Presence of Merkel cell polyomavirus DNA and large-T antigen in keratinocyte carcinomas and its correlation with immunohistochemical markers p16, p53 and ki67.

BACKGROUND: Merkel cell polyomavirus (MCPyV), a human polyomavirus that is unequivocally linked to merkel cell carcinoma (MCC), has been found in association with keratinocytes carcinomas (KC), especially basal cell carcinoma (BCC) and cutaneous squamous cell carcinoma (cSCC). Nevertheless, there is scarce information about the possible involvement of MCPyV in the development of KC. OBJECTIVES: To assess the presence of MCPyV DNA and Large-T Antigen (LT-Ag) via Polymerase Chain Reaction (PCR) and Immunohistochemistry (IHC) in cases of KC, and to correlate its presence with immunohistochemical markers p16, p53, and ki67, tumor type and subtype, sun-exposed location, and epidemiological data. METHODS: The prevalence of MCPyV DNA, LT-Ag, and immunohistochemical markers p16, p53, and ki67 was assessed by PCR and Immunohistochemistry (IHC) in 127 cases of KC, these results were correlated with tumor type and subtype, sun-exposed location, and epidemiological data. RESULTS: The MCPyV DNA was detected in 42.57% (43 of 101) cases by PCR, the LT-Ag was detected in 16.4% (20 of 122) of cases, p16 in 81.5% (97 of 119), p53 in 66.4% (83 of 125), ki67 in 89% (73 of 82). No correlation between MCPyV LT-Ag and DNA confronted with tumor type, subtype, location site, and immunohistochemical markers was found. A single correlation between the MCPyV LT-Ag and cSCC tumors and peri-tumoral lymphocyte cells was noted. STUDY LIMITATIONS: Further steps need to be taken to better evaluate the MCPyV influence and its possible role in KC carcinogenesis, as the evaluation of the virus genome state, the gene sequence that encodes LT-Ag in the KC tumor cells, and in situ hybridization for viral DNA or RNA in these cells. CONCLUSIONS: Despite the frequent detection of MCPyV in KC, the data available so far does not support the hypothesis of a causal relationship between them.

Challenging of treating patients with exfolliative cheilittis: Report of two cases.

Cheilitis is a term given to the inflammation that occurs in the vermillion of the lips. The exfoliative type is an uncommon form of cheilitis, which is characterized by inflammation and desquamation of the lip. It can cause aesthetic problems and compromise daily eating and phonation. The aim of this paper is to describe two cases of exfoliative cheilitis in young persons under periods of emotional stress and parafunctional habits. A 22-year-old white male and an 18-year-old black female presenting edema, intense dryness, and slight desquamation on the vermilion of the lips. In the second case, fissures with bleeding were also observed. Oral lesions were associated with intense emotional stress. The diagnosis of both was made based on the clinical presentation and the exclusion of other conditions. Although the patients have presented a significant improvement after the corticosteroid treatment, they still have a recurrence in stressful episodes. Detailed clinical examination and complementary exams are fundamental for determining associated factors and correctly diagnosing exfoliative cheilitis. Treatment can be challenging, especially in the face of relapses. Key words:Cheilitis, exfoliative cheilitis, oral lesions, stress psychological.

Evaluation of changes in antimicrobial susceptibility in bacteria infecting children and their mothers in pediatric, neonatal-intensive care unit, and gynecology/obstetrics wards of a quaternary referral hospital during the COVID-19 pandemic.

