Épulis congénito gigante: importancia del diagnóstico prenatal / Congenital giant cell epulis: Importance of the prenatal diagnosis

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Tumor del estroma gastrointestinal y schwannoma gástricos: una presentación inusual en un paciente sin neurofibromatosis / Gastric gastrointestinal stromal tumour and schwannoma: an unusual presentation in a patient without neurofibromatosis

Actualmente, la mayoría de las neoplasias presentes en el estómago son de tipo epitelial, constituyendo el subtipo más frecuente. El siguiente subtipo de tumor más común es el estromal, siendo el tumor del estroma gastrointestinal el más frecuente, seguido por el leiomioma y el schwannoma. En el presente artículo mostramos el caso excepcional de un paciente con un tumor del estroma gastrointestinal y sospecha de enfermedad residual gástrica, la cual fue diagnosticada posteriormente como schwannoma tras su exéresis

The majority of gastric neoplasms are of epithelial type. Stromal tumours are the next most frequent and are most commonly gastrointestinal stromal tumours, followed by leiomyoma and schwannoma. We present an exceptional case of a patient with a gastrointestinal stromal tumour with suspicion of residual gastric disease, which was diagnosed post-operatively as a schwannoma

[Gastric gastrointestinal stromal tumour and schwannoma: An unusual presentation in a patient without neurofibromatosis]. / Tumor del estroma gastrointestinal y schwannoma gástricos: una presentación inusual en un paciente sin neurofibromatosis.

The majority of gastric neoplasms are of epithelial type. Stromal tumours are the next most frequent and are most commonly gastrointestinal stromal tumours, followed by leiomyoma and schwannoma. We present an exceptional case of a patient with a gastrointestinal stromal tumour with suspicion of residual gastric disease, which was diagnosed post-operatively as a schwannoma.

Carcinoma tubuloquístico. Una entidad infrecuente en la patología tumoral renal / Tubulocystic renal cell carcinoma. A rare entity in neoplastic renal pathology

El carcinoma renal tubuloquístico es una neoplasia infrecuente dentro de la patología tumoral renal. En el presente caso comentamos la evolución de un paciente con cólicos nefríticos de repetición en el que durante el seguimiento ecográfico se descubrió una lesión nodular de aspecto quístico. Posteriormente la lesión aumentó de tamaño y se decidió nefrectomía parcial (tumorectomía). En el examen histológico e inmunohistoquímico se estableció el diagnóstico de carcinoma renal tubuloquístico

Tubulocystic renal carcinoma is an uncommon neoplasm. We present a case of a patient presenting with multiple renal colic. A nodular cystic lesion was an incidental sonographic finding which increased in size during subsequent follow-ups. The patient underwent radical nephrectomy and tubular renal carcinoma was diagnosed histopathologically and immunohistochemically

[Tubulocystic renal cell carcinoma. A rare entity in neoplastic renal pathology]. / Carcinoma tubuloquístico. Una entidad infrecuente en la patología tumoral renal.

Tubulocystic renal carcinoma is an uncommon neoplasm. We present a case of a patient presenting with multiple renal colic. A nodular cystic lesion was an incidental sonographic finding which increased in size during subsequent follow-ups. The patient underwent radical nephrectomy and tubular renal carcinoma was diagnosed histopathologically and immunohistochemically.

Quiste sinovial de articulación temporomandibular: presentación de un caso / Synovial cyst of the temporomandibular joint: presentation of a case

El quiste sinovial localizado en la articulación temporomandibular es una entidad rara, con pocos casos reportados en la literatura médica. Gaisford et al. fueron los primeros autores que informaron de un caso de quiste sinovial, patología que afecta más frecuentemente a articulaciones como la muñeca o la rodilla. Estos quistes están delimitados por células sinoviales, y pueden estar comunicados o ser independientes de la cavidad articular. Se han propuesto varias teorías para explicar la etiología de esta patología, un incremento de la presión en la cavidad articular causado por una inflamación o traumatismo puede ser clave para producir una herniación de la membrana sinovial dando lugar a la entidad. Entre los diagnósticos que hay que considerar ante una tumoración preauricular se encuentran los tumores de parótida, los quistes sebáceos, los gangliones y los quistes sinoviales, entre otros. El tratamiento quirúrgico es el tratamiento frecuentemente propuesto por la literatura, logrando una escasa tasa de recurrencia (AU)

