Squamous odontogenic tumor (SOT) is a rare benign but locally infiltrative tumor often misdiagnosed as other entities, such as ameloblastoma and squamous cell carcinoma, due to overlapping morphological findings. We document here the clinicopathological and imaging findings of an aggressive intraosseous SOT in the posterior left region of the maxilla in a 25-year-old male patient. On intraoral examination, the tumor extended from the region of the left lateral incisor to the upper left premolar and was covered by reddish mucosa, with discrete areas of ulceration. Imaging exams revealed an osteolytic lesion causing thinning, erosion, and buccal and lingual cortical plate perforation associated with an impacted canine. Microscopically, the tumor showed a proliferation of islands of well-differentiated squamous epithelium in a variably collagenized background. The peripheral cells of the islands were flat or slightly cuboidal and did not exhibit nuclei with peripheral palisade and reverse polarization. The diagnosis of SOT was rendered. The patient underwent surgical resection and has been under clinical follow-up for approximately 12 months with no signs of recurrence. A careful morphological evaluation is essential to avoid misdiagnosis and ensure a satisfactory treatment approach.

Squamous odontogenic tumor (SOT) is a rare benign neoplasm of the jaw that likely arises from remnants of the dental lamina. It is a slow-growing lesion, with a radiolucent appearance in the central variant. Microscopically, SOT shows islands of squamous epithelium supported by fibrous stroma. In rare cases, squamous odontogenic tumor-like proliferation (SOT-LP) can be observed arising from odontogenic cysts (SOT-LPOC). Herein, we describe the case of a 42-year-old man who presented with discreet bleeding in the maxillary gingiva. Imaging revealed a well-defined, ovoid-shaped lesion with sclerotic margins involving tooth #18 in the intraosseous location. Fine needle aspiration supported the cystic nature of the lesion. After surgery, microscopy revealed a dentigerous cyst showing SOT-LP features. There was no recurrence after a 3-year follow-up. To the best of our knowledge, this is the first report of a dentigerous cyst showing SOT-LP features in the maxilla. Such cysts should be identified to avoid misdiagnosis, with the finding having therapeutic and prognostic implications.

Squamous odontogenic tumor (SOT) is a rare benign neoplasm and may be located to multiple sites in the oral cavity mouth. As per the literature, there have been <50 reported cases. The tumor is often asymptomatic, although it can present with symptoms of pain and tooth mobility. Peripheral odontogenic tumor is a rare entity derived from either epithelial or mesenchymal portions of the tooth-forming apparatus. Lesions are common to gingiva and alveolar mucosa. Peripheral granular cell ameloblastoma (GCA) is considered to be even rarer. The purpose of the study is to report a case of SOT with a synchronous association with peripheral GCA of cystic nature in the mandible. The occurrence of SOT with ameloblastoma has not been reported as per the literature search.

Squamous odontogenic tumors (SOT) are rare, benign, odontogenic neoplasms of the jaws. The sporadically reported cases with multifocal SOTs seem to have a marked predilection for younger African American patients. In this case report a 14-year-old Caucasian male presented with swelling of the vestibular alveolar process, slight tooth divergence, and mobility. A multifocal squamous odontogenic tumor was diagnosed and subsequently treated twice with surgical enucleation. Two and a half years earlier his mother was diagnosed and treated for a similar multifocal SOT. Next-Generation-Sequencing targeted resequencing mutational analysis of the maternal surgical specimens was performed. No potential causal mutation could be identified. Postoperative follow-up of the patient showed no recurrence of the SOT after 2 years. This case report substantiates the possibility of a familial relationship in (multifocal) SOT, possibly changing current ideas concerning the etiology and treatment of these neoplasms.

A 9-year-old standard poodle presented for a comprehensive oral health assessment and treatment, at which time a left rostral mandibular swelling was recognized. The mass was biopsied and eventually excised by a left rostral mandibulectomy en bloc resection. Histopathology supported the diagnosis of a benign, intraosseous, epithelial tumor that was otherwise unclassified. The clinical, radiological, and histological features of this case are similar to those reported for squamous odontogenic tumor (SOT) in humans. This case study relays the diagnosis, treatment, and follow-up of the first SOT-like tumor in a dog.

OBJECTIVE: To integrate the available data published on squamous odontogenic tumors (SOT) and squamous odontogenic tumor-like proliferations in odontogenic cysts (SOT-LPOC) into a comprehensive analysis of their clinical/radiologic features.
METHODS: An electronic search was undertaken in January 2017. Eligibility criteria included publications having enough clinical/radiological/histological information to confirm a definite diagnosis.
RESULTS: A total of 74 publications reporting 110 SOTs (102 central, 8 peripheral) and 60 SOT-LPOC were included. Compared to SOT-LPOC, SOT showed lower mean age, no preference regarding maxilla or mandible localization, significant association with cortical bone perforation, multilocular radiographic appearance, and mobility of the tooth/teeth associated with the lesion. While 5 recurrent SOT were reported after enucleation, no recurrent SOT-LPOC was found.
CONCLUSIONS: SOT shows a more aggressive biologic behavior than SOT-LPOC, which supports the hypothesis that the two lesions are distinct clinicopathological conditions.

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.

Odontogenic tumors (OTs) arising from the periodontium are quite rare. Squamous OT (SOT) is one such neoplasm which has an exceedingly rare occurrence. According to the literature, there have been very few reported cases of SOT with a very small percentage involving the gingiva. Most of the times, these tumors are located within the bone, only a very few peripheral cases been noted so far. Although predominantly benign, the possibility of a malignant transformation prevails. Our case describes a rare presentation of a peripheral SOT involving the upper anterior gingiva in a 59-year-old patient, which presented clinically as a firm and fibrotic swelling. An excisional biopsy was performed as a part of surgical management and for microscopic evaluation to confirm the clinical diagnosis. A soft tissue graft was harvested from an edentulous area to cover the deficit at the surgical site. The patient was evaluated at periodic intervals to assess the healing outcome and for any recurrence of the lesion.

Squamous odontogenic tumors (SOTs) are benign, locally infiltrative neoplasms that localize to the periodontium. In total, <50 cases have been reported since the first description of SOTs in 1975. Although the exact etiology of SOTs is unknown, the tumors are considered to derive from the epithelial cell rests of Malassez. SOTs are characterized by radiological and clinical signs and symptoms, including pain with increased sensitivity in the affected area, bone expansion and increased tooth mobility. The present study describes the case of a patient that experienced numerous SOT recurrences and also discusses recommendations for treatment. A locally invasive mandibular SOT was identified in a Caucasian 41-year-old female patient. The treatment involved recommended conservative surgery, including local curettage. In addition, 49 cases published in the literature were reviewed to assess the treatment strategies. The present patient experienced two recurrences of the tumor during the 6-year follow-up period. Ultimately, the vitality of the adjacent teeth was compromised. An apicoectomy with a small amount of resection of the marginal bone was necessary. In >50% of the reported cases of SOT in the literature the adjacent teeth were extracted. The present case of SOT and the associated literature were also discussed. It was concluded that the treatment of choice appears to be a conservative surgical removal, but the successful management of SOTs often requires the removal of the adjacent teeth.

Since its first publication in 1975, the squamous odontogenic tumor remains the rarest odontogenic lesion, with around 50 cases in the English-language literature in which the microscopic characteristics are frequently very well demonstrated. However, articles which discuss the radiographic aspects are scarce, especially with emphasis on the differential diagnosis. The present treatise proposes an assessment of jaw lesions with the same radiographic characteristics of the squamous odontogenic tumor to clarify the main findings for dental clinicians during routine diagnosis.