Two enemies, one fight: An update of oral cancer in patients with Fanconi anemia.

Fanconi anemia (FA) is a rare inherited genetic condition that may lead to bone marrow failure, leukemia, and/or solid tumors. It is caused by the loss of function of at least 1 gene of the FA/BRCA pathway, which is necessary for DNA repair. Patients with FA have a 200-fold to 1000-fold risk of developing head and neck cancer, mainly oral squamous cell carcinoma (OSCC), and of doing so at a much younger age than individuals within the general population. Also, patients who have FA with OSCC have poor overall survival rates, reinforcing the necessity to detect OSCC early. The scope of the current review is to provide an update on OSCC in patients with FA.

Long-term Survival, Organ Function, and Malignancy after Hematopoietic Stem Cell Transplantation for Fanconi Anemia.

We report on long-term survival in 157 patients with Fanconi anemia (FA) who survived 2 years or longer after their first transplantation with a median follow-up of 9 years. Marrow failure (80%) was the most common indication for transplantation. There were 20 deaths beyond 2 years after transplantation, with 12 of the deaths occurring beyond 5 years after transplantation. Donor chimerism was available for 149 patients: 112 (76%) reported > 95% chimerism, 27 (18%) reported 90% to 95% chimerism, and 8 (5%) reported 20% to 89% donor chimerism. Two patients have < 20% donor chimerism. The 10- and 15-year probabilities of survival were 90% and 79%, respectively. Results of multivariate analysis showed higher mortality risks for transplantations before 2003 (hazard ratio [HR], 7.87; P = .001), chronic graft-versus-host disease (GVHD) (HR, 3.80; P = .004) and squamous cell carcinoma after transplantation (HR, 38.17; P < .0001). The predominant cause of late mortality was squamous cell carcinoma, with an incidence of 8% and 14% at 10 and 15 years after transplantation, respectively, and was more likely to occur in those with chronic GVHD. Other causes of late mortality included chronic GVHD, infection, graft failure, other cancers, and hemorrhage. Although most patients are disease free and functional long term, our data support aggressive surveillance for long periods to identify those at risk for late mortality.

Oral and Maxillofacial Complex Lesions in Adolescents: A Retrospective Study of 20 Years / Lesiones Orales y del Complejo Maxilofacial en Adolescentes: Un Estudio Retrospectivo de 20 Años

Studies assessing the prevalence of oral lesions show that 5.2% to 12.8% of biopsy specimens are from children and adolescents. In Brazil, there are few studies analyzing the distribution of oral lesions in that population. Therefore, the aim of this study was to evaluate the distribution of oral and maxillomandibular lesions of adolescents, admitted to the Department of Oral Diagnosis of the Federal University of Paraná (UFPR) from 1994 to 2013. A study based on the review of the charts was done and the lesions were classified in 11 categories: salivary glands disease, dental pathology, gingival and periodontal pathology, odontogenic cysts, odontogenic tumors, non odontogenic cysts, bone pathology, mucosal pathology, connective tissue pathology, malignant tumors and other pathology. Variables including age, sex, ethnicity, diagnosis and lesion location were also evaluated. Upon analysis, 376 lesions were identified, most in girls, 51.9% and 77.1 % of patients were white. The most frequent site was the lower lip followed by the gingiva. Mucocele was the most common lesion (27.6%), followed by fibroepithelial hyperplasia (8.2%) and pyogenic granuloma (5.3%). Although there is a wide range of pathologies that can occur in the oral cavity, traumatic injuries are the most common in this age group.

Los estudios que evalúan la prevalencia de lesiones orales muestran que entre el 5,2% hasta el 12,8% de las muestras de biopsia corresponden a niños y adolescentes. En Brasil, hay pocos estudios que hayan analizado la distribución de las lesiones orales enesa población. Por lo tanto, el objetivo de este estudio fue evaluar la distribución de las lesiones orales y maxilofaciales de adolescentes, ingresados en el Servicio de Diagnóstico Oral de la Universidad Federal de Paraná (UFPR) entre 1994 a 2013. Se llevó a cabo la revisión de historias clínicas y las lesiones halladas fueron clasificadas en 11 categorías: enfermedad de glándulas salivales, patología dental, patología gingival y periodontal, quistes odontogénicos, tumores odontogénicos, quistes no odontogénicos, patología ósea, patología de la mucosa, patología del tejido conectivo, tumores malignos y otras patologías. También se evaluaron otras variables como edad, género, etnia, diagnóstico y localización de la lesión. Luego del análisis, se identificaron 376 lesiones, la mayoría en niñas (51,9%), siendo el 77,1% de los pacientes de raza blanca. La localización más frecuente fue el labio inferior seguida de la encía. El mucocele fue la lesión más común (27,6%), seguido de hiperplasia fibroepitelial (8,2%) y granuloma piógeno (5,3%). Aunque existe una amplia serie de patologías que pueden ocurrir en la cavidad oral, las lesiones traumáticas son las más comunes en este grupo de edad.

Oral manifestations compatible with chronic graft-versus-host disease in patients with Fanconi anemia.

