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AMELOBLASTOMA A.

Definition Ameloblastoma is a tumor in which the tumor cells form caricatures of the enamel organ and some of them resemble ameloblasts. However, they are incapable of making enamel matrix. This tumor occurs chiefly in middle age people long after odontogenesis has ceased. Presumably a carcinogen converts a cell in one of the epithelial rests to become a tumor cell. As such, it starts to divide endlessly to form a tumor. Remember the axiom, tumor cells tend to resemble the tissues from which they arise. Since cells in rests of Serres and Malassez were at one time capable of becoming ameloblasts, it should not come as a surprise that tumor cells resemble ameloblasts (Charles, 2001). B. Classifications 1. Solid / Multicystic Ameloblastoma Clinical Features Small multicystic ameloblastoma s may be asymptomatic. More commonly, multicystic ameloblastoma present as variably sized swellings of the jaws. Pain or paraesthesia are rare. Multicystic ameloblastoma may be unilocular or multilocular radiolucencies resembling cysts and they may reveal scalloped borders. An unerupted tooth may be associated with multicystic ameloblastoma. Resorption of the roots of adjacent teeth is common. Definitive diagnosis of multicystic ameloblastoma cannot be made radiologically, since similar radiographic features are displayed by e.g. keratocystic odontogenic tumour or myxoma. Particularly for maxillary multicystic ameloblastoma, CT-scans or MRIs are recommended (Gardner, 2001). Histopatology There are two basic histopathologic patterns, the follicular and plexiform, without clinical relevance. The follicular pattern consists of islands of odontogenic epithelium within a fibrous stroma. Typically, the basal cells of these islands are columnar, hyperchromatic, and lined up in a palisaded fashion. Typically

their nuclei are displaced away from the basement membrane, and their cytoplasm is generally vacuolated. The central cells may be loosely arranged, resembling stellate reticulum. These areas often become cystic and at times confluent. If these cells are spindleshaped, basaloid, granular or showing squamous differentiation, the terms spindle cell ameloblastoma, basal cell ameloblastoma, granular ameloblastoma and acanthomatous ameloblastoma have been used. In addition ghost cells may be observed. The plexiform pattern contains basal cells arranged in anastomosing strands with an inconspicuous stellate reticulum. The stroma is usually delicate, often with cystlike degeneration. For both histologic patterns, mitotic activity and cellular pleomorphism are rarely noted. The microscopic differential diagnosis may include ameloblastic fibroma, squamous odontogenic odontogenic tumour, remnants adenomatoid in dental odontogenic follicles, tumour, epithelial-rich

odontogenic fibroma, calcifying odontogenic cyst and adenoid cystic carcinoma arising form the maxillary sinus (Gardner, 2001). 2. Peripheral Ameloblastoma Clinical Features The peripheral ameloblastoma is a painless, firm and exophytic growth with a smooth, pebbly or papillary surface. Rarely, intraosseous ameloblastomas may extend to the gingival tissues and merge with the gingivalepithelium, creating an exophytic peripheral ameloblastoma like lesion. Apart from a superficial erosion or depression (saucerizationor cupping) of the bone crest due to pressure resorption, there is rarely signi ficant bone involvement (Gardner, 2001).

Fig. . peripheral ameloblastoma (Charles, 2001).

Histopatology The peripheral ameloblastoma consists of odontogenic epithelium with the same histomorphological cell types and patterns as seen in multicystic ameloblastoma. Some lesions are located entirely within the connective tissue of the gingiva, showing no continuity with the surface epithelium, whereas others seem to fuse with or originate from the mucosal epithelium. It is generally believed that basal cell carcinoma of the gingival (BCCG) and peripheral ameloblastoma represent the same neoplasm. Squamous cells in the acanthomatous areas of peripheral ameloblastoma may show ghost cell formation, and in some parts of the tumour islands, vacuolated or clear cells occur in discrete clusters. The stroma is that of a mature, fibrous connective tissue. Rare cases of malignant peripheral ameloblastoma (ameloblastic carcinomas) have been reported (Gardner, 2001). 3. Desmoplastic Ameloblastoma Clinical Features A painless swelling of the jaw bone represents the chief initial complaint. The size of the tumour varies between 1.0 and 8.5 cm in diameter. An extraosseous variant of desmoplastic ameloblastoma has not been reported. Radiographically, ameloblastomas show a about 50% of mottled, mixed desmoplastic