The World Health Organization released a statement warning of increased risk for the incidence of multidrug resistant microorganisms and the absence of new drugs to control such infections soon. Since the beginning of the COVID-19 pandemic, the prescription of antimicrobial agents has increased and may have accelerated the emergence of multidrug resistant (MDR) bacteria. This study aimed to evaluate maternal and pediatric infections within a hospital from January 2019 to December 2021. An observational retrospective cohort study was performed at a quaternary referral hospital in a metropolitan area of Niteroi city, Rio de Janeiro state, Brazil. A total of 196 patients' medical records were analyzed. The data from 90 (45.9%) patients were collected before the SARS-CoV-2 pandemic, 29 (14.8%) from the 2020 pandemic period, and 77 (39.3%) from the 2021 pandemic period. A total of 256 microorganisms were identified during this period. Out of those, 101 (39.5%) were isolated in 2019, 51 (19.9%) in 2020, and 104 (40.6%) in 2021. Antimicrobial susceptibility tests were performed on 196 (76.6%) clinical isolates. The exact binomial test showed that the distribution of Gram-negative bacteria was predominant. The most common microorganism was Escherichia coli (23%; n = 45), followed by Staphylococcus aureus (17.9%, n = 35), Klebsiella pneumoniae (12.8%, n = 25), Enterococcus faecalis (7.7%, n = 15), Staphylococcus epidermidis (6.6%, n = 13) and Pseudomonas aeruginosa (5.6%, n = 11). Staphylococcus aureus was the predominant species among resistant bacteria. Among the antimicrobial agents tested, the following were resistant, presented on a descending scale: penicillin (72.7%, p = 0.001, Binomial test), oxacillin (68.3%, p = 0.006, Binomial test), ampicillin (64.3%, p = 0.003, Binomial test), and ampicillin/sulbactam (54.9%, p = 0.57, Binomial test). Infections with S. aureus were 3.1 times greater in pediatrics and maternal units than in other hospital wards. Despite the global reduction in the incidence of MRSA, we observed an increase in MDR S. aureus in this study. No changes were observed in the frequency of resistance profiles of the clinical isolates after the establishment of the global SARS-CoV-2 pandemic. More comprehensive studies are needed to understand the impact of the global SARS-CoV-2 pandemic on the resistance levels of bacteria associated with neonate and pediatric patients.

Head circumference and anthropometric changes and their relation to plexiform and skin neurofibromas in sporadic and familial neurofibromatosis 1 Brazilian adults: a cross-sectional study.

BACKGROUND: Neurofibromatosis 1 (NF1) is a common autosomal dominant syndrome with complete penetrance and highly variable expressivity. The cutaneous neurofibroma (Cnf) and plexiform neurofibroma (Pnf), café-au-lait spots, and freckle-like lesions are common in NF1, but many other manifestations can occur. We aimed to evaluate head circumference, height, weight, body mass index (BMI), head circumference-to-height ratio (HCHR) and waist-hip ratio (WHR) in adult NF1 Brazilian individuals versus a paired control group and investigate their correlation with the presence of clinically visible Pnfs, and number of "skin neurofibromas" (Snf), which include both cutaneous and subcutaneous neurofibromas. METHODS: A case-control study was conducted with 168 individuals, 84 with NF1 and 84 without NF1, paired by sex and age. Head circumference and anthropometric measurements, Snf quantification, evaluation of clinically visible Pnf and familial inheritance were accessed. RESULTS: Prevalence of macrocephaly was significantly higher in NF1 women. Height and weight were significantly lower in both males and females with NF1. HCHR was higher in the NF1 group than in the control group for both sexes. BMI was significantly lower in men with NF1. Waist and hip circumferences were significantly reduced in NF compared with the controls, but the mean WHR was significantly lower only in NF1 women. No correlation was found between the Snf and head circumference and anthropometric measurements, sex or family history. The presence and larger size of clinically visible plexiform neurofibromas were associated with normal stature (p = 0.037 and p = 0.003, respectively). CONCLUSIONS: NF1 individuals have increased prevalence of macrocephaly, short stature, low BMI, and reduced abdominal fat. There is no relation between head circumference and anthropometric data with family history, or neurofibromas.

Oral lichenoid lesion in association with chemotherapy treatment for non-Hodgkin lymphoma or lichen planus? Review of the literature and report of two challenging cases.

