Type I pleuropulmonary blastoma presenting as congenital pulmonary airway malformation: A report of two cases

Pleuropulmonary blastoma (PPB) is a rare aggressive intrathoracic tumor which is believed to originate from embryonic uncommitted lung mesenchymal cells, which are important for developing the lung. Type I PPB is cystic, type II is cystic and solid, while type III is predominantly solid. Diagnosing type 1 PPB is a challenge for both radiologists as well as pathologists. Owing to its purely cystic nature, type I PPB it is often mistaken for unrelated entities such as congenital pulmonary airway malformation and congenital lobar emphysema which delays surgical intervention. Here, we report two such cases presenting clinically and radiologically as congenital pulmonary airway malformation. On histology, a final diagnosis of type I pleuropulmonay blastoma was made. Thereafter, chemotherapy was administered following complete surgical excision.

Frecuencia, tipo y predictores de complicaciones pleuropulmonares en los primeros treinta días del postoperatorio de pacientes pediátricos intervenidos de cirugía cardiovascular sin apoyo de circulación extracorpórea / Frequency and type of pleuropulmonary complications and their predictors during the first thirty days after cardiopulmonary bypass surgery in children

Resumen Objetivo: Determinar la frecuencia, tipo y predictores de complicaciones pleuropulmonares en los primeros 30 días de postoperatorio de pacientes intervenidos de cirugía cardiovascular sin apoyo de circulación extracorpórea. Métodos: Se realizó un estudio de cohorte retrospectivo durante el periodo comprendido del 1 de enero de 2013 al 31 de diciembre 2014. Incluyó a todos los pacientes portadores de cardiopatías congénitas intervenidos de cirugía cardiaca con abordaje esternal o torácico, sin soporte de circulación extracorpórea con ingreso registrado a Unidad de Cuidados Intensivos del Hospital de Pediatría del Centro Médico Nacional Siglo XXI, IMSS. Se cuantificó la frecuencia de eventos de las complicaciones pleuropulmonares y se realizó un análisis multivariado de regresión logística para identificar los factores de riesgo asociados a complicaciones pleuropulmonares, calculándose odds ratio (OR) e intervalos de confianza al 95% (IC 95%). Resultados: Se incluyeron un total de 139 pacientes, en los cuales la frecuencia de complicaciones pleuropulmonares fue del 42.4% (n = 59), y los tipos más frecuentes fueron atelectasia (28 eventos), neumonía asociada a ventilador (24 eventos), neumotórax (20 eventos), pudiéndose encontrar más de una complicación por paciente. Los predictores más significativos de complicaciones pleuropulmonares fueron las cardiopatías congénitas cianógenas (OR = 3.58; IC 95%: 1.10-7.50; p = 0.001), el abordaje por toracotomía (OR = 1.46; IC 95%: 1.18-1.12; p = 0.008) y el evento quirúrgico realizado de urgencia (OR = 3.46; IC 95%: 1.51-7.95; p = 0.002). Conclusiones: La principal complicación pleuropulmonar fue la atelectasia lo cual concuerda con lo reportado en la literatura internacional. Los pacientes que presenten alguno de los predictores identificados en el presente estudio deben ser monitorizados de manera especial para prevenir, detectar y/o tratar oportunamente las complicaciones pleuropulmonares tras cirugía cardiaca.

Abstract Objective: To determine the frequency and type of pleuropulmonary complications and their predictors in the first thirty postoperative days of patients undergoing surgery without cardiopulmonary bypass. Methods: A retrospective cohort study was carried out between January 2013 and December 2014. It included all patients with congenital heart disease who underwent cardiac surgery using a sternal or thoracic approach, without cardiopulmonary bypass with a registered admission to a Neonatal or Paediatric Intensive Care. The frequency of events of pleuropulmonary complications and logistic regression analysis was performed, and the adjusted odds ratio (OR) and confidence intervals at 95% (95% CI) were calculated. Results: A total of 139 patients were included. The frequency of pleuropulmonary complications was 42.4% (N = 59), and the most frequent types were atelectasis (28 events), ventilator-associated pneumonia (24 events), pneumothorax (20 events), with more than one complication per patient occasionally being found. Significant risk factors were cyanogenic congenital heart disease (OR = 3.58, 95% CI: 1.10-7.50, P =.001), thoracotomy approach (OR = 1.46, 95% CI: 1.18-1.12, P = .008), and an emergency surgical event (OR = 3.46, 95% CI: 1.51-7.95, P = .002). Conclusions: The main pleuropulmonary complication was atelectasis, which is consistent with that reported in the international literature. Patients with any of the predictors identified in the present study should be closely monitored in order to prevent, detect and/or treat pleuropulmonary complications in a timely manner after cardiac surgery.

