Ameloblastomas are rare, typically benign tumors that originate in odontogenic epithelial tissue (tissue of the jaw, usually

near the third molar). These tumors are rarely metastatic (they rarely spread to other parts of the body) and they rarely develop into a malignant growth, but they can result in lesions that lead to severe abnormalities in the jaw and face. Neoplastic tissue associated with an ameloblastoma can also easily infiltrate and damage the surrounding bone structures. Thus, wide surgical excision is often necessary to treat and/or reduce symptoms of this disorder. ameloblastoma signs and symptoms An ameloblastoma is often accompanied by the presence of unerupted teeth. Wisdom teeth account for almost all unerupted teeth in adults. The patient may experience, as a result of the growth, loose teeth, ulcers in the mouth, periodontal (gum) disease, swelling, and facial deformities if the lesions are severe enough. These lesions can occur in both the mandible (lower jaw) and maxilla (upper jaw), but 75% of them occur in the ascending ramus of the mandible (the part of the jaw bone that forms the joint with the skull). When ameloblastomas are present in the maxilla, they have a tendency to spread into the sinus cavities and the floor of the nose, resulting in pain and deformities. Furthermore, because the progression of an ameloblastoma is usually very slow, periosteum (a membranous tissue that invests all bones) often has enough time to create a thin shell of bone around the expanding neoplastic tissue. This shell will crack when examined by touch and/or when the tumor has expanded enough to puncture the bone. This cracking of the bone is known as crepitus and is a major factor in the diagnosis of an ameloblastoma. ameloblastoma diagnosis Ameloblastomas are diagnosed using a combination of radiographic examination and biopsy. In radiographic examinations, an ameloblastoma reveals itself as a lucent region in the bone. It is said that the appearance of an ameloblastoma resembles a soap bubble in such examinations. Biopsied tissue confirms the diagnosis. ameloblastoma treatment Typically, ameloblastomas resist radiation and chemotherapy, making surgery the most common and most curative treatment. Unfortunately, this tumor often invades the jawbone through the porous marrow (this invasion is not the same as metastasis), commonly resulting in the surgical removal of adjacent bone matter. Even though an ameloblastoma is not a cancer, surgical removal of the growth is treated similar to that of a cancer. This means that wide surgical margins, far from diseased tissue, are usually required for a good prognosis. Recurrence is frequent with ameloblastoma

Ameloblastoma Dr Yuranga Weerakkody and Dr Frank Gaillard et al.view revision history An ameloblastoma (previously known as an adamantinoma of the jaw) is a benign but locally agressive tumour arising from the mandible, or less commonly from the maxilla. Epidemiology Ameloblastomas are the second most common odontogenic tumour (odontoma is the most common) and account for up to a 3rd of such cases. They are slow growing and tend to present in the 3rd to 5th decades of life, with no gender predilection 4. Clinical presentation Ameloblastomas typically occur as hard painless lesions near the angle of the mandible in the region of the 3rd molar tooth (48 and 38) although they can occur anywere along the alveolus of the mandible (80%) and maxilla (20%). When the maxilla is involved, the tumour is located in the premolar region, and can extend up in the maxillary sinus. Although benign, it is a locally aggressive neoplasm with a high rate of recurrence. Approximately 20% of cases are associated with dentigerous cysts and unerrupted teeth. Pathology Ameloblastomas (not surprisingly) arise from ameloblasts, (part of the odontogenic epithelium, responsible for enamel production and eventual crown formation). Three variants are described:

simple (no nodule) : best prognosis luminal : single nodule projecting into the cyst) mural : multiple nodules (often only microscopic) in the wall of the cyst

Radiographic features Plain film and CT It is classically seen as a multilocualted (80%), expansile "soap-bubble" lesion, with well demarcated borders and no matrix calcification. Occasionally erosion of the adjacent tooth roots can be seen which is highly specific. When larger it may also erode through cortex into adjacent soft tissues. MRI In general ameloblastomas demonstrate a mixed solid and cystic pattern, with a thick irregular wall, often with papillary solid structures projecting into the lesion. These components tend to vividly enhance.

