1. Eur Ann Otorhinolaryngol Head Neck Dis. Feb;(1) doi: / Epub Jul 9. A case of an ameloblastic fibrosarcoma in the mandible is described. The primary tumor was seen in a 5-year-old child. In spite of repeated surgical. Introduction. Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor. It can arise de novo, however one-third of cases may arise from a recurrent.

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Here is described a case of ameloblastic fibrosarcoma AFS affecting the posterior mandible of a woman who was treated surgically and recovered without signs of recurrence or metastasis after 12 years of follow-up. Tumor sections were immunostained for cell cycle, epithelial and mesenchymal markers. Immunohistochemical analysis evidenced ameloblastid Ki positivity in stromal cells mean of Epithelial cells displayed strong positivity for p53, p63 and cytokeratin All these lesions displayed very similar clinical-demographic and prognostic features.

Moreover, the review provided evidence that first treatment, regional metastasis, distant metastasis and local recurrence were significant prognostic values for malignant ifbrosarcoma mesenchymal lesions. Based on the findings, segregation among ameloblastic fibrosarcoma, ameloblastic fibrodentinosarcoma and ameloblastic fibro-odontosarcoma seems illogical, considering all these lesions have similar predilections and outcomes. Foi realizado imunohistoquimica para marcadores epiteliais, mesenquimais e do ciclo celular.

It is defined as an odontogenic tumor composed of malignant ectomesenchyme in which variable quantities of a benign epithelial component can be seen.

It is regarded as the malignant counterpart of ameloblastic fibroma AF. However, most cases were diagnosed as primary malignant processes 12345. Since then, there were about cases of similar microarchitectural features described in the literature.

The essence of this view has remained unaltered since that time 18. All these components are included in a highly cellular malignant connective stromal component, with cells showing variable degrees of anaplasia 1.

Although AFS was first described years ago, information about its epidemiology, treatment, predictive factors and expected outcomes is very limited, and is based mainly on case reports 91011 In those cases, when the inductive process resulted only in the deposition of dentine, the lesions were called ameloblastic fibrodentinosarcomas AFDSs ; when dentine and enamel were identified concurrently, the term ameloblastic fibro-odontosarcoma AFOS was used 1 Moreover, there are few reports on the proliferative potential of AFS, based on cell cycle markers.

There is also a lack of data on the effects of the sarcomatous component of the lesion on proliferative activity and differentiation aspects of the ameloblastic epithelium present in AFS. Taken together, these data could improve the understanding of tumor pathogenesis and progression and aid in the differentiation of subtle malignant transformations of AF.

The aim of this report was to describe a case of AFS with an immunohistochemical evaluation. A year-old non-white woman was referred for evaluation and treatment of a large and persistent malignant swelling in the left mandible. The patient complained of pain and dysphagia for 4 months. An extraoral examination disclosed a large, firm swelling, involving the left mandibular ramus. Examination of other head and neck structures and a general physical evaluation yielded unremarkable findings.

An intraoral examination revealed lingual and buccal cortical bone expansion affecting the mandibular body, with no clinical or imaging evidence of cortical perforation. Oral mucosa and mandibular nerve functions were normal and intact. Plain radiographic examinations showed an ill-demarcated and partially corticated multilocular radiolucency affecting the left posterior mandible.


An incisional biopsy was performed, followed by routine histopathology, resulting in the diagnosis of primary AFS. En bloc surgical resection from the second premolar to the anterior ramus region was performed under general anesthesia. Immediate reconstruction was performed using an autogenous right iliac bone graft, stabilized with a titanium plate.

The patient did not undergo any other treatment. Histopathological examination of the resected specimen showed evidence of scant cords and nests of odontogenic epithelium scattered within the mesenchymal stromal tissue Fig. Stromal components contained hyperchromatic and pleomorphic cells, as well as numerous mitotic structures Fig. There were foci of necrosis near the epithelial islands. Odontogenic epithelial islands, in a follicular or trabecular aspect, showed hyperplasia of peripheral columnar cells; these cells showed hyperchromatic and discrete anisokaryosis Fig.

In some places, dentinoid material of variable dimensions was associated closely with the epithelial islands. B Marked pleomorphism in odontogenic ectomesenchyme adjacent to an epithelial island.

C Odontogenic ectomesenchyme showing high pleomorphism, hyperchromatism and mitotic figure arrow.

Ameloblastic Fibrosarcoma of the Mandible: A Case Report and Brief Review of the Literature

The streptavidin-biotin-peroxidase method was used following standard protocols. Endogenous avidin-biotin binding properties and endogenous peroxidase activity were blocked according to Miller et al Finally, sections were counterstained with Harris hematoxylin. As a negative control, ameliblastic antibodies were replaced with phosphate-buffered saline.