Blastoma pleuropulmonar: reporte de un caso / Pleuropulmonary blastoma: case report

El blastoma pleuropulmonar corresponde a una neoplasia primaria de pulmón, exclusiva de la edad pediátrica, poco frecuente y de características agresivas. La edad de diagnóstico varía entre 1 mes y 12 años. No se identifica una distinción de sexos. Se localiza en la pleura o el pulmón. Suele presentarse como dificultad respiratoria, con o sin neumotórax, dolor torácico y fiebre, por lo que es mal diagnosticada como neumonía. Radiológicamente, suele hallarse una masa de gran tamaño cercana a la pleura en la base pulmonar derecha sin broncograma aéreo. Cuenta con tres tipos histológicos: tipo I (quístico), tipo II (mixto), tipo III (sólido). Su tratamiento incluye resección y quimioterapia multimodal. El tipo I tiene un pronóstico favorable, pero los tipos II y III tienen mal pronóstico. Se presenta un caso de blastoma pleuropulmonar en una niña de 2 años, con énfasis en su diagnóstico clínico-radiológico.

Pleuropulmonary Blastoma corresponds to a malignant primary lung disorder, exclusive of pediatric age, infrequent and of aggressive characteristics. Age on diagnosis is 1 month-12 years. Rates per sex are equal. It can be found inside pleura or lungs. Respiratory distress associated or not with pneumothorax, chest pain and fever are classical clinical signs. These symptoms could be misdiagnosed as pneumonia. Radiologically, a large mass near the pleura at the base of the right lung without air bronchogram is its most common form. It has three histological types: type I (cystic), type II (mixed) and type III (solid). Its treatment requires tumor excision and multimodal chemotherapy. Pleuropulmonary Blastoma type I has good prognosis; type II and III variants have lower survival. We report a 2-year-old girl, pointing to the clinical-radiological diagnosis.

Deformación de la pared torácica como presentación de un blastoma pleuropulmonar, caso clínico / Pleuro-pulmonary blastoma presenting as a chest wall deformity: a case report

INTRODUCCIÓN: El blastema pleuropulmonar (BPP) es la neoplasia primaria maligna más común de los pulmones en la infancia. Se presenta con mayor frecuencia en niños entre 1 y 4 años, con sínto mas respiratorios. Han sido definidos tres tipos por histopatología (tipo I, II y III), relacionados con sobrevida y pronóstico. OBJETIVO: Reportar el primer caso de un paciente con un BPP que se presentó como una deformación de la pared torácica. CASO CLÍNICO: Paciente de un año de vida que se presentó con una deformidad de la pared torácica a los 10 meses de edad. El estudio imagenológico reveló una gran masa quística en el hemitórax derecho. No desarrolló sintomatología respiratoria hasta el ingreso hospitalario. Se realizó una lobectomía superior derecha y la biopsia confirmó un BPP tipo I. Se trató con resección quirúrgica y realización periódica de imágenes torácicas como seguimiento. CONCLUSIONES: Se reporta el caso de un BPP que se manifestó con deformidad torácica, forma de presentación no descrita previamente en la literatura. El BPP es un cáncer poco frecuente que debe ser considerado en el diagnóstico diferencial de lesiones quísticas pulmonares, sobre todo en la edad pediátrica. Reconocerlo como un tumor maligno en vez de una anomalía del desarrollo, es determinante para que el paciente sea sometido a resección quirúrgica, terapia adyuvante y seguimiento apropiado.

