Clinical analysis of primary lung tumors in 56 children / 中华肿瘤杂志

Objective: To investigate the clinical manifestation, pathological type, treatment and prognosis of primary lung tumors in children. Methods: We collected and retrospectively analyzed the clinical manifestation, pathological type, therapeutic method and prognosis of 56 primary lung tumors patients who diagnosed from 2009 to 2019 in Guangzhou Women and Children Medical Center. Results: There were 56 patients identified as the primary lung tumors, including pleuropulmonary blastoma (PPB, n=28), pulmonary inflammatory myofibroblastic tumor(IMT, n=20), mucoepidermoid carcinoma(n=6), infantile hemangioma (n=1), pulmonary sclerosing hermangioma(n=1). Respiratory symptoms were the most manifestation at the time of diagnosis including 26 patients with cough, 3 with hemoptysis, and 17 with dyspnea. Others included 15 with fever, 3 with chest pain, and 2 with epigastiric pain. The primary tumor of 18 cases were located in the lower lobe of left lung, 11 cases in the lower lobe of right lung, 10 cases in the upper lobe of left lung, 7 cases in the upper lobe of right lung, 6 cases in the middle lobe of right lung, and 4 cases in pulmonary hilum. Among the 56 patients, 41 patients underwent thoracotomy, 13 thoracoscopy, and 2 fiberoptic bronchoscopy. Five patients with type Ⅰ PPB were still alive at the end of follow-up without chemotherapy. Among 5 patients with type Ⅱ PPB, 2 patients without chemotherapy died after recurrence, 3 patients suffered postoperative chemotherapy were still alive at the end of follow-up. All of the 18 patients with type Ⅲ PPB underwent postoperative chemotherapy with IVADo regimen. Recurrence occurred in 6 cases, distant metastasis occurred in 3 cases, and cancer-related deaths occurred in 8 cases. For 20 patients with IMT, recurrence occurred in 5 of 13 patients experienced wedge resection, 1 of 6 patients experienced lobectomy and 1 of 6 underwent fiberoptic bronchoscopy, respectively. For 6 mucoepidermoid carcinoma patients, lobectomy was carried on 5 patients, wedge resection on 1 patient, all of them were still alive at the end of follow-up. One hermangioma patient underwent fiberoptic bronchoscopy and other 1 sclerosing hermangioma patient underwent wedge resection, both of them were still alive at the end of follow-up. Conclusions: The clinical manifestations of the primary lung tumors in children are nonspecific. Complete resection and achieving negative marginattribute to the excellent outcome. Adjunctive treatment such as chemotherapy is necessary for patients with type Ⅱ and type Ⅲ PPB.

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

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.

Pediatric lung lesions: a clinicopathological study of 215 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate clinical and pathological features of lung lesions in children.</p><p><b>METHODS</b>Clinical manifestations, radiologic imaging, histopathological features and immunohistochemical results were analyzed in 215 cases of lung lesions in children.</p><p><b>RESULTS</b>A total of 215 cases of lung lesions in children aged 0 day to 13 years (average age of 27.2 months and the median age of 18.0 months) were selected, including 137 male and 78 female patients with a male to female ratio of 1.76:1.00. The incidence of congenital lung disease was higher in patients of less than 1 year old than those of over 1 year old age, and the difference of the two groups was statistically significant (P = 0.004). 142 cases had acquired lung diseases, and 73 cases had congenital bronchopulmonary dysplasia. Lung abscess was the most common lesion seen in 86 cases (40.0%), including 1 case of fungal abscess. Congenital pulmonary airway malformation (CPAM) was the second most common, seen in 44 patients (20.5%), including 20 cases of type 1, 18 cases of type 2 and 6 cases of type 4 CPAM. Pulmonary sequestration was found in 25 cases (11.6%) including 14 cases of intralobar type and 11 cases of extralobar type. Two cases of extralobar pulmonary sequestration showed simultaneous CPAM2 type 2 lesion. Other lesions included tuberculosis (13 cases, 6.0%), emphysema (12 cases, 5.6%), interstitial pneumonia (7 cases, 3.2%), pulmonary hemorrhage (6 cases, 2.8%), bronchogenic cyst (4 cases, 1.9%), bronchiolitis obliterans (2 cases, 0.9%), idiopathic pulmonary hemosiderin deposition disease (2 cases, 0.9%) and 1 cases of lung non-specific changes. 13 cases of neoplastic lesions (6.0%) were found, of which 11 cases were primary tumors (5.1%), including inflammatory myofibroblastic tumor in 5 patients (2.3%), pleuropulmonary blastoma in 5 cases (1 case of type I, 2 type II and 2 type III) and 1 case of mucoepidermoid carcinoma (0.5%) and 2 cases of metastatic tumors (hepatoblastoma and Wilm's tumor, 0.9%).</p><p><b>CONCLUSIONS</b>Infectious diseases are the most common lung diseases in children. Congenital bronchopulmonary dysplasia is the most common in children of less than 1 year old. Malignant lesions are rare.</p>

