Central Dentinogenic Ghost Cell Tumor: An Update on a Rare Aggressive Odontogenic Tumor

Purpose Dentinogenic ghost cell tumor (DGCT) is a very rare odontogenic lesion, with most knowledge based on single case reports. Therefore, a comprehensive analysis was performed of the clinical and radiologic features of reported cases of DGCT with an emphasis on treatment modalities. Material and Methods This is a case series of DGCTs collected from the literature after a systematic search of Medline's PubMed and Google Scholar. Three additional cases were included from the authors' files. Demographic data of the patients, lesion site and size, and radiologic features were analyzed. Treatment approach and events of recurrence were recorded. Results Forty-five cases (42 from the literature) were included. The mean age of patients was 39.7 ± 19.3 years (range, 12 to 79 yr) and the male-to-female ratio was 1.8:1. The mandible-to-maxilla ratio was 1.14:1, with the posterior region of the jaws being the most commonly involved site. Radiographically, 78% lesions were unilocular, 67% were mixed radiolucent and radiopaque, and 68% had well-defined borders. The mean lesion size was 4.0 cm (range, 1.8 to 13.0 cm). The primary treatment for 21 patients was conservative surgery consisting of enucleation or curettage. Follow-up information for longer than 1 year (mean, 6.2 ± 8.3 yr; range, 1 to 31 yr) was known for 15 patients, of whom 11 (73%) had recurrences. The primary treatment in 19 patients was radical surgery consisting of marginal or segmental resection. Follow-up information for longer than 1 year (mean, 3.3 ± 2.6 yr; range, 1 to 10 yr) was known for 12 patients, of whom 4 (33%) had recurrences. Conclusions This study highlighted the potentially aggressive biological behavior of DGCTs that demands extensive surgery and long follow-up. However, owing to the rarity of DGCT, more well-documented cases with long follow-up periods are needed to further define the optimal treatment modalities and prognosis.