Unusual presentation of ameloblastoma and a review of its biological behaviour

Authors

  • Choo Xiang Tan Department of Pathology, Faculty of Medicine, Universiti Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Cheras, Kuala Lumpur, Malaysia.
  • Nurwahyuna Rosli Department of Pathology, Faculty of Medicine, Universiti Kebangsaan Malaysia, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Cheras, Kuala Lumpur, Malaysia.

DOI:

https://doi.org/10.31436/ijohs.v7i2.501

Keywords:

ameloblastoma, maxillary tumour, nasal mass, odontogenic tumour

Abstract

Ameloblastoma is a benign odontogenic tumour occurring primarily in the mandible with rare occurrence outside the jaw. We report a case of an ameloblastoma presenting as a polypoidal mass in the right nasal cavity of a 72-year-old woman. She presented with progressive nasal blockage symptoms and examination revealed a mass occupying the right nostril. Initial tissue biopsy confirmed conventional ameloblastoma. Subsequent imaging study showed a mass involving the right maxilla. Primary tumour from the maxilla extending into the sinonasal tract was favoured over a much rarer primary ameloblastoma of the sinonasal tract, in view of the tumour bulk in the maxilla. Unfortunately, the patient defaulted her follow-up. Ameloblastomas are known to recur locally thus excision with clear margins is paramount. Recurrence is also influenced by factors such as the presence of B-Raf proto-oncogene (BRAF) and Smoothened, frizzled class receptor (SMO) genetic mutations, with SMO mutation found to be associated with maxillary location in the older age group. Ameloblastomas can metastasise without undergoing malignant cytological changes, with mandibular tumours being far more likely to do so. Long-term follow-up and regular computed tomography (CT) monitoring is required post excision.

References

Almeida Rde A., Andrade E.S., Barbalho J.C., Vajgel A., & Vasconcelos B.C. (2016). Recurrence rate following treatment for primary multicystic ameloblastoma: systematic review and meta-analysis. International Journal of Oral & Maxillofacial Surgery, 45(3), 359-367. https://doi.org/10.1016/j.ijom.2015.12.016

Bettoni J., Neiva C., Fanous A., Olivetto M., Demarteleire C., Dakpe S., et al. (2018). Brain ameloblastoma: metastasis or local extension case report and physipathological reflexion. Journal of Stomatology, Oral and Maxillofacial Surgery, 119(5), 436-439. https://doi.org/10.1016/j.jormas.2018.04.017

Brown N.A., Rolland D., McHugh J.B., Weigelin H.C., Zhao L., Lim M.S., et al. (2014). Activating FGFR2-RAS-BRAF mutations in ameloblastoma. Clinical Cancer Research. 20(21), 5517-5526. https://doi.org/10.1158/1078-0432.CCR-14-1069

Darshani Gunawardhana K.S., Jayasooriya P.R., Rambukewela I.K., & Tilakaratne W.M. (2010). A clinico-pathological comparison between mandibular and maxillary ameloblastomas in Sri Lanka. Journal of Oral Pathology & Medicine, 39(3), 236-241. https://doi.org/10.1111/j.1600-0714.2009.00850.x

Kaye F.J., Ivey A.M., Drane W.E., Mendenhall W.M., Allan R.W. (2014). Clinical and radiographic response with combined BRAF-targeted therapy in stage 4 ameloblastoma. Journal of the National Cancer Institute, 107(1), 378. https://doi.org/10.1093/jnci/dju378

Goh Y.C., Siriwardena B.S.M.S., & Tilakaratne W.M. (2021). Association of clinicopathological factors and treatment modalities in the recurrence of ameloblastoma: analysis of 624 cases. Journal of Oral Pathology & Medicine, 50, 927–936. https://doi.org/10.1111/jop.13228

Grynberg S., Vered M., Shapira-Frommer R., Asher N., Ben-Betzalel G., Stoff R., et al. (2024). Neoadjuvant BRAF-targeted therapy for ameloblastoma of the mandible: an organ preservation approach. Journal of the National Cancer Institute, 116(4), 539-546.

https://doi.org/10.1093/jnci/djad232. Journal of the National Cancer Institute, 116(4), 631. https://doi.org/10.1093/jnci/djad264

Gültekin S.E., Aziz R., Heydt C., Sengüven B., Zöller J., Safi A.F., et al. (2018). The landscape of genetic alterations in ameloblastomas relates to clinical features. Virchows Archiv European Journal of Pathology, 472(5), 807-814. https://doi.org/10.1007/s00428-018-2305-5

Hertog D., Bloemena E., Aartman I.H., & van-der-Waal I. (2012). Histopathology of ameloblastoma of the jaws; some critical observations based on a 40 years single institution experience. Medicina Oral, Patologia Oral, Cirugia Bucal. 17(1), e76-82. https://doi.org/10.4317/medoral.18006

Hosalkar R., Saluja T.S., Swain N., & Singh S.K. (2021). Prognostic evaluation of metastasizing ameloblastoma: A systematic review of reported cases in literature. Journal of Stomatology, Oral & Maxillofacial Surgery. 122, 192–198. https://doi.org/10.1016/j.jormas.2020.07.001

Masthan K.M., Anitha N., Krupaa J., & Manikkam S. (2015). Ameloblastoma. Journal of Pharmacy and Bioallied Sciences, Suppl 1, S167-S170. https://doi.org/10.4103/0975-7406.155891

Pandiar D., Anand R., Kamboj M., Narwal A., Shameena P.M., & Devi A. (2021). Metastasizing Ameloblastoma: A 10 Year Clinicopathological Review with an Insight Into Pathogenesis. Head and Neck Pathology, 15(3), 967-974. https://doi.org/10.1007/s12105-020-01258-5

Schafer D.R., Thompson L.D., Smith B.C., & Wenig B.M. (1998). Primary ameloblastoma of the sinonasal tract: a clinicopathologic study of 24 cases. Cancer, 82(4), 667-674. https://doi.org/10.1002/(sici)1097-0142(19980215)82:4<667::aid-cncr8>3.0.co;2-i

Siar C.H., Lau S.H., & Ng KH. (2012). Ameloblastoma of the jaws: a retrospective analysis of 340 cases in a Malaysian population. Journal of Oral and Maxillofacial Surgery, 70(3), 608-615. https://doi.org/10.1016/j.joms.2011.02.039

Sweeney R.T., McClary A.C., Myers B.R., Biscocho J., Neahring L., Kwei K.A., et al. (2014) Identification of recurrent SMO and BRAF mutations in ameloblastomas. Nature Genetics, 46(7), 722-725. https://doi.org/10.1038/ng.2986

Tilakaratne W.M., Odell E.W., Muller S., editors. (2023). Chapter 7: Odontogenic and maxillofacial bone tumours. In: WHO Classification of Head and Neck Tumours. 5th ed. IARC Publications; Lyon, France: 2023.

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Published

2026-07-31

How to Cite

Tan, C. X., & Rosli, N. (2026). Unusual presentation of ameloblastoma and a review of its biological behaviour. IIUM Journal of Orofacial and Health Sciences, 7(2), 245–249. https://doi.org/10.31436/ijohs.v7i2.501

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Section

Case Report