Ameloblastic Fibrosarcoma: A Case Report and Literature Review

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 high Ki-67 positivity in stromal cells (mean of 20.9 cells/High power field). Epithelial cells displayed strong positivity for p53, p63 and cytokeratin 19. In addition to the case report, a systematic review of current knowledge is presented on the AFS’s clinical-demographic features and prognostic factors. Based on the review, 88/99 cases were diagnosed as AFS, 9/99 as ameloblastic fibro-odontosarcoma and 2/99 as ameloblastic fibrodentinosarcoma. 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 odontogenic 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.

Resumo Aqui é descrito um caso de fibrossarcoma ameloblástico afetando região posterior da mandíbula de uma mulher. Após o tratamento, a paciente ficou livre da doença durante os 12 anos de acompanhamento. Foi realizado imunohistoquimica para marcadores epiteliais, mesenquimais e do ciclo celular. Além disso, uma revisão sistemática de literatura também foi realizada, na tentativa de descobrir as características clínico-demográficas e fatores prognósticos da lesão. 88/99 casos foram diagnosticados como fibrossarcoma ameloblastico, 9/99 como fibro-odontosarcoma ameloblastico e 2/99 como fibrodentinosarcoma ameloblastico. Todas estas lesões exibem características clínico-demográficas e prognósticos muito semelhantes. Além disso, esta revisão forneceu evidências de que primeiro tratamento, metástases regionais, metástases à distância e recorrência local são valores prognósticos significativos para lesões odontogênicas mesenquimais malignas. A análise imunohistoquímica demonstrou elevada marcação positiva em células do estroma para Ki-67 (média de 20,9 células /HPF). As células epiteliais exibiram forte marcação para p53, p63 e citoqueratina 19. A segregação entre fibrosarcoma ameloblastico, fibrodentinosarcoma ameloblastico e fibro-odontosarcoma ameloblastico é ilógica, uma vez que todas essas lesões têm predileções e resultados semelhantes.

A case report of ameloblastic fibrosarcoma in the mandible

Amelobastic Fibrosarcoma of the Mandible: A case report

Ameloblastic fibrosarcoma is an extremely rare variety of odontogenic tumor. It has not previously been reported in Korea. The tumor is composed of benign odontogenic epithelium with a mesenchymal part which exhibits the histologic features of fibrosarcoma. We have reported a case of amloblastic fibrosarcoma of the mandible in a 26-year-old man with swelling of right mandible for 2 weeks. The tumor showed yellowish ill-demarcated ulcerating mass involving right premolar and molar area. Light microscopy revealed irregularly arranged strands and islands of odontogenic epithelium surrounded by abundant mesenchymal tissue with the feature of fibrosarcoma. The fibrosarcoma cells were strong positive on immunostain for vimentin and ameloblastic cells were weakly positive for cytokeratin. S-100 and CEA were negative in both epithelial and sarcoma cells. The sarcoma cells were corresponding to fibroblasts on the electron microscopy with abundancy of RER and mitochondria and covering of basal lamina. Two types of virus like particles were distributed in the cytoplasm and nuclei of sarcoma cells. We treated the patient with surgery and chemotherapy. The recovery was uneventful and the prognosis is under observation.