Ameloblastic fibrosarcoma

INTRODUCTION: Odontogenic tumors (OTs) are a heterogeneous group of lesions that are derived from odontogenic apparatus comprised of odontogenic epithelium, ectomesenchyme, and/ or mesenchymal elements. The OTs show marked geographical variation. This study was conducted to analyze the epidemiology and clinicopathological presentation of OTs based on age, sex, and site. MATERIALS AND METHODS: This study was conducted in Department of Oral Pathology and Microbiology...

PURPOSE: To integrate the available data published to date on ameloblastic fibromas (AF) and ameloblastic fibrosarcomas (AFS) into a comprehensive analysis of their clinical/radiological features. METHODS: An electronic search was undertaken in July 2017. Eligibility criteria included publications having enough clinical, radiological and histological information to confirm a definite diagnosis. RESULTS: A total of 244 publications (279 central AF tumours, 10 peripheral AF, 103 AFS) were included...

A 24-year-old womanpresented with ameloblastic fibrosarcoma arising from ameloblastic fibroma. The delayed presentation accounted for the extensive destruction of the mandible and complete occlusion of her oral cavity. This resulted in an inability to eat and maintain oral hygiene. A multidisciplinary team management approach involved nutritional optimisation, segmental mandibulectomy, reconstruction with a reconstructive plate and a free anterolateral thigh flap to line the the floor of mouth. Functional and aesthetic outcome was acceptable, and the patient is planned for secondary free fibular flap bony reconstruction...

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...

Sarcomas are rare diseases of the head and neck region, representing around 1% of all malignancies. Amongst them, ameloblastic fibrosarcoma (AFS) is of even greater rarity, with less than 100 cases reported in the literature. Consequently, no standard treatment or guidelines have been made available. Surgery is often performed as primary therapy, but may be limited due to anatomical or functional reasons. We present a case of AFS successfully treated by postoperative radiation therapy. A detailed case study is provided, followed by a review of the English-language literature focusing on the role of radiation therapy...

Ameloblastic fibroma (AF) is an uncommon true mixed odontogenic tumor, with a relative frequency between 1.5% and 4.5% of all odontogenic tumors. It may behave either as a true neoplasm or as a hamartomatous proliferation of odontogenic epithelium of the enamel organ and odontogenic mesenchyme of the primitive dental pulp. Frequently diagnosed between the first and second decades of life with 75% of cases was diagnosed before the age of 20 and present with a well-defined unilocular or multilocular radiolucencies...

This article reviews a myriad of common and uncommon odontogenic cysts and tumors. The clinical presentation, gross and microscopic features, differential diagnosis, prognosis, and diagnostic pitfalls are addressed for inflammatory cysts (periapical cyst, mandibular infected buccal cyst/paradental cyst), developmental cysts (dentigerous, lateral periodontal, glandular odontogenic, orthokeratinized odontogenic cyst), benign tumors (keratocystic odontogenic tumor, ameloblastoma, adenomatoid odontogenic tumor, calcifying epithelial odontogenic tumor, ameloblastic fibroma and fibroodontoma, odontoma, squamous odontogenic tumor, calcifying cystic odontogenic tumor, primordial odontogenic tumor, central odontogenic fibroma, and odontogenic myxomas), and malignant tumors (clear cell odontogenic carcinoma, ameloblastic carcinoma, ameloblastic fibrosarcoma)...

BACKGROUND: Ameloblastic fibrosarcoma (AFS) is a rare odontogenic neoplasm of the jaw that usually arises de novo or through a malignant change in the mesenchymal component of a preexisting or recurrent benign fibroma. The majority of AFS cases reported in the literature arise in the mandible. CASE REPORT: A 35-year-old male presented with an asymptomatic left maxillary mass that on imaging was found to be effacing most of his maxillary sinus. He underwent a left maxillectomy with free-flap reconstruction and adjuvant radiotherapy to the tumor bed...

Transformation of an ameloblastic fibroma to an ameloblastic fibrosarcoma has been reported rarely in the literature. The present case report describes such evolution in a patient under long-term follow-up. The patient was first treated in 2008, and he developed the malignant counterpart of the disease 2 years later. The patient is currently under careful long-term follow-up and is free of disease. This article describes the clinical and radiographic features, histological characteristics, immunohistochemical findings, and surgical treatment of the tumor...