BACKGROUND: The diagnosis of oral lichenoid lesions (OLL) remains a challenge for clinicians and pathologists. Although, in many cases, OLL cannot be clinically and histopathologically distinguishable from oral lichen planus (OLP), one important difference between these lesions is that OLL has an identifiable etiological factor, e.g. medication, restorative material, and food allergy. The list of drugs that can cause OLL is extensive and includes anti-inflammatory drugs, anticonvulsants, antihypertensives, antivirals, antibiotics, chemotherapeutics, among others. This work aimed to perform a literature review of OLL related to chemotherapy drugs and to report two cases of possible OLL in patients with B-cell and T-cell non-Hodgkin lymphomas in use of chemotherapy and adjuvant medications. We also discuss the challenge to clinically and histopathologically differentiate OLL and OLP. CASE PRESENTATION: In both cases, oral lesions presented reticular, atrophic, erosive/ulcerated, and plaque patterns. The diagnosis of OLL was initially established in both cases by the association of histopathology and history of onset of lesions after the use of medications. Although the patients have presented a significant improvement in the oral clinical picture for more than 2 years of follow-up, they still have some lesions. CONCLUSION: A well-detailed anamnesis associated with the drug history, temporal relationship of the appearance of the lesions, and follow-up of patients are fundamental for the diagnosis of OLL related to drugs. Nevertheless, its differentiation from OLP is still a challenge.

Congenital sebaceous choristoma of the tongue: A rare case report.

The most common oral choristomas are consisted of thyroid tissue and bone. The presence of sebaceous glands in the oral mucosa, especially in the buccal mucosa and labial mucosa, is often considered a normal anatomical variation since they are observed in about 80% of the population and are called ectopic sebaceous glands or Fordyce's granules. However, the presence of these glands on the tongue is rare, with only 11 cases in the dorsum of the tongue reported in the English literature, and it is considered a choristoma. This paper aims to report the third case in the literature of a congenital sebaceous choristoma on the tongue. An 11-year-old white male patient presented a firm sessile papule, without color alteration, measuring 0.4 cm x 0.3 cm in diameter, in the middle third of the dorsum of the tongue with a slight increased size in the last months. Histopathological examination showed an invagination of the epithelium into the connective tissue, forming a ductal structure covered by stratified squamous epithelium. The deeper areas had normal well-differentiated sebaceous glands, with ducts connected to the central duct. Considering clinical and histopathological findings the diagnosis was sebaceous choristoma. Despite being rare, sebaceous choristomas should also be considered in the differential diagnosis of tongue abnormalities or lesions. Although the pathogenesis is not well understood, the present report, as a congenital choristoma case in the midline, reinforces the hypothesis of a disorder with embryological origin and a possible relationship with thyroglossal duct remnants. Key words:Choristoma, Oral Mucosa, Tongue.

Dentinogenic ghost cell tumor with focal atypical features suggesting ghost cell odontogenic carcinoma: Report of a challenging diagnosis.

Dentinogenic ghost cell tumor (DGCT) represents a rare benign odontogenic neoplasm that can appear in a central or peripheral form and may rarely undergo malignant transformation to ghost cell odontogenic carcinoma (GCOC). We aim to report a case of a central DGCT with focal cytological malignant aspects. A 24-year-old woman exhibited a painful enlargement and dental mobility in the left posterior maxilla for about one year, which appeared as an expansive well-defined hypodense maxillary image with hyperdense foci invading ipsilateral maxillary sinus. Incisional biopsy showed a predominantly solid hyperchromatic basaloid epithelium presenting cellular pleomorphism and mitotic activity, admixed with abundant ghost cell aggregates and dentinoid material. The lesion was immunopositive for p53 and had 21% of Ki-67 proliferation index (PI). These microscopic features suggested initially a GCOC diagnosis. Partial left maxillectomy was performed without complications. The surgical specimen presented an exuberant variation of the epithelial parenchyma, including ameloblastomatous, fusiform, and cribriform areas, with numerous ghost cells and dentinoid material, lacking any signs of malignancy. The final diagnosis was DGCT. The patient is in a strict regular follow-up for over two years, and there are no signs of recurrence.

Comparative Analysis Between Dentinogenic Ghost Cell Tumor and Ghost Cell Odontogenic Carcinoma: A Systematic Review.

