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Oral and Maxillofacial Neuroendocrine Carcinoma: A Systematic Review

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Abstract

The aim of the present study was to integrate the available data published in the literature on oral and maxillofacial neuroendocrine carcinomas concerning the demographic, clinical and histopathological features of this condition. An electronic search with no publication date restriction was undertaken in April 2021 in four databases. Eligibility criteria included reports published in English having enough data to confirm a definite diagnosis, always showing a neuroendocrine marker. Cases originating in the oropharynx, including base of the tongue and tonsils, were excluded. Outcomes were evaluated by the Kaplan–Meier method along with Cox regression. Twenty-five articles (29 cases) from nine different countries were detected. Mean patient age was 56.3 (± 17.5) years, with a slight male predilection. Symptomatology was present in 72.2% of informed cases. Regarding clinical presentation, a non-ulcerated nodule located in the gingiva with a mean size of 3.4 (± 2.0) cm was most frequently reported. Concomitant metastasis was identified in seven individuals. Histopathologically, most neoplasms were of the small cell type, and immunohistochemistry for both epithelial and neuroendocrine differentiation was used in 65.5% cases. Radical surgery was the treatment of choice in almost all cases, with or without adjuvant therapy. Mean follow-up was 20.5 (± 21.2) months, and only four patients developed recurrences. Eleven (44.0%) individuals died due to the disease. Ulcerated lesions were a prognostic factor. This study provides knowledge that can assist surgeons, oncologists, and oral and maxillofacial pathologists with the diagnosis and management of neuroendocrine carcinomas. Our findings demonstrated that the long-term prognosis of this lesion continues to be poor.

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Acknowledgements

The authors thank the Coordination for the Improvement of Higher Education Personnel (CAPES, Finance Code 001), Brazil. L.F.S. and L.B.K. are the recipients of fellowships. We also acknowledge the Brazilian National Council for Scientific and Technological Development (CNPq). F.P.F., J.N.S., P.A.V. and M.D.M. are research fellows of CNPq.

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Authors and Affiliations

  1. Department of Oral Diagnosis, Piracicaba Dental School, Universidade Estadual de Campinas, Piracicaba, São Paulo, Brazil

    Lauren Frenzel Schuch & Laura Borges Kirschnick

  2. Department of Oral Pathology, Faculdade de Odontologia, School of Dentistry, Universidade Federal do Rio Grande do Sul, R. Ramiro Barcelos, 2492, room 503, Porto Alegre, Rio Grande do Sul, CEP: 90035-003, Brazil

    Tuany Rafaeli Schmidt, Gisele de Oliveira Zigmundo, Marco Antonio Trevizani Martins & Manoela Domingues Martins

  3. Molecular Pathology Area, School of Dentistry, Universidad de la República (UDELAR), Montevideo, Uruguay

    Felipe Martins Silveira

  4. Department of Oral Medicine, Hospital de Clínicas de Porto Alegre, Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil

    Marco Antonio Trevizani Martins

  5. Pathology Section, Centro Clínico de Cabeza y Cuello/Hospital Herrera Llerandi, Guatemala City, Guatemala

    Roman Carlos

  6. Department of Pathology, Postgraduate Program in Dentistry and Health, School of Dentistry, Universidade Federal da Bahia, Salvador, Bahia, Brazil

    Jean Nunes Dos Santos

  7. Department of Oral Surgery and Pathology, School of Dentistry, Universidade Federal de Minas Gerais, Belo Horizonte, Minas Gerais, Brazil

    Felipe Paiva Fonseca

  8. Department of Oral Diagnosis, Piracicaba Dental School, Universidade Estadual de Campinas, Piracicaba, São Paulo, Brazil

    Pablo Agustin Vargas & Manoela Domingues Martins

  9. Academic Unit of Oral and Maxillofacial Medicine and Pathology, Department of Clinical Dentistry, University of Sheffield, Sheffield, UK

    Vivian Petersen Wagner

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  1. Lauren Frenzel Schuch
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  12. Manoela Domingues Martins
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Contributions

Study concepts and Study design: LFS, MDM; Data acquisition: LFS, TRS, GOZ; Quality control of data and algorithms: LBK, FMS; Data analysis and interpretation and Manuscript preparation: LFS, VPW; Statistical analysis: VPW; Manuscript editing: MATM, RC, JNS, FPF; Manuscript review: PAV, MSM.

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Correspondence to Manoela Domingues Martins.

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Schuch, L.F., Schmidt, T.R., de Oliveira Zigmundo, G. et al. Oral and Maxillofacial Neuroendocrine Carcinoma: A Systematic Review. Head and Neck Pathol 16, 525–537 (2022). https://doi.org/10.1007/s12105-021-01398-2

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