When Jeremy first noticed the swelling in his jaw, he assumed it must be due to a problem with a tooth. He wondered why he didn’t feel any pain, however. One harrowing year later, he found himself a survivor of multiple surgeries to battle a rare disease known as ameloblastic carcinoma.
According to the National Organization for Rare Disorders (NORD), this type of cancer begins in the bones of the jaw. Medical personnel classify it as an odontogenic tumor – one that arises from the same tissue that forms a person’s teeth. In some patients, it metastasizes to other parts of the body.
Symptoms
Some patients who develop ameloblastic carcinoma experience no symptoms. For those who do, typical signs are progressive pain and swelling in the jaw. Bleeding and headache are also common.
Less frequently, individuals complain of being unable to open the mouth or of dysphonia, a voice disorder accompanied by hoarseness, weakness, tingling or numbness, Caring4Cancer reports. In rare cases, a patient might lose his or her voice.
If the tumor reaches a certain size, a patient might have trouble eating or experience dental abnormalities such as the misalignment of upper and lower teeth so that they don’t meet correctly. Some patients with an ameloblastic tumor in the sinuses of the maxilla report discharge and blockage of their nasal passages.
The most frequent origin of this type of tumor is the lower jaw, though the upper jaw can be the primary site. This type of carcinoma has even appeared in the anterior base of the skull.
Causes and risk factors
The precise cause of ameloblastic carcinoma continues to elude scientists. Most patients have no prior history of cancer.
Researchers hypothesize that various factors might play a role in developing this type of malignancy. Among them are abnormalities of the patient’s immune system, environmental exposure to things like ultraviolet rays or certain chemicals, stress and diet. The medical community so far has been unable to identify any genetic predisposition for this kind of cancer.
Some experts believe the disorder could develop from epithelial tissue left behind in the body after teeth and associated structures develop. For some patients, it occurs when an existing ameloblastoma or a benign odontogenic cyst becomes malignant.
The malignancy affects males and females in equal percentages. Although it can strike anyone from childhood through the senior years, the mean age of patients is about 30 years old.
Diagnosis and treatment
Doctors and dentists discover most cases of ameloblastic carcinoma incidentally. Diagnosis is the result of a detailed patient history and looking at a biopsy of the tumor under a microscope. Physicians might order various radiographic techniques to pinpoint the size, location and extension of the tumor prior to treatment. Certain laboratory tests can indicate potential infiltration of lymph nodes and metastases.
The appropriate treatment for an individual with ameloblastic carcinoma depends on the site of the primary tumor, its stage and grade and whether it has metastasized. Doctors also take into consideration the patient’s age and general health.
The most frequent treatment is wide surgical excision, sometimes accompanied by radiation. The use of radiation by itself has not proven effective in controlling these tumors. However, it can sometimes reduce the size of the growth enough to make surgery possible.
Although experts sometimes attempt to control extensive metastases with chemotherapy, it has not proven an effective treatment overall.
Prognosis
According to Caring4Cancer, this type of cancer is often aggressive. It can spread to other parts of the body, particularly the lungs, and cause life-threatening problems. Other common sites to which the tumor metastasizes are the liver, brain and bones.
The typical course of ameloblastic carcinoma is persistent recurrence with local spread. For this reason, periodic exams throughout the patient’s life are essential.
Sources:
National Organization for Rare Disorders (NORD) site
Caring4Cancer site
