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Oral Oncol. 2017 Nov;74:21-29. doi: 10.1016/j.oraloncology.2017.09.010.

The clinicopathological spectrum of olfactory neuroblastoma and sinonasal neuroendocrine neoplasms: Refinements in diagnostic criteria and impact of multimodal treatments on survival.

Author information

1
Unit of Otorhinolaryngology, Department of Biotechnology and Life Sciences, University of Insubria, Ospedale di Circolo e Fondazione Macchi, Varese, Italy; Head and Neck Surgery & Forensic Dissection Research Center (HNS & FDRc), Department of Biotechnology and Life Sciences, University of Insubria, Varese, Italy. Electronic address: tzmario@inwind.it.
2
Unit of Pathology, Department of Medicine and Surgery, University of Insubria, Varese, Italy.
3
Unit of Otorhinolaryngology, Department of Biotechnology and Life Sciences, University of Insubria, Ospedale di Circolo e Fondazione Macchi, Varese, Italy; Head and Neck Surgery & Forensic Dissection Research Center (HNS & FDRc), Department of Biotechnology and Life Sciences, University of Insubria, Varese, Italy.
4
Unit of Biostatistics, Department of Statistics, Monzino Hospital, Milan, Italy.
5
Unit of Radiation Oncology, University of Insubria, Varese, Italy.
6
Unit of Otorhinolaryngology, Spedali Civili di Brescia, University of Brescia, Italy.
7
Unit of Pathology, Spedali Civili di Brescia, University of Brescia, Italy.
8
Ear, Nose and Throat Metropolitan Unit, Azienda Unità Sanitaria Locale di Bologna, Bologna, Italy.
9
Unit of Pathology, Bellaria Hospital, Department of Biomedical and Neuromotor Sciences, University of Bologna, Italy.
10
Service of Clinical Pathology, Institute of Pathology, Lausanne University Hospital, Lausanne, Switzerland.

Abstract

OBJECTIVES:

To provide a comprehensive review of the clinical and histopathological features of olfactory neuroblastoma (ONB) and other sinonasal neuroendocrine neoplasms (NENs), in order to refine diagnostic criteria, analyze treatment outcomes, and identify prognostic factors.

METHODS:

Data from an Italian multi-institutional database were analyzed. Patients were treated surgically via a minimally-invasive endoscopic approach followed by adjuvant radiotherapy or radiochemotherapy. Neoadjuvant cisplatin/etoposide chemotherapy was administered in cases of poorly-differentiated tumors. A centralized pathology review was performed in all cases. Patients were prospectively observed for survival. Overall (OS) and Disease-free survival (DFS) estimates were determined from Kaplan-Meier analysis and compared using the log-rank test. Statistically significant variables were entered in a multivariate Cox regression model.

RESULTS:

98 patients with a median follow-up of 53months were included. Morphology review and the incorporation of cytokeratin 8/18 in the immunohistochemical panel modified the final diagnosis in 8/98 (8.2%) cases. The neoplasms were ultimately classified into four groups with different immunohistochemical profiles and clinical behaviors: ONB in 67 cases (5-year-OS, 91.6%); NEC (poorly-differentiated neuroendocrine carcinoma) in 22 cases (5-year-OS, 42.6%); MiNEN (mixed neuroendocrine/non-neuroendocrine neoplasm) in five cases (5-year-OS, 0%,0/5 cases); and NET (well-differentiated neuroendocrine tumor) in four cases (5-year-OS, 50%, 2/4 cases). Hyams grade and Ki67 index were independent prognostic factors for ONB. Neoadjuvant chemotherapy appeared to be associated with improved OS and DFS for NEC, independent of other clinicopathological variables.

CONCLUSIONS:

Induction chemotherapy improves survival outcomes in patients affected by poorly-differentiated tumors. Recent advances in histopathological diagnosis, including CK8/18 staining, allow to plan the most appropriate range of multimodal treatments.

KEYWORDS:

Cytokeratin; Esthesioneuroblastoma; Ki67; Nasal cavity; Neuroendocrine carcinoma; Olfactory neuroblastoma; Paranasal sinuses

[Indexed for MEDLINE]

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