The products are multiplicative quick-score and score values, ranging from 0 to For Ki analysis, the mean number of positive cell nuclei in 10 consecutive high-power fields HPFs was determined. Most epithelial cells had small numbers of Kipositive cells All compartments of fibrosafcoma tumor were negative for Bcl The atypical mesenchymal cells showed strong diffuse nuclear and cytoplasmic reactivity for p16, whereas the epithelial component showed only weak staining for this marker Fig.

Ameloblastic Fibrosarcoma of the Mandible: A Case Report and Brief Review of the Literature

However, the epithelial cells showed strong diffuse nuclear and cytoplasmic reactivity for p53 Fig. The benign ameloblastomatous component showed weak diffuse cytoplasmic reactivity for CK8 and CK14 Fig. The stromal malignant component was positive for vimentin and SMA Fibrosarcma.

There was no reactivity for CK7, CK18, calretinin, fibronectin or desmin. The following search terms were used: Papers describing clinicopathological features similar to those described for AFS were included in the first instance 4591011121316 – 2528293032343536373839424345 – 6567 – 7274 – 89919394 Studies that could not be accessed and those with inconsistent or equivocal reporting of features, like suboptimal histological illustrations or unclear clinicopathological data, were excluded 6171819202122232426273133404144667390 Data on sociodemographic characteristics, clinical features, treatment, follow-up, and outcomes were collected and tabulated for each study.

Descriptive statistics were used to assess the clinicopathological data. All collected information was considered in the overall survival analysis. Curves for different clinicopathological factors were traced using the Kaplan-Meier method and then compared using univariate analyses log-rank test. The chi-square test was used to analyze any association between clinical and histomorphological factors.

The Spearman rank correlation test was used to determine whether lesion size was correlated with evolution time. All analyses were performed using the GraphPad Prism software v.


Tables 2 and Tables 3 highlight the overall clinicopathological features of AFS from the literature and the present case report.

Of these, 88 Died of disease; ED: For the cases diagnosed as AFS, age ranged widely from 0. Lesions usually affected the posterior mandible ameoblastic cases, Most of the lesions presented large mean, 5.

Only one peripheral lesion was reported and the bone was free of invasion. Out of 74 cases, 11 Surgical treatment was the primary therapy in most cases. There were regional and distant metastases reported in ameloblatic than Metastasis appeared 7 months after the first appointment in The overall mortality rate was The 9 cases diagnosed as AFOS affected the posterior mandible Swelling was present in all cases and pain was the chief complaint in six of eight Six of the eight lesions were considered as de novo malignancies; the remaining two were considered as malignant transformations of one AF and one ameloblastic fibro-odontoma.

There were recurrent primary tumors in four of nine Regional and distant metastatic foci were diagnosed in only one patient After a mean follow-up period of Both patients complained of swelling and pain.

One case was diagnosed as de novo and the other was diagnosed as malignant transformation of a previous AF. The fibroasrcoma received initial aggressive surgical management. None of these cases evolved with relapse or metastatic disease. Treatment was prescribed for only one case and the patient was disease-free for 18 months after diagnosis. An overall survival analysis was performed for all 99 cases. The median overall survival time was months, and the overall 5- and year survival rates were Results of a univariate overall survival analysis are in Table 3.

Statistically significant associations were identified for the following parameters: AFS is the most common of them 66. In Brazilian series, the reported frequency of AFS is up to 1. Because of the secondary types 88initial diagnosis of a malignant phenotype is not always easy. As anaplasia is not distributed uniformly throughout the tumor, definitive diagnosis can be made amelblastic from surgically removed pieces, as in the present case.

This information is required for the detailed evaluation of the clinical history and radiographic aspects to identify preoperatively aspects that may help ameloblqstic surgeon choose a more representative area to be biopsied or even to make multiple biopsies. However, in the case of clinicoradiographic evidence of an aggressive tumor, serial sampling of surgical specimens should be performed to look for a histologically malignant phenotype.

Clinically, most AFS are similar to AF, fibroosarcoma characteristics of aggressive behavior are always identified, as seen in the present case. Based on the present review, AFS is a locally invasive neoplasm predominantly affecting the mandibles of male individuals with a wide age range 4 months to fibrodarcoma years.

The most common clinical features of AFS are facial swelling, often accompanied by pain. Paresthesia, dysesthesia and ulcers are sometimes reported 53747 Radiologically, AFS appears as a uni- or multilocular radiolucent mass with an ill-defined border, frequently causing gross expansion, thinning or even rupture ameloblastc the cortical bone