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.
Original language | English (US) |
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Article number | 105705 |
Journal | Oral Oncology |
Volume | 125 |
DOIs | |
State | Published - Feb 2022 |
Externally published | Yes |
Keywords
- Renal Cell-Like Adenocarcinoma
- Sinonasal
- Surgery
- Von Hippel Lindau syndrome
ASJC Scopus subject areas
- Oral Surgery
- Oncology
- Cancer Research