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.

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.

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.

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.

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.

A Case Report of a Patient with Pleuropulmonary Blastoma Presenting as Congenital Cystic Adenomatoid Malformation / 소아알레르기및호흡기학회지

Congenital cystic adenomatoid malformation(CCAM) is one of the most common congenital lung lesions. Clinical manifestations that show are neonatal respiratory distress, recurrent respiratory infection, pneumothorax, and hemothorax. But, there are patients who are asymptomatic until mid-childhood. The treatment of asymptomatic CCAM is controversial. There is a possibility to resolve it spontaneously, but late complications such as recurrent pulmonary infection, pneumothorax, hemothorax, and cancer, which includes bronchoalveolar carcinoma and rhabdomyocarcinoma, pleuropulmonary blastoma still remain. Some investigators advocate routine surgery for all cases of CCAM that are apparent at birth. A previously healthy 16-months-old girl who had suffered from a cough for 2 weeks was transferred to Asan Medical Center with CCAM. Due to a chest CT and fever, we first thought that she had CCAM with infection. After we treated her with antibiotics for one week, we performed surgery to confirm the diagnosis and to prevent further complication. But by surgical wedge resection, a pleuropulmonary blastoma was found. There were no evidence of metastasis and adjacent involvement. She started her chemotherapy with vincristine, actinomycin D and cyclophosphamide, and is now continuing maintenance chemotherapy with etoposide, vincristine, and Ifosfamide. We report pleuropulmonary blastoma that presented as CCAM. So we recommend surgical resection in asymptomatic CCAM to confirm the diagnosis and to prevent its malignant transformation, even not accompanied by symptoms.

Two Cases of Pleuropulmonary Blastomas in Children / 대한소아혈액종양학회지

Pleuropulmonary blastoma (PPB) is a rare primary malignant neoplasm with poor prognosis in children. PPB originates from the lung, the pleura, or the mediastinum. Histologically, it is characterized by a primitive, mixed blastematous, sarcomatous appearance and the absence of epithelial cell. Initial presenting symptoms are cough, fever and dyspnea. We experienced two cases of PPB (type I and type II). Complete surgical removal is always required for the treatment and chemotherapy and radiotherapy is needed as adjuvant therapy. We report two cases of pleuropulmonary blastoma treated with surgical removal, chemotherapy (vincristine, actinomycin D, cyclophosphamide) and radiotherapy.

Blastoma Pleuropulmonar

Introducción. El blastoma pleuropulmonar es una neoplasia disembriónica rara en pediatría. Ocurre habitualmente en menores de 6 años de edad. Existen 3 tipos: I, componente quístico; II, mixto; y el III, sólido. Su comportamiento clínico y biológico es agresivo. Caso clínico. Paciente femenina de 7 meses de edad, que 3 meses previos a su ingreso presentó afección respiratoria, tratada como neumonía y derrames, sin mejoría clínica ni radiológica. Ingresó con dificultad respiratoria, hipomovilidad del hemitórax derecho, matidez y ausencia de ruidos respiratorios. La tomografía axial computada de tórax mostró masa ocupativa de más de 90% del hemitórax derecho, heterogénea, con predominio de componente sólido sobre el quístico. Se realizó lobectomía media derecha y el reporte histopatológico fue de blastoma pleuropulmonar tipo II y III. Quince días después del procedimiento quirúrgico la paciente falleció sin evidencia radiológica de complicación pulmonar postoperatoria. Conclusión. Se presenta el segundo caso de blastoma pleuropulmonar de tipo II y III a edad tan temprana; el involucramiento de la pleura y mediastino probablemente determinó su mala evolución. Por lo que se considera importante la resección temprana de lesiones pulmonares quísticas.

Introduction. The pleuropulmonary blastoma is a rare malignant lung neoplasm usually observed in children younger than 6 years old. There are 3 types of pleuropulmonary blastoma: I cystic, III solid, and II mixed. Its clinical and biological behavior is aggressive. Case report. A female patient, 7 months old who 3 months previous to admission presented respiratory symptoms treated as neumonia and spillings, without clinical or radiological improvement. She was admitted with respiratory difficulty, hypomobility of the right hemitorax, and absence of respiratory sounds; a CAT scan of the thorax showed a lump that occupied more than 90% of the right hemitorax, heterogenous, with predominance of solid components. A resection of the middle right lobe was carried-out; the histopathologic studied disclosed a pleuropulmonary blastoma type II-III; the patient died 15 days after surgery. Conclusions. We present a case of pleuropulmonary blastoma type II-III at an early age. The involvement of the pleura and mediastinum probably determined its poor evolution. We consider that early resection of pulmonary lesions is advisable.

Imprint Cytologic Feature of Pleuropulmonary Blastoma: A Case Report / 대한세포병리학회지

Pleuropulmonary blastoma (PPB) is an unusual intrathoracic blastoma presenting in childhood and characterized by a biphasic neoplastic population of undifferentiated, small round blastemal cells and larger spindle-shaped sarcomatous cells with entrapped benign epithelial-lined structures. We experienced the cytologic features of PPB in imprint smear from the pleural-based huge mass of the middle lobe of the right lung in a 4-year-old boy. The smears showed high cellularity composed of small ovoid blastemal elements and scattered spindle mesenchymal tumor cells. Lobectomy and pathologic investigation confirmed the diagnosis. PPB seems to be a tumor in which accurate diagnosis may be achieved by cytology if appropriate clinical information were given. Timely and accurate diagnosis of PPB by cytology paves the way for attempting preoperative treatment in future cases.

A Case Report of Pleuropulmonary Blastoma in Childhood

Classic pulmonary blastoma is a variant of carcinosarcoma which is seen almost exclusively in adults. By contrast, most cases of pulmonary blastoma in children have been described as having an exclusive mesenchymal composition, which was proposed as pleuropulmonary blastoma. Recently we experienced a case of pleuropulmonary blastoma, type 1. This 27-month-old male baby was transferred to our hospital due to the left tension pneumothorax. Chest CT revealed a subpleural pulmonary cystic lesion on the left upper lung and an open cystectomy was performed. Histologically the lesion was composed of variable-sized cystic structures lined with a single layer of respiratory-type epithelium. the underlying stroma was composed of sheets of small, round to oval, primitive tumor cells. Some of them had eccentric, eosinophilic cytoplasm, suggestive of rhabdomyoblastic differentiation. These rhabdomyoblastic cells were fuchsinophilic and positive with desmin and vimentin on immunohistochemistry.