Treatment and prognosis Ameoloblastomas tend to be treated by surgical en-bloc resection. Local curettage is associated with a high rate of local recurrance (45 - 90%). Simple unicystic lesions are less common but have a better prognosis. Simple (no nodule) variant will not be diagnosable on radiography, as it will be indistinguishable form other more common cysts. Luminal variant, has a single nodule projecting into the cyst. Mural variant has multiple nodules (often only microscopic) in the wall of the cyst. The latter has an elevated risk of recurrence. Malignant behaviour is seen in two forms 5: 1. ameloblastic carcinoma o frankly malignant histology 2. malignant ameloblastoma o metastases despite well differentiated 'benign' histology Differential diagnosis Considerations include

dentigerous cyst : the relationship between ameloblastomas and dentrigerous cysts is a controversial one: 20% of ameloblastomas thought to arise from pre-existing dentigerous cysts odontogenic keratocyst (OKC) : usually unilocular with thin poorly enhancing walls odontogenic myxoma : can be almost indistiguishable aneurysmal bone cyst (ABC) fibrous dysplasia

Ameloblastoma From Wikipedia, the free encyclopedia Ameloblastoma Classification and external resources

Micrograph of an ameloblastoma showing the characteristic nuclear palisading and stellate reticulum. H&E stain. ICD-10 ICD-9 ICD-O: DiseasesDB MeSH D16.5 213.1 9310/0 31676 D000564

Ameloblastoma (from the early English word amel, meaning enamel + the Greek word blastos, meaning germ[1]) is a rare, benign tumor ofodontogenic epithelium (ameloblasts, or outside portion, of the teeth during development) much more commonly appearing in the lower jaw than theupper jaw.[2] It was recognized in 1827 by Cusack.[3] This type of odontogenic neoplasm was designated as an adamantinoma in 1885 by the French physician Louis-Charles Malassez.[4] It was finally renamed to the modern name ameloblastoma in 1930 by Ivey and Churchill.[5][6] While these tumors are rarely malignant or metastatic (that is, they rarely spread to other parts of the body), and progress slowly, the resultinglesions can cause severe abnormalities of the face and jaw. Additionally, because abnormal cell growth easily infiltrates and destroys surrounding bony tissues, wide surgical excision is required to treat this disorder. Contents [hide]

1 Subtypes 2 Clinical features 3 Histopathology 4 Variants 5 Treatment

o o

5.1 Molecular biology 5.2 Recurrence

6 Epidemiology 7 See also 8 References [edit]Subtypes There are three main clinical subtypes of ameloblastoma: unicystic, multicystic, peripheral.[7] The peripheral subtype composes 2% of all ameloblastomas.[2] Of all ameloblastomas in younger patients, unicystic ameloblastomas represent 6% of the cases.[2] A fourth subtype, malignant, has been considered by some oncologic specialists, however, this form of the tumor is rare and may be simply a manifestation of one of the three main subtypes. Ameloblastoma also occurs in long bones, and another variant is Craniopharyngioma (Rathke's pouch tumour, Pituitary Ameloblastoma.) [edit]Clinical features This section needs additionalcitations for verification.(March 2009)

A CT scan of a patient suffering from an ameloblastoma.

The resected left half of a mandible containing an ameloblastoma, initiated at the third molar Ameloblastomas are often associated with the presence of unerupted teeth. Symptoms include painless swelling, facial deformity if severe enough, pain if the swelling impinges on other structures, loose teeth, ulcers, and periodontal (gum) disease. Lesions will occur in the mandible and maxilla,although 75% occur in the ascending ramus area and will result in extensive and grotesque deformitites of the mandible and maxilla. In the maxilla it can extend into the maxillary sinus and floor of the nose. The lesion has a tendency to expand the bony cortices because slow growth rate of the lesion allows time for periosteum to develop thin shell of bone ahead of the expanding lesion. This shell of bone cracks when palpated and this phenomenon is referred to as "Egg Shell Cracking" or crepitus, an important diagnostic feature. Ameloblastoma is tentatively diagnosed through radiographic examination and must be confirmed by histological examination (e.g., biopsy). Radiographically, it appears as a lucency in the bone of varying size and featuressometimes it is a single, well-demarcated lesion whereas it often demonstrates as a multiloculated "soap bubble" appearance. Resorption of roots of involved teeth can be seen in some cases, but is not unique to ameloblastoma. The disease is most often found in the posterior body and angle of the mandible, but can occur anywhere in either the maxilla or mandible. Ameloblastoma is often associated with bony-impacted wisdom teethone of the many reasons some dentists recommend having them extracted. [edit]Histopathology Histopathology will show cells that have the tendency to move the nucleus away from the basement membrane. This process is referred to as "Reverse Polarization". The follicular type