A synovial cyst located in the temporomandibular joint is rare, with few cases reported in the medical literature. Gaisford et al. were the first authors to report a case of a synovial cyst. This a condition that more frequently affects joints such as the wrist or knee. These cysts are delimited by synovial cells, and may be connected to, or be independent of, the joint cavity. Several theories have been proposed to explain the etiology of this disease: an increase in pressure in the joint cavity caused by inflammation or trauma may be the reason that a herniation of the synovial membrane could be produced. Among the diagnoses to be considered when faced with a tumor are pre-auricular parotid tumors, sebaceous cysts, ganglia and synovial cysts, among others. Surgery is the treatment most frequently proposed in the literature, achieving a low rate of recurrence (AU)

Resultados funcionales del trasplante pulmonar en la enfermedad pulmonar obstructiva crónica / Functional Outcomes After Lung Transplant in Chronic Obstructive Pulmonary Disease

Introducción: El trasplante pulmonar (TP) es una opción terapéutica con resultados controvertidos en la enfermedad pulmonar obstructiva crónica (EPOC). Nuestro objetivo es analizar los resultados del trasplante en términos de función pulmonar, así como identificar los factores pronósticos. Método: Se realizó un análisis retrospectivo de 107 pacientes con EPOC trasplantados en el Hospital Universitario La Fe entre 1991 y 2008. Se analizaron variables preoperatorias, estudio funcional pre y post-TP, variables del procedimiento quirúrgico y del seguimiento a largo plazo, expresadas en media o porcentaje según el caso, comparándose los resultados espirométricos antes y después del TP. Para el análisis multivariante se utilizó regresión lineal o logística según la variable. Resultados: Fueron trasplantados 94 hombres (87,9%) y 13 mujeres (12,1%), con una edad media ± desviación estándar de 52,58 ± 8,05 años; el 71% de TP fueron bipulmonares. Los valores espirométricos mejoraron tras el TP: FVC: +1,22 l (+34,9%), FEV1: +1,66 l (+56,7%) y FEF25-75: +1,85 l (+50,8%); p = 0,001, manteniéndose esta mejoría funcional tras 5 años solo en el grupo con puntuación BODE > 7 (p = 0,001). La talla del receptor, el tipo de TP, la utilización de circulación extracorpórea durante el procedimiento quirúrgico, la presencia de síndrome de bronquiolitis obliterante junto con la edad y la causa de muerte del donante influyeron significativamente en la función pulmonar a largo plazo. Conclusiones: El TP mejora la función pulmonar de los pacientes con EPOC. Esta mejoría se mantiene a los 5 años solo en los pacientes con BODE mayor de 7. El trasplante bipulmonar proporciona mejores resultados funcionales que el unipulmonar

Introduction: Lung transplantation (LT) is a therapeutic option with controversial results in chronic obstructive pulmonary disease (COPD). We aimed to analyze the outcomes of transplantation in terms of lung function and to identify prognostic factors. Method: A retrospective analysis of 107 patients with COPD receiving lung transplants in the La Fe Hospital between 1991 and 2008 was performed. Preoperative variables, pulmonary function tests before and after LT, surgical procedure variables and long-term monitoring, expressed as mean or percentage, as applicable, were analyzed. Spirometric results before and after LT were analyzed. Linear or logistic regression were used for multivariate analysis depending on the variable. Results: Ninety-four men (87.9%) and 13 women (12.1%) were transplanted, with a mean age ± standard deviation of 52.58 ± 8.05 years; 71% of LTs were double-lung transplantations. Spirometric values improved after LT: FVC: +1.22 L (+34.9%), FEV1: +1.66 L (+56.7%) and FEF25-75: +1.85 L (+50.8%); P = .001. This functional improvement was maintained after 5 years only in the group with BODE score > 7 (P = .001). Recipient height, type of LT, use of extracorporeal circulation during the surgical procedure, presence of bronchiolitis obliterans syndrome and the age and cause of death of the donor significantly influenced lung function over time. Conclusions: LT improves lung function in COPD patients. This improvement was maintained at 5 years only in patients with BODE > 7. Double lung transplantation provides better functional results than single-lung transplantation

Functional outcomes after lung transplant in chronic obstructive pulmonary disease.

