Ameloblastic carcinoma is a rare malignant neoplasm arising from the odontogenic epithelium. Ameloblastic carcinoma commonly occurs de novo affecting the posterior segments of the mandible. Presently, only less than 100 cases have been reported arising from the maxilla. We report a unique case of maxillary ameloblastic carcinoma in a 68-year-old male with a 5.6 cm positron emission tomography (PET) avid left maxillary sinus mass. The patient underwent a left maxillectomy which revealed hyperchromatic and pleomorphic tumor cells arranged in a nested and trabecular architecture. The tumor cells showed distinct peripheral palisading with reverse polarization. Areas of bone destruction, necrosis, lymphovascular and perineural invasions, as well as atypical mitoses, were identified. Immunohistochemically, the tumor cells were positive for keratin cocktail (AE1/AE3 and CAM 5.2), keratin 19, p40, and weakly positive for MDM2, while negative for calretinin. Molecular analysis revealed wild-type BRAF; however, alterations in CDKN2A/B, MTAP, RB1, SMARCA4, STK11, FGF12, SETD2, and TP53 were present. This histopathologic and molecular profile supported the diagnosis of ameloblastic carcinoma. There has been no evidence of disease recurrence or metastasis eleven months after the initial diagnosis.
DengLWangRYangMLiWZouL. Ameloblastic carcinoma: clinicopathological analysis of 18 cases and a systematic review. Head Neck. 2019;41(12):4191‐4198. doi:10.1002/hed.25926.
2.
ElzayRP. Primary intraosseous carcinoma of the jaws. Review and update of odontogenic carcinomas. Oral Surg Oral Med Oral Pathol. 1982;54(3):299‐303. doi:10.1016/0030-4220(82)90099-8.
3.
BenlyazidALacroix-TrikiMAzizaRGomez-BrouchetAGuichardMSariniJ. Ameloblastic carcinoma of the maxilla: case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007;104(6):e17‐e24. doi:10.1016/j.tripleo.2007.05.026.
4.
SlootwegPJMüllerH. Malignant ameloblastoma or ameloblastic carcinoma. Oral Surg Oral Med Oral Pathol. 1984;57(2):168‐176. doi:10.1016/0030-4220(84)90207-x.
5.
BarnesLEvesonJWReichartP, et al.World Health Organization Classification of tumours: Pathology and genetics of tumours of the head and neck. IARC Press; 2005.
6.
WHO Classification of Tumours Editorial Board. Head and neck tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2022, (WHO classification of tumours series, 5th ed.; vol. 9). Available from: https://tumourclassification.iarc.who.int/chapters/52.
7.
El-NaggarAKChanJKGrandisJR. World Health Organization Classification of Head and Neck Tumours. 4th ed.International Agency for Research on Cancer; 2017.
8.
MoroAForestaEGaspariniG, et al.Ameloblastic carcinoma of the maxilla: a case report and an updated review of the literature. Oncol Lett. 2016;12(6):4339‐4350. doi:10.3892/ol.2016.5272.
9.
UzawaNSuzukiMMiuraCTomomatsuNIzumoTHaradaK. Primary ameloblastic carcinoma of the maxilla: a case report and literature review. Oncol Lett. 2015;9(1):459‐467. doi:10.3892/ol.2014.2654.
10.
KruseALZwahlenRAGrätzKW. New classification of maxillary ameloblastic carcinoma based on an evidence-based literature review over the last 60 years. Head Neck Oncol. 2009;1:31. Published 2009 Aug 12. doi:10.1186/1758-3284-1-31.
11.
CasarotoARToledoGLFilhoJLSoaresCTCapelariMMLaraVS. Ameloblastic carcinoma, primary type: case report, immunohistochemical analysis and literature review. Anticancer Res. 2012;32(4):1515‐1525.
12.
OdellEMullerSRichardsonMS. Ameloblastic carcinoma. In: WHO Classification of Tumours Editorial Board, ed. Head and neck tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2022. (WHO classification of tumours series, 5th ed.; vol. 9). Available from: https://tumourclassification.iarc.who.int/chapters/52.
13.
