Sinonasal Small Cell Carcinoma-Case Series of a Rare Malignancy.

Sinonasal small cell neuroendocrine carcinoma (SNEC) is an extremely rare and aggressive neoplasm that can arise in the sinonasal region. These tumors are associated with high morbidity and mortality, are difficult to diagnose, and are hard to treat. We describe 2 cases of this poorly understood malignancy and review imaging, pathology, and treatment decisions. A 41-year-old male and a 64-year-old female presented to a tertiary center in 2019 after developing nasal obstruction and were found to have sinonasal masses on imaging. Both biopsies showed strong expression of pancytokeratin with dot-like reactivity and expression of neuroendocrine markers chromogranin and synaptophysin. The findings were diagnostic of SNEC. Staging positron emission tomography/computed tomography and brain MRI were performed, and patients were discussed at a multidisciplinary tumor board. Neither had distant metastatic disease at presentation. One patient had no intracranial or orbital disease and underwent a subtotal endoscopic resection with adjuvant chemoradiation. The other patient demonstrated middle cranial fossa, dural, and orbital involvement as well as cranial nerve V palsy. This patient was treated with induction chemotherapy, followed by concurrent chemoradiation. Both patients are presently alive at 4 months follow-up, but one with persistent local disease and the other distant metastasis. Sinonasal small cell neuroendocrine carcinoma is a rare and poorly understood malignancy with an aggressive clinical course. Continued careful review of pathology and study of molecular features are needed for improved understanding of SNEC, and particularly for head and neck SNEC, to establish a staging system and better formulate treatment protocols.

Treatment of Sinonasal Teratocarcinosarcoma: A Systematic Review and Survival Analysis.

BACKGROUND: Sinonasal teratocarcinosarcoma (SNTCS) is a rare malignancy of the anterior skull base with only 127 cases described in the English literature. Given the rarity of this tumor, new cases and analysis of published reports may assist in future management of SNTCS. OBJECTIVES: 1) Describe findings from a systematic review of all available literature for malignant SNTCS including the clinical presentation, treatment modalities and outcomes. 2) Present two new cases of this rare anterior skull base tumor. 3) Compare treatment outcomes with respect to recurrence and mortality. METHODS: A systematic review of all English literature available in 2 comprehensive databases was conducted by two independent reviewers using PRISMA guidelines. 85 publications were identified. Each case was reviewed for demographics, treatment and survival, and aggregate treatment outcomes were compared using Kaplan-Meier analysis. RESULTS: A total of 64 articles meeting inclusion criteria were reported in the literature between 1977-2018. This represented a total of 127 patients, with a strong male predominance (83%) and mean age of 50 years (range 10-82). Mean follow-up was 21 months. Recurrence rate was 38%, with mean survival at 2 years of 55%. Almost all patients underwent surgery as a primary treatment modality (90%). The majority of cases were treated with multimodal therapy, with 55% receiving surgery and radiation and 20% receiving surgery with adjuvant chemoradiation. Kaplan-Meier analysis demonstrated a significant survival advantage for patients treated with combined therapy compared to surgery alone (p < 0.001) but did not show differences in recurrence (p = 0.085). CONCLUSION: Two-year survival rates for SNTCS are 55%. Multimodality treatment outcomes appear to be superior to surgery alone based on the published data of this rare skull base tumor, although heterogeneity of treatment methods and reporting bias limits the generalizability of these findings.