Hallazgo incidental de neoplasia primaria de pulmón: blastoma pulmonar en mujer adulta. Reporte de caso / Incidental finding of primary lung neoplasm: pulmonary blastoma in an adult woman, case report

El blastoma pulmonar es una neoplasia poco común y agresiva que se origina en el pulmón; está compuesto por tejido mesenquimal y epitelial inmaduro que imita la configuración pulmonar en etapas embrionarias. Tiene una baja incidencia y una alta tasa de mortalidad, con aproximadamente un 60 %. Este blastoma tiende a desarrollarse con mayor frecuencia entre la cuarta y quinta década de vida, siendo más prevalente en mujeres y generalmente asociado al tabaquismo. Se presenta el caso de una mujer de 23 años sin factores de riesgo conocidos, a quien se le diagnosticó incidentalmente un blastoma pulmonar primario. La paciente experimentaba dolor en la región costal izquierda, tos productiva, disnea y hemoptisis desde hace 30 días. Los hallazgos en las imágenes de rayos X y tomografías de tórax llevaron a la realización de una biopsia del pulmón izquierdo, que reveló fragmentos de tejido con una notable presencia de necrosis tumoral. Cinco días después, la paciente consultó nuevamente debido a un empeoramiento de los síntomas, incluyendo dolor torácico de tipo pleurítico. Se decidió realizar una lobectomía inferior izquierda, durante la cual se encontró un tumor sólido adherido a la pleura visceral, con masas tumorales en la pleura parietal. El estudio histopatológico final confirmó el diagnóstico de un tumor maligno de alto grado compatible con blastoma pulmonar. En conclusión, el blastoma pulmonar es una neoplasia rara que puede presentarse en diferentes etapas de la vida, aunque tiene mayor incidencia entre los 40 y 50 años. Es importante que los patólogos consideren esta enfermedad en sus diagnósticos diferenciales debido a los desafíos que implica su diagnóstico. Detectar el blastoma pulmonar en etapas tempranas es crucial para el tratamiento adecuado, aunque actualmente no se conocen marcadores predictivos confiables y el pronóstico de esta enfermedad es generalmente desfavorable.

Pulmonary blastoma is a rare and aggressive neoplasm that originates in the lung. It is composed of immature mesenchymal and epithelial tissue that mimics the embryonic configuration of the lung. It has a low incidence and a high mortality rate, of approximately 60%. Pulmonary blastoma tends to occur more frequently between the fourth and fifth decades of life, being more prevalent in women and generally associated with smoking. The case of a 23-year-old woman with no known risk factors is presented, who incidentally was diagnosed with a primary pulmonary blastoma. The patient experienced pain in the left costal region, productive cough, dyspnea, and hemoptysis for 30 days. Findings on X-ray and chest tomography led to a biopsy of the left lung, which revealed tissue fragments with significant tumor necrosis. Five days later, the patient consulted again due to worsened symptoms, including pleuritic chest pain. It was decided to perform a left lower lobectomy, during which a solid tumor attached to the visceral pleura with tumor masses in the parietal pleura was found. The final histopathological study confirmed the diagnosis of a high-grade malignant tumor compatible with pulmonary blastoma. In conclusion, pulmonary blastoma is a rare neoplasm that can occur at different stages of life, although it has a higher incidence between the ages of 40 and 50. Pathologists need to consider this disease in their differential diagnoses due to the challenges involved in its diagnosis. Detecting pulmonary blastoma at early stages is crucial for appropriate treatment, although currently there are no reliable predictive markers, and the prognosis of this disease is generally unfavorable.

