Four camels (Camelus dromedarius) presented to the Veterinary Teaching Hospital at King Faisal University with maxillary masses. On radiographs, the masses were multicystic and expanded the maxillary bone. The tumors were diagnosed by histopathologic examination as conventional ameloblastoma, two cases as intraosseous squamous cell carcinoma, and central odontogenic fibroma with ossification. To the authors\' knowledge, this is the first report of ameloblastoma in a camel, the first detailed description of maxillary squamous cell carcinoma in camels, and the first report of central odontogenic fibroma in any animal species.

BACKGROUND: The World Health Organization\'s (WHO) chapter on odontogenic and maxillofacial bone tumors provides a global reference for diagnosis of these tumors. In the fifth edition, the inclusion of consensus definitions and development of essential and desirable diagnostic criteria help improve recognition of distinct entities. These are key enhancements since the diagnosis of odontogenic tumors is largely based on histomorphology which is taken in combination with clinical and radiographic appearances.
METHODS: Review.
RESULTS: Despite delineation of diagnostic criteria for ameloblastoma, adenoid ameloblastoma, and dentinogenic ghost cell tumor, a subset of these tumors continues to show overlapping histological features that can potentially lead to misdiagnosis. Accurate classification may be challenging on small biopsies, but potentially enhanced by refining existing diagnostic criteria and utilization of immunohistochemistry and/or molecular techniques in a specific cases. It has become clear that the clinical and histologic features of the non-calcifying Langerhans cell-rich subtype of calcifying epithelial odontogenic tumor and the amyloid-rich variant of odontogenic fibroma converge into a single tumor description. In addition, this tumor shows remarkable clinical, histological overlap with a subset of sclerosing odontogenic carcinoma located in the maxilla. Benign perineural involvement vs perineural invasion is an underexplored concept in odontogenic neoplasia and warrants clarification to reduce diagnostic confusion with sclerosing odontogenic carcinoma.
CONCLUSIONS: While controversial issues surrounding classification and discrete tumor entities are addressed in the WHO chapter, ambiguities inevitably remain. This review will examine several groups of odontogenic tumors to highlight persistent knowledge gaps, unmet needs and unresolved controversies.

BACKGROUND: Granular Cell Odontogenic Fibroma (GCOF) is a rare odontogenic neoplasm reported over time with different names. The purpose of this study is to review all available data on the GCOF in the scientific literature, with a summary of all reported cases and a report of a new case.
METHODS: This review was conducted following the PRISMA guidelines. An electronic search was performed up to November 2022.
RESULTS: Thirty-nine studies reporting fifty-three cases were included. GCOF is a rare neoplasm among the odontogenic tumors, with a higher prevalence in women of the middle-aged and white population. This lesion occurs mostly on the posterior region of the mandible. Furthermore, based on clinical, radiographic, and histopathologic features, conservative treatment was the most reported choice with recurrence reported in two cases.
CONCLUSIONS: GCOF remains controversial due to the still unsolved histogenesis.