will have outer arrangement of columnar or palisaded ameloblast like cells and inner zone of triangular shaped cells resembling stellate reticulum in bell stage. The central cells sometimes degenerate to form central microcysts. The plexiform type has epithelium that proliferates in a "Fish Net Pattern". The plexiform ameloblastoma shows epithelium proliferating in a 'cord like fashion', hence the name 'plexiform'. There are layers of cells in between the proliferating epithelium with a well-formed desmosomal junctions, simulating spindle cell layers.

Ameloblastoma [edit]Variants The six different histopathological variants of ameloblastoma are desmoplastic, granular cell, basal cell, plexiform, follicular, and acanthomatous.[8] The acanthomatous variant is extremely rare.[9] One-third of ameloblastomas are plexiform, one-third are follicular. Other variants such as acanthomatous occur in older patients.[2] In one center, desmoplastic ameloblastomas represented about 9% of all ameloblastomas encountered.[10] [edit]Treatment

Tracheal intubation is anticipated to be difficult in this child with a massive ameloblastoma. While chemotherapy, radiation therapy, curettage and liquid nitrogen have been effective in some cases of ameloblastoma, surgical resection or enucleation remains the most definitive treatment for this condition. In a detailed study of 345 patients, chemotherapy and radiation therapy seemed to be contraindicated for the treatment of ameloblastomas.[2] Thus, surgery is the most common treatment of this tumor. Because of the invasive nature of the growth, excision of normal tissue near the tumor margin is often required. Some have likened the disease to basal cell carcinoma (a skin cancer) in its tendency to spread to adjacent bony and sometimes soft tissues without metastasizing. While not a cancer that actually invades adjacent tissues, ameloblastoma is suspected to spread to adjacent areas of the jaw bone via marrow space. Thus, wide surgical margins that are clear of disease are required for a good prognosis. This is very much like surgical treatment of cancer. Often, treatment requires excision of entire portions of the jaw. Radiation is ineffective[2] in many cases of ameloblastoma.[11] There have also been reports of sarcoma being induced as the result of using radiation to treat ameloblastoma.[12] Chemotherapy is also often ineffective.[12] However, there is some controversy regarding this[13] and some indication that some ameloblastomas might be more responsive to radiation that previously thought.[14][6] While the Mayo Clinic recommends surgery for almost all ameloblastomas, there are situations in which a Mayo Clinic physician might recommend radiation therapy. These include malignancy, inability to completely remove the ameloblastoma, recurrence, unacceptable loss of function, and unacceptable cosmetic damage.[15] In the case of radiotherapy, oncologists at the Mayo Clinic would use intensity-modulated radiotherapy.[15]

[edit]Molecular biology There is evidence that suppression of matrix metalloproteinase-2 may inhibit the local invasiveness of ameloblastoma, however, this was only demonstrated in vitro.[16] There is also some research suggesting that 51 integrin may participate in the local invasiveness of ameloblastomas.[17] [edit]Recurrence Recurrence is common, although the recurrence rates for block resection followed by bone graft are lower than those of enucleation and curettage.[18]Follicular variants appear to recur more than plexiform variants.[2] Unicystic tumors recur less frequently than "non-unicystic" tumors.[2] Persistent follow-up examination is essential for managing ameloblastoma.[19] Follow up should occur at regular intervals for at least 10 years.[20] Follow up is important, because 50% of all recurrences occur within 5 years postoperatively.[2] Recurrence within a bone graft (following resection of the original tumor) does occur, but is less common.[21] Seeding to the bone graft is suspected as a cause of recurrence.[19] The recurrences in these cases seem to stem from the soft tissues, especially the adjacent periosteum.[22] Recurrence has been reported to occur as many as 36 years after treatment.[23] To reduce the likelihood of recurrence within grafted bone, meticulous surgery[21] with attention to the adjacent soft tissues is required.[22][19] [edit]Epidemiology The annual incidence rates per million for ameloblastomas are 1.96, 1.20, 0.18 and 0.44 for black males, black females, white males and white females respectively.[24] Ameloblastomas account for about one percent of all oral tumors[12] and about 18% of odontogenic tumors.[25] Men and women tend to be equally affected, although women tend to be 4 years younger than men when tumors first occur and tumors appear to be larger in females.[2] [edit]See also