Pituitary blastoma, expanding the spectrum of newly recognized entities. A case report

Tumors of the pituitary gland and sellar region represent about 15% of all brain tumors, with pituitary adenoma being the commonest and pituitary carcinoma being very rare. Pituitary tumors in children are even rarer. Pituitary blastoma, a pediatric adenohypophysial tumor, is a new entity described in the 2017 WHO classification of pituitary tumors. This is a very rare tumor with only 21 cases reported so far. Hence, we are reporting this unusual case seen in a 7-month-old infant who presented with a large sellar/suprasellar mass with pressure symptoms of short duration. Typically, they present between 7–24 months of age. On histopathology, a cellular tumor was seen with primitive-looking round cells with scanty cytoplasm with few well-defined gland or rosette-like structures. The immunohistochemical stains showed diffuse strong staining for synaptophysin with a very high MIB-1 index. Other markers for common round cell tumors in this age group and hormonal markers of pituitary tumors were negative with INI-1 being intact. The initial cases described by Scheithauer presented with Cushing's disease and at least focally expressed adrenocorticotrophic hormone on immunohistochemistry. However, nonfunctioning tumors are also seen, albeit rarely. These are known to be associated with DICER 1 mutations and have a poor prognosis. Hence, morphologic recognition in the right clinical context and excluding other differential diagnoses in infants help make the correct diagnosis.

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.

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.

Blastoma pleuropulmonar: Presentación de un caso / Pleuropulmonary Blastoma: Case Report

El blastoma pleuropulmonar es una neoplasia mesenquimal rara que se debe considerar siempre en el escenario de una masa pulmonar sólida o quística en niños menores de 5 años. Se localiza usualmente en la periferia del pulmón; sin embargo, puede existir compromiso extrapulmonar de mediastino, diafragma y pleura. Se clasifica según su patrón histológico e imagenológico en tres tipos: quístico, mixto (sólido-quístico) y sólido. La cirugía radical es el tratamiento de elección y la única que ha demostrado disminución en la tasa de recurrencia. Se indica el uso de la quimioterapia neoadyuvante y la radioterapia según el tipo histológico y las márgenes libres de tumor. Se estudia el caso de una niña de 3 años con hallazgos, en radiografía convencional y tomografía de tórax, de masa sólida con diagnóstico histopatológico de blastoma pleuropulmonar.

Pleuropulmonary blastoma is a rare mesenchymal neoplasm that should always be considered in the setting of a solid or cystic lung mass in children under 5 years old. It is usually located in the periphery of the lung. However extra pulmonary involvement of the mediastinum, diaphragm, and pleura can exist. It is classified according to its histological and imaging pattern in three types: cystic, mixed (solidcystic) and solid. Radical surgery is the treatment of choice and the only one that has demonstrated a decrease in the rate of recurrence. The use of neoadjuvant chemotherapy and radiotherapy are defined according to the histological type and presence of tumor-free margins. We present the case of a 3-year-old female patient with findings on conventional radiography and thoracic tomography of a solid mass with histopathological diagnosis of pleuropulmonary blastoma.

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.

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.

Clinicopathological features of cystic lung diseases in children / 临床与实验病理学杂志

Purpose To investigate the histopathological features of cystic lung diseases ( CLD) , and to discuss the timing of clinical interventions. Methods HE and immunohistochemical staining were performed and reviewed in 125 cases of CLD. Results 125 ca-ses of CLD aged from birth to 11 years and 6 month, with an average age of 23. 0 months, median age 15 months, of which 60 cases were less than 1 year (48. 0%). 75 cases were male and 50 cases female, with male to female ratio of 1. 5 ∶ 1. Grossly, 50 cases showed single or multiple cysts with the size 0. 5 ~8. 0 cm in diameter, which did not communicate with bronchial cavity. 18 cases showed honeycomb cysts with the diameter of 0. 1~2. 0 cm. 26 cases were solid lesions without visible cysts. 21 cases were observed lung abscess with thick and rough wall and pus inside. 7 cases of emphysema showed microcysts with crepitation. 2 cases were identi-fied cystic and solid masses, with fish-fresh like cut surface. Histopathologically, 94 cases (75. 2%) were related to congenital bron-chopulmonary dysplasia in 125 cases of CLD, in which there were 59 patients (47. 2%) of congenial pulmonary airway malformation (CPAM), including 29 cases of type 1 (49. 2%), 18 cases of type 2 (30. 5%), and 12 cases of type 4 (20. 3%), there were 26 ca-ses (20. 8%) of pulmonary sequestration, including 15 cases of intralobar type (57. 7%) and 11 of extralobar cases (42. 3%), 5 ca-ses were complicated with CPAM type 2, 8 cases were bronchial cyst (6. 4%) and 1 case of enteric cyst (0. 8%). Acquired lesions were detected in 31 cases (24. 8%), including 21 cases of infected lung abscess, 1 case of fungal abscess. 7 cases of emphysema, and 3 cases of pleuralpulmonary blastoma (typeⅠ1 case and typeⅡ2 cases). Conclusion Pediatric CLD is characterized as com-plexed categories. The prognosis depends on correct pathological diagnosis, combined with imaging evaluation and appropriate timing of surgery.

