Leucoplasia verrugosa proliferativa: estudio de 32 casos.

RESUMEN La leucoplasia verrugosa proliferativa (LVP) es una forma especialmente agresiva de leucoplasia oral, de etiología desconocida y con unas características clínicas típicas que la definen como una entidad diferente aparte del resto de leucoplasias orales. La edad media es mayor, la distribución por sexos es diferente, así como la localización y evolución de las lesiones. La LVP muestra una gran tendencia a recurrir tras el tratamiento y a desarrollar carcinomas. Estos carcinomas aparecen en muy baja frecuencia en las localizaciones típicas afectadas en el carcinoma oral de células escamosas convencional, además estos carcinomas desarrollados presentan menos agresividad. La alta proporción de cancerización de campo que existe en estos pacientes (>50% de los casos de LVP que desarrollan cáncer posteriormente desarrollarán segundos tumores primarios en diferentes zonas de la cavidad oral). Esto apoya la hipótesis de un posible agente etiológico viral especialmente por el corto espacio de tiempo entre la aparición de los tumores en un mismo paciente. Los objetivos de nuestro estudio fueron analizar las características clínicas de un grupo de 32 pacientes con LVP, examinar su tendencia a recidivar tras el tratamiento y la tendencia a desarrollar cáncer, estudiar las características clínicas del cáncer en aquellos pacientes que desarrollaron carcinomas y detectar y tipificar en aquellos pacientes que desarrollaron cáncer. El material y métodos consistió en un grupo de 32 pacientes con leucoplasia verrugosa proliferativa del Servicio de Estomatología del Hospital General Universitario de Valencia. Nosotros analizamos sus características clínicas, su evolución, su tratamiento y la detección y tipificación de virus mediante el estudio biomolecular en aquellos pacientes que desarrollaron carcinomas. En los resultados obtuvimos que la edad media de los pacientes era de 7303 años de edad, con una predilección claramente por las mujeres (78´1% mujeres: 21´9% hombres). La proporción de no fumadores respecto a los fumadores era de 7187%: 28´12%). Las áreas más afectadas por las lesiones de leucoplasia era la encía inferior (813%) y la encía superior ((719%). El tipo clínico más frecuente fue la leucoplasia homogénea múltiple y la leucoplasia verrugosa en igual proporción (906%). El 25 % de los pacientes desarrollaron un carcinoma verrugoso y el 531% un carcinoma oral de células escamosas a lo largo de su evolución. La recidiva tras el tratamiento fue del 813%, el 781% en forma de leucoplasia, el 406% en un carcinoma oral de células escamosas y un 156% en forma de un carcinoma verrugoso. Detectamos virus de Epstein-Barr en un alto porcentaje de pacientes de leuucoplasia, en un 60%, subiendo la proporción a un 66% en aquellos pacientes de LVP que presentaban un carcinoma. Tras nuestro estudio llegamos a las siguientes conclusiones: la leucoplasia verruosa proliferativa es una entidad con predilección femenina, en no fumadoras, con una edad media de unos 70 años. Es una enfermedad multifocal, siendo la localización más frecuente la encía alveolar. La agresividad es debida a su alta tendencia a recurrir tras el tratamiento y a la elevada incidencia de cáncer. El cáncer se manifiesta con alta frecuencia en la encía. Loa pacientes de LVP pueden desarrollar varios carcinomas, esto demuestra la alta proporción de cancerización de campo de esta entidad. A pesar de alta presencia de virus de Epstein-Barr en nuestros pacientes de LVP, no podemos decir que exista una evidencia del papel directo entre la infección por el virus y la etiología de la enfermedad dabido a que el grupo de muestras estudiado es pequeño y porque nos encontramos ante un epitelio con múltiples alteraciones durante un largo periodo de tiempo. __________________________________________________________________________________________________Proliferative verrucous leukoplakia (PVL) is a special aggressive form of oral leukoplakia, the aetiology is unknown and with typically clinical characteristics which they define like a different entity a part from the rest of oral leukoplakias. The average age is higher, sex distribution is different just like location and evolution of lesions. PVL exhibits a high tendency to recur after treatment and to develop cancer. These carcinomas appear with very low frequency in the typical locations affected in conventional oral squamous cell carcinomas and they are less aggressive. The high field cancerization in this patients (more than 50% of cases of PVL which will develope a carcinoma, afterwards will develope second primary tumors in different parts of oral cavity), lend support to the hypothesis that PVL has a viral infection, specially because for the short evolution time between tumours in the same patient. Our main objectives were to analyze the clinical characteristics of a group of 32 patients with PVL, examining their tendency to develop cancer and studying the characteristics of this cancer in that patients who developed carcinomas, and detect virus in those patients wich develope cancer. Materials and methods were forms by the study group consisted of 32 patients with PVL in the Stomatology Service of the University General Hospital of Valencia. We analyzed their clinical characteristics, their evolution and treatment, and detection of virus by nested PCR for biomarkers (human papillomavirus and Epstein-Barr virus (EBV)). The mean age of our patients was de 7303 years old, with a distinct femal predilection (781% females: 219 males). There were 7187% of no cigarette smokers and 2812% of cigarette smokers. The areas more affected were the lower gingiva (813%) and upper gingiva (719%). The most frequent clinical type was multiple homogeneous leukoplakia and verrucous leukoplakia (90´6%). The 25% of patients developed a verrucous carcinoma (CV) and the 531% an oral squamous cell carcinoma (OSCC) in their evolution. The relapse after treatment was 813% of PVL, 781% were leukoplakia, 40´6% were OSCC and 156% were CV. We detected EBV in a high rate of patients, 60%, and we detected in a rate of 66% in those patients with PVL and carcinoma. PVL is an entity with female predilection, no smokers, with a mean age over 70 years old. This is a multifocal illness and the most frequent location is alveolar gingiva. The aggressiveness is due to its high tendency to recur after treatment and to the increased incidence of cancer. The cancer is manifested with highest frequency in gingiva. Patients with PVL could develop several carcinomas, thus demostrate the high rate of field cancerization of this entity. Although we detected a high proportion of patients with Epstein-Barr virus in our patients we can not say that exists a direct relation between the viral infection and the etiology of PVL because our study group is small and the epithelial cells of these patients show disturbances during a long period of time