INTRODUCTION: Lung transplantation (LT) is a therapeutic option with controversial results in chronic obstructive pulmonary disease (COPD). We aimed to analyze the outcomes of transplantation in terms of lung function and to identify prognostic factors. METHOD: A retrospective analysis of 107 patients with COPD receiving lung transplants in the La Fe Hospital between 1991 and 2008 was performed. Preoperative variables, pulmonary function tests before and after LT, surgical procedure variables and long-term monitoring, expressed as mean or percentage, as applicable, were analyzed. Spirometric results before and after LT were analyzed. Linear or logistic regression were used for multivariate analysis depending on the variable. RESULTS: Ninety-four men (87.9%) and 13 women (12.1%) were transplanted, with a mean age±standard deviation of 52.58±8.05 years; 71% of LTs were double-lung transplantations. Spirometric values improved after LT: FVC: +1.22L (+34.9%), FEV1: +1.66L (+56.7%) and FEF25-75: +1.85L (+50.8%); P=.001. This functional improvement was maintained after 5 years only in the group with BODE score >7 (P=.001). Recipient height, type of LT, use of extracorporeal circulation during the surgical procedure, presence of bronchiolitis obliterans syndrome and the age and cause of death of the donor significantly influenced lung function over time. CONCLUSIONS: LT improves lung function in COPD patients. This improvement was maintained at 5years only in patients with BODE>7. Double lung transplantation provides better functional results than single-lung transplantation.

Expresión de calretinina en el tumor de células granulares: análisis del gradiente de intensidad en la inmunotinción con correlación clínico-patológica / Calretinin expression in granular cell tumour: an analysis of the immunostaining intensity gradient and its clinicopathological correlation

Introducción. El tumor de células granulares (TCG) es una lesión poco frecuente que aparece predominantemente en mujeres y en el área de cabeza y cuello. Múltiples estudios apuntan a que deriva de las células de Schwann y su perfil inmunohistoquímico ha sido ampliamente estudiado. La expresión de calretinina (CRT) es, sin embargo, la peor caracterizada en el TCG, por ello en este estudio se pretende analizar en detalle su expresión en esta forma tumoral. Material y método. Se analizó una amplia muestra de TCG (n = 43), de los que se recogieron datos clínico-epidemiológicos y se realizó inmunotinción en todos ellos con anticuerpo anti CRT estudiando su patrón de expresión. Resultados. Se encontró que este tumor muestra positividad global frente a CRT en una amplia mayoría de los casos (74%), encontrando un gradiente en la inmunotinción en el 53% de los TCG estudiados, situación observada en los tumores con superficies epiteliales en proximidad y que no ocurre cuando estos epitelios se encuentran ausentes o alejados del TCG. Conclusiones. La tinción frente a CRT es útil para el diagnóstico del TCG y se demuestra la existencia de un gradiente en la inmunotinción cuando el TCG se encuentra en la proximidad de epitelios escamosos, sobre todo cuando estos muestran hiperplasia epitelial pseudoepiteliomatosa, lesión a la que frecuentemente se encuentran asociados (AU)

Introduction. Granular cell tumour (GCT) is a rare lesion which predominately affects women and occurs most frequently in the head and neck. Many reports suggest that the tumour cells are probably derived from Schwann cells. The immunohistochemical profile of GCT has been extensively studied, although calretinin expression (CRT) is the least understood. Our aim is to undertake a detailed analysis of its expression in this tumour. Materials and methods. A large sample of GCT (n = 43) was analysed. Clinical-epidemiological data were collected and immunostaining with CRT was performed in all of them with anti-CRT antibody to study its expression pattern. Results. We found that most of the GCT cases were positive to CRT (74%). Furthermore, in 53% of cases, we detected an intensity gradient in the immunostaining in tumour cells with epithelial surfaces in proximity, which does not occur when these epithelia are absent or distant from the GCT. Conclusions. CRT is a useful marker for the diagnosis of GCT. We found the existence of an immunostaining intensity gradient when the tumour is in close proximity to squamous epithelia, especially when these show pseudoepitheliomatous epithelial hyperplasia, which is often associated with GCT (AU)

[Immunophenotypic classification of 3 cases of hepatocellular adenoma. Differential diagnosis with focal nodular hyperplasia]. / Clasificación inmunofenotípica de 3 casos de adenomas hepatocelulares. Diagnóstico diferencial con hiperplasia nodular focal.

