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.

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.

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.

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 of Huge Pulmonary Blastoma With Multiorgan Invasion / 결핵및호흡기질환

A pulmonary blastoma is a rare malignant tumor of the lung that is composed of epithelial and mesenchymal elements and resembles the structure of an embryonic lung. Pulmonary blastomas have a very poor prognosis and make up 0.25 to 0.5 percent of all primary malignant lung tumors. A pulmonary blastoma usually manifests as a solitary parenchymal mass or nodule and multiple subpleural mass with effusion on chest X-ray and computed tomography. We encountered a very rare case of pulmonary blastoma in a 52 years old male. He complained of abdominal pain, fullness, and dyspnea. The radiology examination revealed a huge lung mass invading the mediastinum, heart, diaphragm, and liver. The percutaneous needle biopsies were performed, and this tumor was diagnosed as a pulmonary blastoma. We report a biopsy confirmed case of a huge pulmonary blastoma invading multiple organs.

Fatal Aortic Tumor Embolism Presenting as Acute Paraplegia

We report a case of fatal aortic tumor embolism presenting as acute paraplegia. A four-year-old girl was referred from a local hospital with sudden paraplegia and a poor medical condition. A neighbor had noticed her fall from a bike, and she could not walk. She had no previous illness. Emergency spine MRI revealed no remarkable findings. During the process of evaluation, her general condition deteriorated progressively. Chest and abdominal CT showed a large mass in the left lung field, and a diagnosis of aortic occlusion was made. An emergency transfemoral embolectomy was attempted. However, the patency of the aorta was not recovered. On pathological examination of tissues taken from the embolectomy, a pleuro-pulmonary blastoma was found. The patient died 22 hours after the onset of her symptoms. We describe a possible mechanism for the tumor embolism. To the best of our knowledge, this is the first case report of aortic occlusion caused by an embolic malignancy, presenting as acute paraplegia.

Biphasic Pulmonary Blastoma detected by Pneumothorax / 결핵

Pulmonary blastoma is a rare primary lung neoplasm composed of a mixture of immature mesenchyme and epithelium that morphologically mimics embryonal pulmonary structure. Microscopically, tumors could be divided into two classes; those composed solely of malignant glands of epithelial components (well-differentiated fetal adenocarcinoma) and those with biphasic appearance. A 51-year-old man was admitted with left sided pneumothorax. After closed thoracostomy, chest X-ray showed a circular mass in the left lower zone. The tumor was diagnosed as adenocarcinoma as a result of percutaneous needle aspiration biopsy, and left lower lobectomy was performed. The patient was diagnosed as having biphasic pulmonary blastoma on postoperative histological assessment. This case is the first reported patient with biphasic pulmonary blastoma detected by pneumothorax.

Pleuropulmonary Blastoma in a Young Adult Presenting as a Ruptured Cystic Teratoma in Radiology

Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting children and is characterized histologically by a variably mixed blastematous and sarcomatous patterns. We herein report a very exceptional adult case of PPB. A 21-yr-old male patient presented with a left chest pain of two weeks' duration. A computed tomography scan revealed a large, multicystic tumor occupying the left lower hemithorax, leading to the impression of a ruptured mediastinal cystic teratoma. A thoracotomy for resection of the tumor was performed. On histologic examination, the tumor consisted of cystic walls and associated solid lesions which showed undifferentiated blastemal tissues with focal fibrosarcomatous and rhabdoid features. Immunohistochemically the tumor cells only showed diffuse strong positivity for vimentin. The histologic findings corresponded to a type II PPB. The authors suggest that PPB, especially of type I or II, should be included in the radiologic differential diagnosis of mediastinal cystic neoplasms in a young adult.

Incidentally Founded Biphasic Pulmonary Blastoma / 결핵

Pulmonary blastoma is a family of tumors in which the glands or mesenchyme composing the neoplasm are primitive or embryonic in appearance. There are three subtypes, which include well differentiated fetal adenocarcinoma (pulmonary endodermal tumor), biphasic pulmonary blastoma, and cystic and pleuropulmonary blastomas in children. Among them, biphasic pulmonary blastoma is a primary malignancy of the lung originating from multipotential pulmonary blastema including both the malignant fetal epithelial and mesenchymal components. These make up 0.25 to 0.5 percent of all primary malignant lung tumors. This tumor is usually symptomatic and appears as a large, solitary peripheral mass, with a tendency to favor the upper lobe. Here we report a case where small sized asymptomatic peripheral lung mass was diagnosed as a biphasic pulmonary blastoma, prior to the operation, A subsequent percutaneous needle biopsy was performed, which revealed features of a large cell neuroendocirne tumor. In addition, a review of the relevant literature is provided.

CT Diagnosis of Pulmonary Blastoma / 实用放射学杂志

Objective To evaluate the efficacy of CT in diagnosis of pulmonary blastoma (PB).Methods 10 patients with surgically and pathologically proved PB were collected in this study,and CT findings of the tumors were retrospectively analyzed. Results Of 10 cases,1 were central form (the tumors localized under pleura) and 9 was periphery form.The tumors were localized at right lobus in 6( including upper lobus,middle and lower lobus in 1,1 and 4,respectively) and left lobus in 4(upper lobus in 3 and lower lobus in one).The lesions were more than 3 cm in diameters in all cases.Necrosis of tumors were seen in 7 cases.The lesions had obvious enhancement after intravenous injection of contrast medium.Enlarged lymph nodes were identified at hilum of the lung and mediastinum in one,bony destruction and distant metastases were seen in one and 2,respectively.Conclusion PB is not of characteristic CT features,CT-guided percutaneous needle biopsy and the immunohistochemical method are helpful to the diagnosis of pulmonary blastoma.