INTRODUCTION: Pleuropulmonary blastema (PPB) is the most common primary malignancy of the lungs in childhood. It occurs more frequently in children between one and four years of age, and respiratory symptoms are a common manifestation. Three types have been defined (type I, II and III), which are related to survival and prognosis. OBJECTIVE: To report the first case of a patient with a PPB who presented with a chest wall deformity. CASE REPORT: One year old male patient who had a chest wall deformity at ten months of age. Imaging revealed a giant cyst in the right hemithorax. He did not develop respiratory symptoms until hospital admission. A right upper lobectomy was perfor med and the biopsy confirmed a type I pleuropulmonary blastoma. He was considered successfully treated with complete surgical excision and routine follow-up with thoracic imaging is conducted. CONCLUSIONS: PPB is a very rare cancer that needs to be considered in the differential diagnosis of cystic lung diseases in children. The recognition of this lesion as a malignant tumour rather than a developmental cystic malformation is vital so the child can receive complete excision and appropriate follow-up care.

Malformación adenomatoidea quística y riesgo de tumores malignos pulmonares / Adenomatoid lung malformation and malignant tumors

Cystic adenomatoid malformation is the most frequent congenital pulmonary malformation. The usual treatment is surgical resection. However there is controversy over management in asymptomatic patients. The possible malignization would justify surgery of cystic lesions. Relation with pleuropulmonary blastoma has been described, however it is not clear whether this is a primary tumor or cyst malignization. Cystic adenomatoid malformation also has association with adenocarcinoma and rhabdomyosarcoma. Currently available evidence suggests surgical resection, despite the natural course of congenital lung cystic lesions is uncertain

La malformación adenomatoidea quística (MAQ) es la anomalía del desarrollo pulmonar más frecuente. El tratamiento habitual es la resección quirúrgica, no obstante existe controversia sobre el manejo en pacientes asintomáticos. La posible malignización de las lesiones quísticas es uno de los argumentos que justifican la cirugía en estos pacientes. Se ha descrito relación con blastoma pleuropulmonar, sin embargo no está claro si se trataría de una lesión quística que se maligniza o es una entidad diferente. También hay asociación con adenocarcinoma y rabdomiosarcoma . Actualmente se sugiere la resección quirúrgica como el tratamiento más adecuado, sin embargo la evolución natural de las lesiones quísticas pulmonares congénitas es incierta

Clinical analysis of seven cases of pulmonary malignant tumors in children / 中华儿科杂志