RooperLMBishopJFluckeUWeinrebI. Ewing sarcoma. In: WHO Classification of Tumours Editorial Board, ed. Head and neck tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2022. (WHO classification of tumours series, 5th ed.; vol. 9). Available from: https://tumourclassification.iarc.who.int/chapters/52.
14.
Agaimy A, Din NU, JainDRooperLMBalMMittalN. SWI/SNF complex-deficient sinonasal carcinoma (SMARCB1 & SMARCA4). In: WHO Classification of Tumours Editorial Board, ed. Head and neck tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2022. (WHO classification of tumours series, 5th ed.; vol. 9). Available from: https://tumourclassification.iarc.who.int/chapters/52.
15.
French CA, Stelow EB, MinatoH. NUT Carcinoma. In: WHO Classification of Tumours Editorial Board, ed. Head and neck tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2022. (WHO classification of tumours series, 5th ed.; vol. 9). Available from: https://tumourclassification.iarc.who.int/chapters/52.
16.
BishopJA, Wenig BM. Basaloid squamous cell carcinoma. In: WHO Classification of Tumours Editorial Board, ed. Head and neck tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2022. (WHO classification of tumours series, 5th ed.; vol. 9). Available from: https://tumourclassification.iarc.who.int/chapters/52.
17.
Bilodeau EA, Maiorano E, NevilleBW. Clear cell odontogenic carcinoma. In: WHO Classification of Tumours Editorial Board, ed. Head and neck tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2022. (WHO classification of tumours series, 5th ed.; vol. 9). Available from: https://tumourclassification.iarc.who.int/chapters/52.
18.
BilodeauEAWeinrebIAntonescuCR, et al.Clear cell odontogenic carcinomas show EWSR1 rearrangements: a novel finding and a biological link to salivary clear cell carcinomas. Am J Surg Pathol. 2013;37(7):1001‐1005. doi:10.1097/PAS.0b013e31828a6727.
19.
KoutlasIG, Sloan P. Primary intraosseous carcinoma, NOS. In: WHO Classification of Tumours Editorial Board, ed. Head and neck tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2022. (WHO classification of tumours series, 5th ed.; vol. 9). Available from: https://tumourclassification.iarc.who.int/chapters/52.
20.
JoVY, Agaimy ADinNUFranchiA. Sinonasal undifferentiated carcinoma. In: WHO Classification of Tumours Editorial Board, ed. Head and neck tumours [Internet; beta version ahead of print]. International Agency for Research on Cancer; 2022. (WHO classification of tumours series, 5th ed.; vol. 9). Available from: https://tumourclassification.iarc.who.int/chapters/52.
21.
BrunnerPBihlMJundtGBaumhoerDHoellerS. BRAF P.V600E mutations are not unique to ameloblastoma and are shared by other odontogenic tumors with ameloblastic morphology. Oral Oncol. 2015;51(10):e77‐e78. doi:10.1016/j.oraloncology.2015.07.010.
22.
DinizMGGomesCCGuimarãesBV, et al.Assessment of BRAFV600E and SMOF412E mutations in epithelial odontogenic tumours. Tumour Biol. 2015;36(7):5649‐5653. doi:10.1007/s13277-015-3238-0.
23.
KawauchiSHayatsuYTakahashiM, et al.Spindle-cell ameloblastic carcinoma: a case report with immunohistochemical, ultrastructural, and comparative genomic hybridization analyses. Oncol Rep. 2003;10(1):31‐34.
24.
KhojastehAKhodayariARahimiF, et al.Hypermethylation of p16 tumor-suppressor gene in ameloblastic carcinoma, ameloblastoma, and dental follicles. J Oral Maxillofac Surg. 2013;71(1):62‐65. doi:10.1016/j.joms.2012.04.033.
25.
NobusawaASanoTYokooSOyamaT. Ameloblastic carcinoma developing in preexisting ameloblastoma with a mutation of the p53 gene: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol. 2014;118(5):e146‐e150. doi:10.1016/j.oooo.2014.03.021.
26.