Nasal chondromesenchymal hamartoma in an adolescent with pleuropulmonary blastoma

Nasal chondromesenchymal hamartoma [NCMH] is an extremely rare benign lesion arising in the sino-nasal tract. They usually affect children below one year of age. The pathogenesis of these lesions is poorly understood however, they have been associated with pleuropulmonary blastoma [PPB]. Although benign, malignant transformation has been reported in the literature. On literature review, we found 32 reported cases, most of them occurring in children below one year. We report a 14-year-old adolescent male with history of PPB at the age of 6, presenting with bilateral nasal obstruction and decreased sense of smell. In our case report we highlight the association between PPB and NCMH, and describe an unusual presentation

Childhood pleuropulmonary blastoma: a clinicopathologic study of 16 cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathological and immunohistochemical features, histogenesis and prognosis of pleuropulmonary blastoma (PPB) in children.</p><p><b>METHODS</b>PPB specimens from 16 pediatric cases with an age ranging from 1 year and 7 months to 5 years and 3 months (mean age of 3 years) were retrieved and analyzed by routine histological, immunohistochemical and electron methods.</p><p><b>RESULTS</b>Among 16 patients, there were 2 type I, 7 type II and 7 type III PPB cases. Type I PPB as multilocular cystic structure, consisted of thin fibrous wall lining the respiratory epithelium, subepithelial primitive blastema or immature mesenchymal cells, with or without rhabdomyoblastic differentiation or cartilage; Type II PPB as cystic-solid tumor, comparing with type I, consisted of intracystic components with appearance of anaplastic tumor cells. Type III PPB consisted of completely solid mass, the same as the solid region of type II, had mixed pattern including blastema, undifferentiated spindle-cell proliferations and sarcomas. In addition, anaplastic tumor cells and intra-and extra- cytoplasmic eosinophilic globules were also commonly present. Epithelial components in PPB were benign. Immunohistochemical study showed primitive mesenchymal differentiation of tumors. All cases were positive for vimentin, desmin, myogenin and SMA in tumors with skeletal muscle differentiation, S-100 was positive in tumors with cartilage differentiation. All tumors were negative for synaptophysin, CD99, and CD117. Benign epithelial components were positive for AE1/AE3 and EMA. In 12 cases, electron microscopy revealed few organelles in the primitive mesenchymal cells and rich heterochromatin in mesenchymal cells, the latter also demonstrating cytoplasmic myofilament dysplasia. Nine cases had clinical follow-up ranging from 5 to 48 months, of which 4 patients died.</p><p><b>CONCLUSIONS</b>PPB is a rare lung neoplasm of children under the age of 6 years, with distinct pathological morphology. PPB may arise from lung or pleura mesenchymal cells and has a poor clinical outcome.</p>

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.

A Case of Locally Advanced Well-Differentiated Fetal Adenocarcinoma of the Lung Treated with Concurrent Chemoradiation Therapy / 결핵및호흡기질환

Fetal adenocarcinoma is a rare adenocarcinoma subtype of pulmonary blastoma. A 48-year-old male patient is being referred to our hospital due to progressive dyspnea. A chest X-ray showed a lung mass of unknown origin that was obstructing the right main bronchus. After relieving the airway obstruction with stent insertion via bronchoscopy, a diagnosis of fetal adenocarcinoma is being confirmed through thoracoscopic biopsy. Due to the locally advanced state of the lung cancer, it seemed to be inoperable, and concurrent chemo-radiation therapy was being administered with docetaxel. The stent was removed after improvements in the airway obstruction followed by a lung mass shrinkage. Comparing to other contexts which describe fetal adenocarcinoma as lower grade malignancy with low-associated mortality, herein, we describe a case of locally-advanced fetal adenocarcinoma (T4N3M0). This is the first documented case being treated with concurrent chemoradiation therapy. The followed-up image studies represent a partial response and the patient is currently under further observations.