radiolucency / radiopacity with diffuse margins, suggesting a fibroosseous lesion. Resorption of tooth roots and bone formation may occur. The ill-defined borders of desmoplastic ameloblastomas make high-resolution CT and MRI helpful in treatment planning (Gardner, 2001). Histopatology In desmoplastic ameloblastomas the stromal component dominates, compressing the odontogenic epithelial components. The epithelial tumour islands are very irregular or bizarre in shape with a pointed,

stellate appearance. The epithelial cells at the periphery of the islands are cuboidal with occasional hyperchromatic nuclei. Columnar cells with nuclear polarity are rarely conspicuous. The islands have a swirled, hypercellular centre with spindle-shaped or squamous, epithelial cells. Microcysts may occur centrally. Myxoid changes of the juxtaepithelial stroma are often found. Formation of metaplastic osteoid trabeculae (osteoplasia) may be present {2035}. A fibrous capsule is not present corresponding to the radiographically poorly defined tumour margin. A combination of desmoplastic ameloblastoma with multicystic ameloblastoma is known and has been termed as hybrid lesion (Gardner, 2001).

Fig. . Desmoplastik ameloblastoma (Gardner, 2001).

4. Unicystic Ameloblastoma Clinical Features Some cases are asymptomatic, sometimes presenting as a swelling of the posterior mandible. Up to 80% are associated with an unerupted mandibular third molar. The lesion presents radiographically as a well corticated unilocular, often pericoronal radiolucency. Root resorption may occur. The clinical radiographic diagnosis is frequently a dentigerous (follicular) cyst (Gardner, 2001). Histopatology Two histopathologic variants exist. The luminal variant is a cystic lesion lined by ameloblastomatous epithelium. In addition

intraluminal extensions may occur. These extensions usually exhibit a plexiform epithelial pattern. There is no tumour infiltration into the fibrous wall. The mural variant, the cyst wall is infiltrated by ameloblastomatous epithelium that exhibits either a follicular or plexiform pattern. Sometimes both variants may occur in the same lesion. The mural variant of unicystic ameloblastoma may be confused with either dentigerous cysts or dental follicles containing a lot of odontogenic epithelial remnants. These epithelial nests, however, do not show the typical histiologic features of ameloblastoma: peripheral palisading and nuclear polarization (Gardner, 2001).

Fig. . Unicystic ameloblastoma showing ameloblastomatous epithelium lining the cyst wall (Gardner, 2001).

C. Etiology and Pathogenesis Etiology A benign, aggressive jaw tumor of odontogenic epithelial (ectodermal) origin. The most common odontogenic tumor after the odontoma. Incidence of 0.3 cases per million people. (Sciubba J, 2002) Pathogenesis This neoplasm originates within the mandible or maxilla from epithelium that is involved in the formation of teeth. Potential epithelial sources include the enamel organ, odontogenic rests (rests of Malassez, rests of Serres), reduced enamel epithelium, and the epithelial lining of odontogenic cysts,

especially dentigerous cysts. The trigger or stimulus for neoplastic transformation of these epithelial residues is totally unknown. Mechanisms by which ameloblastomas gain a growth and invasion advantage include overexpression of anti-apoptotic proteins (Bcl-2, Bcl-xL) and interface proteins (fibroblast growth factor [FGF], matrix metalloproteinases [MMPs]). Ameloblastomas, however, have a low proliferation rate. Mutations of the p53 gene do not appear to play a role in the development or growth of ameloblastoma. D. Treatment 1. Surgery Surgery is the optimal treatment for patients with ameloblastoma and ameloblastic carcinoma. The optimal surgical approach remains controversial. Conservative options include enucleation, curettage, cryotherapy, electrocautery, marsupialization, or any combination of the above. Wide resection involves segmental or rim resection of the mandible or maxilla (Sampson DE, 1999) Conservative resections have resulted in high local recurrence rates. Sehdev et al reported local recurrences in excess of 90% in a series of 92 patients with ameloblastoma treated with curettage alone (Sehdev MK, 1974). With a mean follow-up of 3 years, Sampson and Pogrel reported a 100% local recurrence rate in 11 patients undergoing curettage for ameloblastoma (Sampson DE, 1999). Some authors have emphasized the importance of distinguishing unicystic from the solid or multicystic variant of ameloblastoma; the latter warrants more aggressive surgical intervention. Muller and Slootweg reported a series of 84 patients and observed local recurrence rates of 75% versus 20% after conservative resection of multicystic versus unicystic ameloblastomas (Muller H, 1985). Following wide resection for multicystic tumours, the recurrence rate declined to 15%. In addition, they noted that 95% of local recurrences developed within 5 years after surgery. Gardner and Pecak reported that conservative surgical treatment should be considered only in the presence of unicystic lesions when extraosseous spread has not occurred (Gardner DG, 1980). They concluded that unacceptably high