A Case of Biphasic Pulmonary Blastoma / 결핵

Pulmonary blastoma is a primary lung tumor consisting of a mixture of immature embryonal-like mensenchymal and epithelial components and in some areas resemble embryonal lung tissue of up to 3-4 months gestational age. The tumor was first described in 1945 by Barrett and Barnard and again in 1952 by Barmard, who named it an embryoma of the lung which resembled fetal lung histologically, with glandular structures lined by non-ciliated epithelium and a surrounding stroma resembling mesenchyme. In 1961 Spencer renamed them Pulmonary blastoma as he believed they represented neoplasms similar in pathogenesis to nephroblastoma. The prognosis of these tumors is poor, and the clinical course is not readily predicted from histological appearance. Untill now, more than one hundred cases have been reported in the literature in the word and there is no report in Korea. We report a case of biphasic pulmonary blastoma, which ruptured spontaneously, in a 31-year-old young man who was presented as sharp pain under the right subscapular area.

A Case of Biphasic Pulmonary Blastoma / 결핵

Pulmonary blastoma is a primary lung tumor consisting of a mixture of immature embryonal-like mensenchymal and epithelial components and in some areas resemble embryonal lung tissue of up to 3-4 months gestational age. The tumor was first described in 1945 by Barrett and Barnard and again in 1952 by Barmard, who named it an embryoma of the lung which resembled fetal lung histologically, with glandular structures lined by non-ciliated epithelium and a surrounding stroma resembling mesenchyme. In 1961 Spencer renamed them Pulmonary blastoma as he believed they represented neoplasms similar in pathogenesis to nephroblastoma. The prognosis of these tumors is poor, and the clinical course is not readily predicted from histological appearance. Untill now, more than one hundred cases have been reported in the literature in the word and there is no report in Korea. We report a case of biphasic pulmonary blastoma, which ruptured spontaneously, in a 31-year-old young man who was presented as sharp pain under the right subscapular area.

A Case of Pulmonary Blastoma / 결핵

Pulmonary blastoma is a rare lung tumor resembling fetal lung tissue. Pathologically the tumor can be classified to 2 groups, well-differentiated fetal adenocarcinoma(WDFA) and biphasic blastoma. WDFA has more favorable prognosis with fewer metastasis at initial presentation and fewer recurrence after treatment. We experienced a case of pulmonary blastoma in 32-year-old female patient. The patient was referred to our hospital because of abnormal mass shadow in right middle lobe. The diagnosis of pulmonary blastoma(WDFA type, Stage I T2NOMO) was confirmed after right middle lobectomy. We followed up 22 months without an evidence of recurrence.

Pulmonary Blastoma with Rhabdomyoblastic Differentiation: A case report with immunohistochemical and electron microscopic examination

Pulmonary blastoma is a rare lung tumor composed of epithelial and mesenchymal element : the latter element may show various pattern of differentiation toward mature tissue, such as cartilage, smooth muscle, and bone. Rhabdomyoblastic differentiation in pulmonary blastoma is quire rare. In th literature, only seven cases have been reported. We report a case of pulmonary blastoma with rhabdomyoblastic differentiation which occured in a 3 year old girl. Microscopically, cytoplasmic cross-striation was present. Immunohistochemically, strong positivity for vimentin and desmin was observed. Electron microscopy demonstrated A and I bands which documented rhabdomyoblastic differentiation.

Pulmonary Adenocarcinoma of Fetal Type: Report of a case

Adenocarcionma of fetal type is in lung is a newly recognized malignant tumor sharing morphologic features with the epithelial component of the pulmonary blastoma devoid of sarcomatous component. We present a case of adenocarcinoma of fetal type in a 28-year-old female, consisting of numerous branching tubules or glands and morula-like epithelial complexes. Histologically, the tubules and glands were composed of glycogne-rich nonciliated epithelial cells showing in part argyrophilia. Some of tubular and morula-like epithelial cells revealed immunoreactivity for neuron-specific enolase. We report this case with a review of literatures with special references on the histogenisis. This report is the pathologically confirmed second case of the pulmonary adenocarcinoma of fetal type in Korea, following the report of Cho and Lee, 1990.

Pulmonary Adenocarcinoma of Fetal Type: Report of a case

Pulmonary adenocarcinoma of fetal type is a very uncommon tumor of the lung which simulates an early stage of lung differentiation. This is a primitive appearing epithelial tumor similar to the epithelial component of pulmonary blastoma but lacking the sarcomatous features. Since the report of Kradin et al, 8 more cases have been reported by a variety of name. These tumors are composed of glycogen-rich, non-ciliated tubular epithelial cells forming irregularly shaped tubules or arranged in a papillary pattern. A very remarkable findings of this tumor is the presence of endocrine cells which is confirmed by argyrophilia, immunohistochemistry or electron microscopy. We experienced a case of this tumor which showed hepatocytoid differentiation in addition to the characteristic histologic findings. Immunohistochemical studies performed on a resected tumor tissue showed immunoreactivity for alpha-fetoprotein, neuron-specific enolase and somatostatin, and endocrine type granules were found ultrastructurally. Although this tumor seems to have a relation with pulmonary blastoma in its histology, immunohistochemistry and ontogeny, a distinction between these should be attained because the average survival of the former group is longer as 23 months, while that of the latter is only 4 months.