Interest in adenomas has been renewed by the discovery of the molecular changes in these tumors. The latest World Health Organization publication on gastrointestinal tract tumors (2010) includes four types of hepatic adenomas, which are well characterized immunohistochemically, genotypically and phenotypically. In these tumors, medical history and morphological behavior play an important role in determining the risk of malignancy, mainly in adenomas with a b-catenin mutation. The presence of steatosis, inflammation, vascular changes linked to response to L-FABP, serum amyloid A, and glutamyl synthetase help to classify these tumors into four groups: hepatocellular adenomas with the HNF1A mutation (H-HCA), those with the b-catenin mutation (b-HCA), inflammatory HCA (IHCA), and HCA without markers. The absence of glypican 3 expression, HSP 70 and perivenular mapping of glutamyl synthetase helps to distinguish these tumors from well differentiated hepatocellular carcinoma. We describe the clinical, morphological and immunophenotypic features of three patients diagnosed with hepatic adenomas in a 2-year period.

[Complications after lung transplantation in chronic obstructive pulmonary disease]. / Complicaciones del trasplante de pulmón en la enfermedad pulmonar obstructiva crónica.

BACKGROUND AND OBJECTIVE: Lung transplantation (LT) in chronic obstructive pulmonary disease (COPD) is a procedure with a high rate of morbimortality. The aim of this paper is to analyze the early and late rates of complications and mortality in COPD patients undergoing LT. PATIENTS AND METHOD: Retrospective study of 107 COPD patients transplanted in the Hospital Universitario La Fe, between 1991 and 2008. Preoperative variables were collected as well as all the complications, medical and surgical, occurred in the follow-up, which are expressed as mean or percentage as appropriate. The 30-day mortality and long term survival were established. RESULTS: A total of 94 men (87.9%) and 13 women (12.1%) were transplanted with a mean age (SD) of 52.58 (8.05) years with 71% of double-lung LT. BODE score was 7.24 (1.28). The rate of primary graft dysfunction was 39.3%. The most common surgical complications were phrenic paralysis (16.8%), hemothorax (17.8%) and pleural effusion (30.8%). There was a high number of postoperative hospitalization (30%) and medical complications such as hypertension (36%), diabetes mellitus (16.7%) and renal failure (40%), secondary to treatment. Perioperative mortality was 14% and 34.5% after a year, being the most frequent causes infections (34.6%) and chronic rejection (BOS) (17.8%). Five-year survival was 40.9% with bronchiectasis and smoking history being the risk factors. CONCLUSIONS: LT is a procedure with a high early mortality rate associated with high medical and surgical complications that affect the outcome.

Osteonecrosis maxilar relacionada con bisfosfonatos (OMRB): consideraciones patogenéticas con posibles implicaciones terapéuticas / No disponible

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Follicular adenomatoid odontogenic tumor: immunohistochemical study.

Adenomatoid odontogenic tumor (AOT) is an uncommon benign odontogenic lesion that affects young patients, with female predominance, mainly in second decade, showing a radiolucent unilocular image associated with an unerupted tooth, usually a canine. In spite of previous and confusing denominations, such as adenoameloblastoma or adenomatoid ameloblastic tumor, AOT is a benign tumor with a very low rate of recurrence, that show a peculiar morphological picture (basaloid appearance with glandular-like structures, calcifying areas, and amiloid-like material) that allow its histopathological recognition. We present a clinicopathological analysis of a case of follicular AOT affecting the mandible in a 9 years-old female patient associated with unerupted lower left canine. Immunohistochemical study showed some data previously unrecognised. All cellular types that composed AOT showed nuclear positivity for p63 indicating a basal characterization in the different cellular components. According to its benign character and low potential for recurrence, AOT revealed a scant proliferative activity (2-3% nuclei showed Ki-67 positivity) limited to some epithelial nodules (AE1-3 +) of fusiform appearance. Absence of reactivity for hormonal receptors (RE and RPg) excluded a possible hormonodependence in AOT that could explain the observed female predominance.