BACKGROUND: Odontogenic fibroma (OF) is a rare benign tumor of mesenchymal odontogenic tissue with variable global incidence. Two variants comprising extraosseous /peripheral (POF) and intraosseous /central (COF) have been identified based on the anatomical origin of the tumor. OF isslow growing, and often asymptomatic with characteristic radiographic appearance of a unilocular or multilocular cyst frequently associated with unerupted or displaced teeth while histologically, varying amount of inactive odontogenic epithelium embedded in a mature relatively dense collagenous stroma is diagnostic. The absence of odontogenic epithelium does not exclude the diagnosis of odontogenic fibroma.
METHODS: A 15 months old female presented with 3 months history of left lower jaw swelling which showed aggressive growth following incisional biopsy. Parents said lesion was noticed for the first time as pinpoint on the submandibular area and was too small to warrant any worries. Initial radiograph revealed absence of bony involvement but the second showed evidence of bony involvement. An impression of soft tissue sarcoma was made and incisional biopsy was sent for pathological diagnosis which revealed peripheral odontogenic fibroma. The rapidity of growth following incisional biopsy bellied a benign lesion.
CONCLUSIONS: An aggressive odontogenic fibroma with multifocal points in a 15months old girl.
BACKGROUND: Le fibrome odontogène (FO) est une tumeur bénigne rare du tissu odontogène mésenchymateu dont l’incidence globale est variable. Deux variantes comprenant des fibromes extra-osseux /périphérique (POF) et intra-osseuse / centrale (COF) ont été identifiés en fonction de l’origine anatomique de la tumeur. La POF est à croissance lente, et souvent asymptomatique avec l’apparence radiographique caractéristique d’un kyste uniloculaire ou multiloculaire fréquemment associé à des dents non érigées ou déplacées alors que histologiquement, une quantité variable d’épithélium inactif noyé dans un stroma collagène mature relativement dense est le diagnostic. L’absence d’épithélium odontogène n’exclut pas le diagnostic de fibrome odontogène.
UNASSIGNED: Une femme de 15 mois a présenté un gonflement de la mâchoire inférieure gauche depuis 3 mois de gonflement de la mâchoire inférieure gauche qui a montré une croissance agressive après une biopsie incisionnelle. Les parents ont déclaré que la lésion avait été remarquée pour la première fois sur forme de point d’épingle dans la zone submandibulaire et était trop petite pour justifier une quelconque inquiétude. La radiographie initiale a révélé l’absence d’atteinte osseuse mais la seconde a montré des signes d’atteinte osseuse. Une impression de sarcome des tissus mous et une biopsie incisionnelle a été envoyée pour un diagnostic pathologique qui a révélé un fibrome odontogène périphérique. La rapidité de la croissance après la biopsie incisionnelle a fait croire à une lésion bénigne.
CONCLUSIONS: Un fibrome odontogène agressif avec des points multifocaux chez une jeune fille de 15 mois.
UNASSIGNED: Fibrome odontogène, Périphérique, Central, Agressif.

Tuberous sclerosis complex (TSC) is an autosomal dominant inherited disease characterized by systemic hamartoma and diverse systemic features. TSC1 and TSC2 are the causative genes, and mental retardation, epileptic seizures, and facial angiofibroma develop in many patients with the disease. The case of a patient with TSC who developed a central odontogenic fibroma of the mandible is reported here. The patient was a 21-year-old woman who was referred with a swelling of the labial gingiva in the region of the right lower lateral incisor and canine. Dental radiography revealed a multilocular radiolucent region with a clear boundary. The right lower lateral incisor and canine were continuous with the lesion and thus were excised en bloc. The lesion was encapsulated and easily dissected. The diagnosis on immunohistological staining was odontogenic fibroma without an epithelial component. TSC1/2 gene mutation causes abnormal activation of mammalian target of rapamycin (mTOR) downstream of the PI3K-AKT pathway. The odontogenic fibroma in this patient was positive for mTOR, suggesting that the development of the odontogenic fibroma was the result of abnormal activation of mTOR, as in angiofibroma. The clinical course of this patient is presented and the developmental mechanism of central odontogenic fibroma is discussed.

The oral cavity is uncommon site for metastatic disease usually discovered secondary to malignancy. We encountered with a rare case in which metastasis to mandibular bone was the first clinical sign in the diagnosis of breast cancer without any radiographic findings. A 49-yr-old premenopausal woman, was referred to the Department of Medical Oncology of Imam Hossein Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran in 2014, presented with pain and tenderness in the left mandibular and temporal bone and paresthesia of the lower left lip and chin. CT scan of mandible showed no significant finding. Four months later, she was referred with complaints left breast pain for 4 wk and worsening swelling, pain and paresthesia. Breast examination revealed a 2 cm firm nodule on the left breast. Based on her medical history and histopathological study, metastatic carcinoma of the breast was suspected. She has received chemoradiotherapy that led to complete relief of her symptoms and remission of the disease. In the presence of an ambiguous sign in oral cavity such as jaw pain or paresthesia, diagnostic examination of malignancy is recommended.

