Ameloblastoma is an aggressive odontogenic tumor, which arises from odontogenic epithelium. Ameloblastomas can present in several clinical and histomorphological patterns. The granular cell variant accounts for only 3.5% to 5% of ameloblastomas. We have presented two cases of granular cell ameloblastoma (GCA) occurring in a 44-year-old and 50-year-old man, respectively. Case No. 1 on incisional biopsy was diagnosed as unicystic ameloblastoma (UA), which later after excisional biopsy was finally diagnosed as GCA owing to the features observed in excisional biopsied tissue. Case No. 2 on incisional biopsy showed darker and lighter stained cells arranged in small follicular pattern, and anastomosing cords. Meticulous immunohistochemistry, histochemical examination and careful literature search helped us to diagnose it as GCA. We have made an attempt to elucidate the diagnosis of GCA especially in cases of GCA with unusual granular component.
The term Unicystic Ameloblastoma (UA) refers to those cystic lesions that show clinical and radiological characteristics of an odontogenic cyst but on histological examination show a typical ameloblastomatous epithelium lining part of the cyst cavity, with or without luminal and/or mural tumor growth. Till date, lot of controversies exist among oral surgeons and oral pathologists regarding this entity. An attempt is being made here to discuss all the diagnostic dilemmas associated with UA.
In the recent World Health Organization classification of odontogenic tumours, desmoplastic ameloblastoma has been characterized as a variant of ameloblastoma, with specific clinical, radiographical, and histological features. Till date, 145 cases have been reported in Japanese, Chinese, Malaysian, Western, and African populations, with very few cases described in Indians. Here, we report five cases in the Indian population. The male to female ratio was 3:2. The mean age at diagnosis was 33.2 years. Four of the tumours were located in the maxilla, in the anterior premolar region. The lone mandibular tumour was located anteriorly, crossing the midline. Three of the tumours had a mixed radiologic appearance with poorly defined borders. Unilocular change was seen in one of them. Two tumours presented as unilocular radiolucencies with specks of radiopacities and well-circumscribed borders. Histologically, irregular odontogenic islands, with a stretched-out 'kite-tail' appearance, were seen in a dense desmoplastic stroma. The peripheral layer of the epithelial islands was made up of flattened cells and the inner core was made up of spindle-shaped and, in some instances, squamous-shaped cells. In two cases, odontogenic epithelium in the form of follicles, typical of solid/multicystic ameloblastoma, was seen and these were typed as 'hybrid' variants. All the cases were treated by resection.
Mandibular swellings may occur as a result of many benign lesions of odontogenic or non-odontogenic origin. Ameloblastomas are benign tumours of odontogenic origin, whose importance lies in its potential to grow into enormous size with resulting bone deformity, it is a slow-growing, persistent, and locally aggressive neoplasm. The unicystic ameloblastoma (UA) represents an ameloblastoma variant, presenting as a cyst clinically and radiographically, but showing typical ameloblastomatous epithelium lining histologically. It commonly occurs in second and third decades of life and is rare in children under 12 years of age, and better response to conservative treatment. It shares many clinical and radiographic features with odontogenic cysts/tumours and/or periapical disease of endodontic origin. Reported here is an unusual case of unicystic ameloblastoma involving the crown of an unerupted mandibular first premolar in a 9-year boy in an uncommon location, which was misdiagnosed as periapical lesion of inflammatory origin clinically, and as a dentigerous cyst radiographically. This highlights the importance to routinely submit the removed surgical specimen for histopathological examination.