Tumor odontogenico adenomatoide folicular: Estudio inmunohistoquimico / Follicular adenomatoid odontogenic tumor: immunohistochemical study

El tumor odontogénico adenomatoide (TOA) es una infrecuente lesión odontogénica benigna, que aparece en pacientesjóvenes, generalmente mujeres en la segunda década de la vida, a menudo como una lesión radiolúcida de aspecto quístico unilocular, en asociación a un diente, usualmente canino, no erupcionado.A pesar de haberse denominado también adenoameloblastoma o tumor ameloblástico adenomatoide, el TOA es una lesión benigna con una muy baja tendencia a la recidiva, mostrando una morfología muy peculiar (apariencia basaloide con estructuras glanduliformes, calcificaciones esferulares, presencia de material amiloide) que facilitan su reconocimientohistológico. Se presenta un análisis clínico-patológico de un TOA de tipo folicular de maxilar inferior, en asociación a la inclusiónde un canino inferior izquierdo, en una paciente pediátrica de 9 años. El estudio inmunohistoquímico realizado muestra algunos datos previamente no referidos. A pesar de existir distintos tipos celulares en el TOA se observo una universal inmunorreactividad para p63, demostrando el carácter basal de los distintos elementos que lo constituyen. En concordancia con su benignidad y con su baja tasa de recidiva existe una escasa actividad proliferativa (2-3% de núcleos marcados por el antígeno Ki-67), estando la proliferación reducida a pequeños nódulos de células epiteliales (AE1-3 +) de núcleos elongados o fusiformes; de otra parte la mayor incidencia en pacientes de sexo femenino no puede ser explicada en base a la existencia de una hormonodependecia tumoral dada la ausencia de expresión de receptores hormonales (RE y RPg)

Adenomatoid odontogenic tumor (AOT) is an uncommon benign odontogenic lesion that affects young patients, with female predominance, mainly in second decade, showing a radiolucent unilocular image associated with an unerupted tooth, usually a canine.In spite of previous and confusing denominations, such as adenoameloblastoma or adenomatoid ameloblastic tumor, AOT is a benign tumor with a very low rate of recurrence, that show a peculiar morphological picture (basaloid appearance with glandular-like structures, calcifying areas, and amiloid-like material) that allow its histopathological recognition.We present a clinicopathological analysis of a case of follicular AOT affecting the mandible in a 9 years-old female patient associated with unerupted lower left canine. Immunohistochemical study showed some data previously unrecognised.All cellular types that composed AOT showed nuclear positivity for p63 indicating a basal characterization in the different cellular components. According to its benign character and low potential for recurrence, AOT revealed a scant proliferative activity (2-3% nuclei showed Ki-67 positivity) limited to some epithelial nodules (AE1-3 +) of fusiform appearance. Absence of reactivity for hormonal receptors (RE and RPg) excluded a possible hormonodependence in AOT that could explain the observed female predominance

Características citopatológicas del carcinoma de células acinares (CCA) de glándula salival. A propósito de cuatro observaciones / Cytological characteristics of acinic cell carcinoma (ACC) diagnosed by fi ne-needle aspiration biopsy (FNAB). A study of four cases

