Expression of CD24 gene in human malignant pleural mesothelioma and its relationship with prognosis / 中华劳动卫生职业病杂志

Objective: To investigate the expression of CD24 gene in human malignant pleural mesothelioma (MPM) cells and tissues, and evaluate its relationship with clinicopathological characteristics and clinical prognosis of MPM patients. Methods: In February 2021, UALCAN database was used to analyze the correlation between CD24 gene expression and clinicopathological characteristics in 87 cases of MPM patients. The TIMER 2.0 platform was used to explore the relationship between the expression of CD24 in MPM and tumor immune infiltrating cells. cBioportal online tool was used to analyze the correlation between CD24 and MPM tumor marker gene expression. RT-qPCR was used to analyze the expressions of CD24 gene in human normal pleural mesothelial cell lines LP9 and MPM cell lines NCI-H28 (epithelial type), NCI-H2052 (sarcoma type), and NCI-H2452 (biphasic mixed type). RT-qPCR was performed to detect the expressions of CD24 gene in 18 cases of MPM tissues and matched normal pleural tissues. The expression difference of CD24 protein in normal mesothelial tissue and MPM tissue was analyzed by immunohistochemistry. A Kaplan-Meier model was constructed to explore the influence of CD24 gene expression on the prognosis of MPM patients, and Cox regression analysis of prognostic factors in MPM patients was performed. Results: The CD24 gene expression without TP53 mutation MPM patients was significantly higher than that of patients in TP53 mutation (P<0.05). The expression of CD24 gene in MPM was positively correlated with B cells (r(s)=0.37, P<0.001). The expression of CD24 gene had a positive correlation with the expressions of thrombospondin 2 (THBS2) (r(s)=0.26, P<0.05), and had a negative correlation with the expression of epidermal growth factor containing fibulin like extracellular matrix protein 1 (EFEMP1), mesothelin (MSLN) and calbindin 2 (CALB2) (r(s)=-0.31, -0.52, -0.43, P<0.05). RT-qPCR showed that the expression level of CD24 gene in MPM cells (NCI-H28, NCI-H2052 and NCI-H2452) was significantly higher than that in normal pleural mesothelial LP9 cells. The expression level of CD24 gene in MPM tissues was significantly higher than that in matched normal pleural tissues (P<0.05). Immunohistochemistry showed that the expressions of CD24 protein in epithelial and sarcoma MPM tissues were higher than those of matched normal pleural tissues. Compared with low expression of CD24 gene, MPM patients with high expression of CD24 gene had lower overall survival (HR=2.100, 95%CI: 1.336-3.424, P<0.05) and disease-free survival (HR=1.800, 95%CI: 1.026-2.625, P<0.05). Cox multivariate analysis showed that compared with the biphasic mixed type, the epithelial type was a protective factor for the prognosis of MPM patients (HR=0.321, 95%CI: 0.172-0.623, P<0.001). Compared with low expression of CD24 gene, high expression of CD24 gene was an independent risk factor for the prognosis of MPM patients (HR=2.412, 95%CI: 1.291-4.492, P=0.006) . Conclusion: CD24 gene and protein are highly expressed in MPM tissues, and the high expression of CD24 gene suggests poor prognosis in MPM patients.

Mesotelioma pleural maligno en ocho pacientes y revisión del tema / Malignant pleural mesothelioma in eight patients and review of topic