BartelsSAdisaAAladelusiT, et al.Molecular defects in BRAF wild-type ameloblastomas and craniopharyngiomas-differences in mutation profiles in epithelial-derived oropharyngeal neoplasms. Virchows Arch. 2018;472(6):1055‐1059. doi:10.1007/s00428-018-2323-3.
27.
AnsariHARayPSKhanNKhanAH. Spindle-cell ameloblastic carcinoma of the maxilla with adenoid cystic carcinoma-like areas: a new variant. Indian J Pathol Microbiol. 2015;58(4):513‐515. doi:10.4103/0377-4929.168856.
28.
YıldırımAEDivanlıoğluDKaraoğluD, et al.Massive skull base ameloblastic carcinoma with intracranial extension. Turk Neurosurg.2017;27(6):1016‐1020.
29.
FitzpatrickSGHirschSAListinskyCMLyuDJBaurDA. Ameloblastic carcinoma with features of ghost cell odontogenic carcinoma in a patient with suspected Gardner syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol. 2015;119(4):e241‐e245. doi:10.1016/j.oooo.2014.09.028.
30.
FometeBAdebayoETAyubaGIOkekeUA. Ameloblastic carcinoma of the maxilla: a report of two cases and a review of the literature. J Korean Assoc Oral Maxillofac Surg. 2016;42(1):43‐46. doi:10.5125/jkaoms.2016.42.1.43.
31.
MahmoudSAMAmerHWMohamedSI. Primary ameloblastic carcinoma: literature review with case series. Pol J Pathol. 2018;69(3):243‐253. doi:10.5114/pjp.2018.79544.
32.
YamagataKIshikawaHSaitoTBukawaH. Proton beam therapy for ameloblastic carcinoma of the maxilla: report of a rare case. J Oral Maxillofac Surg. 2019;77(1):227.e1‐227.e5. doi:10.1016/j.joms.2018.08.014.
33.
LandeenKSpanosWCPowellS. A rare presentation of ameloblastic carcinoma of the Sinus cavity and skull base. Cureus. 2019;11(12):e6265. Published 2019 Dec 1. doi:10.7759/cureus.6265.
34.
McLean-HoldenACBishopJAKesslerHP, et al.Spindle-cell variant of ameloblastic carcinoma: a report of 3 cases and demonstration of epithelial-mesenchymal transition in tumor progression. Oral Surg Oral Med Oral Pathol Oral Radiol. 2019;128(3):e113‐e121. doi:10.1016/j.oooo.2019.01.071.
35.
AokiTAkibaTKondoYSasakiMKajiwaraHOtaY. The use of radiation therapy in the definitive management of ameloblastic carcinoma: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol. 2019;127(2):e56‐e60. doi:10.1016/j.oooo.2018.09.009.
36.
KosanwatTPoomsawatSJuengsomjitR. Ameloblastic carcinoma ex ameloblastoma of the maxilla. J Oral Maxillofac Pathol. 2019;23(Suppl 1):58‐62. doi:10.4103/jomfp.JOMFP_116_17.
37.
TakayamaKNakamuraTTakadaA, et al.Proton beam therapy combined with retrograde intra-arterial infusion chemotherapy for an extremely rapid growing recurrent ameloblastic carcinoma: a case report. Mol Clin Oncol. 2020;13(4):34. doi:10.3892/mco.2020.2104.
38.
TarleMMüllerDTarleABlivajsIAljinović RatkovićNKneževićP. Challenges in the diagnostics and treatment of ectopic ameloblastic carcinoma: a case report. Croat Med J. 2020;61(3):271‐275. doi:10.3325/cmj.2020.61.271.
IsmailTHaumerALungerA, et al.Case report: reconstruction of a large maxillary defect with an engineered, vascularized, prefabricated bone graft. Front Oncol. 2021;11:775136. Published 2021 Dec 6. doi:10.3389/fonc.2021.775136.
41.
XuZShangJYangNLongLZhangP. Reconstruction of soft tissue defect after maxillectomy of ameloblastic carcinoma with submental island flap: a case report and literature review. Ann Transl Med. 2021;9(23):1746. doi:10.21037/atm-21-5196.
