Ameloblastoma
2020081
218321258
2008-06-10T02:56:14Z
DumZiBoT
6085301
robot Adding: [[it:Ameloblastoma]]
{{Infobox_Disease |
Name = Ameloblastoma |
Image = |
Caption = |
DiseasesDB = 31676 |
ICD10 = {{ICD10|D|16|5|d|10}} |
ICD9 = {{ICD9|213.1}} |
ICDO = 9310/0 |
OMIM = |
MedlinePlus = |
eMedicineSubj = |
eMedicineTopic = |
MeshID = D000564 |
}}
'''Ameloblastoma''' is a rare, [[benign]] tumor of odontogenic [[epithelium]] (ameloblasts, or outside portion, of the teeth during development) much more commonly appearing in the mandible than the maxilla. While these [[tumors]] are rarely [[malignant]] or [[metastatic]] (that is, they rarely spread to other parts of the body), and progress slowly, the resulting [[lesions]] 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. Further, dentists caution that wide surgical excision is not invasive enough to adequately treat this disorder.
==Subtypes==
There are three main clinical subtypes of ameloblastoma: unicystic, multicystic, peripheral. 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..
==Clinical features==
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 [[radiograph]]ic examination and must be confirmed by histological examination (e.g., [[biopsy]]). Radiographically, it appears as a lucency in the bone of varying size and features--sometimes 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 teeth--one of the many reasons dentists recommend having them extracted.
==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.
==Variants==
Desmoplastic Ameloblastoma,
Granular Cell variant,
Basal Cell variant.
==Treatment==
Ameloblastomas are relatively resistant to [[chemotherapy]] or [[radiation therapy]], 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. Recurrence is common.
=References=
*'''Zahid,Arsalan''' from Contemporary Oral and Maxillofacial Pathology
==Support link==
[http://survivor-support.rare-cancer.org/forum/groupcp.php?g=1514 Adult Rare Brain Tumors]
{{Oral pathology}}
{{Soft tissue tumors and sarcomas}}
[[Category:Oral pathology]]
[[Category:Oral and maxillofacial surgery]]
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[[it:Ameloblastoma]]
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