Dentinogenic ghost cell tumor (DGCT) and ghost cell odontogenic carcinoma (GCOC) form a spectrum of rare benign and malignant odontogenic neoplasms, respectively. The aim of this study was to perform a comparative systematic review of the clinicopathological, genetic, therapeutic, and prognostic features of DGCT and GCOC. The electronic search was performed until December 2020 on seven electronic databases. Case reports, series, and research studies with enough histopathological criteria for diagnosis and all genomic studies were included. Both DGCT and GCOC showed a male prevalence (p = 0.043), with mandibular and maxillary predilections, respectively (p = 0.008). Peripheral DGCT (DGCTp) affected most elderly people (p < 0.001), and central DGCT (DGCTc) and GCOC occurred mainly in younger individuals. Unilateral enlargement of maxilla or mandible was the most common clinical sign associated with a radiolucent or mixed image. Ameloblastomatous epithelium was often present in both neoplasms. Basaloid and large cells with vesicular nuclei were also frequently seen in GCOC. ß-catenin expression and mutations (CTNNB1 gene) were found in DGCT and GCOC. Conservative surgery was mostly used for DGCTp, while radical resection was chosen for DGCTc and GCOC. High recurrence rates were found in DGCTc and GCOC. Metastasis occurred in 16.7% of GCOC cases and the 5-year survival rate was 72.6%. DGCT and GCOC share numerous clinicopathological features and demand a careful histopathological evaluation, considering the overlap features with other odontogenic tumors and the possibility of malignant transformation of DGCT. A strict regular post-operative follow-up is mandatory due to high recurrence rates and metastatic capacity in GCOC.

Neurofibromin expression by normal salivary glands.

INTRODUCTION: Neurofibromin, a protein encoded by the NF1 gene, is mutated in neurofibromatosis 1, one of the most common genetic diseases. Oral manifestations are common and a high prevalence of hyposalivation was recently described in individuals with neurofibromatosis 1. Although neurofibromin is ubiquitously expressed, its expression levels vary depending on the tissue type and developmental stage of the organism. The role of neurofibromin in the development, morphology, and physiology of salivary glands is unknown and a detailed expression of neurofibromin in human normal salivary glands has never been investigated. AIM: To investigate the expression levels and distribution of neurofibromin in acinar and ductal cells of major and minor salivary glands of adult individuals without NF1. MATERIAL AND METHOD: Ten samples of morphologically normal major and minor salivary glands (three samples of each gland: parotid, submandibular and minor salivary; and one sample of sublingual gland) from individuals without neurofibromatosis 1 were selected to assess neurofibromin expression through immunohistochemistry. Immunoquantification was performed by a digital method. RESULTS: Neurofibromin was expressed in the cytoplasm of both serous and mucous acinar cells, as well as in ducts from all the samples of salivary glands. Staining intensity varied from mild to strong depending on the type of salivary gland and region (acini or ducts). Ducts had higher neurofibromin expression than acinar cells (p = 0.003). There was no statistical association between the expression of neurofibromin and the type of the salivary gland, considering acini (p = 0.09) or ducts (p = 0.50) of the four salivary glands (parotid, submandibular, minor salivary, and sublingual gland). Similar results were obtained comparing the acini (p = 0.35) and ducts (p = 0.50) of minor and major salivary glands. Besides, there was no correlation between the expression of neurofibromin and age (p = 0.08), and sex (p = 0.79) of the individuals, considering simultaneously the neurofibromin levels of acini and duct (n = 34). CONCLUSION: Neurofibromin is expressed in the cytoplasm of serous and mucous acinar cells, and ductal cells of salivary glands, suggesting that this protein is important for salivary gland function.

A Clinicopathologic Study on the Role of Estrogen, Progesterone, and Their Classical and Nonclassical Receptors in Cutaneous Neurofibromas of Individuals With Neurofibromatosis 1.

OBJECTIVES: To evaluate the expression of progesterone receptor (PR), estrogen receptor (ER), and G protein-coupled estrogen receptor 1 (GPER-1) in cutaneous neurofibromas (cNFs) and their correlation with demographic, clinical, and laboratory data of individuals with neurofibromatosis 1 (NF1). The association of PROGINS polymorphism and PR expression in cNFs, as well as the serum steroidal hormones and the number of cNFs, was investigated. METHODS: The sample comprised 80 large and 80 small cNFs from 80 individuals with NF1. PR, ER, GPER-1, and Ki-67 expression were investigated by immunohistochemistry in tissue micro- and macroarrays and quantified using a digital computer-assisted method. The number of cNFs, the levels of serum 17ß estradiol and progesterone, and the PROGINS polymorphism were identified. RESULTS: Twelve (8.5%) small cNFs were weakly positive for ER, 131 (92.3%) cNFs expressed PR, and all (100%) cNFs expressed GPER-1. Large cNFs showed a higher expression of PR (P < .0001) and GPER-1 (P = .019) and had a higher intensity of staining for these receptors (P < .0001). The cell proliferation index was positively correlated with PR (P = .001). Persons with more cNFs had higher serum levels of progesterone (P = .001). CONCLUSIONS: These findings emphasize the role of estrogen and progesterone in cNF development and suggest that these hormones may act on cNF cells via a noncanonical pathway through GPER-1.

