Paediatric gingival extra-axial chordoma: an extremely rare entity

Area of Science:

• Oncology
• Pathology
• Surgical Oncology

Background:

• Extra-axial chordomas are rare malignant neoplasms originating outside the axial skeleton.
• These tumors are often misdiagnosed due to their rarity and varied presentations.

Observation:

• A 13-year-old male presented with a gingival mass, initially suspected as a calcifying epithelial odontogenic tumor (CEOT).
• Pathological examination confirmed the diagnosis of chordoma, specifically an extra-axial variant located in the maxillary gingiva with bone infiltration.

Findings:

• The patient underwent stepwise radical resection of the maxillary chordoma with wide safety margins.
• Reconstruction was successfully achieved using a free latissimus dorsi flap.
• One-year follow-up showed no evidence of tumor recurrence or metastasis.

Implications:

• This case underscores the importance of considering extra-axial chordomas in the differential diagnosis of maxillofacial bone and soft tissue tumors.
• Highlights the potential for chordomas to occur in unusual locations distant from the midline.
• Demonstrates the feasibility of complete surgical resection and successful reconstruction for these rare tumors.