Gigantiform cementoma

The most famous case is of Novemthree Siahaan (who died on September 15, 2005), a young Indonesian boy from Batam Island who received medical care in Haulien, Taiwan through a Buddhist missionary from the Tzu Chi Foundation, which was documented on the Discovery Health Channel.

Cases like Novemthree Siahann and Ayun Lee are included to illustrate the condition's worldwide and heritable nature.

[1] The condition typically affects adults between the ages of 20 and 50, which there is a higher incidence in women compared to men.

The term has been used in the past to describe florid cemento-osseous dysplasia, but it is now reserved for an autosomal dominant condition affecting the maxillae.

Symptoms of Gigantiform cementoma usually occurs in the adolescent age which is from 10-19 years old, and in some cases, it may not be detected or diagnosed in later in life.

It has a rapid growth of a tumor that causes the expansion of the maxilla and mandible that results in the deformity of the facial skeleton and malocclusion.

[4] The cause of Gigantiform cementoma was unknown in previous years but there is a new studies that the gene ANO5 also known as TMEM16E has been linked to mutations.

The ANO5 gene encodes a protein called Anoctiamin-5 which is involved with physiological processes that includes ion channel regulation and cell signaling.

It is an autosomal dominant genetic disorder meaning both females and male are able to develop the tumor.

It is classified as a benign fibro-osseous lesion of the jaw, which means it contains both fibrous tissue and bone like structures.

The tumor affects the jaw, which is more commonly found in the mandible and there has been hardly any cases with the maxillary but it can still happen.

Biopsy may be suggested by an Oral Surgeon to be able to collect tissues from the tumor to get samples for histological examine, this is necessary to confirm diagnosis.

Gigantiform cementoma affects the jaw and has a rapid growth and can be found in multiple quadrants and has high reoccurring rates.

Therefore the goal for treatment is to be able to reset the lesion, preserve the jaw and help restore its function, reduce complications and simply to improve/maintain the patients quality of life.

There is a slight chance it can be inherited because it is an autosomal disorder which means that a single copy of the mutant allele is present.

It is possible for recurrence because during the skeletal growth of lesions are more cellular and biologically active and it has high chances of recurring even after surgery.

Life for young adolescents will be difficult with these changes that can grow fast and big in a short period of time.

There has been high recurrence rates because there is a rapid expansion of the tumor which can cause a difficult surgery when trying to remove it.

Lower jaw swollen
Panoramic X-ray with a view of the jaw and teeth
Cone Beam Computed Tomography (CBCT) gives a 3D view of the jaw and of a specific area