Analysis of risk factors for maxillary denture-related oral mucosal lesions: A cross-sectional study

Background: To evaluate the frequency of maxillary dentures-related lesions and the possible associated risk factors. Material and Methods: Ninety-seven participants were selected, and a complete anamnesis, physical examination and tests of occlusion vertical dimension (OVD), retention and stability of the denture, biofilm quantification, cytopathology, sialometry, pH analysis and buffer capacity of the saliva were performed. Statistical analyses were performed with the Pearson's chi-square, Mann-Whitney tests, and Pearson’s coefficient (p < 0.05). Results: In 78% of the participants at least one denture-related lesion was found. Denture-associated stomatitis (63%), inflammatory fibrous hyperplasia (19%) and traumatic ulceration (11%) were the 3 most frequent lesions. The habit of night use of the denture was considered an independent risk factor for the development of oral lesions [OR = 3.0 (95% CI 1.09-8.56); p < 0.05]. Furthermore, the longest period of use of the same denture and biofilm also had statistically significant relation to oral lesions. The biofilm seems to be more related to the prevalence of oral lesions according to the multiple logistic regression [OR = 1.3 (95% CI: 1.01-1.83) p < 0.05]. The lack of a dentures' cleaning solution and detrition of the prothesis were independent risk factors for denture-associated stomatitis. Male gender, loss of OVD and bad buffer capacity were risk factors for angular cheilitis. Fractures of the base and repair of broken dentures were risk factors for traumatic ulcers. Conclusions: These results show a high frequency of denture-related lesions. Besides, participants hygiene habits and poor quality of the dentures were the main factors for the development of these lesions

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Behaviour and Prevention of 5'Fluorouracil and Doxorubicin-induced Oral Mucositis in Immunocompetent Patients with Solid Tumors: A Randomised Trial.

PURPOSE: This study evaluated the efficacy of four methods to prevent chemotherapy-induced oral mucositis (OM) in patients with solid tumors (ST). In addition, the behaviour of OM was investigated in these oncological patients. MATERIALS AND METHODS: Forty-eight patients, aged 27-84, were randomly allocated to different groups from the first day of chemotherapy (CT), in the following sequence: group 1: intensive oral care programme (IOCP); group 2: 0.12% chlorhexidine mouthrinse; group 3: 0.03% triclosan mouthrinse; group 4: low-level laser therapy (LLLT). Oral mucositis was evaluated on the 7th and 14th days by means of the Oral Mucositis Assessment Scale (OMAS). RESULTS: Thirty-one (64.5%) patients developed OM in the first cycle of CT and the pain was significantly associated with OM severity (p < 0.0001). The statistically significantly worst OMAS score was found for the lips and buccal mucosa (p < 0.0001). Despite a lack of statistical significance, IOCP and LLLT notably demonstrated potential effects to prevent OM in patients who presented with only oral erythema (75%) and lower peak of severity during the follow up, respectively. CONCLUSIONS: Improved oral care awareness is needed in patients undergoing 5'fluorouracil and doxorubicin, mainly to avoid pain caused by oral mucositis. Oral mucositis was more prevalent and aggressive in oral sites exposed to chronic trauma. The IOCP and LLLT approaches showed positive results to prevent oral mucositis and should be further investigated in similar and larger samples.

Prevalence and clinicopathological characteristics of lipomatous neurofibromas in neurofibromatosis 1: An investigation of 229 cutaneous neurofibromas and a systematic review of the literature.