Reacción granulomatosa facial por rellenos cosméticos inyectados: presentación de cinco casos

El uso de sustancias para el aumento de tejidos blandos por motivos estéticos puede ocasionar la aparición de granulomas a cuerpo extraño, entre otros efectos indeseables. Las mejoras introducidas en dichas sustancias han conseguido la disminución de la incidencia de reacciones adversas pero no su desaparición. Presentamos cinco casos de reacción a cuerpo extraño por tres productos diferentes, dimetilpolisiloxano (silicona), colágeno bovino, y ácido poliláctico, que habían sido infiltrados en el tejido celular subcutáneo de las pacientes (las cinco eran mujeres) entre dos y dieciséis años antes de la aparición de la reacción a cuerpo extraño. Las cinco presentaron un cuadro de tumefacción facial difusa, no dolorosa y de consistencia duroelástica. Los estudios de imagen con resonancia magnética mostraron signos de reacción inflamatoria intensa de la zona afectada. La histología mostró la presencia de granulomas a cuerpo extraño con células gigantes multinucleadas. Las pacientes fueron tratadas con corticoides administrados por vía sistémica, excepto una de ellas que no precisó tratamiento farmacológico.The use of substances to augment soft tissues as aesthetic purpose is associated with, among other undesirable effects, the appearance of foreign body granulomas. The improvements made to these substances have reduced the incidence of adverse reactions, but not eliminated them. We present five cases of foreign body reactions to three different products, dimethylpolysiloxane (silicone), bovine collagen, and polylactic acid, which were injected into the subcutaneous cellular tissue of the patients (all five were women), between two and sixteen years before the appearance of the foreign body reaction. All five presented painless, diffuse facial tumefaction, of firm, elastic consistency. The magnetic resonance image (MRI) studies showed signs of intense inflammatory reaction in the affected areas. The histology revealed the presence of foreign body granulomas with giant multi-nucleated cells. The patients were treated with systemically administered corticoids, except in one case which did not require pharmacological treatment

Granulomatous facial reaction to injected cosmetic fillers : a presentation of five cases

