Ameloblastic Fibrosarcoma in Pregnancy: an Unreported Entity!

Abstract

AFS is a rare tumor of odontogenic origin. Less than 100 cases have been reported so far in the literature. Due to its extreme rarity, we do not have clear management guidelines at present. The entire clinical spectrum and natural history of this uncommon malignancy is unknown. Most of the cases present with facial swelling along with varying degrees of oral signs and symptoms. AFS has higher incidence in male and common in third decade of life. However, presentation in female can be unique and may have a different course and outcome then what is known to us. The association of pregnancy and AFS is interesting and has hardly been emphasized before. Pregnancy can dramatically boost the growth of this tumor leading to impending airway obstruction or fatal hemorrhage. We report a case of an 18-year-old pregnant female who presented with rapidly enlarging bleeding mandibular mass and respiratory distress and present the challenges in its surgical management along with it longterm outcome on follow-up. We also discuss and explore the probable role of AFS in context of pregnancy and its oncological outcome.

Ameloblastic Fibrosarcoma of the Jaw: Case Report, Genetic Profiling, and Literature Review

“…A systemic review was published in 2017 [ 11 ], highlighting the clinical features in 99 previously reported cases, although full data were missing in a handful of cases. In the period of 2017–2020, only 8 cases were reported, one in 2019 [ 12 ] and 7 in 2020 [ 9 ]. We have summarized the clinical findings of all 107 cases in Table 2 .…”