• Journal of Chest Surgery
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• 제37권11호
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• pp.959-962
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• 2004

흉막폐아세포종은 주로 5세 이하의 소아의 폐나 흉막에서 발생하는 드문 악성종양으로, 매우 빠른 성장으로 예후가 불량하다. 흉강 내의 원발성 태생 신생물인 성인형 폐아세포종은 악성 모세포, 상피세포와 간엽세포 모두를 가지고 있지만 흉막폐아세포종은 악성 상피세포를 보이지 않는 것이 특징이다. 흉막폐아세포종이 성인에서 발견된 경우는 매우 드물어 단지 수 예가 보고되어 있다. 21세의 남자에서 발생한 흉막폐아세포종 1예를 수술 치험하였기에 문헌고찰과 함께 보고한다.

• Journal of Chest Surgery
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• 제36권8호
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• pp.614-618
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• 2003

흉막폐아세포종은 소아에 국한하여 생기는 매우 드문 원발성 악성종양이며 극히 나쁜 예후를 보인다. 주 증상은 흉부불쾌감, 호흡곤란, 반복적인 상기도 감염, 발열, 마른기침, 그리고 흉통 등이다. 흉막폐아세포종은 매우 빠른 진행양상을 보이며, 폐문 및 종격 림프절에 전이될 수 있다. 원격전이는 뇌, 골조직, 그리고 복강 내 장기들에서 보인다 흉막폐아세포종의 치료는 다각적 접근을 요한다 수술에 의한 종괴의 일차적 제거가 우선적인 치료법이나, 종양의 크기나 침범 범위로 인해 일차적으로 수술적 제거가 힘든 경우 수술 전 신보조항암요법으로 종양의 크기를 줄일 수 있으며, 이후 수술적인 완전절제를 고려할 수 있다. 본원에서는 소아에서 발생한 흉막폐아세포종에 대해 신보조항암요법, 수술적 절제 및 보조항암요법을 통해 성공적으로 치료한 2예를 경험하고 이를 보고하고자 한다.

• Radiation Oncology Journal
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• 제38권2호
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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.

• 대한세포병리학회지
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• 제12권1호
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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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• 제39권9호
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• pp.722-724
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• 2006

흉막폐아세포종은 소아에서 발생하는 아주 드문 흉강 내 원발성 종양으로 폐아세포종과는 다른 병리학적 소견을 가진다. 수술적 완전 절제가 우선적인 치료법이며, 빠른 진행 및 전이를 보이기 때문에 신보조항암요법, 보조항암요법 등의 다각적인 방법을 고려하여야 한다. 저자들은 수술적 절제 및 보조항암요법으로 치료한 흉막폐아세포종 1예를 보고한다.

• Tuberculosis and Respiratory Diseases
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• 제50권5호
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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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• 제61권1호
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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.