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.

Pleuropulmonary blastoma in children: a case report

Pleuropulmonary Blastoma [PPB] is a very rare, highly aggressive and malignant tumor that originates from either the lungs or pleura. It occurs mainly in children aged less than five or six years. It has poor prognosis with three different subtypes: cystic [type I], combined cystic and solid [type II] and solid [type III]. PPB is treated with aggressive multimodal therapies including surgery and chemotherapy. We present a case of 3.5 years old boy with PPB type II successfully treated with complete surgical resection followed by neoadjuvant chemotherapy

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.

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: a clinicopathological analysis / 中国肺癌杂志

<p><b>BACKGROUND AND OBJECTIVE</b>Pleuropulmonary blastoma (PPB) is a rare malignant tumor with unique clinicopathological features. The aim of this study is to investigate the clinicopathological features, the diagnosis and differential diagnosis of pleuropulmonary blastoma.</p><p><b>METHODS</b>Five cases of PPB were analyzed by light microscopy, immunohistochemistry and their clinical data, and the relative literatures were reviewed.</p><p><b>RESULTS</b>Five cases of patients suffered from PPB were aged from 21 to 47 months (mean 32.8 months). Most of the masses were located in the thoracic cavities and 4 cases accompanied with pleural effusions. Histologically, these tumors included 1 case of type I PPB which showed pure cystic architecture; 2 cases were type II PPB which showed cystic and solid masses accompanied with rhabdomyoblastic differentiation and nodules of cartilage; the other 2 cases were type III PPB and characterized by absolute solid masses with anaplastic undifferentiated sarcomatous components. Immunohistochemical studies showed that tumor cells were positive for Vimentin and some for Desmin and Myogenin, the nodules of cartilage were positive for S-100. The tumor cells were negative for PCK, EMA and CD99.</p><p><b>CONCLUSION</b>Pleuropulmonary blastoma is a rare and highly aggressive malignancy arising in the lung and pleural of infancy and early childhood. The type I, II and III PPB have unique clinicopathological features respectively. This kind of tumor should be distinguished from some benign and malignant diseases such as congenital cystic adenomatoid malformation (CCAM) and embryonal rhabdomyosarcoma.</p>

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.

Primary Neoplasms of the Lung in Children and Adolescents: 22 Cases from a Single Institute

PURPOSE: Primary lung tumors in children and adolescents are uncommon. We report here on the clinical presentations, the methods of treatment, the results and the outcomes of a series of children with these rare tumors. MATERIALS AND METHODS: We conducted a retrospective review on all the patients less than 19 years of age with primary lung tumors and who were treated at Samsung Medical Center between 1995 and 2009. Twenty two cases of primary lung tumors were reviewed and the clinical-pathological information concerning the tumor characteristics, the therapy and the follow-up was collected. RESULTS: The average age of the 10 male patients and 12 female patients was 11.5 years (range: 3 months to 18 years). Of the 22 primary lung tumors, 18.2% (4/22) were benign, 36.4% (8/22) were of borderline malignancy and 45.4% (10/22) were malignant. The pathologic types were inflammatory myofibroblastic tumor (6/22), mucoepidermoid carcinoma (4), pleuropulmonary blastoma (2), adenocarcinoma (1), alveolar soft part sarcoma (1), malignant lymphoma (1), leiomyoma (1), pulmonary leiomyomatous hamartoma (1), congenital pulmonary myofibroblastic tumor (1), Langerhans' cell histiocytosis (1), neurilemmoma (1), sclerosing pneumocytoma (so-called sclerosing hemangioma) (1) and atypical carcinoid tumor (1). After surgery for the benign tumors, no postoperative recurrence was been observed during long-term follow-up. All the patients with the benign tumors were alive with no evidence of disease. For the patients with the tumors of borderline malignancy, one patient experienced recurrence, but there was no death from disease. For the group with malignant tumors, two patients died from untreatable metastatic disease and 8 patients survived their disease on the long-term follow-up. CONCLUSION: The primary lung tumors during childhood and adolescence had different characteristics compared with their adult counterparts with respect to the incidence and prognosis. These facts must be considered when making the diagnosis and deciding on proper therapy.