The term primary intraosseous odontogenic carcinoma (PIOC) has been primarily used to describe a squamous cell carcinoma within the jaws arising either from a previous odontogenic cyst or, more rarely, de novo. Diagnostic criteria of PIOC require absence of initial connection with the overlying mucosa or skin and exclusion of metastasis from a distant primary tumor by physical and radiographic examination during at least a 6-month follow-up. We report 5 patients with PIOC treated at the University of Maryland, Baltimore, during the period from 1997 to 2004. Three of the patients were female and 2 were male. Their age ranged from 18 to 84 years with a mean age of 52.8 years. One case involved the anterior maxilla, and 4 cases occurred in the mandible, 3 of them being located posteriorly. One patient complained of pain and paresthesia, and the remaining patients were asymptomatic. The typical radiographic presentation of our cases was that of a radiolucent lesion with well defined or irregular margins. Histopathologically, 4 cases were diagnosed as PIOC arising from previous odontogenic cysts (including 2 odontogenic keratocysts and 2 periapical cysts); all 4 were well differentiated keratinizing carcinomas. The remaining case, a poorly differentiated nonkeratinizing squamous cell carcinoma, was not associated with a cystic component and appeared to have arisen de novo. Four patients underwent surgical removal with postoperative radiotherapy, and 1 patient was treated with surgery only. Lymph node involvement and distance metastasis were not present in any of our patients at the time of diagnosis and did not develop during a follow-up period ranging from 15 to 77 months. To date, all patients are alive and free of disease. Knowledge of the clinical, radiographic, and histopathologic features of PIOC will allow accurate diagnosis and appropriate treatment of this rare malignancy. The term primary intraosseous odontogenic carcinoma (PIOC) has been primarily used to describe a squamous cell carcinoma within the jaws arising either from a previous odontogenic cyst or, more rarely, de novo. Diagnostic criteria of PIOC require absence of initial connection with the overlying mucosa or skin and exclusion of metastasis from a distant primary tumor by physical and radiographic examination during at least a 6-month follow-up. We report 5 patients with PIOC treated at the University of Maryland, Baltimore, during the period from 1997 to 2004. Three of the patients were female and 2 were male. Their age ranged from 18 to 84 years with a mean age of 52.8 years. One case involved the anterior maxilla, and 4 cases occurred in the mandible, 3 of them being located posteriorly. One patient complained of pain and paresthesia, and the remaining patients were asymptomatic. The typical radiographic presentation of our cases was that of a radiolucent lesion with well defined or irregular margins. Histopathologically, 4 cases were diagnosed as PIOC arising from previous odontogenic cysts (including 2 odontogenic keratocysts and 2 periapical cysts); all 4 were well differentiated keratinizing carcinomas. The remaining case, a poorly differentiated nonkeratinizing squamous cell carcinoma, was not associated with a cystic component and appeared to have arisen de novo. Four patients underwent surgical removal with postoperative radiotherapy, and 1 patient was treated with surgery only. Lymph node involvement and distance metastasis were not present in any of our patients at the time of diagnosis and did not develop during a follow-up period ranging from 15 to 77 months. To date, all patients are alive and free of disease. Knowledge of the clinical, radiographic, and histopathologic features of PIOC will allow accurate diagnosis and appropriate treatment of this rare malignancy.
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