2025 Poster Presentations
P313: RECURRENT CALCIFYING EPITHELIAL ODONTOGENIC TUMOR WITH MALIGNANT TRANSFORMATION, ORBITAL EXTENSION, AND PULMONARY METASTASIS: A RARE CASE REPORT AND LITERATURE REVIEW
Zachary A Wykoff, BS1; Joseph Lee, MD2; Kyle VanKoevering, MD2; 1The Ohio State University College of Medicine; 2The Ohio State University Wexner Medical Center
Introduction: Calcifying epithelial odontogenic tumor (CEOT), also known as a Pindborg tumor, is a typically benign but locally invasive tumor likely of stratum intermedium origin. We report a case of an exceedingly aggressive, recurrent CEOT of maxillary origin, with malignant transformation, orbital extension, and pulmonary metastasis. The case is supplemented by a review of the literature.
Case Presentation: A 71-year-old male with seventeen prior surgical resections for recurrent left maxillary CEOT presented with new onset diplopia. Prior pathology showed foci with features concerning for malignant transformation. MRI revealed tumor recurrence in the left maxillary sinus with extension superiorly into the orbital floor, inferiorly into the remnants of the pterygopalatine fossa, laterally into the medial most components of the infratemporal fossa, and posteriorly to the skull base at the fovea ethmoidalis ([Fig. 1]). The patient opted for re-resection without orbital exenteration followed by adjuvant external beam radiation therapy despite the high likelihood of subtotal resection. Surgery was performed by otolaryngology in collaboration with oculoplastics via a combined transconjunctival, transoral, and endoscopic endonasal approach. Orbital preservation necessitated leaving positive margins along the inferior rectus muscle and medial orbital apex. Two years after surgery, the patient had minimal vision or movement in the affected eye. Local tumor control was reassuring ([Fig. 2]), but PET and subsequent biopsy revealed metastasis to the lungs ([Fig. 3]). Pathology showed PDL-1 positivity, and the patient was started on pembrolizumab. The patient has undergone wedge resection and adjuvant radiation and chemotherapy with continued progression of metastatic disease.
Discussion: CEOTs are uncommon, constituting less than 1% of odontogenic neoplasms. Mandible origin is more common over maxilla at a ratio of 2:1. Malignant transformation is rare and more common from the mandible, with only ten previous reports in the literature. Recurrence is more common with malignant transformation. Orbital involvement is exceedingly rare. To our knowledge, there have been only two previous reported cases of CEOT orbital extension. Furthermore, only two cases of pulmonary metastasis have been reported. The presentation of CEOT in this case is exceedingly rare, and its aggressive nature necessitated an interdisciplinary treatment approach and close follow-up.
Conclusion: CEOTs are rare, benign neoplasm. However, aggressive extension, recurrence, malignant transformation, and metastasis are possible. Treatment may require an interdisciplinary approach. Early diagnosis and intervention are crucial. Recurrent or malignant cases should be followed closely for metastasis.