Objective@#To understand the clinical manifestation, imaging characteristics and outcomes of pulmonary malignant tumors in children.@*Method@#We retrospectively collected information about seven cases of pulmonary malignant tumors in children in our hospital from Jan 2010 to Dec 2016. The information included clinical manifestation, imaging characteristics, pathologic results, and treatment.@*Result@#(1) All the seven patients firstly visited pediatric internal medicine departments. Symptoms included cough (n=6), dyspnea (n=4), fever (n=2), anorexia (n=2), chest tightness (n=1), chest pain (n=1), lameness (n=1), abdominal distension and constipation (n=1). We did not find hemoptysis, wheezing or weight loss in those patients. Physical examinations revealed unilateral reduced breath sounds (n=5), moist rales and wheezes (n=1), and normal (n=1). Extrapulmonary signs included abdominal distension (n=2), left hip tenderness (n=1), and mass in left scrotum. We did not identify clubbing finger, anemic appearance, lymph node enlargement, or hepatosplenomegaly etc. (2) Laboratory examination results: complete blood count showed white blood cells in normal range except one case (17.44×109/L). Neutrophil percentage ranged from 0.348 to 0.767. C reactive protein ranged between<1 and 162 mg/L. Hemoglobin was normal. Three out of four cases had abnormal blood tumor markers.(3) Imaging results showed multiple nodes (n=3), multiple cystic lesions in lungs (n=2) (both with pleuropulmonary blastoma), endobronchial soft tissue mass (n=1), pulmonary round-shaped mass (n=1), and mediastinal mass (n=1). Imaging results also found atelectasis (n=3), pneumonia (n=2), pneumothorax (n=2), longitudinal diaphragmatic hernia (n=2), pleural effusion (n=1), subcutaneous emphysema (n=1). (4) All the patients underwent tumor puncture biopsy or tumor resection. Pathology revealed the final diagnosis of pleuropulmonary blastoma (n=3), endodermal sinus tumors (n=2), squamous cell carcinoma of lung (n=1), and thyroid papillary carcinoma (n=1). All of them were malignant tumors. We followed up them. Two patients died (both with pleuropulmonary blastoma) after their parents refused any medical help. Two cases were lost to follow-up. Three patients survived (followed up for 19 months, 11 months and tow months, respectively). One case with thyroid papillary carcinoma pulmonary metastasis underwent right thyroid cancer radical plus left thyroid lobe resection plus modified selective central lymph node dissection, then iodine 131 treatment. One case with endodermal sinus tumor pulmonary metastasis underwent three times of chemotherapies, resection of left retroperitoneal tumor and left testicular tumor, and six additional chemotherapies. Another one case of endodermal sinus tumor pulmonary metastasis underwent three times of chemotherapies and was discharged.@*Conclusion@#Pulmonary malignant tumors are rare diseases in children. Clinical signs are often non-specific. For those with chest CT showing multiple cystic lesions, endobronchial soft tissue mass or multiple nodes, but no significant infection manifestation or no response to anti-infection therapy, pulmonary malignant tumors should be considered. Biopsy may be needed to confirm the final diagnosis.

Pleuropulmonary nocardiosis due to Nocardia otitidiscaviarum in a debilitated host.

Nocardia otitidiscaviarum is a rare cause of pulmonary nocardiosis. We present a case of pulmonary nocardiosis with pleural involvement in an adolescent with rheumatic heart disease and congestive cardiac failure presenting with right lower lobe consolidation and pleural effusion. Direct gram‑stain of pleural fluid showed pus cells with Gram‑positive filamentous branching bacilli. Empiric treatment with parenteral ceftriaxone and supportive therapy for cardiac failure was initiated. Pleural fluid culture yielded growth of N. otitidiscaviarum at 72 h. Antibiotic susceptibility testing showed resistance to cephalosporins. The patient expired due to congestive cardiac failure on the 5th day.

Blastoma pleuropulmonar: caso clínico / Pleuropulmonary blastoma: case report

El blastoma pleuropulmonar es un tumor pulmonar raro de la infancia, que puede manifestarse con lesiones quísticas o sólidas, como un hallazgo radiológico o con clínica respiratoria. Presentamos el caso de un niño de 2 años de edad que consultó en su primer cuadro obstructivo con imagen sospechosa de malformación pulmonar en el lóbulo superior izquierdo en la radiografía y la tomografía de tórax. Se realizó cirugía, que evidenció una malformación quistica en el segmento ápico posterior del lóbulo superior izquierdo. Recibimos el informe de anatomía patológica con diagnóstico de blastoma pleuropulmonar tipo I. Comenzó el seguimiento por Oncología e inició el tratamiento con ciclofosfamida y vincristina, con buena tolerancia.

Pleuropulmonary blastoma is a rare lung tumor of childhood that can occur with cystic or solid lesions, as a radiological finding with or without respiratory symptoms. We report the case of a 2 year old toddler in his first pulmonary obstructive episode with suspected toracic malformation of the left upper lobe in his chest x-ray and tomography. Surgery was performed showing cystic malformation of the left upper lobe. We received the pathology report with diagnosis of type I pleuropulmonary blastoma. He began follow-up with Oncology initiating treatment with cyclophosphamide and vincristine, well tolerated.