recurrence rates occur in the multicystic or solid variant, which often exhibits invasion into the intertrabecular spaces of the cancellous bone, making complete resection with conservative methods exceedingly difficult. In addition, Gardner and Pecak concluded that ameloblastomas in the posterior part of the maxilla should be treated more aggressively than similar lesions in the mandible, due to the proximity of vital structures and difficulty of treating subsequent recurrences (Gardner DG, 1980). Due to the high risk of local recurrence, optimal resection requires a more than 1 cm margin of uninvolved cancellous bone surrounding the primary tumour (Feinberg SE, 1996). However, local recurrence rates are as high as 15% to 25% after wide resection. Reportedly, salvage surgery can control 80% of mandibular tumours but only 40% of maxillary tumours (Sehdev MK, 1974). Therefore, patients at high risk for developing recurrence after surgery should be considered for adjuvant therapy. 2. Radiotherapy There are relatively few data pertaining to the efficacy of radiotherapy. Robinson reported one of the first series, in which 18 patients were treated with radiotherapy alone; 13 patients (72%) developed a local recurrence (Robinson HB.1937). Radiotherapy consisted of orthovoltage external beam radiotherapy, radium needles, or radon seeds (Robinson HB.1937). Sehdev et al reported on 11 patients treated at the Memorial Sloan Kettering Cancer Center with radiotherapybetween 1921 and 1951 (Sehdev MK, 1974). Although the tumour initially responded in some patients, all eventually experienced progression of persistent disease or a local recurrence. Recently published studies analyzing the efficacy of megavoltage therapy in the management of ameloblastoma have questioned the proposition that these tumours are inherently radioresistant. Gardner reported on 3 patients treated with megavoltage radiotherapy (40, 45 and 55 Gy, respectively); all 3 responded initially but later recurred. Based on these results, Gardner concluded that radiotherapycan produce regression of an ameloblastoma, particularly the part which causes expansion of the jaw or has invaded the adjacent soft tissues but that

it is not appropriate treatment for ameloblastomas and should be reserved for unresectable tumours (Gardner DG, 1988). Atkinson et al published a case series of 10 patients treated at Princess Margaret Hospital between 1958 to 1982.27 Two patients underwent total excision and postoperative radiotherapyand 1 patient underwent subtotal excision and radiotherapy; all 3 remained alive and disease-free at 27 months, 30 months, and 5 years after treatment. Seven patients were treated with radiotherapyalone; 1 patient had persistent disease and required further treatment and the remainder experienced slow regression of the tumour which remained locally controlled in all 6 patients. In a case report and review of the literature, Miyamoto et al asserted that ameloblastoma is radiosensitive.32 They proposed guidelines for treatment planning as follows: 1) radiotherapyportals should include the entire tumour volume with a 2 cm margin, 2) lymph nodes should not be included unless clinical involvement is suspected, and 3) doses of at least 45 to 50 Gy in 4 to 5 weeks using 1.8 Gy fractions are necessary to control the tumour. All 5 of our patients treated with surgery and radiotherapy (4 patients) or radiotherapy alone (1 patient) have remained disease free after treatment. 3. Chemotherapy Experience with chemotherapy is minimal in the treatment of ameloblastoma and is largely limited to isolated cases. Lanham described a case report of ameloblastoma metastatic to the lungs and submandibular nodes treated with doxorubicin, cisplatin, cyclophosphamide, dacarbazine, and 5-fluorouracil; the tumour failed to respond (Lanham RJ, 1987). Duffey et al reported a case of ameloblastoma with dissemination to cervical lymph nodes, liver, and lungs treated with multi-agent chemotherapy (Duffey DC, 1995). The tumour did not respond to treatment. In contrast, Grunwald et al described a case of ameloblastoma metastatic to the lungs and pleura, which exhibited response to paclitaxel and carboplatin (Grunwald V, 2001)

Dapus

Charles Dunlap, DDS. 2001. Odontogenic Tumors. UMKC School of Dentistry D.G. Gardner et. al. 2000. Ameloblastomas. Availlable at http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb9/bb9chap6.pdf . Access on 24 April 2013 15.02. Sciubba J, Regezi J, and Rogers R. 2002. PDQ Oral Disease: Diagnosis and Treatment. Hamilton: BC Decker Inc p.260