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 and provide a literature review of case reports and case series on non-syndromic multiple DOCs, particularly dentigerous cysts (DCs) and odontogenic keratocysts (OKCs).

The study included histologically 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.

Of a total of 377 patients, 286 had DCs, 85 OKCs, 5 orthokeratinizing odontogenic cysts (OOCs), 1 botryoid cyst, and 1 calcifying odontogenic cyst. The proportion of patients with multiple DCs in our study (6.6%) was higher than usually reported in the literature. The study also found that 100% of patients with multiple DCs did not exhibit any syndromic associations. On the other hand, 66% of multiple OKCs were associated with the Naevoid Basal Cell Carcinoma Syndrome (NBCCS) and the proportion of OKC patients with NBCCS (7%) was relatively higher than in other studies. Recurrence of OKCs was also significantly associated with NBCCS (p < 0.05). Only one patient presented with bilateral OOCs, without any association with a syndrome.

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. Still, we conclude that patients with multiple DOCs should be carefully considered for examination by other specialists to rule out possible syndromic involvement.

The association between genetic syndromes and odontogenic tumors encompasses several entities, reflecting the intricate interplay between genetic factors and the development of these lesions. The present study aimed to comprehensively investigate the associations between genetic syndromes and odontogenic tumors. We delineated the diverse spectrum of syndromic connections, including key syndromes such as Gardner syndrome, Gorlin syndrome, Schimmelpenning syndrome, and others. Our findings underscore the clinical significance of recognizing odontogenic tumors associated with genetic syndromes as diagnostic indicators for early intervention. We advocate for multidisciplinary collaboration among clinicians, geneticists, and researchers to deepen our understanding of the underlying mechanisms driving these syndromic associations. In light of this, our study contributes to the growing body of knowledge in dentistry and medical genetics, offering insights that may inform clinical practice and enhance patient care for individuals affected by genetic syndromes and odontogenic tumors.

Background: The most common odontogenic tumor is the solid ameloblastoma. This is concerning due to the progressive bone destruction in its intraosseous variant; this type of pathology often gets a delayed diagnosis due to the asymptomatic characteristic it has. Objective: The aim of the article is to propose a conservative treatment for multicystic ameloblastoma, reviewing the latest concepts, controversies, and treatment options described in the literature. Case Report: A patient arrives to our department with a diagnosis of unicystic ameloblastoma, so decompression and subsequent enucleation were decided as treatment. However, the new histopathological result was a multicystic ameloblastoma; with this result, we decided to continue with the initial treatment, which in fact had an adequate response. A 5-month radiographic follow-up was performed in which a decrease in size was evident and enucleation was decided. The postoperative results were favorable after a 2.5-year follow-up. Conclusion: The solid ameloblastoma may have a cystic component, in which there is the possibility of diagnostic errors when studying an incisional biopsy; for this reason, the definitive diagnosis should be established until complete enucleation of the lesion is performed. This characteristic in a solid ameloblastoma makes possible a positive response to decompression, making it easier to perform the enucleation subsequently, avoiding other aggressive treatments that can dramatically affect the patient's quality of life and also avoiding costly reconstruction bills.