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.

Pancreatoblastoma in an Adult / 대한췌담도학회지

A blastoma is a type of cancer, which is common in children; it is caused by malignancies derived from in the precursor cells, often called blasts. Examples are nephroblastomas, retinoblastomas, pleuropulmonary blastomas, and pancreatoblastomas. Pancreatoblastomas are extremely rarely in adults. It is difficult preoperatively to distinguish this tumor from other pancreatic tumors including solid and papillary epithelial neoplasm of the pancreas (SPEN), acinar cell carcinoma, islet cell tumor, and ductal adenocarcinoma with cystic degeneration. To our knowledge, this case may be the second report of a pancreatoblastoma occurring in an adult in Korea. We report a case of a pancreatoblastoma that was confirmed by pathology, despite the radiologic finding that assumed it was a SPEN.

Diagnosis and multi-modality treatment of adult pulmonary plastoma: Analysis of 18 cases and review of literature

The clinical data of 18 patients with PB from April 1989 to April 2013 was analyzed retrospectively, including 11 men and 7 women, aged 45 and 76 years old (mean 53 years). There were 12 cases of PB occurring in right lung and other cases in left lung. Among them, 3 patients had no symptoms, and 15 patients displayed symptoms of cough, chest pain, asthenia or minor haemoptysis. Overall, 11 patients had a preoperative diagnosis of lung cancer, 7 patients were preoperatively diagnosed as the other diseases, which included lung benign tumor (n=5) and mediastinal mass (n=2). All patients received a radical resection. Six patients received postoperative cisplatin-based chemotherapy, and two patients received postoperative irradiation with the dose of 55 Gy. Histologically, 14 cases of 18 patients had biphasic pulmonary blastoma and four cases had well differentiated fetal adenocarcinoma. A total of 12 patients died in a period of 6-36 months after operation, and 1 case was lost after 2 years of follow up. The median survival time was 19 months. PB is a rare primary lung malignant embryonal neoplasm. Despite its assumed embyonal origin, the tumor has a predilection for adults. A preoperative correct diagnosis is very difficult in spite of modern diagnostic imaging and biopsy techniques. Surgical resection is the main method for diagnosis and treatment. Postoperative chemotherapy or irradiation can help eliminate tumor remnants. Its prognosis is very poor, especially for the biphasic type.

Biphasic Pulmonary Blastoma: a Case Report and Literature Review.

Introduction: Biphasic Pulmonary blastoma (BPB) is classified as one of the rare primary lung malignancies. It is composed of a mixture of epithelial and mesenchymal tissues resembling embryonic lung tissue. BPB is considered to be distinct from other lung tumors based on pathological features, clinical course and prognosis. Presentation of Case: The authors report an atypical case of BPB in a 27 -year-old man presented with complaints of dyspnea and left-sided chest pain for the previous four months. A chest radiograph showed the presence of an opaque left hemithorax, and the mediastinum was pushed toward the left. Computed tomography (CT) of the chest revealed a mixed solid and cystic process with variable contrast enhancement measuring 15,4 x 13,7 cm occupying the totality of the left hemithorax, pleural effusion, and a collapsed left lung, with contralateral mediastinal shift. A transthoracic needle pleural biopsy yielded a diagnosis of BPB.A general examination shows a peritoneal effusion. One month after diagnosis, the tumor grew rapidly, and therefore he was treated only by palliative care. He died from respiratory failure one month later. Although BPB is rare, this entity is increasingly described. Conclusion: The purpose of presenting this case report is to raise awareness among clinicians to consider this clinical entity as a differential diagnosis when a pleural mass is identified. Histological examination is the most reliable and conclusive method of diagnosing BPB and differentiating it from other primary or metastatic lung malignancies.