Pleuropulmonary blastoma：one case report / 临床儿科杂志

ObjectiveTo investigate the early diagnosis and treatment of pleuropulmonary blastoma in children. Meth-ods The progress of pleuropulmonary blastoma from type 1 to type 3 was retrospectively analyzed.Results The male infant had no obvious abnormality in chest X-ray at 18 days. At one year old, X-ray iflm of the chest showed cystic lesions near hilum of the left lung (about 5 cm × 4 cm × 4 cm). The congenital cystic adenomatoid malformation was considered and an operation resection was suggested. However, it had been rejected by his parents. At 3 years and 2 months old, chest X-ray and CT showed left lung had a solid cystic lesion, the volume was signiifcantly enlarged, the back side was solid and the upper part had a large gas cavity. Two months later, the patient had asthmatic suffocation. The chest CT showed the tumor was completely solid. Pleuropulmonary blastoma type 3 was considered. The patient received the conventional chemotherapy after operation excision, and was followed up for 1 year. No recurrence was observed after the treatment.Conclusions Type 1 pleuropulmonary blastoma and congenital cystic adenomatoid malformation are hard to be differentially diagnosed. Early diagnosis and treatment of type 1 pleuropulmo-nary blastoma can improve the prognosis.

Pleural fluid characteristics of pleuropulmonary paragonimiasis masquerading as pleural tuberculosis

BACKGROUND/AIMS: Pleuropulmonary paragonimiasis produces no specific symptoms or radiologic findings, allowing for the possibility of misdiagnosis. We evaluated the specific clinical and pleural fluid features of pleuropulmonary paragonimiasis masquerading as pleural tuberculosis. METHODS: We retrospectively analyzed the clinical and radiologic characteristics of 20 patients diagnosed with pleuropulmonary paragonimiasis between 2001 and 2011. RESULTS: In total, 17 patients presented with respiratory symptoms, including dyspnea (30%), hemoptysis (20%), cough (20%), and pleuritic chest pain (15%). Chest radiographs revealed intrapulmonary parenchymal lesions, including air-space consolidation (30%), nodular opacities (20%), cystic lesions (15%), ground-glass opacities (10%), and pneumothorax (5%). A pleural f luid examination revealed eosinophilia, low glucose levels, and high lactate dehydrogenase (LDH) levels in 87%, 76%, and 88% of the patients, respectively. These traits helped to distinguish pleuropulmonary paragonimiasis from other pleural diseases such as parapneumonic effusion, malignancy, and pleural tuberculosis. CONCLUSIONS: Pleuropulmonary paragonimiasis is often initially misdiagnosed as other pleural diseases. Therefore, it is important to establish the correct diagnosis. In patients with unexplained pleural effusion living in paragonimiasis-endemic areas, pleural fluid obtained by thoracentesis should be examined to distinguish pleuropulmonary paragonimiasis. When marked eosinophilia, high LDH levels, and low glucose levels are identified in pleural fluid, physicians could consider a diagnosis of pleuropulmonary paragonimiasis.

Características clínicas y epidemiológicas de la tuberculosis en niños hospitalizados en el servicio de Pediatría del Hospital Hipólito Unanue. 2005-2012 / Clinical and epidemiological characteristics of tuberculosis in children hospitalized in the Pediatric Service at the Hospital Hipolito Unanue. 2005-2012

