Ameloblastoma is one of the most common benign odontogenic tumors of the jaw that constitutes about 10% of all tumors that arise in the mandible and maxilla. It is a slow-growing but locally invasive tumor that presents with painless swelling of the mandible or maxilla. The World Health Organization (WHO) classification of 2017 describes ameloblastomas of the following four types: ameloblastoma; unicystic ameloblastoma; extraosseous/peripheral ameloblastoma; and metastasizing ameloblastoma. The diagnosis of ameloblastoma requires computerized tomography (CT) imaging as well as a biopsy. A biopsy is helpful in differentiating ameloblastoma from ossifying fibroma, osteomyelitis, giant cell tumor, cystic fibrous dysplasia, myeloma, and sarcoma. The best treatment of ameloblastoma is aggressive en bloc resection with simultaneous reconstruction. The high recurrence rate and large tissue defects have been long-standing issues in the treatment of ameloblastoma. Recent molecular developments strongly suggest the possibility of targeted therapy with better outcomes in ameloblastomas. We present a detailed updated narrative review of our current understanding and management of this enigmatic tumor.

Introduction Ameloblastoma is a locally destructive tumor with a propensity for recurrence if not entirely excised. Management of ameloblastoma poses a challenge for all involved in the field of head and neck surgery because successful treatment requires not only adequate resection but also a functional and aesthetically acceptable reconstruction of the residual defect. Methods Patients who had histologically proven ameloblastoma between 1991 and 2009 were identified from the database of Aga Khan University Hospital. A review of all medical records, radiological images, operative reports and pathology reports was undertaken. Results A total of 15 patients with histologically confirmed ameloblastoma were identified. Out of 15 patients nine were males and six were females with age range from 20 to 60 years (mean age 43 years). The most common symptom found in our patient group was painless facial swelling. In 13 patients the origin of tumor was mandible and in the remaining two the tumor originated from maxilla. Eleven out of 15 patients underwent segmental mandibulectomy, two had maxillectomy and two had enucleation. All patients who underwent segmental mandibulectomy required reconstruction. Reconstruction was done with microsurgical free tissue transfer in eight patients, non-vascularized iliac crest bone graft was used in one patient and two had plating only. All free flaps survived with no evidence of flap loss. The mean follow-up was eight years. There was no evidence of graft failure which was used in one patient. Complication was seen in only one of our patients in the form of plate exposure. Recurrence was seen in two of our cases who primarily underwent enucleation. All patients had satisfactory speech, cosmesis and mastication. Conclusion The management of ameloblastoma still poses a big challenge in spite of being the most common odontogenic tumor. In our study we have found that segmental mandibulectomy with disease-free margin of around 1 cm and immediate reconstruction with free tissue transfer have shown good results.

This retrospective study investigated the reduction rate and speed of shrinkage after marsupialization in mandibular cystic ameloblastoma and clarified whether marsupialization is appropriate for unicystic ameloblastoma and multicystic ameloblastoma.
Sixty-three patients with mandibular cystic ameloblastoma were initially treated with marsupialization. Premarsupialization and postmarsupialization panoramic radiographs were reviewed for reduction rate and speed of shrinkage, and then were evaluated with age, sex, tumor location, and tumor type.
The overall recurrence rate was 4.5% (2/44). The average reduction rate after marsupialization was 65.6%. No significant difference was found between unicystic ameloblastoma and multicystic ameloblastoma in reduction rate. The speed of shrinkage of unicystic ameloblastoma was significantly faster than that of multicystic ameloblastoma (P < .05). Similarly, patients with multicystic ameloblastoma had longer marsupialization periods than those with unicystic ameloblastoma (P < .05).
Marsupialization is effective in reducing tumor size for both unicystic ameloblastoma and multicystic ameloblastoma. Marsupialization plus second-stage curettage is recommended as the primary treatment for mandibular cystic ameloblastoma.