Ameloblastic carcinoma is a rare form of malignant

odontogenic tumor An odontogenic tumor is a neoplasm of the cells or tissues that initiate odontogenic processes. Examples include: * Adenomatoid odontogenic tumor * Ameloblastic fibroma * Ameloblastic fibro-odontoma * Ameloblastoma, a type of odontogenic tumor ...

, that develops in the

jaw The jaw is any opposable articulated structure at the entrance of the mouth, typically used for grasping and manipulating food. The term ''jaws'' is also broadly applied to the whole of the structures constituting the vault of the mouth and serv ...

bones from the

epithelial cells Epithelium or epithelial tissue is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. It is a thin, continuous, protective layer of compactly packed cells with a little intercellula ...

that generate the

tooth enamel Tooth enamel is one of the four major Tissue (biology), tissues that make up the tooth in humans and many other animals, including some species of fish. It makes up the normally visible part of the tooth, covering the Crown (tooth), crown. The ...

. It is usually treated with surgery;

chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemotherap ...

has not been proven to be effective.

Signs and Symptoms

Common symptoms of ameloblastic carcinomas are pain and swelling either localized in the jaw or throughout the entire face, dysphagia, and

trismus Trismus, commonly called ''lockjaw'' as associated with tetanus, is a condition of limited jaw mobility. It may be caused by spasm of the muscles of mastication or a variety of other causes. Temporary trismus occurs much more frequently than perma ...

. Less common symptoms include ulceration, loosening of the teeth, chronic

epistaxis A nosebleed, also known as epistaxis, is bleeding from the nose. Blood can flow down into the stomach, and cause nausea and vomiting. In more severe cases, blood may come out of both nostrils. Rarely, bleeding may be so significant that low bloo ...

, facial pressure, and nasal dyspnea.

Causes

It is speculated that some cases of ameloblastic carcinoma arise from remnants of epithelial tissue left behind after the development of the teeth and related structures. Other times, it may be caused by a benign odontogenic cyst becoming malignant, or a pre-existing ameloblastoma.

Treatment

Chemotherapy has not proven effective in treating ameloblastic carcinoma, leaving surgical removal of the tumor one of the only options. Surgical resection with wide margins is the main treatment.

Prognosis

Followup after surgery is important, as over 50% of recurrences occur within 5 years.

References

External links

{{Medical resources , ICD10 = C41.1 , ICD9 = , ICDO = , OMIM = , DiseasesDB = , MedlinePlus = , eMedicineSubj = , eMedicineTopic = , MeSH = , GeneReviewsNBK = , GeneReviewsName = , Orphanet = 314422 Dentistry Rare diseases