Se actualizan aspectos etiopatogénicos, clínicos, diagnósticos y terapéuticos en el mesotelioma pleural maligno, enfermedad temida e infrecuente en nuestro medio. Nos impresionó sobremanera, una profesional de salud tratada recientemente y en etapa temprana que apenas sobrevivió un año. El objetivo es elevar el conocimiento sobre el tema para tratar de mejorar la sobrevida. Se presentan una síntesis de ocho pacientes estudiados y tratados con este diagnóstico en los hospitales "Amalia Simoni", "Manuel Ascunce Domenech", "Madam Curie" de Camagüey y el "Martín Chang Puga" de Nuevitas desde 1998 hasta 2015, señalando el cuadro clínico, exámenes complementarios, diagnóstico, tratamiento médico quirúrgico y los resultados. Más de la mitad de los pacientes eran fumadores con pequeño derrame pleural inicial que hicieron pensar en la enfermedad, todo lo contrario cuando no existió derrame. Hubo tres enfermos donde el diagnóstico nos sorprendió por lo inesperado. La sobrevida fue baja con una media alrededor de 11 meses, solo uno vivió dos años. Los complementarios utilizados se ajustan a otros reportes y nuestras posibilidades. El tratamiento fue actualizado y acorde a otras series en el momento del diagnóstico. Se compara nuestra casuística, la cual se asemeja a publicaciones foráneas en cuanto a diagnóstico, tratamiento y sobrevida. Señalamos que, independiente de algunos recursos desde el punto de vista diagnóstico y terapéutico con que no contamos, los resultados se ajustan a la literatura actual y la sobrevida lograda fue sin dudas, adversa(AU)

Several etiopathogenetic, clinical, diagnostic and therapeutic aspects of the malignant pleural mesothelioma, fearful and infrequent disease in our context, are updated. It was really impressive the case of a female health professional that was recently treated at early stage of disease and barely survived one year. The objective of this review was to raise the level of knowledge on this disease in oder to improve survival rates. To this end, eight patients with this diagnosis, who were studied and treated in "Amalia Simoni", "Manuel Ascunce Domenech", "Madame Curie" hospitals in Camaguey and in "Martin Chang Puga" in Nuevitas from 1998 to 2015 were presented. Their clinical picture, supplementary tests, diagnosis, medical and surgical treatment and final results were described. Half of them were smokers with initial small pleural effusion that made the specialists suspect the existence of the disease. There were three patients whose diagnoses surprised the physicians because they were unexpected. Survival was low and the average survival rate was 11 months, although one managed to live two years. The indicated supplementary tests were similar to those of other reports and adjusted to our setting. Treatment was updated and consistent with other series at the time of diagnosis. The casuistry in our conditions was compared to others and it was similar in terms of diagnosis, treatment and survival rates to the one shown in foreign publication. Regardless of some unavailable diagnostic and therapeutic resources, the results of the treatment agree with those of the current literature on the topic and the survival rate was undoubtedly negative(AU)

Tumor adenomatoide pleural / Adenomatoid pleural tumor

Introduction: Adenomatoid tumors are rare benign neoplasms, pleural is a very rare location, reporting only 4 cases worldwide and none in our country. Case report: A 30 year-old female patient, that in the context of a preventive health examination, presents a mediastinal solid mass in the right hemithorax, in the thorax radiography. The lesion of 8 cm x 7 cm x 2.4 cm was surgically removed. Histology shows a lesion compatible with pleural adenomatoid tumor and inmunohistochemistry is positive for markers such as calretinin and cytokeratin 5/6. Discussion: The pleural adenomatoid tumor is an uncommon neoplasm with benign behavior and several differential diagnoses.

Introducción: Los tumores adenomatoides son neoplasias benignas poco frecuentes, cuya ubicación pleural es de muy baja frecuencia, reportándose sólo 4 casos a nivel mundial y ninguno nacional a la fecha. Las muestras parciales en biopsia intraoperaratoria, en ausencia de antecedentes clínico-radiológicos puede representar un desafío diagnóstico. Caso clínico: Paciente de sexo femenino, 30 años que en contexto de un examen preventivo de salud se pesquisa en la radiografía de tórax una masa sólida mediastínica en hemitórax derecho. Se extirpa lesión redondeada de 8 cm. La histología muestra lesión compatible con tumor adenomatoide pleural y la inmunohistoquímica positividad para marcadores Calretinina y Citoqueratina 5/6. Discusión: El tumor adenomatoide pleural en una entidad poco frecuente, de comportamiento benigno, con diversos diagnósticos diferenciales.

