Oral & Dental
Four out of every five people affected by BCCNS will develop aggressive tumors in their jaws. These keratocystic odontogenic tumors (KCOTs), previously known as odontogenic keratocysts (OKC) or jaw cysts, grow rapidly and are capable of disrupting the formation of new teeth and the alignment of the jaws. Because people with BCCNS are likely to develop multiple KCOTs during their adolescent years and beyond, the following treatment protocol is recommended by Dr. John Hellstein, clinical professor of Oral Pathology at the University of Iowa College of Dentistry and member of our Medical Advisory Board.
- Annual Panorex. A person with BCCNS should have a panoramic X-ray taken of the mouth every year, starting at age 8. These should continue until age 21 or the first occurrence of a KCOT (whichever comes first). After a KCOT is found and treated, the next Panorex should be taken at six months, and then every year until the patient has had 5 years free of tumors. After five years, the schedule can be relaxed to every two years; after ten years, the patient and oral surgeon may decide how often to take any follow-up scans. Remember to “reset the clock” after every new occurrence.
- Get them treated. This advice may seem obvious, but some dentists or oral surgeons may ask if they can wait a few months and observe the KCOT. KCOTs, however, do not resolve on their own — in fact, left to their own devices, they only get larger and more destructive. Any new tumor should be treated as soon as possible.
- Avoid resection. Resection is the complete removal of the tumor, plus a margin of bone all around it — or the entire jaw. Because people with BCCNS are likely to form multiple KCOTs during their lives, repeated resection would slowly destroy an affected individual’s face. The goal of treating KCOTs in an affected individual is to preserve as much of the jaw and teeth as possible. The overlying epithelium should also be preserved, if possible.
- Decompress large lesions. Decompression is the insertion of a tube or a vent into a KCOT, allowing it to be flushed out with water or saline solution every day. This slowly cleans out the contents of the tumor and allows it to shrink to a more manageable size while the jaw regrows healthy bone to take its place. A similar process calledmarsupialization simply stitches the cyst open and exposes its contents, but decompression generally results in a more thorough recovery.
- Enucleate small lesions. Enucleation is the careful removal of a KCOT. Enucleation by itself is reported to have a high recurrence rate, which is why an oral surgeon will generally supplement it with extra measures. These include curettage (scraping the surrounding cavity with a sharp knife or burr to remove any remaining KCOT matter), cryotherapy (freezing and killing the tumor with liquid nitrogen), and Carnoy’s solution (a chemical fixative used to cauterize the area around the tumor). If Carnoy’s solution is used, a blend without chloroform is recommended.