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선천성 낭선종성 기형이 의심되었던 환아에서 진단된 흉막폐모세포종 1례

A Case Report of a Patient with Pleuropulmonary Blastoma Presenting as Congenital Cystic Adenomatoid Malformation

임호준|김효빈|홍수종|송준섭|우성일|구현령
대한 소아알레르기 호흡기학회 (The Korean Academy Of Pediatric Allergy And Respiratory Disease)|2006.06|Allergy Asthma & Respiratory Disease (알레르기 천식 호흡기질환)|제16권 제2호|pp. 177-182

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참고문헌

주제분류소아과학

초록

저자들은 이전에 증상이 없던 16개월 여아에서 선천성 낭선종성 기형 의심하에 수술적 절제 후 병리학적 검사에서 흉막폐모세포종으로 진단된 증례를 경험하였기에 보고하는 바이며, 선천성 낭선종성 기형이 의심되는 경우 증상의 동반 여부와 관계없이 확진과 발생할 수 있는 합병증의 예방, 악성 종양 발생 가능성을 없애기 위해 수술적 절제가 필요할 것으로 생각한다.

Abstract

Congenital cystic adenomatoid malformation(CCAM) is one of the most common congenital lung lesions. Clinical manifestations that show are neonatal respiratory distress, recurrent respiratory infection, pneumothorax, and hemothorax. But, there are patients who are asymptomatic until mid-childhood. The treatment of asymptomatic CCAM is controversial. There is a possibility to resolve it spontaneously, but late complications such as recurrent pulmonary infection, pneumothorax, hemothorax, and cancer, which includes bronchoalveolar carcinoma and rhabdomyocarcinoma, pleuropulmonary blastoma still remain. Some investigators advocate routine surgery for all cases of CCAM that are apparent at birth. A previously healthy 16- months-old girl who had suffered from a cough for 2 weeks was transferred to Asan Medical Center with CCAM. Due to a chest CT and fever, we first thought that she had CCAM with infection. After we treated her with antibiotics for one week, we performed surgery to confirm the diagnosis and to prevent further complication. But by surgical wedge resection, a pleuropulmonary blastoma was found. There were no evidence of metastasis and adjacent involvement. She started her chemotherapy with vincristine, actinomycin D and cyclophosphamide, and is now continuing maintenance chemotherapy with etoposide, vincristine, and Ifosfamide. We report pleuropulmonary blastoma that presented as CCAM. So we recommend surgical resection in asymptomatic CCAM to confirm the diagnosis and to prevent its malignant transformation, even not accompanied by symptoms.

참고문헌 (22)

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관련 논문AI추천

산전 진단된 선천성 낭종성 선종양 폐기형 1예김석배, 박재동, 박학열, 손경락, 안광준, 이홍우대한산부인과학회 · Obstetrics & Gynecology Science2003 · 제46권 제10호선천성 낭성 선종양 폐기형의 임상적 고찰박희주, 전소은대한 소아알레르기 호흡기학회 · Allergy Asthma & Respiratory Disease2008 · 제18권 제2호조기수술이 필요한 폐 선천성 낭포성 샘 모양 기형아의 특성과 수술 전 중재적 시술김암, 김기수, 김동관, 김애란, 김현경, 서영은, 심재윤, 원혜성, 윤종현, 이병섭, 이필량, 최용성대한신생아학회 · Neonatal medicine2013 · 제20권 제1호흉막 삼출을 동반한 흉선 성숙 기형종 1례강보훈, 김규언, 김명준, 김세훈, 손명현, 윤지현대한 소아알레르기 호흡기학회 · Allergy Asthma & Respiratory Disease2005 · 제15권 제1호태아의 횡격막을 가로지르는 외엽형 폐 격리증과제 2형 선천성 낭종성 선종양 폐기형의 혼합기형 1예김지영, 배성근대한산부인과학회 · Obstetrics & Gynecology Science2003 · 제46권 제8호소아 폐과오종 1례류민혁, 이동진, 이상배, 이선영대한 소아알레르기 호흡기학회 · Allergy Asthma & Respiratory Disease2003 · 제13권 제1호

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