Primary Neuroendocrine Carcinoma of the Palate: A Case Report
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Abstract
Primary neuroendocrine carcinomas (NECs) of the oral cavity represent exceptionally rare malignancies (< 30 reported cases globally), posing significant diagnostic challenges due to histological mimicry of other round cell tumors and nonspecific clinical presentation. This report details a novel case with atypical clinicopathological features and favorable therapeutic outcomes. A 38-year-old male presented with a 3-month history of a right hard palate ulceration. Clinical examination revealed an indurated endophytic lesion. Histopathology and immunohistochemistry confirmed poorly differentiated large cell NEC. Due to unresectable pterygopalatine fossa involvement, chemoradiation was administered. Given the extreme rarity of oral large cell neuroendocrine carcinoma (LCNEC), clinicians must consider this entity even in patients lacking traditional risk factors (e.g., smoking). Its aggressive growth pattern and metastatic propensity necessitate prompt specialist referral for early intervention. Keywords: Oral pathology; Neuroendocrine carcinoma; Large cell carcinoma.
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2. El-Naggar, Chan AK, Grandis J, Takata JR, Slootweg T. World Health Organization classification of head and neck tumours. 4. IARC Press; 2017.
3. Schuch LF, Schmidt TR, de Oliveira Zigmundo G, Kirschnick LB, Silveira FM, Martins MAT, et al. Oral and maxillofacial neuroendocrine carcinoma: A systematic review. Head Neck Pathol. 2021;16(2):525–37
4. Agea Martínez M, Navarro Cuéllar I, Sada Urmeneta A, Alijo Serrano F, Navarro Cuéllar C. Large cell neuroendocrine carcinoma on the floor of the mouth: a rare entity in an unusual location. Int J Oral Maxillofac Surg. 2022;51(6):742–5
5. Benzerdjeb N, Traverse-Glehen A, Philouze P, Bishop J, Devouassoux-Shisheboran M. Poorly differentiated neuroendocrine carcinoma of the head and neck: human papillomavirus tumour status/p16 status and impact on overall survival. Histopathology. 2020;76(4):581–91
6. Vanoli A. The spectrum of neuroendocrine neoplasia: A practical approach to diagnosis, classification and therapy by Sylvia L. Asa, Stefano La Rosa, Ozgur Mete. Endocr Pathol. 2023; 34:361–2
7. Bal M, Mittal N, Rane S, Rabade K, Patil A. Primary neuroendocrine carcinoma in oral and maxillofacial region: a comprehensive study of twelve rare cases including mixed neuroendocrine non-neuroendocrine carcinoma. Oral Surg Oral Med Oral Pathol Oral Radiol. 2024;138(2):e43
8. Bellizzi AM. Immunohistochemistry in the diagnosis and classification of neuroendocrine neoplasms: What can brown do for you? Hum Pathol. 2020; 96:8–33
9. Terada T. Pathologic diagnosis of large cell neuroendocrine carcinoma of the lung in an axillary lymph node: a case report with immunohistochemical and molecular genetic studies. Int J Clin Exp Pathol. 2013 May 15;6(6):1177-9
10. Perez-Ordoñez B. Neuroendocrine carcinomas of the larynx and head and neck: Challenges in classification and grading. Head Neck Pathol 2018;12(1):1–8
11. Gadducci A, Carinelli S, Aletti G. Neuroendrocrine tumors of the uterine cervix: A therapeutic challenge for gynecologic oncologists. Gynecol Oncol. 2017;144(3):637–46
12. Yan O, Xie W, Teng H, Fu S, Chen Y, Liu F. Nomograms forecasting long-term overall and cancer specific survival of patients with head and neck neuroendocrine carcinoma. Front Oncol. 2021; 11:619599.
| Files | ||
| Issue | Vol 12, No 3 (Summer 2025) | |
| Section | Case Report(s) | |
| Keywords | ||
| Oral pathology; Neuroendocrine carcinoma; Large cell carcinoma. | ||
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Fattahi B, Esmaeili A, Fallah A, Khalesi S. Primary Neuroendocrine Carcinoma of the Palate: A Case Report. J Craniomaxillofac Res. 2025;12(3):200-204.
