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Background The rarity of juvenile psammomatoid ossifying fibroma (JPOF) and lack of cytogenetic studies prompted us to report a novel SETD2 gene mutation in a benign odontogenic tumour. Case presentation A 21-year-old man presented with a hard, expanded mandibular cortex. Computed tomography revealed multilocular radiopacity in the mandible; this was reconstructed via segmental mandibulectomy using a vascularised iliac crest flap. Based on the clinical and histological findings, we diagnosed JPOF associated with an aneurysmal bone cyst. Microscopically, the solid area was characterised by many rounded or angular ossicles in a cellular fibrous stroma. The stromal cells were spindle-like or stellate. Next-generation sequencing detected a frame shift mutation of the SETD2 gene, while the copy number was normal. Conclusions Our findings suggest further genetic studies should be performed to assess whether this mutation is related to tumour genesis.

Idiopathic condylar resorption (ICR), though a rare event, is associated with severe detrimental sequelae for the patient. To date, the etiology remains unknown, and treatment strategies are highly controversial. Therefore, the aim of this study is to present an analysis of the consensus- and evidence-based approach to ICR by a German interdisciplinary guideline project of the AWMF (Association of the Scientific Medical Societies in Germany). Following a systematic literature search, including 56 (out of an initial 97) publications, with a predominantly low level of evidence (LoE), two independent working groups (oral and maxillofacial surgery and interdisciplinary, respectively) voted on a draft comprising 25 recommendations in a standardized anonymized and blinded Delphi procedure. While the results of the votes were relatively homogeneous, the interdisciplinary phase required a significantly higher number of rounds (p < 0.001). Most of the controversial recommendations were related to initial imaging (with consensus on CT/CBCT as the current diagnostic standard for imaging), pharmacotherapy (no recommendation due to lack of evidence), discopexy (no recommendation possible due to low LoE) and timing of orthognathic surgery (with consensus on two-staged procedures after invasive TMJ surgery, except for single-stage procedures if combined with total joint reconstruction). Overall, the Delphi procedure resulted in an interdisciplinary guideline offering the best possible evidence- and consensus-based expertise to date in the diagnosis and treatment of ICR.

Management of central giant cell granuloma (CGCG) presents a clinical challenge. While eradicating a lesion known for its high recurrence rate calls for radical surgical approaches, these cause significant esthetic and functional impairment. We present an eight-year-old boy suffering from an extraordinarily large CGCG expanding into the mandible and base of the mouth in the whole anterior region. Combined treatment with surgical intervention and corticosteroid application was successfully applied, and all six attached dental germs could be preserved. Different approaches for clinical management in pediatric cases are discussed.