Bone grafting Epithelial cell rests of Malassez List of cutaneous conditions Matrix Metalloproteinase-2 Tooth development and Odontogenesis

[edit]References

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73. doi:10.1002/1097-0142(19840215)53:4<869::AID-CNCR2820530409>3.0.CO;2V. PMID 6420036. 14. ^ Miyamoto CT, Brady LW, Markoe A, Salinger D (June 1991). "Ameloblastoma of the jaw. Treatment with radiation therapy and a case report.". Am J Clin Oncol. 14 (3): 225 30.doi:10.1097/00000421-199106000-00009. PMID 2031509. 15. ^ a b "Ameloblastoma Treatment". 3 January 2009. 16. ^ Anxun Wang, Bin Zhang, Hongzhang Huang, Leitao Zhang, Donglin Zeng, Qian Tao, Jianguang Wang and Chaobin Pan (2008). "Suppression of local invasion of ameloblastoma by inhibition of matrix metalloproteinase-2 in vitro". BMC Cancer 8 (182): 182.doi:10.1186/1471-2407-8-182. PMC 2443806. PMID 18588710. 17. ^ Souza Andrade ES, da Costa Miguel MC, Pinto LP, de Souza LB. (June 2007). "Ameloblastoma and adenomatoid odontogenic tumor: the role of alpha2beta1, alpha3beta1, and alpha5beta1 integrins in local invasiveness and architectural characteristics.". Ann Diagn Pathol. 11 (3): 199 205. doi:10.1016/j.anndiagpath.2006.04.005. PMID 17498594. 18. ^ Vasan, NT (March 1995). "Recurrent ameloblastoma in an autogenous bone graft after 28 years: a case report". N Z Dent J. 91 (403): 123. PMID 7746553. 19. ^ a b c Choi YS, Asaumi J, Yanagi Y, Hisatomi M, Konouchi H, Kishi K. (January 2006). "Recurrence of an ameloblastoma in an autogenous iliac bone graft.". Dentomaxillofac Radiol35 (1): 436. doi:10.1259/dmfr/13828255. PMID 16421264. 20. ^ Su, T; Liu, B; Zhao, JH; Zhang, WF; Zhao, YF (February 2006). "Ameloblastoma recurrence in the grafted iliac bone: report of three cases". Shanghai Kou Qiang Yi Xue. 15 (1): 10911.PMID 16525625. 21. ^ a b Dolan EA, Angelillo JC, Georgiade NG. (April 1981). "Recurrent ameloblastoma in autogenous rib graft. Report of a case.". Oral Surg Oral Med Oral Pathol. 51 (4): 357 60.doi:10.1016/0030-4220(81)90143-2. PMID 7015222. 22. ^ a b Martins WD; Fvaro DM. (December 2004). "Recurrence of an ameloblastoma in an autogenous iliac bone graft.". Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 98 (6): 657659. doi:10.1016/j.tripleo.2004.04.020. PMID 15583536. 23. ^ Zachariades N. (October 1988). "Recurrences of ameloblastoma in bone grafts. Report of 4 cases.". Int J Oral Maxillofac Surg. 17 (5): 3168. doi:10.1016/S09015027(88)80011-0.PMID 3143780. 24. ^ Shear M, Singh S (July 1978). "Age-standardized incidence rates of ameloblastoma and dentigerous cyst on the Witwatersrand, South Africa". Community Dent Oral Epidemiol 6 (4): 1959. doi:10.1111/j.1600-0528.1978.tb01149.x. PMID 278703.

25. ^ Jonathan Gordon, M.D., Ph.D. (30 December 2008). "Clinical Quiz: Painless Mass". Appl Radiol Online 3 (8).