Aspectos diagnósticos y terapéuticos en el mesotelioma pleural maligno / Diagnostic and therapeutic aspects in malignant pleural mesothelioma

Para el diagnóstico de mesotelioma se requiere distinguir entre afectación mesotelial benigna y maligna, y entre mesotelioma maligno y carcinoma metastásico. Para ello son necesarias técnicas inmuno-histoquímicas realizadas sobre biopsias amplias. La toracoscopia es la técnica de elección, aunque la biopsia con aguja usando técnicas de imagen en tiempo real puede ser muy útil si hay marcado engrosamiento nodular. Es improbable que la cirugía radical (pleuroneumonectomía) sea realmente curativa, por lo que está ganando adeptos la reducción de masa tumoral mediante pleurectomía/decorticación, con asociación de quimioterapia y radioterapia a la cirugía (terapia multimodal). Cuando la resección no es factible se plantea quimioterapia, con pleurodesis o colocación de un catéter pleural tunelizado si se requiere el control del derrame pleural y se reserva la radioterapia para tratar la infiltración de la pared torácica. También es esencial un completo control del dolor (que adquiere particular protagonismo en esta neoplasia) en unidades especializadas.

Diagnosis of malignant pleural mesothelioma requires making the distinction between benign mesothelial hiperplasia and true mesothelioma, and between malignant mesothelioma and metastatic pleural adenocarcinoma. This involves immunohisto-chemical techniques applied on large biopsy specimens, and thoracoscopy is the best choice for obtaining them. Real-time image-guided needle biopsy can also be very helpful in presence of marked nodular pleural thickening. Radical surgery (ie, extrapleural pneumonectomy) is unlikely to cure completely the patient, and cyto-reduction surgery with preservation of the underlying lung (pleurectomy/decortication), with addition of chemo and radiation therapy (muiltimodal treatment) is gaining adepts in the last few years. When surgery is not feasible at all, early chemotherapy -with pleurodesis or placement of a indwelling pleural catheter (to control the effusion if necessary)- is advisable. Radiation therapy should be reserved to treat chest wall infiltration in those cases, and complete control of pain in specialized units is also essential in those patients.

The Pleural Sandwich Sign in Two Cases of Primary Pleural Lymphoma

The sandwich sign is used to describe mesenteric lymphoma in which mesenteric vessels and fat are enveloped by enlarged mesenteric lymph nodes. We present two cases of primary pleural lymphoma demonstrating the "pleural sandwich sign". Contrast-enhanced computed tomography showed conglomerated parietal pleural and extrapleural masses encasing the intercostal arteries. Histopathological examinations confirmed low grade marginal zone B-cell lymphoma in an 80-year-old man and diffuse large B-cell lymphoma in a 68-year-old man. The pleural sandwich sign may suggest the diagnosis of primary pleural lymphoma.

A Pleural Loose Body Mimicking a Pleural Tumor: A Case Report

We present a rare case of a pleural loose body, thought to be a pedunculated pleural tumor, found incidentally in a 58-year-old female. Computed tomography showed a non-enhancing mass, which migrated along the mediastinum and paravertebral area. Thoracoscopic surgery revealed a 4 cm, soap-like mass that was found to be a fibrin body consisting of hyalinized collagen histopathologically. Mobility and the lack of contrast enhancement of a pleural mass are important clues to diagnosing this benign condition.

