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Exceptional response to radiotherapy in unresectable pleuropulmonary blastoma of a child

Radiation Oncology Journal 2020년 38권 2호 p.148 ~ 150
김재식, 이주호,
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김재식 ( Kim Jae-Sik ) - Seoul National University Hospital Department of Radiation Oncology
이주호 ( Lee Joo-Ho ) - Seoul National University Hospital Department of Radiation Oncology

Abstract


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.

키워드

Child; Radiotherapy; Pleuropulmonary blastoma

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