La tuberculosis es un problema de salud pública y la forma de presentación clínica en niños presenta características diferentes al adulto. Objetivo: Determinar las características clínicas y epidemiológicas de tuberculosis infantil en pacientes hospitalizados en el Departamento de Pediatría del Hospital Nacional Hipólito Unanue (DP-HNHU) durante los años 2005 al 2012. Materiales y Métodos: Estudio retrospectivo de revisión de casos de pacientes menores de 15 años con diagnóstico de tuberculosis atendidos en el DP-HNHU. Se realizó análisis univariado y bivariado presentados en tablas y gráficos. Resultados: Se analizaron 113 casos, donde 65 por ciento fueron varones. El grupo de 10 a 14 años representó 69.9 por ciento. El estado nutricional normal se observó en 77 por ciento. El 43.7 por ciento de pacientes presentó contacto intradomiciliario. Síntomas más frecuentes fueron tos (78.7 por ciento), fiebre (73.7 por ciento), hiporexia (46.9 por ciento) y diaforesis (40.7 por ciento). Signos más frecuentes: disminución de murmullo vesicular (61.1 por ciento), egofonía (33.7 por ciento), disminución de vibraciones vocales (20.3 por ciento) y presencia de crepitantes (20.3 por ciento). Muestras de esputo y líquido pleural fueron las muestras más frecuentes. La proporción de baciloscopías positivas fue 21 por ciento, donde el cultivo estuvo disponible en 6 casos (5.3 por ciento); con 4 positivos (3.54 por ciento), y un aislamiento de TBC-MDR. Efusión pleural (46 por ciento), infiltrado alveolar (45 por ciento), infiltrados retículo-nodulares (31.8 por ciento), y engrosamiento hiliar (26.5 por ciento) fueron frecuentes. Tuberculosis pleuropulmonar (40.7 por ciento), tuberculosis pulmonar (34.5 por ciento) y tuberculosis sistémica 9.7 por ciento fueron más frecuentes. El PPD fue positivo en 83.2 por ciento, y más frecuente a mayor edad y en niños eutróficos. Conclusiones: La presentación clínica más frecuente es tuberculosis pleuropulmonar, afectando...

Tuberculosis is a public health problem and the clinical presentation in adult children has different characteristics. Objective: To determine the clinical and epidemiological characteristics of childhood tuberculosis in patients hospitalized in the Department of Pediatrics of the National Hospital Hipolito Unanue (DP-HNHU) during the years 2005 to 2012. Materials and Methods: Retrospective case review of patients younger than 15 years diagnosed with tuberculosis and treated at the DP-HNHU. Univariate and bivariate analysis was performed; all data is presented in tables and graphs. Results: 113 cases were analyzed; 65 per cent were male. The group of 10 to 14 years accounted for 69.9 per cent. Normal nutritional status was observed in 77 per cent. 43.7 per cent of patients had intradomiciliary contact. Most common symptoms were cough (78.7 per cent), fever (73.7 per cent), decreased appetite (46.9 per cent) and sweating (40.7 per cent). Most common signs: decreased breath sounds (61.1 per cent), egophony (33.7 per cent), decreased vocal vibrations (20.3 per cent) and crackles (20.3 per cent). Sputum and pleural fluid samples were the most frequent. The proportion of positive smears was 21 per cent, where the culture was available in 6 cases (5.3 per cent); with 4 positive (3.54 per cent), and one isolate of MDR-TB. Pleural effusion (46 per cent), alveolar infiltrates (45 per cent), infiltrated reticulo-nodular (31.8 per cent) and hilar thickening (26.5 per cent) were common. Pleuropulmonary tuberculosis (40.7 per cent), pulmonary tuberculosis (34.5 per cent) and 9.7 per cent systemic tuberculosis were more frequent. The PPD was positive in 83.2 per cent, and more frequent with increasing age and eutrophic children. Conclusions: The most common clinical presentation is pleuropulmonary tuberculosis, mainly affecting the age group 10 to 14 years, eutrophic with cardinal symptoms and history of intradomiciliary contact...

Anaerobes in nosocomial and community acquired pleural infections.

Anaerobes are important causes of pleural space infections. The aim of the study is to evaluate the role of the anaerobic bacteria in pleural infections. The study involved 278 consecutive clinical samples sent to the Clinical Microbiology Laboratory of Tertiary Chest Hospital. Anaerobes were isolated in 39 community acquired and fi ve nosocomial cases out of 278 anaerobic cultivations (15.8%). Total of 56 anaerobe strains were identifi ed and 21 aerobes were accompanied to anaerobic isolates. Aerobe isolates were associated with anaerobic microorganisms in 19 cases (43.2%). Bacteroides species (21.4%) and Pseudomonas aeruginosa (33.3%) were the most common anaerobic and aerobic isolates.