INTRODUCTION: Ameloblastic fibro-odontosarcoma is an extremely rare subtype of odontogenic sarcoma, with only 13 cases reported in the literature. CASE REPORT: A 4-year-old male presented with a painless mandibular swelling, which appeared 4months previously. Cone beam computed tomography revealed an extensive, ill-circumscribed, multilocular radiolucency of the right mandible extending from the first deciduous molar to the ramus with slightly dense opacities. Histological examination of the incisional biopsy specimen revealed a biphasic tumor with sarcomatous mesenchyme and benign ameloblastic epithelial component compatible with a diagnosis of ameloblastic fibrosarcoma...

OBJECTIVE: Determine survival and factors affecting survival for patients with malignant tumors of odontogenic origin. STUDY DESIGN: Retrospective analysis of the National Cancer Institute's SEER database (Surveillance, Epidemiology, and End Results). SETTING: Tertiary medical center. SUBJECTS AND METHODS: All cases of malignant tumors of odontogenic origin were extracted from the SEER database for the period of 1973 to 2011...

BACKGROUND Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor with a mesenchymal component, showing sarcomatous features and epithelial nests resembling ameloblastic fibroma (AF). CASE REPORT We report a case of AFS showing epithelial dysplasia arising in a recurrent AF in the left mandible after 3 years in a 26-year-old man, which is regarded as an uncommon histopathologic finding in AFS. We also emphasize the comprehensive clinical, radiographic, and histopathologic evaluation, and immunohistochemical staining of this patient...

Ameloblastic fibrosarcoma is a mixed odontogenic tumor that can originate de novo or from a transformed ameloblastic fibroma. This report describes the case of a 34-year-old woman with a recurrent, rapidly growing, debilitating lesion. This lesion appeared as a large painful mandibular swelling that filled the oral cavity and extended to the infratemporal fossa. The lesion had been previously misdiagnosed as ameloblastoma. Twenty months after final surgery and postoperative chemotherapy, lung metastases were diagnosed after she reported respiratory signs and symptoms...

Ameloblastic fibro-odontosarcoma (AFOS) is an extremely rare malignant odontogenic tumor. Complete surgical excision is the treatment of choice. Deaths due to disease recurrence and/or progression are documented. Here, we report the case of a 15-year-old female with multiple recurrent AFOS. She responded to chemotherapy with ifosfamide and doxorubicin consolidated by stereotactic reirradiation using cyberknife and remained in complete remission 14 months from the end of reirradiation therapy. Chemotherapy with ifosfamide and doxorubicin should be considered in advanced cases of AFOS...

Odontogenic carcinosarcoma is an extremely rare malignant odontogenic tumor with only a few reported cases. It is characterized by a true mixed tumor showing malignant cytology of both epithelial and mesenchymal components. It has been assumed to arise from pre-existing lesions such as ameloblastoma, ameloblastic fibroma, and ameloblastic fibrosarcoma. To date, the reported cases have exhibited considerably aggressive clinical behavior. The case of an odontogenic carcinosarcoma in the mandible of a 61-year-old male is described herein...

Odontogenic sarcomas and their subtypes are very rare tumors. The authors' objectives were to report an additional case of ameloblastic fibrosarcoma, review the clinicopathologic features, discuss their treatment approach, and complete a thorough review of the literature.

The surgical treatment of some odontogenic tumors often leads to tooth and maxillary bone loss as well as to facial deformity. Therefore, the identification of genes involved in the pathogenesis of odontogenic tumors may result in alternative molecular therapies. The PRKAR1A gene displays a loss of protein expression as well as somatic mutations in odontogenic myxomas, an odontogenic ectomesenchymal neoplasm. We used a combination of quantitative RT-PCR (qRT-PCR), immunohistochemistry, loss of heterozygosity (LOH) analysis, and direct sequencing of all PRKAR1A exons to assess if this gene is altered in mixed odontogenic tumors...

Ameloblastic fibrosarcoma is an uncommon odontogenic tumor composed of a benign epithelial component and a malignant ectomesenchymal component most frequently seen in the third and fourth decades of life. It mainly presents as a painful maxillary or mandibular swelling. Radiographs show a radiolucent mass with ill-defined borders. Radical surgical excision and long-term follow-up are the suggested treatment. We report the case of a 22-year-old female with a 2-month history of an asymptomatic swelling in her left mandible...

Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic neoplasm of the jaw. AFS is characteristically composed of a benign odontogenic epithelium and a malignant mesenchymal component. The posterior region of the mandible is the predominantly occupied site. In the present report, a new case of AFS in a 22-year-old male that originated from ameloblastic fibroma was described. Histologically, the tumor showed biphasic components: Benign epithelium and a malignant mesenchymal component. Immunochemical findings revealed that the tumor cells were positive for cluster of differentiation (CD) 34, vimentin, Ki-67 and p53, but negative for smooth muscle actin, S-100, CD68 and desmin...

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