Ghost cell odontogenic carcinoma: a case report and review of the literature

Ghost cell odontogenic carcinoma (GCOC) is an exceptionally rare malignant odontogenic neoplasm representing the aggressive end of the calcifying cystic odontogenic tumor spectrum, with fewer than 50 well-characterized cases reported in the English-language literature and a marked predilection for Asian males in the fifth to seventh decades of life. A 38-year-old African American female presented with an expansile anterior mandibular radiolucency initially misdiagnosed as mandibular tori, then as a calcifying odontogenic cyst on incisional biopsy; progressive neurological symptoms and lesion growth prompted definitive surgical resection. Final histopathologic examination revealed infiltrating odontogenic epithelium with ameloblastoma-like features, cellular atypia, increased mitotic activity, ghost cell populations, and vascular invasion. Immunohistochemistry demonstrated an elevated Ki-67 index and β-catenin nuclear reactivity, confirming the diagnosis of GCOC, and postoperative PET scan showed no distant metastatic disease. This case represents a significant epidemiological departure from established GCOC demographics and underscores the importance of correlating clinical, radiographic, histopathologic, and immunohistochemical findings to reach an accurate diagnosis, highlighting the necessity of a multidisciplinary approach for optimal management of this rare malignancy.