Beyond the mandible: aggressive maxillary presentation of Pindborg tumour

An 89-year-old woman presented with progressive right facial swelling and pain that began shortly after a maxillary tooth extraction and progressively worsened over 1 year. Computed tomography and magnetic resonance imaging demonstrated a large lobulated expansile mass centred in the right maxilla, extending into the ipsilateral nasal cavity and orbit, with remodeling of the orbital floor and associated proptosis (Figures 1–3). No intracranial extension or distant metastasis was identified. Imaging findings suggested a locally aggressive odontogenic neoplasm, with differential diagnoses including vascular tumours, chondroid lesions and metastatic disease.

Incisional biopsy confirmed calcifying epithelial odontogenic tumour (Pindborg tumour). Histopathology revealed sheets of polygonal epithelial cells with prominent intercellular bridges and extracellular eosinophilic amyloid-like material. Congo red staining demonstrated apple-green birefringence under polarised light, and concentric lamellar calcifications consistent with Liesegang rings were observed. Immunohistochemistry supported the diagnosis, without cytologic atypia.

Calcifying epithelial odontogenic tumour accounts for less than 1% of odontogenic neoplasms and typically involves the posterior mandible.¹ Maxillary involvement is uncommon and may exhibit more aggressive behavior due to thin cortical bone and proximity to the orbit and skull base.^2,3 Early radiologic–pathologic correlation is essential for accurate diagnosis and therapeutic planning in anatomically complex regions.

Treatment options for calcifying epithelial odontogenic tumour include surgical resection ranging from curettage to partial maxillectomy depending on tumour extent.^1–3 In this case, due to the patient’s advanced age and tumour extension to the orbit and nasal cavity, a conservative surgical approach was chosen. The patient underwent incisional biopsy followed by clinical management planning. A focal radiopaque structure in the right maxillary alveolar region, possibly representing a retained tooth root, was identified adjacent to the lesion. While not considered causative, this finding is relevant in the context of odontogenic tumours, which are frequently associated with impacted or previously treated teeth. Notably, the previously extracted tooth was located in the same region. Clinical follow-up was recommended due to the known potential for local recurrence.

View Figure 1–3.