Fanconi anemia (FA) is a genetic disease that is characterized by several congenital abnormalities and progressive bone marrow failure and is associated with an increased susceptibility to malignant disorders. Currently, the only potential cure for hematological disorders is hematopoietic stem cell transplantation (HSCT). However, 1 of the most common complications after HSCT is the development of oral chronic graft-versus-host disease (cGVHD), which is also a risk factor for the development of cancer, particularly oral squamous cell carcinoma. Therefore, the purpose of this study was to describe the prevalence and characteristics of oral manifestations compatible with cGVHD in patients diagnosed with FA according to the National Institutes of Health (NIH) consensus criteria. A total of 96 patients (51 females, 45 males; median age, 16 years) with FA, who were in medical follow-up after HSCT at the outpatient clinic of the bone marrow transplantation unit (Hospital de Clínicas from the Universidade Federal do Paraná) underwent an oral evaluation between January 2013 and December 2013. Post-HSCT periods varied from 1 to 261 months and were divided into 3 periods: immediate post-HSCT period; intermediate post-HSC period, and late post-HSCT period. Among the evaluated patients, 40 of 96 (42%) presented with oral manifestations of cGVHD, with 29 of 40 (73%) of these patients in the late post-HSCT period. NIH scale scores varied from 0 to 10, and lichenoid and hyperkeratotic lesions were the abnormalities most frequently observed (100%). Overall, a high prevalence of oral manifestations was observed for cGVHD patients with FA. These data highlight the importance of monitoring oral manifestations compatible with cGVHD to identify and treat individuals with a higher risk of developing oral cancer.

Performance of distant diagnosis of dental caries by teledentistry in juvenile offenders.

BACKGROUND: Telehealth has been considered a practical and potentially cost-effective method of providing healthcare to the underprivileged population. The aim of this study was to determine whether a teledentistry system is a valid tool to screen for the presence of dental caries in a cohort of 102 Brazilian juvenile offenders. MATERIALS AND METHODS: Clinical examinations were performed to evaluate the decayed, missing, and filled teeth (DMFT) index and oral conditions of each adolescent, documented using a digital camera. For distant diagnosis, two methods of image transmission were tested. In the first, images were uploaded on a file-sharing service, and the link was sent via e-mail to one distant consultant. The other consultant received the images recorded on a compact disc. Both assessed the files and calculated the DMFT index for each case. The degree of agreement between the traditional examination and distant consultant assessments was measured by the kappa coefficient of agreement. Sensitivity and specificity were also calculated. RESULTS: According to the traditional clinical examination, 93.1% of the adolescents had at least one instance of dental caries with a mean DMFT index of 5.9. Kappa statistics ranged from "strong" to "almost perfect." Sensitivity ranged from 48% to 73%, and specificity was either 97% or 98%. CONCLUSIONS: Adolescent inmates could benefit from oral health screening using digital photography. Teledentistry appears to be a reliable alternative to the traditional oral examination for dental caries assessment.

Teledentistry: distant diagnosis of oral disease using e-mails.

OBJECTIVE: The purpose of this study was to evaluate the applicability of telediagnosis in oral medicine, through the transmission of clinical digital images by e-mail. SUBJECTS AND METHODS: The sample included 60 consecutive patients who sought oral medicine services at the Federal University of Paraná, in the state of Paraná, located in southern Brazil. The clinical history and oral lesion images were recorded using clinical electronic charts and a digital camera, respectively, and sent by e-mail to two oral medicine consultants. The consultants provided a maximum of two clinical hypotheses for each case, which were compared with biopsy results that served as the gold standard. RESULTS: In 31 of the 60 cases (51.7%), both consultants made the correct diagnosis; in 17 cases (28.3%), only one consultant made the correct diagnosis; and in 12 cases (20%), neither consultant made the correct diagnosis. Therefore, in 80% of cases, at least one consultant provided the correct diagnosis. The agreement between the first consultant and the gold standard was substantial (κ=0.669), and the agreement between the second consultant and the gold standard was fair (κ=0.574). CONCLUSIONS: The use of information technology can increase the accuracy of consultations in oral medicine. As expected, the participation of two remote experts increased the possibility of correct diagnosis.

[Strategies for management of oral cancer in primary and secondary healthcare services]. / Abordagem do câncer da boca: uma estratégia para os níveis primário e secundário de atenção em saúde.

Progress in cancer management by health systems involves improvements in surveillance, organization of healthcare services, specific programs focused on primary and secondary prevention, and scientific and technical advances in diagnosis and treatment. Despite well-known progress in the management of malignant neoplasms in all the above areas, oral cancer displays persistently high morbidity and mortality rates, apparently failing to reflect the accumulated scientific knowledge on the disease. The current article discusses the reasons for this mismatch, the need for redefining priorities in oral cancer management, and the implementation of such priorities as a public health policy.

Abordagem do câncer da boca: uma estratégia para os níveis primário e secundário de atenção em saúde / Strategies for management of oral cancer in primary and secondary healthcare services

O avanço verificado no enfrentamento de neoplasias malignas por meio dos sistemas de saúde envolve melhorias nas áreas de vigilância, organização de redes de assistência, programas específicos voltados às prevenções primária e secundária e, obviamente, aos avanços técnico-científicos que caracterizam a abordagem diagnóstica e terapêutica. Embora seja notável o reconhecimento de avanços no manejo de neoplasias malignas em todas as áreas citadas, o câncer da boca permanece com indicadores de morbidade e mortalidade que parecem não acompanhar o acúmulo científico no conhecimento da doença. O presente manuscrito objetiva discutir os motivos desse descompasso, a necessidade de reorientação de prioridades na abordagem do câncer da boca e sua efetivação como política pública de saúde.

Progress in cancer management by health systems involves improvements in surveillance, organization of healthcare services, specific programs focused on primary and secondary prevention, and scientific and technical advances in diagnosis and treatment. Despite well-known progress in the management of malignant neoplasms in all the above areas, oral cancer displays persistently high morbidity and mortality rates, apparently failing to reflect the accumulated scientific knowledge on the disease. The current article discusses the reasons for this mismatch, the need for redefining priorities in oral cancer management, and the implementation of such priorities as a public health policy.