Tumor fibroso localizado de pleura: reporte de un caso / Localized fibrous tumor of pleura: a case report

Fundamento: los tumores fibrosos localizados de pleura son raros, de etiología incierta; la mayoría de los pacientes con esta enfermedad sobrepasan la quinta década de vida y más de la mitad están asintomáticos al diagnóstico, principalmente cuando los tumores son pequeños. Objetivo: describir el caso de una paciente con tumor fibroso localizado de pleura que, por su edad, sintomatología y por las características histológicas del tumor, representa una situación clínica infrecuente, con dificultades para el diagnóstico. Presentación de caso: mujer saludable, de 39 años, con dolor tóraco-lumbar persistente, de un año de evolución; en radiografía de tórax se visualizó imagen retro cardíaca izquierda corroborada por tomografía computarizada. Se realizó exéresis total de una masa de 4,8 cm, pedunculada, con origen en la pleura visceral, cuyo diagnóstico histopatológico fue un tumor fibroso localizado de pleura con características quísticas. Después de varios meses de seguimiento la paciente permanecía saludable y sin signos de recurrencia. Conclusiones: este caso constituye una forma infrecuente de presentación del tumor fibroso localizado de pleura por tratarse de una paciente menor de 40 años, con una sintomatología no frecuente y por el comportamiento benigno que ha tenido el tumor aunque presentó características quísticas.

Background: localized fibrous tumors of the pleura are rare, with uncertain etiology; most of the patients with this disease exceed the fifth decade of life and more than a half are without symptoms at the diagnosis, mainly when tumors are small. Objective: to describe a case of a female patient with a diagnosis of a localized fibrous tumor of pleura in which due to her age, the symptomatology and histological features of the tumor, it represents an infrequent clinical situation with difficulties for the diagnosis. Case presentation: a 39-year-old healthy woman with one year history of persistent thoracic lumbar pain; in chest radiography a left retrocardiac image was observed and it was corroborated by chest CT scan. Total exeresis was performed in a 4.8 cm mass, pedunculated, originating from the visceral pleura, whose histopathological diagnosis was a localized fibrous tumor of the pleura with cystic features. After several months of follow-up, the patient remained healthy and did not show recurrent signs. Conclusions: this case is an infrequent presentation of a localized fibrous tumor of the pleura for having been diagnosed in a patient under 40 years old, with an infrequent symptomatology and because the tumor has shown a benign behavior even with cystic features.

Dificuldades no diagnóstico diferencial entre mesotelioma pleural e adenocarcinoma metastático para pleura patológica / Difficulties in the differential diagnosis between pleural mesothelioma and metastatic adenocarcinoma for pathological pleura

As dificuldades diagnósticas entre mesotelioma pleural e adenocarcinoma metastático na pleura exigem estudo amplo. Os autores ilustram um caso clínico cujo diagnóstico só foi estabelecido após realização de toracotomia, com retirada de material para análise imuno-histoquímica. A diferenciação diagnóstica é de fundamental importância, uma vez que envolvem conduta terapêutica e prognóstico distintos. O estudo adequado deve utilizar material obtido através de toracoscopia ou toracotomia e empregar coloração imuno-histoquímica, estudos com anticorpos monoclonais, microscopia eletrônica e pesquisa de marcadores tumorais.

The diagnosis difficulties distinguishing mesothelioma from pleural metastatic adenocarcinoma request wide study. The authors illustrate a clinical case whose diagnosis was only established after thoracotomy removing material for immune-histochemical analysis. The diagnosis differentiating is very important, because involves distinct treatment and prognosis. The appropriate study should use material obtained through thoracoscopy or thoracotomy and submitted to immune-histochemical coloration, studies with monoclonal antibodies, electronic microscopy and research of tumor makers.

Mesotelioma pleural sarcomatoide, con diseminación masiva a peritoneo y pericardio. Un caso de autopsia / Malignant pleural mesothelioma, with generalized dissemination to peritoneum and pericardium: a descriptive autopsy report

El mesotelioma maligno es una neoplasia originada a partir de las células mesoteliales de las membranas serosas (pleura, peritoneo, pericardio y otros). Es 5 veces más frecuente en la cavidad pleural que en la peritoneal, y puede observarse en ambas por extensión directa a través del diafragma(1). Se presenta el caso de autopsia en una mujer de 83 años, sin antecedentes de exposición al asbesto, oligosintomática, con mesotelioma pleural maligno tipo sarcomatoide, en estadio avanzado (Estadio IV). El mesotelioma es una neoplasia letal, su diagnóstico a veces resulta dificultoso debido al crecimiento lento, las manifestaciones clínicas tardías y el diagnóstico en estadios avanzados. En primer lugar debe descartarse secundarismo y ante clínica e imágenes compatibles debe plantearse su diagnóstico.