BACKGROUND: Lipomatous neurofibroma (Lnf) is a histopathological variant with adipocytes noted among cells of cutaneous neurofibromas. We aimed to investigate the prevalence and clinicopathological features of Lnfs of neurofibromatosis 1 (NF1)-associated cutaneous neurofibromas and to review the literature systematically. We also evaluated the expression of leptin (a hormone involved in lipid metabolism) in neurofibromas to better understand the pathogenesis of Lnfs. METHODS: A prospective histologic study was conducted on 229 cutaneous neurofibromas from 85 NF1 individuals. Leptin expression was immunohistochemically evaluated in 111 cutaneous neurofibromas. To systematically review the literature, two authors independently performed literature searches without year restriction. RESULTS: Forty (17.5%) neurofibromas were lipomatous. Lnfs were significantly larger lesions and associated with females. Eighteen (7.9%) of all neurofibromas had multinucleated floret-like giant cells, and these were associated with Lnfs. All neurofibromas expressed leptin. We systematically reviewed 13 articles. Three large studies investigated Lnfs mainly in sporadic neurofibromas and suggested that 0.3% to 8.0% of tumors (NF1 and sporadic) are Lnfs. CONCLUSION: In NF1, Lnfs are common, mainly in larger tumors and women. All cutaneous NF1-neurofibromas express leptin. It is unknown if the expression of leptin accounts for the lipomatous variant, but it may have a role in the pathogenesis of cutaneous neurofibroma.

Receptor of ghrelin is expressed in cutaneous neurofibromas of individuals with neurofibromatosis 1.

BACKGROUND: Multiple cutaneous neurofibromas are a hallmark of neurofibromatosis 1 (NF1). They begin to appear during puberty and increase in number and volume during pregnancy, suggesting a hormonal influence. Ghrelin is a hormone that acts via growth hormone secretagogue receptor (GHS-R), which is overexpressed in many neoplasms and is involved in tumorigenesis. We aimed to investigate GHS-R expression in NF1 cutaneous neurofibromas and its relationship with tumors volume, and patient's age and gender. RESULTS: Sample comprised 108 cutaneous neurofibromas (55 large and 53 small tumors) from 55 NF1 individuals. GHS-R expression was investigated by immunohistochemistry in tissue micro and macroarrays and quantified using a digital computer-assisted method. All neurofibromas expressed GHS-R, with a percentage of positive cells ranging from 4.9% to 76.1%. Large neurofibromas expressed more GHS-R than the small ones. The percentage of GHS-R-positive cells and intensity of GHS-R expression were positively correlated with neurofibromas volume. GHS-R expression was more common in female gender. CONCLUSIONS: GHS-R is expressed in cutaneous neurofibromas. Larger neurofibromas have a higher percentage of positive cells and higher GHS-R intensity. Based on our results we speculate that ghrelin may have an action on the tumorigenesis of cutaneous neurofibromas. Future studies are required to understand the role of ghrelin in the pathogenesis of NF1-associated cutaneous neurofibroma.

High prevalence of hyposalivation in individuals with neurofibromatosis 1: a case-control study.

BACKGROUND: Neurofibromatosis type 1 (NF1) is one of the most common genetic diseases in humans and has widely variable expressivity. Oral manifestations are common, but there are no studies that investigated functional alterations in salivary glands in NF1. Our aim was to evaluate the salivary flow rate in NF1 individuals, comparing to a control group, and to investigate the possible causes and some consequences of salivary gland alteration. METHODS: This is a case-control study that evaluated the salivary flow rate of NF1 individuals (n = 49) and compared to an age and sex-matched control group. We have also investigated the possible causes and consequences of hyposalivation in NF1 individuals through anamnesis, a specific questionnaire, physical examination, tongue coating evaluation and cytopathological exam to assess the prevalence of oral candidiasis. RESULTS: Hyposalivation at rest was present in 59% (29/49) of NF1 individuals in contrast to 22% (11/49) in the control group, being statistically significant (P <0.0001; Wilcoxon rank-sum test). The analysis of the adjusted residual showed that the prevalence of hyposalivation in NF1 individuals (46.9%) was 4-fold higher than in controls (10.2%). None of the possible causes of hyposalivation (medications, low liquid intake, caffeinated or stimulant drink use, mouth breathers, alcohol, smoke and plexiform neurofibroma close to or involving major salivary glands areas) had important impact on the salivary flow rate in NF1 individuals. CONCLUSIONS: Hyposalivation may be a consequence of NF1, as occurs in other genetic diseases. More studies are necessary to understand if there is and what is the relationship between NF1 and hyposalivation.

