| Summary: | Background: In general, central giant cell granuloma (CGCG) is a very rare benign osseous lesion of the jaws, more frequently occurring in the mandible than the maxilla. Other regions, such as skull base, ethmoid sinus or temporal bone, are far less common and only seldomly reported. Patients are usually younger than 30 years with a slightly higher occurrence rate in females than males. CGCG usually presents as unspecific incidental radiological finding or with unspecific clinical symptoms, e.g., indolent swelling. Lesions can show aggressive behaviour. Its aetiology remains unclear, syndromic occurrence is reported. Final diagnosis of CGCG is based on histopathological and molecular findings but remains difficult due to numerous differential diagnoses. Surgical therapy is the most common treatment, nevertheless other treatment modalities including several primary and adjuvant drug therapies, e.g., topical injection of steroids, systemic calcitonin, interferon-α, RANKL-inhibitors and tyrosine kinase inhibitors, as well as radiotherapy have been discussed with varying effects. Though, recurrence rates differ widely and range up to 72%. Due to the rarity of CGCG randomized controlled trials are still missing. Case Description: We present a seldom case of a 28-year-old female with extended CGCG of the temporal bone and temporomandibular joint representing a detailed example of diagnostical procedures, surgical approach, histological differentiation, treatment options and 12 months follow-up. Conclusions: The presented case highlights the possibility of CGCG as a differential diagnosis of osteolytic lesions of the skull. But more importantly, to this point no widely accepted follow-up protocol has been established and further investigations are needed. © Frontiers of Oral and Maxillofacial Medicine. All rights reserved.
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