Malignant mesotheliomas are tumors derived from mesothelial cells that form the serous membranes. The incidence of mesotheliomas show a rate 5 times greater in the pleural cavity than in the peritoneum; but they can be detected in both, as a result of direct invasion through the diaphragm. A case out from an autopsy is reported, of a 83 years old female patient, with no previous history of exposure to asbestos, oligosymptomatic, with malignant pleural mesothelioma of sarcomatoideal type, at an advanced stage (Stage IV). Malignant mesotheliomas are relatively rare being a highly lethal neoplasia its diagnosis is sometimes difficult because the have a gradual development and growth, with late clinical manifestions, and with diagnosis at an advanced evolutive stages. First of all, secondarism must be discarged, and in presence of compatibles images, its diagnosis must be hypothesized.

Tumor fibroso solitario pleural de lenta evolución / Giant solitary fibrous tumour of the pleura of slow evolution

El tumor fibroso solitario pleural es una neoplasia infrecuente originada en las células submesoteliales de la superficie pleural. Generalmente se manifiesta como una masa exofítica que crece hacia dicho espacio comprimiendo estructuras adyacentes. Su diferencia con el mesotelioma pleural maligno no sólo yace en su origen histológico, sino también en los antecedentes epidemiológicos y el curso clínico de la enfermedad. Presentamos el caso de un paciente con una masa pleural gigante de 11 años de evolución, los inconvenientes suscitados con el diagnóstico y, finalmente, la resolución quirúrgica definitiva.

The solitary fibrous tumor of the pleura is a rare neoplasm originating from submesothelial cells of the pleural membrane. Its usual manifestation is as an exophytic mass that grows into the pleural cavity compressing adjacent structures. The difference with malignant pleural mesothelioma does not only lie in its histological origin, but also in the epidemiological history and clinical course of the disease. We report a patient with a giant pleural mass of 11 years of evolution, the problems arising for the diagnosis, and finally the definitive surgical treatment.

Determinación de péptidos solubles relacionados a mesotelina para la detección precoz del mesotelioma maligno / Soluble mesothelin-related protein for malignant pleural mesothelioma screening

Introduction: Malignant Pleural Mesothelioma (MPM) is a tumor of the mesothelial cells related to asbestos exposure. This malignancy is extremely aggressive, with poor response to different treatment modalities, and it has a mean survival of 8 months since diagnosis. With the introduction of new chemotherapeutic agents and trimodality protocols, five-year survival of 40 percent in initial stages has been reported. Serum detection of Soluble Mesothelin-related Protein (SMRP) could be used for screening of MPM. Using the MESOMARK® test, 53 percent of MPM patients had levels greater than 1,5 nM, while 99 percent of control patients had lower concentrations. The aim of this study is to evaluate the use of this test in Chile and determine its utility for screening ofMPM. Methods: Quantitative blind measurement of serum SMRP by MESOMARK® test. We studied 3 groups: 8 workers exposed to asbestos, 5 patients with diagnosed MPM and 14 age matched workers without known exposure to asbestos. Participants were informed of the study. Results: Mean +/- standard deviation SMRP levels in the control group was 0,53 +/- 0,4 nM, 0,89 +/- 0,46 nM in patients exposed to asbestos and 10,68 +/- 10,28 nM in MPMpatients. Differences between the groups were statistically significant (p = 0,02). In the MPM group, 3 patients were found to have SMRP levels greater than 1,5 nM (17,27 nM; SD 6,95) and 2 patients normal values (0,79 nM; SD 0,32). Using a cut-off value of 1,5 nM, sensitivity of the test was 60 percent and specificity was 100 percent. Conclusions: Detection of SMRP levels allowed to identify patients with MPM, three of whom had very high concentrations. The sensitivity and specificity found is similar to that previously reported. If our results are confirmed in greater studies, SMRP detection could be used for screening of MPM.