The use of substances to augment soft tissues as aesthetic purpose is associated with, among other undesirable effects, the appearance of foreign body granulomas. The improvements made to these substances have reduced the incidence of adverse reactions, but not eliminated them. We present five cases of foreign body reactions to three different products, dimethylpolysiloxane (silicone), bovine collagen, and polylactic acid, which were injected into the subcutaneous cellular tissue of the patients (all five were women), between two and sixteen years before the appearance of the foreign body reaction. All five presented painless, diffuse facial tumefaction, of firm, elastic consistency. The magnetic resonance image (MRI) studies showed signs of intense inflammatory reaction in the affected areas. The histology revealed the presence of foreign body granulomas with giant multi-nucleated cells. The patients were treated with systemically administered corticoids, except in one case which did not require pharmacological treatment

Stimulated whole salivary flow rate:the most appropriate technique for assessing salivary flow in Sjögren syndrome

We sought to determine the most appropriate method for measuring salivary flow to aid the diagnosis of Sjögren's syndrome (SS). Specifically, we compared the unstimulated whole salivary flow rate (UWSFR) with the stimulated whole salivary flow rate (SWSFR). This case-control study comprised one group of 103 patients with SS and a control group of 50 healthy people. We measured the UWSFR and SWSFR in both groups according to the guidelines established by Navacet (1993). The UWSFR and SWSFR were significantly lower in the patient group compared with the controls (p < 0.01). Among the participants in the patient group, we found a decreased UWSFR in 84 individuals (81.5%) and a decreased SWSFR in 90 individuals (87.4%). We encountered difficulties obtaining saliva in 37 (35.9%) patients during the UWSFR test, and in 12 (11.7%) patients during the SWSFR test. There was no significant statistical difference in the UWSFR or SWSFR between patients with primary and secondary SS. Compared with the UWSFR, the SWSFR is a more suiTable and effective method for measuring salivary flow in patients with SS, as well as for qualitative analysis of the obtained saliva

Prevalence of salivary Epstein-Barr virus in potentially malignant oral disorders and oral squamous cell carcinoma

Background: To analyze the presence of salivary Epstein-Barr virus (EBV) DNA in oral squamous cell carcinoma and potentially malignant oral disorders. Material and Methods: Three groups were studied: Group 1 (12 oral squamous cell carcinomas (OSCC)), Group 2 (12 potentially malignant oral disorders (PMD)) and Group 3 (47 healthy controls). EBV DNA salivary analysis was performed by PCR. Results: The highest percentage of positive salivary EBV DNA corresponded to the OSCC group (58.3%), followed by the PMD group (41.7%) and the controls (40.4%). The differences between groups were not statistically significant, however (p>0.05). Conclusions: Salivary EBV DNA was more prevalent in OSCC than in PMD or the controls

Benign tumors of the oral mucosa: A study of 300 patients

Objectives: To analyze the frequency and type of the most common benign tumors of the oral mucosa found at the Hospital Stomatology Service, and to study the clinical characteristics and possible etiological factors. Material and Methods: This is a retrospective study of 300 patients with histologically diagnosed benign tumors of the oral mucosa. Data was compiled for each case, documenting information relating to age, gender, medication, habits (smoking, oral hygiene), anamnesis (reason for consultation, symptomatology, evolution), and the characteristics of the lesion (site, color, size, surface, consistency, and base). Results: Of all the tumors studied, 53% were histologically diagnosed as fibroma. In the study of prevalence of benign tumors of the oral mucosa, no differences were found for age; however there were differences according to gender, finding a greater prevalence of fibromas, pyogenic granulomas, and giant cell granulomas in women, at a ratio of 2:1. The group of tumors studied showed a significantly asymptomatic behaviour, and self-limiting and slow growth. With respect to the possible etiologic agents, we found no statistically significant differences between them. Conclusions: Following the study of 300 patients histologically diagnosed with benign tumor of the oral mucosa, we can state that with regard to prevalence, we found significant differences with respect to gender, being more frequent in women. The fibroma is the most frequent benign tumor of the oral cavity

