Radiological Case: Odontogenic Ghost Cell Carcinoma

Robert Crowder, MD; Scott A Jorgensen, MD; Alexander J Towbin, MD; Richard Towbin, MD

Disclosures

Appl Radiol. 2020;49(4):42-43. 

In This Article

Discussion

Odontogenic ghost cell carcinoma (OGCC) is an extremely rare, malignant odontogenic tumor within a spectrum of lesions composed of odontogenic epithelium-containing "ghost cells." This term refers to characteristic eosinophilic epithelial cells that have lost their nuclei but otherwise have retained their underlying cell morphology.[1] This group of tumors is classified according to their solid or cystic nature, as well as malignant behavior. The three classifications recognized by the World Health Organization consist of calcifying cystic odontogenic tumors, dentinogenic ghost cell tumors, and ghost cell odontogenic carcinomas.[1] The majority of these tumors fall into the benign cystic category; only 33 cases of the malignant OGCC have been reported in the literature worldwide. These tumors show a peak occurrence in the fourth decade of life, ranging from 13 to 72 years of age.[2] OGCC occurs four times more commonly in males and seems to have a predilection for patients of Asian descent.[3] Clinical presentation is normally characterized as a long-term swelling followed by more rapid onset of pain and swelling at the tumor site. Many of these lesions are believed to represent malignant transformation of a preexisting calcifying odontogenic tumor.

Imaging features vary, with no specific radiologic features that allow confident diagnosis based on imaging characteristics alone. Therefore, OGCC remains a predominantly histologic diagnosis. Most reported tumors have occurred in the maxilla (70%), with the balance occurring within the mandible. Also, in most of the reported cases the lesions are described as mixed radiolucent and radiopaque with poorly defined margins. These tumors often invade surrounding structures, particularly the paranasal sinuses and orbits. Displacement of teeth and root resorption may also be seen.

Clinical outcomes in the reported cases of OGCC are variable and unpredictable. Recurrence is common; the 5-year patient survival rate among the reported cases is 73%.[1] Wide resection is recommended for all tumors; supplemental postoperative radiation and adjuvant chemotherapy may be recommended.[3]

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