Resumen Introducción: El Mesotelioma Maligno (MM) es un tumor de las células mesoteliales relacionado a la exposición a asbesto, altamente agresivo, con pobre respuesta al tratamiento y con una sobrevida promedio de 8 meses después del diagnóstico. Sin embargo, nuevos agentes quimioterapéuticos y protocolos de terapia trimodal han logrado sobrevidas de hasta 40 por ciento en etapas iniciales. La detección en sangre periférica de Péptidos Solubles Relacionados a Mesotelina (SMRP) podría ser útil para el diagnóstico precoz de MM. Utilizando el test MESOMARK® para la determinación de SMRP, 53 por ciento de los pacientes con MM tenían valores mayores a 1,5 nM mientras que 99 por ciento de los controles mostraron valores inferiores. El objetivo del presente trabajo es evaluar la implementación de este test en Chile y determinar su utilidad para el diagnóstico precoz en MM. Métodos: Medición cuantitativa de SMRP en suero humano por test MESOMARK®. Se realizaron mediciones en forma ciega a 8 trabajadores con exposición a asbesto, a 5 pacientes con MMy a 14 voluntarios sin exposición. Todos los participantes fueron informados del estudio. Resultados: El promedio +/- desviación estándar de SMRP en el grupo control fue de 0,53 +/- 0,4 nM, de 0,89 +/- 0,46 nM en los expuestos sin MMy de 10,68 +/- 10,28 nM en el grupo con MM; encontrándose una diferencia estadísticamente significativa entre los valores de estos tres grupos (p = 0,02). En el grupo con MM, 3 pacientes tuvieron concentraciones mucho mayores a 1,5 nM (17,27 nM; DS 6,95) y 2 valores normales (0,79 nM; DS 0,32). Utilizando un valor de 1,5 nM como punto de corte, la sensibilidad fue de 60 por ciento y la especificidad de 100 por ciento. Conclusiones: La medición de SMRP permitió diferenciar a los pacientes con MM, presentando 3 de ellos concentraciones muy elevadas. La sensibilidad y especificidad encontrada es similar con datos previamente reportados. De confirmarse estos resultados en estudios con mayor ...

Peripheral primitive neuroectodermal tumor of the pleura in a 41-year-old female

Peripheral primitive neuroectodermal tumor [pPNET] is a rare, very aggressive neoplasm that belongs to a small round cell tumor, and most often arises from the chest wall. Here, we report a female case with proven pPNET who was treated in our institution. She presented with a history of left side chest pain, cough, and significant weight loss. Contrast enhanced CT imaging of the chest showed multiple left pleural-based enhancing masses with left diaphragmatic involvement. She underwent chemotherapy followed by tumor debulking through thoracotomy. However, she died of rapid growth from recurrent local tumors 3 months thereafter

Massive pleural effusion due to metastasis of prostate cancer / Efusión pleural masiva debido a metástasis de cáncer de próstata

We describe the case of a 72-year old male with pleural effusion associated with prostate cancer. There was a previous history of tobacco smoking (pack/year: 47) and of total prostatectomy followed by external beam radiation therapy seven years previously for prostate cancer. Furthermore, he was submitted to orchiectomy plus non-steroidal anti-androgen blockage, in addition to docetaxel-based chemotherapy and prednisone. After the beginning of chemotherapy, a progressive elevation in prostate specific antigen (PSA) levels was observed. On admission, he presented with fever, weight loss, and respiratory symptoms due to a massive right pleural effusion. Fluid samples obtained by needle aspiration showed haemorrhagic exudates without malignant cells. Pleural metastasis were detected by thorax imaging studies, and biopsy samples revealed prostate adenocarcinoma as the origin of his pleural effusion. Pleural fluid was drained and talc pleurodesis was performed. This report aims to describe the occurrence of massive pleural effusion due to metastasis of prostate cancer, and emphasizes the role of pleural biopsy with immunohistochemical studies to characterize this diagnosis.

