Ameloblastic carcinoma

Definition

Ameloblastic carcinoma is a malignant epithelial proliferation that is associated with an ameloblastoma (carcinoma exameloblastoma) or histologically resembles an ameloblastoma (de novo amelob lastic carcinoma). Ameloblastic carcinoma is an aggressive neoplasm that is locally invasive and can spread to regional lymph nodes or distant sites, such as lung and bones.

Epidemiology

More than 3600 cases of ameloblastomas have been described in the literature,but less than 60 cases of ameloblastic carcinoma have been reported so for, among which two thirds occurred in the mandible.

Symptoms

The most common symptom is a rapidly progressing painful swelling, but it may present as a benign cystic lesion or as a large, rapidly growing mass with ulceration, bone resorption and teeth mobility, as well. The tumor may metastasize, most commonly to the cervical lymph nodes and the lungs.

Cause

Exact cause is unkown. Researchers have speculated that diet, stress, environmental factors, and immunologic abnormalities play a part in causing certain types of cancer including Ameloblastic Carcinoma.

Inheritance

The majority of cases appear sporadically in patients without a past history of cancer.

Diagnosis

Ameloblastic carcinoma subjectively demonstrates greater cytologic atypia and mitotic activity than ameloblastoma. Some ameloblastomas exhibit basilar hyperplasia and an increased mitotic index. These findings might warrant a designation of ‘atypical ameloblastoma’ or ‘proliferative ameloblastoma’, but they are probably insufficient to permit a diagnosis of ameloblastic carcinoma in the absence of nuclear pleomorphism, perineural invasion, or other histologic evidence of malignancy. Radiological investigations include both the plain X-ray and computerized axial tomography. They appear as osteolytic processes, exhibiting a unilocular or multilocular appearance on radiograph.

Treatment

Usually, ameloblastic carcinoma is managed as a squamous cell carcinoma with attempted complete surgical excision, elective or therapeutic neck dissection. Regional lymph node dissection should be considered and performed selectively. Radiotherapy and chemotherapy have limited role in the treatment of ameloblastic carcinomas. Close periodic reassessment of the patient is mandatory. The prognosis is poor.

NIH genetic and rare disease info

• NIH info on Ameloblastic carcinoma

Ameloblastic carcinoma is a rare disease.

Ameloblastic carcinoma Resources
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