HPV-Related Multiphenotypic Sinonasal Carcinoma: A Case Report and Literature Review.

OBJECTIVES: Human papilloma virus-related multi phenotypic sinonasal carcinoma (HMSC), a recently characterized sinonasal malignancy, is discussed including histology, clinical presentation, and treatment outcomes. STUDY DESIGN: Case report with literature review. METHODS: A case of HMSC is reported, as well as a retrospective review of all cases of HMSC reported in the English literature from January 2000 through May 2018 in the MEDLINE, EMBASE, and Scopus databases. Case data from selected articles was pooled along with the presented case and analyzed. RESULTS: Including the present case report, a total of 57 cases of HMSC were identified through literature review. Of the 42 cases with staging information, 25 (60%) presented as early-stage disease (T1/T2). No nodal metastasis or disease-specific mortalities were reported. Among the 44 cases with posttreatment follow-up data, 16 cases (36.4%) developed local recurrence. The majority of recurrences occurred 24 to 60 months posttreatment, although reports of recurrence 10 and 29 years posttreatment exist. Local recurrence occurred in 40% and 60% of patients with perineural invasion and bone invasion, respectively. Patients who developed local recurrence had a longer disease-free interval when treated with adjuvant radiotherapy, which approached statistical significance. CONCLUSIONS: HMSC is a distinct entity with paradoxically aggressive morphology paired with an indolent clinical course characterized by high rates of local recurrence but no reported disease-specific mortalities to date. Surgery with or without adjuvant radiotherapy is the most common treatment modality, and adjuvant radiotherapy may be associated with an increased disease-free interval among patients with local recurrence. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:106-110, 2021.