Ameloblastic fibrosarcoma – A rarity?

Munisekhar, Manay Srinivas; Shylaja, S.; Kumar, R.; Rao, Krishna A; Patil, Santosh; Alam, Mohammad Khursheed

doi:10.1016/j.jpra.2019.05.001

Cited by 2 publications

(3 citation statements)

References 11 publications

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“…Nonetheless, several reports in the literature documented that the epithelial components of odontogenic sarcoma will gradually disappear or become extremely sparse as the malignant transformation progresses, as occurred in this case. Therefore, we speculate that multiple reports of fibrosarcoma of the mandible could have been an odontogenic sarcoma with a disintegration of the odontogenic epithelium [ 12 ]. It is known that odontogenic sarcomas may arise from the malignant transformation of ameloblastic fibromas.…”

Section: Discussionmentioning

confidence: 99%

“…Recent molecular analysis studied seven cases of odontogenic sarcomas and found five out of seven cases to be positive for the BRAF-V600E mutation. Targeted therapy for the BRAF-V600E mutation is currently available, and it would be an excellent option for patients with this aggressive, rare disease [ 12 ]. In this case, the BRAF V600E immunostain was performed and was negative.…”

Section: Discussionmentioning

confidence: 99%

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Odontogenic Sarcomas of the Mandible

Akers,

Geisler,

Qiu

et al. 2024

Biomedicines

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Odontogenic sarcomas are exceedingly rare and account for less than 5% of all Maxillofacial Sarcomas. It usually affects the younger population. The posterior mandible is the most commonly affected site. Radiographically, it appears as a large destructive radiolucent lesion with ill-defined margins. Histopathological diagnosis is usually difficult. Surgery is the mainstay treatment. The role of chemotherapy and radiation therapy is not clear. Here, we present a case study of a 30-year-old female patient diagnosed with odontogenic sarcoma that impinged on her airway. The treatment and postoperative course will be discussed in detail.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Odontogenic Sarcomas of the Mandible

Akers,

Geisler,

Qiu

et al. 2024

Biomedicines

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“…AFS is believed to be a malignant spectrum of the dysplastic changes arising in ameloblastic fibroma (AF). Both tumors are exceedingly rare, with 107 AFS cases reported in the literature [5][6][7].…”

Section: Introductionmentioning

confidence: 99%

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

Barakeh,

Alsolami,

Abedalthagafi

2023

Case Rep Oncol

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Ameloblastic fibrosarcoma (AFS) is considered a malignant progression resulting from dysplastic changes in an ameloblastic fibroma (AF). Both tumors are extremely rare, with only a few cases reported in the scientific literature. Notably, BRAF mutations have been identified in ameloblastomas, suggesting a connection between ameloblastic morphology and BRAF mutations, as AF is believed to be the precursor neoplasm leading to AFS. In this study, we present a case of AFS in a 25-year-old male. The tumor tissue underwent molecular analysis, specifically next-generation sequencing (NGS) using the Oncomine Comprehensive Assay v3 System. The analysis revealed pathogenic mutations in TP53 and RB genes, as well as copy number gains in NTRK1, MDM4, and BRAF. Additionally, we provide a summary of the literature’s findings from the analysis of 107 previously reported AFS cases. Our findings suggest the existence of a molecularly distinct subtype, emphasizing the importance of comprehensive molecular testing for these patients.

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Ameloblastic fibrosarcoma – A rarity?

Cited by 2 publications

References 11 publications

Odontogenic Sarcomas of the Mandible

Odontogenic Sarcomas of the Mandible

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

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