Ghost Cell Odontogenic Carcinoma: Case Series and Literature Review.

Ghost cell odontogenic carcinoma (GCOC) is rare. These tumors can arise from odontogenic precursors or de novo, displaying a spectrum of clinical behaviors with potential for rapid local recurrence, distant metastasis, and death from disease. Considering limited data, we present two cases of GCOC in female patients and perform a comprehensive review of the literature related to GCOC with a focus on adjuvant therapies. The first case, a de novo occurrence, had an aggressive course, while the second, arising from an ameloblastoma, was more indolent. A review of 65 previously published cases revealed that 82% of GCOCs occurred in males. Recurrence rates were higher in cases arising from precursor lesions (64%) compared with de novo lesions (26%). Of the 59 patients who had documented follow-up, 37% had an isolated local recurrence, 3% had an isolated distant metastasis, and 5% had both a local recurrence and distant metastasis. Overall, GCOC is a rare but aggressive odontogenic carcinoma requiring a multidisciplinary approach. Radical surgical excision with clear margins remains the primary treatment. Adjuvant therapies, such as radiotherapy, are indicated for aggressive features like rapid growth, positive margins, and local recurrence. While both local and distant recurrences are a risk, local recurrence is the most common mode of treatment failure. Further research into prognostic markers such as CTNNB1, p53, and Ki-67 may lead to more personalized therapies and improved outcomes. Additional research should also focus on intensifying adjuvant therapies to improve outcomes.