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HPV-Related Multiphenotypic Sinonasal Carcinoma: Four Cases that Expand the Morpho-Molecular Spectrum and Include Occupational Data

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Abstract

HPV-related multiphenotypic sinonasal carcinoma (HMSC) is a recently described distinct tumor entity of the sinonasal tract associated with high-risk subtypes of human papilloma virus (HPV), predominantly type 33. The biological behavior seems to be less aggressive than the often high-grade, highly proliferative morphology implies; however, recurrences are frequent. Most of the cases present as polypoid tumors within the nasal cavity. Microscopic morphology frequently encompasses adenoid cystic-like features or features reminiscent of other salivary gland tumors. Here, we describe four cases of this rare entity, all observed in women. The polypoid tumors were within the nasal cavity, leading to obstruction, facial pain and epistaxis. The morphology was predominantly basaloid, solid and adenoid cystic-like in two of four cases, one with additional glomeruloid features. Another case showed basaloid tumor cells with prominent mature squamous differentiation and extensive keratinization. A single case showed a predominantly solid and reticular growth pattern. All cases were diffusely positive for p16 (100%), expressed SOX10, LEF-1 and partially S-100, and harbored HPV high-risk types 33, 56 (2×) and 82. No recurrences or metastases were detectable after 3–50 months of follow-up. Of note, three of four patients were nurses/nursing assistant. We expand the morphological spectrum by describing a glomeruloid growth pattern and extensive mature keratinization, and add HPV type 82 to the molecular spectrum. The finding of HMSC among predominantly nurses in our cohort warrants further epidemiological studies in larger cohorts.

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Contributions

Study conception and design was established by Niels J. Rupp and Grégoire B. Morand. Material preparation and data collection were performed by Niels J. Rupp, Ulrike Camenisch, Kati Seidl, Elisabeth J. Rushing, Martina A. Broglie, David Holzmann and Grégoire B. Morand. David Holzmann was mainly responsible for patient care and surgery. Analyses were performed by Niels J. Rupp, Nanina Anderegg and Grégoire B. Morand. The first draft of the manuscript was written by Niels J. Rupp and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Niels J. Rupp.

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All patients gave written consent and the study was approved by the local ethics committee (Protocol number 2016–0162 incl. amendment of February 24th, 2018).

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Rupp, N.J., Camenisch, U., Seidl, K. et al. HPV-Related Multiphenotypic Sinonasal Carcinoma: Four Cases that Expand the Morpho-Molecular Spectrum and Include Occupational Data. Head and Neck Pathol 14, 623–629 (2020). https://doi.org/10.1007/s12105-019-01079-1

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