Se describe un hombre de 72 años con efusión pleural asociada con cáncer de próstata. Había antecedentes de tabaquismo (47 paquetes por año) así como una historia de prostatectomía radical, seguida por terapia de radiación externa, siete años antes. Además, se le sometió a orquiectomía junto con bloqueo antiandrogénico no esteroideo, además de quimioterapia a base de docetaxel y prednisona. Después de iniciada la quimioterapia, se observó una elevación progresiva en los niveles de PSA. En el momento del ingreso, el paciente presentaba fiebre, pérdida de peso, y síntomas respiratorios debidos a una efusión pleural derecha voluminosa. Las muestras de fluido obtenidas mediante punción aspirativa con aguja fina, mostraron exudados hemorrágicos sin células malignas. Se detectaron implantes pleurales con los estudios imaginológicos del tórax, y las muestras de la biopsia revelaron que el origen de su efusión pleural, era un adenocarcinoma de la próstata. Se drenó el fluido pleural, y se procedió a practicar una pleurodesis con talco. Este reporte tiene por objetivo describir la ocurrencia de la efusión pleural masiva debido a la metástasis del cáncer de la próstata, y enfatiza el papel que desempeña la biopsia pleural junto a los estudios inmunohistoquímicos a la hora de caracterizar este diagnóstico.

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.

Pleuropulmonary blastoma type II: a case report

Pleuropulmonary blastoma (PPB) is a rare embrionary mesenchymal neoplasm but is recognized as the most common pulmonary malignancy of childhood. It may present metastasis to the brain and also be indicative of other neoplasms in affected individuals or in their relatives. Being such a rare disease, it is considered a difficult diagnosis to be made. A 3-year-old female presented with fever and respiratory distress. At first, she had been treated for pneumonia with antibiotics for 14 days in another hospital with no response. Computed tomography (CT) scan showed a right lung/mediastinal mass. The patient was referred to our institution and a new CT-scan evidenced a complex mass of irregular borders, cystic areas and solid projections, along with a right pneumotorax. The mass was biopsied and hematoxylin-and-eosin (HE) stained histological sections showed a neoplasm composed of small and round cells with hyperchromatic nuclei and scant cytoplasm. The immunohistochemical profile demonstrated positivity for desmin, myogenin and Myo-D1, suggesting the diagnosis of rhabdomyosarcoma. After two weeks of hospitalization, the patient was clinically stable and initiated the first chemotherapy cycle. Surgical resection of the mass was performed and the HE slides demonstrated a neoplasm composed of anaplastic and condrossarcomatous cells with extensive necrosis. The correlation of clinical data, radiological and morphological features were conclusive of a PPB type II. The recognition and diagnosis of this entity is of great importance due to its clinical and prognostic particularities.

Extranodal Interdigitating Dendritic Cell Sarcoma Presenting in the Pleura: A Case Report

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare neoplasm arising from the antigen-presenting cells of the immune system. This disease usually involves the lymph nodes, and rarely, extranodal sites may be affected. The authors report a case of extranodal IDCS presenting in the pleura. A 32-yr-old man presented with progressive chest pain. Imaging studies showed diffuse pleural thickening with pleural effusion. Morphological and immunohistochemical analysis of an incisional biopsy of the pleura were consistent with a diagnosis of IDCS; tumor cells were positive for S100 and CD45, but negative for CD1a, CD21, CD35, B cell and T cell markers. The patient was administered chemotherapy, but died of progressive disease. Although its incidence is extremely rare, this case suggests that extranodal IDCS should be considered in the differential diagnosis of undifferentiated neoplasms and that immunohistochemical staining be performed using appropriate markers.