Background
This retrospective study aims to evaluate the relative representation of individual types of developmental odontogenic cysts (DOCs), especially from the perspective of syndromic and non-syndromic multiple DOCs in the Czech population. In addition, we also summarize the previous studies on the occurrence of multiple DOCs as well as provide a literature review of case reports and case series on non-syndromic multiple DOCs, mainly dentigerous cysts and odontogenic keratocysts.
Methods
The study included histologicaly confirmed DOCs retrieved between January 1, 2012, and August 8, 2023, at the Clinic of Maxillofacial Surgery, University Hospital Brno, Czech Republic. All specimens were re-classified according to the fifth edition of the World Health Organization Classification of Head and Neck Tumors, 2022. Patients with an uncertain histological diagnosis were excluded from the study.
Results
Of a total of 377 patients, 286 had dentigerous cysts (DCs), 85 odontogenic keratocysts (OKCs), 5 orthokeratinizing odontogenic cysts (OOCs), 1 botryoid cyst and 1 calcifying odontogenic cyst. 19 multiple cysts were observed in the DC group, all without any association with a syndrome. Among the OKCs, 6 patients were diagnosed with the Naevoid Basal Cell Carcinoma Syndrome (NBCCS) and 3 were non-syndromic. Recurrence of OKCs was associated with NBCCS (p < 0.05). OKCs appeared more frequently in younger patients and predominantly in women, while the opposite was observed in DCs (p < 0.05 for all). Both these lesions affected the mandible in the vast majority of cases. Only one patient presented with bilateral OOCs, without any association with a syndrome.
Conclusion
Multiple OKCs are more likely to develop in syndromic patients, while none of the multiple DCs were associated with a syndrome. The incidence of multiple OOCs and other DOCs is extremely rare.