CAJ | 학술논문

目的探讨儿童胸膜肺母细胞瘤的误诊原因.方法将1979年1月至2007年1月临床病理证实的小儿胸膜肺母细胞瘤32例,根据病理切片、免疫组织化学按Dehner分型分为Ⅰ、Ⅱ、Ⅲ三型;根据不同分型的X线和CT特点,结合临床表现,分析初诊诊断,总结漏诊原因,并对其存活率进行分析.结果 32例患儿平均年龄(4.0±2.7)岁(3个月～10岁),男女比例为3:2.32例患儿最早出现的症状均为咳嗽,28例伴发热,最终有18例伴呼吸困难.首诊肺囊性病6例,脓气胸、胸膜炎5例,肺炎18例,纵隔肿瘤2例.2例尸检,3例活检,27例手术,术后均化疗.术后随访至2007年7月,平均随访时间(36.5±40.1)个月(1～144个月).13例存活,18例死亡,1例失访.2年存活率35.5%,5年存活率29.0%.从诊断到死亡的时间为1d～26个月.结论由于小儿肺母细胞瘤临床表现与成人肺母细胞瘤有明显不同,因此是一种极易漏诊的少见恶性肿瘤.提高对该病的认识,早期诊断、完整切除、综合治疗是决定预后的关键.
목적 탐토인동흉막폐모세포류적오진원인.방법 장1979년1월지2007년1월림상병리증실적소인흉막폐모세포류32례,근거병리절편、면역조직화학안Dehner분형분위Ⅰ、Ⅱ、Ⅲ삼형;근거불동분형적X선화CT특점,결합림상표현,분석초진진단,총결루진원인,병대기존활솔진행분석.결과 32례환인평균년령(4.0±2.7)세(3개월～10세),남녀비례위3:2.32례환인최조출현적증상균위해수,28례반발열,최종유18례반호흡곤난.수진폐낭성병6례,농기흉、흉막염5례,폐염18례,종격종류2례.2례시검,3례활검,27례수술,술후균화료.술후수방지2007년7월,평균수방시간(36.5±40.1)개월(1～144개월).13례존활,18례사망,1례실방.2년존활솔35.5%,5년존활솔29.0%.종진단도사망적시간위1d～26개월.결론 유우소인폐모세포류림상표현여성인폐모세포류유명현불동,인차시일충겁역루진적소견악성종류.제고대해병적인식,조기진단、완정절제、종합치료시결정예후적관건.
Objective To analyze the reasons of misdiagnosis of pleuropulmonary blastoma in children. Methods Thirty two cases of pleuropulmonary blastoma from January 1979 to January 2007 were reviewed. They were divided into 3 types according to Dehuer classification with pathological and immunohistochemical evidences. The reasons of misdiagnosis of this disease were analyzed by clinical manifestations, X-ray and CT examination. And the survival rate was also recorded. Results The mean age of diagnosis gained was 4.0 ± 2.7 years (3 months to 10 years). The initial symptom was cough in all children. Twenty eight children had fever. Lately dyspnea appeared in 18 children. Chest radiography showed lung cyst formation in 6 children, pyopneumothorax or pleurisy in 5, pneumonia in 18, and mediastinal mass in 2. Of all these children, except for 2 with autopsy and 3 with biopsy, operations were performed on the other 27 children, and followed by postoperative chemotherapy. Mean follow-up time was 36. 5 months (1 to 144 months). Thirteen children survived, 18 children died and 1 child lost follow-up. Two-year survival rate and 5-year survival rate was 35.5% and 29.0%, respectively. Mean duration from diagnosis to death ranged from 1 day to 26 months. Conclusions Pleuropulmonary blastoma in children is a rare malignant tumor, whose clinical manifestation is quit different from such a tumor in adults, and is very easy to get misdiagnosis. Recognition of this disease, early diagnosis, complete resection, and comprehensive therapy are the key measures to get satisfactory prognosis of pleuropulmonary blastoma in children.