Validity and interexaminer reliability of a new method to quantify skin neurofibromas of neurofibromatosis 1 using paper frames.

BACKGROUND: Skin neurofibromas represent one of the main clinical manifestations of neurofibromatosis 1, and their number varies greatly between individuals. Quantifying their number is an important step in the methodology of many clinical studies, but counting neurofibromas one by one in individuals with thousands of tumors is arduous, time-consuming, and subject to intra and interexaminer variability. We aimed to evaluate the efficacy of a new methodology for skin neurofibromas quantification using paper frames. METHODS: The sample comprised 92 individuals with NF1. Paper frames, with a central square measuring 100 cm2, were placed on the back, abdomen and thigh. Images were taken, transferred to a computer and two independent examiners counted the neurofibromas. The average number of neurofibromas/100 cm2 of skin was obtained from the mean of the three values. The differences in the quantity of neurofibromas counted by two examiners were evaluated with Intraclass correlation coefficient (ICC), paired t-test, Bland-Altman and survival-agreement plots. To evaluate the predictive value of the method in obtaining the total number of neurofibromas, 49 participants also had their tumors counted one by one. Reproducibility was assessed with Pearson's correlation coefficients and simple linear regression model. RESULTS: There was excellent agreement between examiners (ICC range 0.992-0.997) and the total number of skin neurofibromas could be predicted by the adhesive frames technique using a specific formula (P < 0.0001). CONCLUSIONS: In this article we describe a reliable, easy and rapid technique using paper frames to quantify skin neurofibromas that accurately predicts the total number of these tumors in patients with NF1. This method may be a useful tool in clinical practice and clinical research to help achieve an accurate quantitative phenotype of NF1.

Gingival Anaplastic Large-Cell Lymphoma Mimicking Hyperplastic Benignancy as the First Clinical Manifestation of AIDS: A Case Report and Review of the Literature.

This paper presents an unusual case of gingival ALCL, which mimicked a benign hyperplastic lesion that occurred in a 57-year-old white man representing the first clinical manifestation of acquired immunodeficiency syndrome (AIDS). The patient was referred to the Dental Clinic of PUCPR complaining of a lobulated nodule on the gingiva of his upper central incisors. The presence of advanced chronic periodontitis and dental plaque raised suspicion for a benignancy. An excisional biopsy was performed, and large pleomorphic cells with an abundant cytoplasm, sometimes containing prominent nucleoli and "Hallmark" cells, were observed through hematoxylin and eosin staining. The tumor cells showed strong CD30 expression, EMA, Ki-67, and LCA, and negative stain for p80(NPM/ALK), CKAE1/AE3, CD20, CD3, CD56, and CD15. The final diagnosis was ALCL (ALK-negative). Further laboratory tests revealed positivity for human immunodeficiency virus (HIV). The patient was submitted to chemotherapy, but four months after diagnosis, the patient died due to pneumonia and respiratory failure. Oral anaplastic large-cell lymphoma (ALCL) is a rare disorder. Only 5 cases involving the gingiva have been reported, and to our knowledge, this is the first case reported of the ALCL, which mimicked a hyperplastic benignancy as the first clinical manifestation of AIDS.

Intraoral nerve sheath myxoma: case report and systematic review of the literature.

BACKGROUND: Oral nerve sheath myxoma (NSM) is an uncommon benign neoplasm with Schwann-cell origin, which is frequently mistaken for neurothekeoma. We report a case of NSM on the buccal mucosa in a 42-year-old woman. This case is compared with previously reported cases and a systematic review is performed. METHODS AND RESULTS: We conducted a case report and systematic review of oral cases considered true NSMs. A literature search was performed using PubMed, Lilacs, Scielo, Cochrane, SciVerse Scopus, Web of Science, and Embase electronic database. Twenty-five cases of oral NSM were included in the systematic review. CONCLUSION: Oral NSM is rare and may represent a diagnostic challenge for pathologists. To confirm the diagnosis of NSM, the evaluation of S-100 protein expression or other neural marker is essential. The use of the terms NSM and neurothekeoma as synonymous or as variants of the same tumor should be avoided, because they are clearly distinct lesions.