• Journal of Chest Surgery
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• v.37 no.11
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• pp.959-962
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• 2004

Pleuropulmonary blastoma is a rare malignant neoplasm which originates from either the lungs or pleura. Pleuropulmonary blastoma usually develops in the first decade of life, mostly younger than 5 years old and shows aggressive biological behavior. Pleuropulmonary blastoma is discriminated from classic pulmonary blastoma of adulthood by its morphological features like primitive mesenchymal and sarcomatous component without carcinomatous portions. To our knowledge, report of pleuropulmonary blastoma in adulthood is very rare. Our case support the possibility that primitive neoplasm recognized as pediatric tumors can develop in adulthood. We report a case of surgical experience of pleuropulmonary blastoma which developed in 21 years old man with literature review.

• Journal of Chest Surgery
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• v.36 no.8
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• pp.614-618
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• 2003

Pleuropulmonary blastoma (PPB) is a rare intrathoracic neoplasm, found solely in childhood. The usual symptoms are dyspnea, chest discomfort, recurrent respiratory infections, fever, dry cough, and chest pain. The progress of PPB is usually aggressive and its progress is generally poor. Lymphatic spread to the hilar and mediastinal nodes can occur Distant metastasis is found in brain, bones, and intra-abdominal organs. Surgical resection is the treatment of choice. When the disease Is too extensive for surgical resection, neoadjuvant chemotherapy can be used. We report 2 cases of pleuropulrnonary blastoma in children successfully treated with multimodal therapy.

• 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.

• 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.

• 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.

• Tuberculosis and Respiratory Diseases
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• v.50 no.5
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• pp.641-644
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• 2001

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 a 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 neuroendocrine tumor. In addition, a review of the relevant literature is provided.

• Clinical and Experimental Pediatrics
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• v.61 no.1
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• pp.30-34
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• 2018

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 ($GGT{\rightarrow}GAT$), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia. Mucinous adenocarcinoma is the most common lung tumor associated with CPAM, but it generally occurs in older children and adults. Further, all cases in the literature are of type I CPAM. This case in a neonate indicates that malignant transformation can occur very early in type II CPAM.