Ameloblastic Fibrosarcoma - A Review of the Literature
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2017, Vol 1, Issue 1
Abstract
Ameloblastic Fibrosarcoma is an extremely rare odontogenic tumor with unclear etiology, which has clinical and radiological features similar to other odontogenic tumors. The rarity of these neoplasms plus their overlapping features with other odontogenic tumors can make diagnosis challenging. These cases should be discussed and reviewed by oral pathologists in multidisciplinary conferences, dealing with sarcoma, at national and international level. The first description of the rare odontogenic tumor, ameloblastic fibrosarcoma (AFS), was in 1887 [1]. Various terminologies like ameloblastic dentinosarcoma and ameloblastic odontosarcoma were employed in the past to denote these types of lesions, which depended upon the presence of enamel or dentin. In the recent past, multiple authors considered these lesions to be the histological variants of the same lesion and were thought to be for a long time. Recently, the World Health Organization classification of the odontogenic tumors, ameloblastic odontosarcoma, and dentinosarcoma are again separately listed. Reported cases of AFS had ages ranging from 3 to 89 years, [2] with a mean age of all the reported cases summing upto 27.3 years [3], which shows that it has a major tendency towards the younger population. The usual clinical presentation is painful, but occasionally painless facial mass. Paresthesia and dysesthesia are the next progressive symptoms. Continuance of the symptomatic episodes is also gradual and has a varied range, from a few days to two years. When analyzing various published articles reporting cases of AFS and literature reviews, it can be seen that more than 32% of the reported cases of AFS were recurrences and this points towards a slightly increased rate of recurrence which further necessitates meticulous removal of the lesion [4]. Radiographic AFS, as per reports, is an expansile destructive mass with ill-defined and irregular borders. The gross tumor appearance varies from cystic or solid with a fleshy whitish to yellow consistency, which is the cause of bone destruction. Histologically, the epithelial component mimics the developing enamel organ, and the mesenchymal cells vary from hyperchromatic spindle to satellite and exhibit moderate to marked nuclear pleomorphism with a high number of mitotic figures. Fibroblastic presence is seen ultra structurally. Immuno histochemically, multiple reports showed that while ameloblastic fibroma shows negativity towards the p 53 and proliferating cell nuclear antigen (PCNA), AFS show positivity towards the same [4]. Recurrent AFS shows higher labeling indices for Ki-67 as compared with non- recurrent AFS, and the same can be used to identify between the same and can be managed accordingly [5,6].
Authors and Affiliations
Shermil Sayd, Suresh Vyloppilli
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