• Journal of Chest Surgery
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• v.39 no.9 s.266
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• pp.722-724
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• 2006

Pleuropulmonary bastoma (PPB) is a rare intrathoracic malignant neoplasm in children, differ from pulmonary blastoma in adults. PPB is usually aggressive and has wide-metastases at the time of diagnosis. The therapeutic medality of PPB is extensive surgical resection with neoadjuvant or adjuvant chemotherapy. We report a case of a cystic pieuropulmonary blastoma treated with surgical resection and adjuvant chemotherapy.

• The Korean Journal of Cytopathology
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• v.12 no.1
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• pp.39-43
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• 2001

Pleuropulmonary blastoma (PPB) is an unusual intrathoracic blastoma presenting in childhood and charac-terized 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 cytoiogy if appro-priate clinical information were given. Timely and accurate diagnosis of PPB by cytology paves the way for attempting preoperative treatment in future cases.

• Radiation Oncology Journal
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• v.38 no.2
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• pp.148-150
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• 2020

Pleuropulmonary blastoma (PPB) is a rare intrathoracic neoplasm in children. Although surgery with or without chemotherapy mainly conducted, the response of radiotherapy (RT) has not been evaluated yet. For unresectable tumor, RT might be considered as one option to decrease tumor extent to relieve obstructing symptoms or to facilitate successive treatment. We report one child in whom PPB with DICER1 mutation recurred after surgery and lead to respiratory distress. She emergently received palliative RT with a relatively low dose (20 Gy), and symptoms sufficiently relieved. Even she showed an 84.3% reduction in diameter and maintained the remission status for 1 year. These might reflect possible radiosensitivity of PPB, and further investigations of RT might be necessary for unresectable PPB.

• Journal of Chest Surgery
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• v.22 no.2
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• pp.357-363
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• 1989

We experienced very rare case of pulmonary blastoma in a 5 year old girl. She complained of right chest pain and productive cough for 3 months. With computerized tomography and echocardiography it is disclosed that huge mediastinal solid tumor is occupied to whole right thoracic cavity and compressed mediastinal structures to left and extended to left atrium. We removed the thoracic tumor and its extended intracardiac portion completely using the technique of intrapericardial pneumonectomy with cardiopulmonary bypass. Postoperatively the patient recovered without any problem and received adjuvant chemotherapy and radiotherapy. But she was succumbed 2 months later because of opportunistic pulmonary infection with pneumocystis carinii.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.774-778
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• 1996

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 Barren and Barmard 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 rumors is poor, and the clinical course is not readily predicted from histological appearance. Untill now, more than one hundred cases have been reponed 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.

• Journal of Korean Neurosurgical Society
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• v.39 no.1
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• pp.72-74
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• 2006

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.

• Korean Journal of Bronchoesophagology
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• v.2 no.2
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• pp.248-252
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• 1996

Pulmonary blastoma is rare malignant neoplasm usually situated peripherally in lung and composed of epithelial and mesenchymal components that resemble the lung at about 3 months gestation. Fortysix year-old man had no symptoms and signs, but solitary pulmonary nodule was found accidentally in a routine simple chest X-ray occupying right upper lung field. Preoperative chest CT-guided aspiration cytology revealed adenocarcinoma. We had done right upper lobectomy with Iymph node dissecton therapeutically. Final postoperative pathology reported this tumor as double pulmonary blastomas(well differentiated fetal adenocarcinoma). We report a case of pulmonary blastoma with review of literature.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.388-391
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• 2009

Well-differentiated fetal adenocarcinoma (WDFA) of the lung is a rare lung tumor that's composed of glycogen rich neoplastic glands and tubules that resemble the fetal lung at 10 to 15 weeks of gestation. Although WDFA is classified as a subtype of pulmonary blastoma or pulmonary adenocarcinoma, its prognosis is better than conventional pulmonary blastoma (biphasic blastoma) or pulmonary adenocarcinoma. It is important to identify this low grade malignancy tumor in younger patients because it is associated with low mortality. We report here on 3 cases of WDFA and the patients are all currently alive without tumor recurrence.

• Journal of Chest Surgery
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• v.34 no.7
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• pp.566-568
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• 2001

WDFA(Well-differentiated fetal adenocarcinoma) histologically resembles pulmonary blastoma. It is also thought to be a subtype of pulmonary blastoma, which has differentiated epithelial features resembling the fetal lung among its epithelial features and sarcomatous features. We recently encounted a patient who underwent surgery for WDFA. A 19-year-old man had a mass shadow in the upper lobe of the left lung. The tumor was diagnosed as pulmonary blastoma as a result of fine needle aspiration biopsy, and left upper lobe lobectomy was performed. No sarcomatous features was observed on postoperative histologic assessment, and the patient was diagnosed as having WDFA. This case is reported with a discussion of the literatures.

• The Korean Journal of Cytopathology
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• v.8 no.1
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• pp.69-75
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• 1997

Well differentiated fetal adenocarcinoma of the lung Is a subtype of pulmonary blastoma. In this report, CT-guided fine needle aspiration smears were performed at the right upper lobe of the lung in a 45 year-old male patient who had the smoking history of one pack per day for 25 years. The smears disclosed round, papillary, and tubular patterns of cell clusters. The individual cells had relatively uniform, small to medium sized nuclei without nucleoli, and showed vesicular or eosinophilic cytoplasm with Indistinct cell border. The morules were seen in the central area of papillary clusters. They were composed of two cell types, outer single layered cuboidal cellular lining and central three-dimensional cluster of cells simulating fetal lung. These cytologic features need to be differentiated from usual pulmonary adenocarcinoma, carcinoid, and pulmonary blastoma. On histologic findings, the tumor arised in the bronchial epithelium. And the tumor cells had abundant intracytoplasmic glycogen with neuroendocrine feature on histochemical study. In addition, the multiplicity of this tumor is the unique point comparable to the previous reports.