An clinicopathological analysis and literature review of pleuropulmonary blastoma in an adult / 重庆医学

Objective To explore the clinicopathological features ,diagnosis and differential diagnosis of pleuropulmonary blasto-ma(PPB) .Methods A case of PPB was reported by light microscopic observation ,immunohistochemistry and molecular pathology study with review of related literature .Results A 45-year-old female was admitted to the hospital because of cough and dyspnea . Chest radiogram revealed a solid mass in the left lung .Grossly ,the tumor was described as a firm lesion with lumina or multicystic components and well-circumscribed margins .Microscopically ,the tumor was composed of sheets of malignant primitive small cells and fascicles of embryonal rhabdomyosarcoma-like cells with foci hyalinized stroma .Beneath the benign epithelium ,the primitive mesenchymal cells showed as mixed blastematous and sarcomatous characteristics that plump spindle shaped cells presented poor differention with abundant eosinophilic cytoplasms and brisk mitotic activities .Immunohistochemically ,vimentin and MyoD-1 were positive in malignant small cells but some epithelial markers are negative .Meanwhile ,K-RAS extron 3 mutation was detected by high resolution melting analysis(HRMA) .Conclusion Pleuropulmonary blastoma(PPB) is a rare malignant tumor with unique clinicopathological features .It should be distinguished from some mimics such as pulmonary blastoma and embryonal rhabdomyo-sarcoma .

Recién nacido con masa pulmonar de etiología infrecuente / Newborn whit lung mass of uncommon etiology

En neonatología, las masas torácicas generalmente responden a anomalías congénitas del aparato respiratorio. Comprenden un extenso número de patologías que pueden comprometer el desarrollo de laringe, tráquea, bronquios, parénquima pulmonar, diafragma o pared torácica. El diagnóstico, en la mayoría de los casos, se efectúa en el período prenatal mediante ecografía. El resto se diagnostica por la clínica, difcultad respiratoria en el recién nacido, o es un hallazgo radiológico en el transcurso de la vida. Presentamos el caso de un recién nacido de término con diagnóstico prenatal de "masa pulmonar" con imágenes quísticas en su interior. Al examen físico solo presentó disminución de la entrada de aire en base derecha. Se realizaron diferentes estudios por imágenes para caracterizar mejor la lesión y estudiar su extensión. Al quinto mes de vida, el paciente fue sometido a tratamiento quirúrgico y quimioterápico. El estudio anatomopatológico de la pieza quirúrgica arrojó como diagnóstico "blastoma pleuropulmonar" (BPP), tumor intratorácico maligno, muy poco frecuente, que aparece casi exclusivamente en niños menores de 7 años.

Thoracic masses in neonates usually respond to congenital anomalies of the respiratory system. They comprise a large number of diseases that can compromise the development of larynx, trachea, bronchi, pulmonary parenchyma and diaphragm or chest wall. Diagnosis is carried out during prenatal period by ultrasound in most cases. In others, respiratory distress is diagnosed during post-birth examination or later as a radiological fnding. We present the case of a full term newborn with prenatal diagnosis of cystic "lung mass". Physical examination was unremarkable except for decreased breath sounds on the right lung. Different image studies were carried out to characterize the lesion. The patient underwent surgery and chemotherapy at ffth month of life. Pleuropulmonary blastoma diagnosis was confrmed by pathological study of the surgical specimen. This is a rare intrathoracic malignant tumor, appearing almost exclusively in children less than 7-years-old.

Pleuropulmonary Blastoma With Cervical Lymph Node Metastasis at Presentation.

Pleuropulmonary blastoma is a rare childhood neoplasm accounting for less than one percent of all primary malignant lung tumors of children less than six years of age. Metastasis to CNS, orbit and iris, bone, contralateral lung and rarely adrenal glands, liver, kidney and pancreas has been described. This report presents a rare case of pleuropulmonary blastoma with cervical lymph node metastasis at the time of presentation.

