Rabdomiosarcoma mediastínico diagnosticado mediante punción aspiración guiada por ecografía endobronquial / Mediastinal rhabdomyosarcoma diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration

El rabdomiosarcoma es una neoplasia mesenquimal maligna y, además, es la segunda neoplasia cardiaca después del angiosarcoma, y su incidencia global es inferior al 0,07%. Otras denominaciones que se le han dado son rabdomioblastoma, miosarcoma y rabdomioma maligno. Habitualmente la clínica de presentación es inespecífica, y el diagnóstico se alcanza con el estudio histológico e inmunohistoquímico de la lesión obtenida mediante biopsia intracavitaria o bien abierta. Presentamos el caso de un paciente de 35 años, con clínica de disnea y alteraciones electrocardiográficas al que se le diagnostica de un rabdomiosarcoma mediante una ultrasonografía endobronquial (EBUS). Revisada la bibliografía existente este es el primer caso descrito de diagnóstico de rabdomiosarcoma mediante la punción de una adenopatía mediastínica por EBUS (AU)

Rhabdomyosarcoma is a malignant mesenchymal neoplasm with an incidence of less than than 0.07%, although it is the second most frequent cardiac neoplasia after cardiac angiosarcoma. It has also been named rhabdomyoblastoma, myosarcoma and malignant rhabdomyoma. Its clinical presentation is usually non-specific and histological and immunohistochemical studies of the lesion obtained by intracavitary or open biopsy are required for diagnosis. We report a case of a 35 year old patient, with dyspnea and electrocardiographic changes, diagnosed with rhabdomyosarcoma by endobronchial ultrasound (EBUS). To our knowledge, this is the first case of a rhabdomyosarcoma diagnosed by puncturing a mediastinal adenopathy using EBUS (AU)

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

El descubrimiento de los cambios moleculares de los adenomas ha dado lugar a un renovado interés en este tipo de tumor. En la última edición de la OMS de los tumores del tracto gastrointestinal (2010) se incluyen 4 tipos de adenomas hepáticos, bien caracterizados inmunohistoquímicamente, genotípicamente y fenotípicamente, en los que tienen un papel importante los antecedentes clínicos y su comportamiento morfológico para determinar el posterior riesgo de malignidad, fundamentalmente en aquellos con mutación de la b-catenina. La presencia de esteatosis, inflamación y cambios vasculares, unidos a la respuesta frente a la FABP, el amiloide sérico A y la glutamina sintetasa nos permite clasificarlos en 4 grupos: con mutación de HNF1A (H-HCA), con mutación de b-catenina (b-HCA), inflamatorios (IHCA) y sin marcadores. La ausencia de expresión frente al glypican 3, el HSP 70 y el mapeo perivenular frente a la glutamina sintetasa ayuda a excluirlos frente a los hepatocarcinomas bien diferenciados. En este trabajo describimos el comportamiento clínico, morfológico e inmunofenotípico de 3 casos de pacientes diagnosticados de adenomas hepáticos en un período de 2 años (AU)

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 (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.

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.