six cases were reported from 2007 to 2012 to the oral and maxillofacial unit of annaswamy mudaliar general hospital , bangalore , out of which four patients were males with the lesion occurring between the second decade of life and sixth decade of life [ figures 1a3c ] . in all cases , the mandible was involved with the greatest size of tumor seen being 11 cm  9.6 cm [ figure 1a ] . all cases exhibited enlargement of cortical plates with perforation , pain and tenderness with regional lymph node enlargement , with three cases showing fixed submandibular groups of lymph nodes ( level ib ) and in one case parasthesia of inferior alveolar nerve . five cases showed unilocular radiolucency and one case showed multilocular radiolucency with erosion of bone , including inferior border of mandible in one case [ figure 1c ] . maximum follow - up was for 39 months with one patient being lost to the follow - up [ table 1 ] . all patients had a chest x - ray and an abdomen ultrasound done to avoid missing distant metastasis as we were aware of the potential of this lesion to metastasize . incisional biopsy was done for diagnosis for all cases and was diagnosed as features suggestive of ameloblastoma with areas showing dysplastic changes in the epithelium suggestive of a neoplastic change . ( a ) patient exhibiting large extra oral tumor with draining sinus , ( b ) intra oral view of tumor : ( c ) orthopantomogram showing bicortical involvement of mandible in one patient , wide excision was done as the lesion was small and the patient being young did not wish aggressive measures that may have had postsurgical cosmetic deformity [ figure 2a  c ] . he chose the  wait and watch  policy and there is no evidence of disease after 32 months . ( a ) preoperative view showing tumor involving left mandible and sulcus , ( b ) preoperative orthopantomogram , ( c ) intra oral view of tumor being excised two patients underwent hemimandibulectomy and neck dissection because of increased involvement of mandible with palpable level ib nodes where fine - needle aspiration cytology showed positive for carcinoma , as also one more patient who had underwent a wide excision with hemimandibulectomy and neck dissection due to skin involvement and palpable fixed nodes at ib level [ figure 1a  c ] . one patient had a segmental resection of the mandibleone patient had a hemimandibulectomy [ figure 3a  c]all the resected specimens were sent for histological examination . 
  one patient had a segmental resection of the mandible one patient had a hemimandibulectomy [ figure 3a  c ] all the resected specimens were sent for histological examination . ( a ) preoperative view of tumor involving left mandible , ( b ) intra oral view of tumor , ( c ) orthopantomogram showing involvement of left mandible the largest diameter of the tumors was 10 cm  9 cm [ figure 1a ] showing increased vascularity with numerous cystic spaces and areas of necrosis . typical features found were multiple pieces of tissue with predominant tumor islands within connective tissue seen under scanner . under higher magnification tumor islands appearing epithelial odontogenic in origin showed infiltrative pattern with each island showing peripheral columnar cells with central stellate reticulum exhibiting feature of ameloblastoma , but with atypical features such as bizarre mitosis , altered nuclear chromatin ratio , hyperchromatisim , and mild pleomorphism . individual cell keratinization with keratin pearl formation with stroma showed predominant inflammatory component and endothelial lined blood vessels [ figure 4 ] . immunohistochemical findings showed that ameloblastic carcinomas reacted strongly with antibodies directed against cytokeratin chm and aei and ae3 . histopathological picture showing bizarre mitosis , altered nuclear chromatin ratio , hyperchromatisim , mild pleomorphism , and central stellate reticulum confirming ameloblastic carcinoma no evidence of disease or metastasis was seen postsurgery in any of the patients . it is interesting to note that up to date of completion of this study 71 cases of ameloblastoma had reported to our department between february 2007 and may 2012 . in this series , ameloblastic carcinoma is a lesion with histologic behavior that dictates a more aggressive surgical approach than that of a simple ameloblastoma . mean age for occurrence is 30.5 years , male : female ratio is 1.5:1 , and 80% are located in the mandible posterior region . involvement of the maxilla is less frequent than that of the mandible . the clinical symptoms of ameloblastic carcinoma are more aggressive than conventional ameloblastoma , with swelling , pain , rapid growth , trismus , dysphonia , expansion of the jaws , and frequently perforation of the cortex . these were mirrored in our series also . radiographic appearance of ameloblastic carcinoma is consistent with that of ameloblastoma except for occasional presence of some focal radiopacities , reflecting dystrophic calcification . histologically , ameloblastic carcinoma does not show uniform proliferation , and has pleomorphic and hyperchromatic cells arranged in the form of sheets and chords with reversed polarity . an additional consideration in the differential diagnosis is the squamous cell carcinoma arising in the lining of an odontogenic cyst . histologically , this lesion tends to more closely resemble oral squamous cell carcinoma ameloblastic carcinoma . the squamous odontogenic tumor may also be mistaken for ameloblastic carcinoma , being composed of islands of squamous epithelium that lack stellate reticulum like zones , peripheral palisading , microcystic changes and dystrophic calcifications . however , the epithelium of the squamous odontogenic tumor lacks any cytologic evidence of malignant disease . thus , the term ameloblastic carcinoma can be applied to our series , where all showed evidence of malignant disease including cytologic atypia and mitoses with indisputable features of classic ameloblastoma . whether ameloblastoma may transform biologically and histologically from a classic ameloblastoma to a malignant lesion is controversial . many authors have shown that metastasizing ameloblastomas are histologically indistinguishable from classic ameloblastomas and others have identified malignant features in the tumor , usually after repeated surgical excisions . the high rate of recurrence maybe due to the mode of growth and surgical mismanagement rather that any inherent malignant properties and metastases are  exceedingly rare . wide local excision is the treatment of choice as most investigators have recommended , which we had followed in all our cases . regional lymph node dissection should be performed selectively as we did in three of our cases where in the regional lymph node was palpable . the efficacy of adjuvant radiation or chemotherapy as a postsurgical treatment is not clear and seems to have a limited value . lanham treated a patient with doxorubicin , cisplatin , cyclophosphamide , dacarbazine , and 5 fu ; but , the tumor failed to respond . yoon et al . have reported distant metastasis as early as 4 months and as late as 47 months after surgery . none of our patients showed recurrence , but one patient was lost to follow - up . none of our patients were sent for radiotherapy or chemotherapy . in our case series , least follow - up until date is 22 months and maximum follow - up being 39 months . this small series of cases illustrates the malignant portion in the spectrum of ameloblastomas and it is possible that ameloblastoma may show a wide variety of histologic and biologic behavior ranging from benignity to frank malignancy . cases of ameloblastoma should be studied carefully , correlating their histologic pattern with biologic behavior to detect changes in histology that may predict aggressive behavior . when a case is diagnosed as ameloblastic carcinoma , assessment of nodal metastasis and evidence of distant metastasis treatment of ameloblastic carcinoma is wide surgical resection and if evidence of nodal metastasis is present , neck dissection should also be considered .
ameloblastic carcinoma is a rare odontogenic tumor exhibiting not only features of ameloblastoma , but also features of carcinoma . 
 clinical dissemination of this lesion is more aggressive and rapid than that of ameloblastoma and it can metastasize to the lung or regional lymph node . 
 histologically , there are features of both ameloblastoma and carcinoma . 
 < 50 cases have been reported until 2011 . 
 we report a series of six cases with our treatment modalities .