Odontogenic fibroma (OF) is considered an unusual, benign mesenchymal neoplasm and one of the most little-understood lesions amongst all odontogenic neoplasms. The incidence rate of the tumor is from 0% to 5.5%. WHO classified them into intraosseous or central and extraosseous or peripheral variants. It chiefly consists of fibroblastic tissue with an inconsistent amount of inactive appearing odontogenic epithelium. The lesion has a slow growth along with cortical expansion with equal predilection in the anterior maxilla and posterior mandible. Radiologically, multilocular radiolucency is the most frequent finding with few cases being associated with root resorption or displacement. Microscopically, mature collagen fibers and numerous fibroblasts along with odontogenic epithelial islands are characteristically found. Central Odontogenic Fibroma responds well to surgical enucleation with no tendency for malignancy or recurrence. Here we report a rare case report of an 18 yr old male patient with Odontogenic fibroma of the posterior maxilla.

Cherubism is a rare autosomal dominant condition affecting the jaws and caused by mutations in the gene encoding for the adapter protein SH3BP2 that maps to chromosome 4p16.3. Cherubism is characterized by symmetrically developing bone lesions in the maxilla and mandible. The lesions have been radiographically and histopathologically well-described. Here, we present a family with cherubism with two of its members featuring odontogenic tumorous proliferations in association with persistent central giant cell lesions (CGCL). Specifically, the proband, a 25-year-old male, developed a radiolucent lesion characterized histologically by central odontogenic fibroma-like proliferation in association with a CGCL component, while his mother, at age 57, was diagnosed with primary intraosseous odontogenic carcinoma with areas of benign fibro-osseous lesions. In both patients the lesions occurred in the anterior mandible and presented with clinical enlargement. The son underwent incisional biopsy and did not have additional treatment. His mother underwent extensive mandibulectomy due to widespread tumor. The son has two affected children with classic cherubism while a third child at age 5, had not shown any features of the disease. Mutation analysis of three affected members resulted in the identification of a heterozygous mutation in SH3BP2 (c.1244G>C; p.Arg415Pro). To the best of our knowledge, association of cherubism with odontogenic neoplastic lesions has hitherto not been reported in the literature, thus suggesting a relationship between cherubism with disturbed odontogenesis.

Central odontogenic fibroma (COF) is an uncommon tumor that accounts for 0.1% of all odontogenic tumors; it has been defined as a benign neoplasm of the jaw. Clinically, the lesion grows slowly and leads to cortical expansion. Radiologically, the most common finding is multilocular radiolucency. The lesions are associated with the crown of an unerupted molar, premolar, or incisor tooth and in some cases, with root resorption or displacement. Histologically, the lesion is characterized by mature collagen fibers and numerous fibroblasts. COF responds well to surgical enucleation with no tendency for malignancy or recurrence. We report a case of a 15-year-old female patient presented with painless swelling of the left side of the maxilla since her childhood. Radiographs revealed an expanding ill-defined radiolucency with a displacement of the adjacent tooth. The impacted tooth was pushed posteriorly. The lesion was removed surgically. There were no postoperative complications.

Odontogenic fibroma is a benign ectomesenchymal tumor classified as central and peripheral on the basis of its location and as an epithelium rich or epithelium poor based on its histological features. Radiological findings consist of radiolucent areas with well-defined bony margins. The lesion is detected early because of its location and usually treated with surgical excision and curettage. We present a case of giant odontogenic fibroma of right maxilla presenting as gross facial deformity and posing a dual challenge of excising the tumor mass and reconstructing the ensuing defect.