RESUMO
Ghost Cell Odontogenic Carcinoma is a rare malignant odontogenic tumor that can appear as "de novo " or arises from malignant transformation of preexisting benign calcifying odontogenic cysts or dentinogenic ghost cell tumors after multiple recurrences. Ghost cell odontogenic carcinoma is histopathologically characterized by ameloblast-like islands of epithelial cells with aberrant keratinization, simulating a ghost cell, with varying amounts of dysplastic dentine. This article reports an extremely rare case of ghost cell odontogenic carcinoma with foci of sarcomatous change, involving maxilla andnasal cavity which arose from a previously existing recurrent calcifying odontogenic cysts in a 54-year-old man and reviews the features of this unusual and rare tumor. To the best of our knowledge, this is the first case of ghost cell odontogenic carcinoma with sarcomatous transformation to be reported till date. Owing to its rarity and unpredictability of clinical course, long -term follow up of patients with ghost cell odontogenic carcinoma, is mandatory for observation of recurrence and distant metastasis. Keywords: Ghost cell odontogenic carcinoma, maxilla, sarcoma, calcifying odontogenic cysts, ghost cells, odontogenic tumour.