Objetivo: Presentar las características citopatológicas del carcinomaacinar (CCA), así como su correlación cito-histológica, comentando los problemas de diagnóstico diferencial de esta entidad en base a cuatro observaciones estudiadas mediante PAAF.Casos clínicos: Dos varones de 52 y 53 años, una mujer de 79 años, y una niña de 12 años que presentaron tumoraciones localizadas en territorio parotídeo (casos 1, 2 y 4), y a nivel laterocervical (caso 3). En tres pacientes, la PAAF fue, junto a los estudios de imagen,la primera aproximación diagnóstica; correspondiendo el caso 3 a una punción de adenopatías laterocervicales metastásicas en una paciente con antecedentes de CCA de parótida. Hallazgos citológicos: Las extensiones citológicas mostraron abundante celularidad tumoral dispuesta en pequeñas placas monocapa, formando estructuras acinares, o como células aisladas. Es de destacar la abundancia de núcleos desnudos en el fondo de los frotis, y la ausencia de grasa y de epitelio ductal. Las células poseían núcleos monomorfos redondeados u ovales, nucleólo poco evidente y abundante citoplasma granular ofinamente vacuolado. Discusión: La PAAF proporciona información esencial en la actuación diagnóstico-terapeútica de las tumoraciones de laglándula salival, resultando esta metodología muy sensibleen su eficacia diagnóstica. La identificación de los CCA frecuentemente presenta dificultades, debido a la gran similitud citológica de las células tumorales con el componente acinar normal propio de la glándula salival. El diagnóstico diferencial se plantea, fundamentalmente, con carcinomas de células claras,con carcinomas mucoepidermoides, con el tumor de Warthiny con los oncocitomas. Nuestras observaciones confirman la validez de la PAAF en una primera aproximación diagnóstica de estas lesiones tumorales accesibles a la punción directa

Objective: To present the cytopathological characteristics of a cinic cell carcinoma (ACC) as well as its cyto-histological correlation, commenting on the differential diagnostic problems of this entity based on four observations studied using fine-needle aspiration biopsy (FNAB).Clinical Cases: Two males of 52 and 53 years of age, one 79 year-old woman and a girl of 12 years of age, who presented tumors located in the parotid area (cases 1, 2 and 4) and at the laterocervical level (case 3).In 3 patients, the FNAB was, together with the image studies,the first diagnostic indication; case 3 corresponding to a puncture of metastatic laterocervical adenopathies in a patient with a history of parotid ACC. Cytological findings: The cytologic smears revealed abundant tumoral cellularity arranged in small monolayered sheets,forming acinar structures or isolated cells. The abundance of bare nuclei at the background of the smears, and the absence of adipose tissue and ductal epithelium are highlighted. The cells possessed round or oval monomorphic nuclei, few nucleoli and abundant granular or finely vacuolate cytoplasm. Discussion: FNAB provides essential information on the diagnostic-therapeutic management of salivary gland tumors; this methodology is highly sensitive in its diagnostic efficacy. The diagnosis of ACCs frequently presents difficulties, owing to the great cytologic similarity of the tumor cells with the normal acinar component of the salivary gland. The differential diagnosis is considered, fundamentally, with clear cell carcinomas, mucoepidermoid carcinomas, Warthin`s tumor, and oncocytomas. Our observations confirm the validity of FNAB in a first diagnostic approximation for those lesions accessible to direct puncture

Cytological characteristics of acinic cell carcinoma (ACC) diagnosed by fine-needle aspiration biopsy (FNAB). A study of four cases.

OBJECTIVE: To present the cytopathological characteristics of acinic cell carcinoma (ACC) as well as its cyto-histological correlation, commenting on the differential diagnostic problems of this entity based on four observations studied using fine-needle aspiration biopsy (FNAB). CLINICAL CASES: Two males of 52 and 53 years of age, one 79 year-old woman and a girl of 12 years of age, who presented tumors located in the parotid area (cases 1, 2 and 4) and at the laterocervical level (case 3). In 3 patients, the FNAB was, together with the image studies, the first diagnostic indication; case 3 corresponding to a puncture of metastatic laterocervical adenopathies in a patient with a history of parotid ACC. CYTOLOGICAL FINDINGS: The cytologic smears revealed abundant tumoral cellularity arranged in small monolayered sheets, forming acinar structures or isolated cells. The abundance of bare nuclei at the background of the smears, and the absence of adipose tissue and ductal epithelium are highlighted. The cells possessed round or oval monomorphic nuclei, few nucleoli and abundant granular or finely vacuolate cytoplasm. DISCUSSION: FNAB provides essential information on the diagnostic-therapeutic management of salivary gland tumors; this methodology is highly sensitive in its diagnostic efficacy. The diagnosis of ACCs frequently presents difficulties, owing to the great cytologic similarity of the tumor cells with the normal acinar component of the salivary gland. The differential diagnosis is considered, fundamentally, with clear cell carcinomas, mucoepidermoid carcinomas, Warthin s tumor, and oncocytomas. Our observations confirm the validity of FNAB in a first diagnostic approximation for those lesions accessible to direct puncture.