Intraoral Schwannomas: presentation of a series of 12 cases

Introduction: Schwannomas are benign and not very frequent tumors of the peripheral nerves, derived from the nerve supporting Schwann cells. Study Design: Data were collected on the clinical manifestations (sex, age), location, size and symptonts of the lesions as well as the evolution time and the initial (presumption) diagnosis. Results: Twelve patients were documented, with a mean age of 29,5 ± 12,1 years (range 16-50) and a balanced gender distribution. The mean duration of the lesions was 42,17± 45,3 months. The lesion located in the floor of the mouth was the largest tumor, measuring about 4 cm in maximum diameter, while the average size of the 12 schwannomas was 2.04± 1.1 cm. Conclusion: We present 12 oral schwannomas diagnosed and treated over a period of 10 years

Osteonecrosis of the jaws by intravenous bisphosphonates and osteoradionecrosis : a comparative study

Aims: We analyze the possible clinical differences between bone jaw exposed areas in ONJ (osteonecrosis of the jaws) and ORN (osteoradionecrosis). Patients and method: Group 1 was composed with 53 ONJ cases and group 2 with 20 ORN cases. In both groups we analyzed, the major size of the exposed bone areas, the number of exposed areas, the location on the jaws and the presence of others associated and severe complications, such as skin fistulas and jaw fractures. We also investigated the possible local aetiology or trigger factor of the lesions. Results: The major size of the bone exposed areas was 2.29±2.02(mean ± std.dev) in group 1 and 2.7±2.9 (mean ± std.dev) in group 2 (p>0.05). The number of exposed areas was 1.8±1.34 (mean ± std.dev) in group 1 and 1.2±0.55 (mean ± std.dev) in group 2 (p>0.05). There were more fractures in the second group (20%) (p<0.05), and skin fistulas (35%) (p<0.05). We found more patients in group 1 in which the dental extraction was the local aetiology of the bone necrosis (35 cases, 66.03%), while in group 2 there were 8 (40%) (p<0.05). Conclusions: In our study with ONJ there were not differences in the major size of the bone exposed areas, but there were more lesions per patient than in group with ORN. The severity of the complications, such as jaw fractures and skin fistulas were higher in ORN, and in this group it was more frequent the spontaneous lesions than in the ONJ where it is more frequent following dental extractions

Epstein-Barr virus in oral proliferative verrucous leukoplakia and squamous cell carcinoma : a preliminary study

The aim of this study was to analyze proliferative verrucous leukoplakia (PVL) and oral squamous cell carcinoma (OSCC) for the possible presence of Epstein-Barr virus (EBV). We studied three groups: Sub-Group 1 was composed of 10 patients with PVL, (6 of whom had developed OSCC); Sub-Group 2 comprised 5 patients with OSCC but no preceding PVL; and Sub-Group 3 were 5 controls with clinically normal oral mucosa. Oral biopsies from all cases were examined for Epstein-Barr virus (EBV) by nested PCR. EBV was detected in 60% of Sub-Group 1 patients (PVL ) and in 40% of Sub-Group 2 (OSCC), but in 0% of SubGroup 3 (controls)

CIBERER : Spanish national network for research on rare diseases: A highly productive collaborative initiative

Altres ajuts: Instituto de Salud Carlos III (ISCIII); Ministerio de Ciencia e Innovación.CIBER (Center for Biomedical Network Research; Centro de Investigación Biomédica En Red) is a public national consortium created in 2006 under the umbrella of the Spanish National Institute of Health Carlos III (ISCIII). This innovative research structure comprises 11 different specific areas dedicated to the main public health priorities in the National Health System. CIBERER, the thematic area of CIBER focused on rare diseases (RDs) currently consists of 75 research groups belonging to universities, research centers, and hospitals of the entire country. CIBERER's mission is to be a center prioritizing and favoring collaboration and cooperation between biomedical and clinical research groups, with special emphasis on the aspects of genetic, molecular, biochemical, and cellular research of RDs. This research is the basis for providing new tools for the diagnosis and therapy of low-prevalence diseases, in line with the International Rare Diseases Research Consortium (IRDiRC) objectives, thus favoring translational research between the scientific environment of the laboratory and the clinical setting of health centers. In this article, we intend to review CIBERER's 15-year journey and summarize the main results obtained in terms of internationalization, scientific production, contributions toward the discovery of new therapies and novel genes associated to diseases, cooperation with patients' associations and many other topics related to RD research