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Middle-aged and old adults (≥ 50 years) diagnosed with Cowden syndrome (CS) with orofacial manifestations are uncommon. We describe a case of CS in a 53-year-old female showing “narrow bird-like” face, macrocephaly, acral keratoses, oral candidiasis, burning in the mouth, and multiple asymptomatic papillomatous lesions with a cobblestone pattern distributed on the alveolar ridge, tongue, buccal mucosa, and commissure. The histopathological features of lesions of the oral mucosa were those of papillary fibroepithelial hyperplasia. Immunohistochemistry revealed strong positivity for PTEN and p53 in most epithelial cells, while the expression of Bcl-2, S-100, and Ki-67 was weak/negative. According to a review conducted in PubMed, Web of Science, Embase, and Scopus for the analysis of reports of CS individuals ≥ 50 years with orofacial manifestations, 56 cases have been described in literature. Predilection for women was observed, with a female:male ratio of 2.3:1. Thirty-five (62.5%) individuals developed some malignant neoplasms. Oral health providers should be aware of the orofacial aspects of CS, including multiple papillomatosis, which can be an important criterion for diagnosis. Since malignancies may occur in older adults with CS, the need for strict surveillance is necessary. The present case has been under follow-up for 7 years without evidence of other manifestations.

The microenvironment of oral cancer is highly dynamic and has been proved to affect tumor progression. Pericytes are blood vessels surrounding cells that have recently gained attention for their roles in vascular and cancer biology. The objective of the present study was to survey the scientific literature for conclusive evidence about whether pericytes are part of blood vessels in oral squamous cell carcinoma (OSCC) and their roles in the tumor microenvironment and clinical outcomes. A systematic electronic search was undertaken in Medline Ovid, PubMed, Web of Science, and Scopus. Eligibility criteria were: publications adopting in vivo models of OSCC that included pericyte detection and assessment by pericyte markers (e.g., α-smooth muscle actin, neuron-glial antigen 2 and platelet-derived growth factor receptor-β). The search yielded seven eligible studies (from 2008 to 2018). The markers most commonly used for pericyte detection were α-smooth muscle actin and neuron-glial antigen 2. The studies reviewed showed the presence of immature vessels exhibiting a reduction of pericyte coverage in OSCC and indicated that anti-cancer therapies could contribute to vessel normalization and pericyte regain. The pericyte population is significantly affected during OSCC development and cancer therapy. While these findings might suggest a role for pericytes in OSCC progression, the limited data available do not allow us to conclude whether they modify the tumor microenvironment and clinical outcome. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1007/s12105-020-01188-2) contains supplementary material, which is available to authorized users.

Data on the occurrence and clinicopathological characteristics of actinic cheilitis (AC) and lip squamous cell carcinoma (LSCC) are well studied; however, they are based on studies limited to a single centre. Herein, we described the frequency of AC and LSCC submitted to microscopic examination from representative geographic regions of Brazil. A retrospective multicentre study was performed on biopsies obtained from 1953 to 2018 at 10 Brazilian oral and maxillofacial pathology centres. A total of 198,709 biopsy specimens were surveyed. Sociodemographic data and clinicopathologic characteristics were analysed. A total of 2017 cases of ACs (1.0%) and 850 cases of LSCCs (0.4%) were recorded. A strong fair-skinned (> 87%) male (> 70%) predilection was observed in both conditions. The mean age was 54.8 ± 18.7 for individuals with AC and 57.8 ± 19.0 for individuals with LSCC. The most commonly affected site was the lower lip (> 90%). This is a large multicentre study of AC and LSCC from Brazil. The frequency and clinicopathological features of AC and LSCC were similar to those described worldwide. This study provides robust and representative epidemiological data of these conditions for the scientific community.

The aim of this study was to describe a series with 66 cases of infected cemento-osseous dysplasia (COD) and to discuss the demographic distribution, clinicoradiographic features and treatment of this condition. A study looking back on the diagnoses made at a single Brazilian centre within a 28-year timeframe was performed. A literature review with searches across five databases was also conducted to identify reports on osteomyelitis/infected COD. Descriptive and statistical analyses were performed. The case series study showed a female/male ratio of 21:1. Affected individuals’ mean age was 57.4 years. Mandible was the most affected site (95.5%) and florid subtype was the most frequent infected COD (62.1%). Tooth extraction was the main factor associated with the development of infection associated within a COD lesion. The literature review retrieved 30 studies reporting 46 cases of this condition. Asian women in their 40 s and 50 s were more affected. Surgery for removal/curettage of necrotic bone was acknowledged as an appropriate approach to the treatment of this infection. The clinicodemographic data of the study were similar to data collected across the literature. Clinicians, maxillofacial surgeons and oral rehabilitation providers should be alert to the diagnosis of COD, since infection is a frequent complication whose management is challenging.

Liposarcomas of the oral cavity are rare. Those originating in the buccal mucosa cause challenging diagnostic and therapeutic issues since less than 40 cases of liposarcomas of the buccal mucosa and cheek have been reported in the worldwide literature. Herein, we present a case of atypical lipomatous tumor/well-differentiated liposarcoma affecting a 45-year-old female patient. Ultrasonography and magnetic resonance imaging confirmed a well-defined mass located in the right buccal mucosa, extending to the submucosal layers of the cheek. Histopathologically, a well-differentiated fatty neoplasm with presence of prominent stromal inflammatory cells was observed. Multifocally scattered bizarre hyperchromatic stromal cells, some of which multinucleated, were also observed. An immunohistochemical panel comprising vimentin, S-100, CD10, CD34, CD20, CD3, CD68, CD138, MDM2, Ki-67, and P53 was employed to better characterize the lesion. A local recurrence event occurred during a 10-year follow-up period. Surgical resection was performed during both episodes. We also provided an overview of demographic and clinicopathological characteristics, immunohistochemical features, imaging findings, and the differential diagnosis of liposarcoma of the oral cavity. Knowledge of the etiopathological and clinical aspects of this rare neoplasm is fundamental in order to rule out other conditions, including lipomatous lesions that affect the buccal mucosa.