Blastoma pleuropulmonar: reporte de caso y revisión de la literatura / Pleuropulmonary blastoma: case report and review of the literature

The pleuropulmonary blastoma is an aggressive primary lung tumor. Is most frequent in paediatric population, and there are a few cases reported worldwide. It consists of embrionary primitive mesenquimal tissue, and is different of the adult Pulmonary Blastoma. The clinical presentation can be missed by other prevalent diseases or may be an accidental diagnosis. The outcome following diagnosis is poor, overall for types ii and iii, with bad response to surgery and quimiotherapy, high rates or recurrence to more aggressive forms (eg. BPP type i to type ii o iii). This report describes the clinical picture of a two years old preschool child with aggressive BPP. We reviewed the actual literature about this topic.

El blastoma pleuropulmonar (BPP), es un tumor agresivo primario de pulmón. Afecta sobre todo en la edad pediátrica, habiendo sido reportado pocos casos a nivel mundial. El BPP consiste de tejido mesenquimal primitivo embrionario, de características diferentes al blastoma pulmonar del adulto. La presentación clínica suele confundirse con otras patologías frecuentes o puede ser incidental. La sobrevida luego del diagnóstico es pobre, sobre todo para los tipos ii y iii, con poca respuesta a la quimioterapia- cirugía, y alta frecuencia de recaídas a formas más agresivas. Se describe el caso de una pre-escolar de 2 años, con diagnóstico de BPP, que presentó una evolución clínica agresiva, se realizó la revisión de la literatura sobre los principales tópicos concernientes a esta patología.

Primary pleuro-pulmonary synovial sarcoma.

Primary pleuro-pulmonary synovial sarcoma (PPSS) is a rare tumour and poses a diagnostic challenge particularly when unusual histological features are present. We report a case of a 30-year-old immunocompromised human immunodeficiency virus (HIV) sero-positive male who was referred to us with complaints of cough, breathlessness and left-sided chest pain for the past two months. The PPSS can be confirmed on tru-cut biopsy.

Pleuropulmonary blastoma in an infant.

Pleuropulmonary blastomas (PPB) are rare and highly aggressive tumors. Herein, we report an infantile case of type III PPB. A 9-mth-old boy presented to our unit with a history of cough and tachypnea for 2 days. Chest computed tomography revealed a mass in the left upper lobe, emphysema in the left upper lung, and mediastinum and heart shifted towards the right. The mass was removed completely by left upper lobectomy and histology confirmed diagnosis of type III PPB. The immature blastematous tissue was positive for vimentin while benign epithelium was positive for epithelial membrane antigen and cytokeratin. No lymph nodule metastasis was found in the 7 lymph nodules obtained from the hilum of the lung near the tumor. Currently, the patient is under close follow-up and is doing well.

Historia de laluucha antituberculosa / History ofanti ttuberculosis fihtt

La tuberculosis es una de las enfermedades más terribles que afectan al hombre; su antigüedad se estima entre 15 000 a 20 000 años. La historia de la lucha antituberculosa se inicia con el surgimiento y desarrollo de la civilización, vinculada a los conocimientos que sobre la enfermedad existían en cada época especifica de la evolución humana. Un sinnúmero de rituales, remedios y fármacos se han empleado a lo largo de la historia en aras de erradicar este peligroso flagelo; en la actualidad numerosas investigaciones y cuantiosos recursos se dedican a la búsqueda de nuevos medicamentos y vacunas con posibilidades terapéuticas futuras, pues aún, en los inicios del siglo XXI, el Mycobaterium tuberculosis sigue causando la muerte de millones de personas y enfrentando nuevos retos como TB/VIH & MDR-TB.

Tuberculosis is one of the most terrible diseases that affect man, it is known since 20 000 years. The history of the fight against tuberculosis initiates since the beginning and development of civilization, linked to the knowledge of the disease in every age period of human evolution. An uncountable amount of rites, remedies, and medicines have been employed along the history to eradicate this disease. Currently, numerous resources and investigations are dedicated to the search for new medications and vaccines with therapeutic possibilities, because even now, at the beginning of the 21st Century, Mycobacterium Tuberculosis is still causing death to millions of people and facing new challenges such as TB/HIV & MDR-TB.