenocarcinoma arising in von {Hippel} {Lindau} ({VHL}) syndrome},
volume = {125},
issn = {1879-0593},
doi = {10.1016/j.oraloncology.2021.105705},
abstract = {Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare and relatively novel diagnosis. Hereditary and somatic genomic signatures are not well defined in this disease. We report the case of a 35-year-old African-American male with von Hippel Lindau (VHL) syndrome who developed SNRCLA. He underwent surgical resection followed by adjuvant radiation and has no recurrence one year from diagnosis. A review of the literature yielded two similar cases in the setting of VHL. In our case with associated VHL syndrome, next generation sequencing detected MST1R mutation, a possible driver. SNRCLA is an emerging tumor associated with VHL syndrome and it is hoped that future studies shed light on the underlying biology of this unique tumor.},
language = {eng},
journal = {Oral Oncology},
author = {Maharaj, Satish and Seegobin, Karan and Wakeman, Kristina and Chang, Simone and Potts, Kevin and Williams, Brian and Redman, Rebecca},
month = feb,
year = {2022},
pmid = {34998175},
pages = {105705},
}
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