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Published on: February 28, 2019
Sinonasal small cell neuroendocrine carcinoma: a systematic review of 80 patients
Alexander Rivero1, Jonathan Liang1
1Kaiser Permanente, Oakland, CA.
Background:
The objective of this study was to review the published literature related to sinonasal small cell neuroendocrine carcinomas (SCNECs). Clinical presentation, demographics, treatment, and outcomes of this uncommon disease are reported.
Methods:
A systematic review of studies for sinonasal SCNECs in PubMed and Ovid databases from 1970 to 2014 was conducted. Bibliographies of selected articles were also examined. Articles were examined for patient data that reported disease outcome.
Results:
Thirty-four articles were included in this analysis, comprising a total of 80 cases. The mean age of presentation was 53.0 years. Nasal obstruction (53.8%) and epistaxis (48.8%) were the most common presenting symptoms. The nasal cavity and septum (32.5%) was the most common site of involvement. Seventy percent of patients presented with American Joint Committee on Cancer (AJCC) stage IV disease. Combination chemotherapy and radiation therapy was the most common treatment modality, used in 21 cases (26.3%). The second and third most common treatment modalities was combination surgery and chemoradiation therapy (21.3%), and surgery alone (18.8%), respectively. A total of 37 patients (46.3%) were alive after a mean follow-up of 30.8 months (median 15.5 months), independent of treatment modality. Of the treatment modalities, multimodality therapy remained the most common therapy.
Conclusion:
This review contains the largest pool of sinonasal SCNEC patients to date. Sinonasal SCNEC is a rare and aggressive neoplasm, and there is currently no standard of care for treatment. Various treatment modalities have been employed. Our systematic review suggests that multimodality therapy remains the most common treatment of sinonasal SCNEC.
Insights
This review of sinonasal small cell neuroendocrine carcinomas (SCNECs) found that multimodality therapy is the most common treatment. Despite being aggressive, some patients survive long-term with various treatment approaches.
Area of Science:
- Oncology
- Otorhinolaryngology
- Pathology
Background:
- Sinonasal small cell neuroendocrine carcinomas (SCNECs) are rare and aggressive neoplasms.
- There is currently no established standard of care for treating SCNECs.
Purpose of the Study:
- To review published literature on sinonasal SCNECs.
- To report on clinical presentation, demographics, treatment, and outcomes.
Main Methods:
- Systematic review of PubMed and Ovid databases (1970-2014).
- Examination of bibliographies of selected articles.
- Analysis of patient data focusing on disease outcomes.
Main Results:
- Eighty cases from 34 articles were analyzed.
- Common symptoms include nasal obstruction (53.8%) and epistaxis (48.8%).
- Most patients presented with AJCC stage IV disease (70%); multimodality therapy was most common (46.3% alive).
Conclusions:
- This review represents the largest patient cohort for sinonasal SCNEC to date.
- Multimodality therapy is the most frequently utilized treatment approach.
- Sinonasal SCNEC remains an aggressive neoplasm with no standardized treatment protocol.