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 .

Pulmonary blastoma in a young adult.

Pulmonary blastoma is a rare but aggressive malignancy of the lung comprising epithelial and mesenchymal elements that resemble fetal lung tissue. This report described a case of an 18-year-old male who presented with cough and weight loss for a month. Computed tomography (CT) of the thorax revealed a large mass with mixed solid and cystic lesions on the right side of chest along with pleural effusion and mediastinal lymphadenopathy. Massive debulking was performed followed by chemotherapy. A biphasic pulmonary blastoma was diagnosed on histopathology.

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.

Autophagy of SO-Rb50 cells induced by arsenic trioxide / 中华实验眼科杂志

Background Cellular autophagy is a non-apoptosis death form of tumor tissue.Research determined that arsenie trioxide (As2O3) leads to apoptosis of tumor cells.But whether As2O3 induce autophagy of SO-Rb50 cells or not is unclear.Objective This study was to assess the effects of As2O3 on autophagy of SO-Rb50 cells.Methods As2O3 with the concentration of 0,0.5,1.0,2.0,4.0 μmol/L was used to treat the SO-Rb50 cell line for 48 hours,and the growth and proliferation of SO-Rb50 cells were detected using MTT assay (A570).pGFP-LC3,a marker of autophagy,was constructed to transfer SO-Rb50 cells,and the cells were then divided into RPMI-1640 culture group (untreated group),As2O3 + RPMI-1640 culture group (As2O3 treated group) and rapamycin culture group (positive control group).Autophagy of SO-Rb50 cells was examined by laser confocal microscope and monodansylcadaverine (MDC) influorescence staining,respectively,48 hours following cell culture.Ultrastructural features of autophagy were examined with transmission electron microscope (TEM).The percentage of autophagy positive cells in different concentrations of As2O3 treated groups was calculated with flow cytometer.Results The A570 values of SO-Rb50 cells were 2.194±0.066,1.841 ±0.213,1.035±0.046,0.374±0.042 and 0.167±0.019 in 0,0.5,1.0,2.0,4.0 μmol/L As2O3 treated groups,with a significant difference among these 5 groups(F=547.636,P＜0.05),and those of 0.5,1.0,2.0,4.0 μmol/L As2O3 treated groups were significantly reduced in comparison with untreated group (P =0.000).The positive granular spots for GFP-LC3 chimeric protein were seen to aggregate in autophagic vacuoles in the As2O3 treated group and positive control group,but diffuse cytoplasmic signal for GFP-LC3 was found in the untreated group.Normal ultrastructure of SO-Rb50 cells was exhibited in the untreated group,and many double-membrane-like bound vesicles and autlysosomes were documented in the As2O3 treated group and positive control group under the TEM.A lots of MDC fluorescence granule were found in the As2O3 treated group and positive control group rather than the untreated group.Flow cytometry showed that the percentages of SO-Rb50 cells were 0,15.6％,42.7％,57.9％,79.5％ and 89.0％ in the 0,0.5,1.0,2.0,4.0 μmol/L As2O3 groups and positive control group,respectively,showing a As2O3 concentration-dependent increase.Conclusions As2O3 can induce the autophagy of SO-Rb50 cells and inhibit the proliferation of SO-Rb50 cells.Autophagic response of SO-Rb50 cells appears prior to the nuclear change after exposed to As2O3.The degree of autophagy of SO-Rb50 cells is associated with As2O3 dose.

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.