Head & Neck Institute Outcomes
Head and Neck Surgery and Oncology
Clinical Management of Emerging Sinonasal Malignancies
Several emerging sinonasal malignancies have recently been described in the pathology literature. Although not all distinctly classified by the World Health Organization, these rare tumors present a management challenge to surgeons and oncologists. While prior studies have summarized histologic details, a clinically focused review is currently lacking in the literature. This review describes the presentation, histopathology, imaging, treatment, and prognosis of newly described or recently evolving sinonasal malignancies while highlighting the distinguishing features of these entities. It includes teratocarcinosarcoma, human papillomavirus-related multiphenotypic carcinoma, biphenotypic sinonasal sarcoma, sinonasal renal cell-like adenocarcinoma, NUT-midline carcinoma, squamous cell carcinoma associated with inverted papilloma, sinonasal undifferentiated carcinoma, and INI-1-deficient sinonasal carcinoma. By describing the diagnosis, treatment, and prognosis of these recently defined entities, this clinical review aims to help guide oncologists in the clinical management of these patients.
Presentation and Diagnosis of Emerging Sinonasal Malignancies
| Pathology | Presentation | Histopathology | Imaging Findings | Differential |
|---|---|---|---|---|
| Teratocarcinosarcoma | 7:1 male, 40-60 y | Neuroectodermal and epithelial and mesenchymal elements | Contrast enhancing on MRI | Esthesioneuroblastoma, SCC, adenocarcinoma small/ large cell carcinoma |
| HPV-related multiphenotypic sinonasal carcinoma | 1.5:1 female, 40-60 y | Salivary appearance with overlapping squamous dysplasia. HPV subtype 33 | Often locally destructive | Basaloid SCC, adenoid cystic, adenosquamous carcinoma |
| Biphenotypic sinonasal sarcoma | 2-3:1 female, 40-60 y | Infiltrative spindled cells in intersecting fascicules, “herringbone” pattern | Superior sinonasal tract and ethmoids | Nerve sheath tumors, synovial sarcomas, rhabdomyosarcomas |
| Renal cell-like adenocarcinoma | 2:1 female, 50-70 y | Cuboidal or columnar cells with clear cytoplasm forming nests and follicles | Vascular, T2 heterogeneous, avid enhancement | Adenocarcinoma, renal or clear cell carcinoma, esthesioneuroblastoma |
| NUT midline carcinoma | 1.5 female, 10-30 y | NUT expression, “abrupt” keratinization, monotonous primitive round cells | T1 hypointense, T2 heterogeneous, bony hyperostosis | Small round blue cell tumors, SNUC |
| SCC associated with inverted papilloma | Slight male, 50-70 y, smokers | Disorganized papillary epithelia, dysplasia, basement membrane loss | Maxillary sinus, loss of convoluted cerebriform pattern | NUT midline, INI-1-deficient sinonasal carcinoma |
| SNUC | 2-3:1 male, 50-60 y | Undifferentiated cells, highgrade, no glandular or squamous features | Low apparent diffusion coefficient ratio, FDG avid | Neuroendocrine, INI-1-deficient carcinoma, SCC, esthesioneuroblastoma |
| INI-1-deficient sinonasal carcinoma | Slight male, 50-60 y | Plasmacytoid or rhabdoid tumor cells in a population of basaloid cells | Low-intermediate T2, enhancing, FDG avid, calcifications | Basaloid SCC, NUT carcinoma, SNUC |
Treatment and Prognosis of Emerging Sinonasal Malignancies
| Pathology | Stage/Classification | Metastasis | Treatment | Recurrence | All-cause survival |
|---|---|---|---|---|---|
| Teratocarcinosarcoma | Advanced | 10% regional or distant | Surgery with adjuvant chemoradiation | 30% at 40 mo | 60% at 39 mo |
| HPV-related multiphenotypic sinonasal carcinoma | Locally destructive, 40% T3-T4 | No regional reported 5% distant | Surgery +/- radiation | 40% at 42 mo | No disease-related deaths at 42 mo |
| Biphenotypic sinonasal sarcoma | 20%-30% extra-sinonasal extension | None reported | Surgery +/- radiation | 50% at 5-8 y | 90%-95% at 5-8 y |
| Renal cell-like adenocarcinoma | Indolent, low grade | None reported | Surgery | 15% at 2 y | 100% at 2 y |
| NUT midline carcinoma | Infiltrating, Intradural/orbit involvement | 25% regional, 6% distant | Surgery with adjuvant chemoradiation | 75% progression at 2 y | 30% at 2 y |
| SCC associated with inverted papilloma | 70% T3-T4 | < 5% regional, 10% distant | Surgery +/- radiation, systemic therapy for T4 | 30% at 5 y | 65% at 3 y |
| Sinonasal undifferentiated carcinoma | Early invasion, advanced 80% T4 | 5%-20% regional and distant | Surgery with radiation or chemoradiation | 50% at 3 y | 40% at 5 y |
| INI-1-deficient sinonasal carcinoma | Universally advanced, 80% T4 | 10% regional, 35% distant | Surgery with chemoradiation or radiation | 30% at 1 y | 30% at 2 y |
Several emerging pathologies have been described for sinonasal carcinoma, including teratocarcinosarcoma, HPV-related multiphenotypic carcinoma, biphenotypic sarcoma, renal cell-like carcinoma, NUT-midline, SCC associated with IP, SNUC, and INI-1-deficient sinonasal carcinoma. By describing the diagnosis, treatment, and prognosis of these malignancies, this review aids providers in the clinical management of patients with these rare and poorly understood diseases.
