小儿肺囊性病变临床病理学观察  

Clinicopathological features of cystic lung diseases in children

作　　者：牛会林 刘威 王凤华 陈峥嵘 高秋 曾荣新 王勇[2] 伊鹏 

机构地区：[1]广州市妇女儿童医疗中心病理科,广州510623 [2]广州市妇女儿童医疗中心外科,广州510623 [3]广州市妇女儿童医疗中心小儿内科,广州510623

出　　处：《临床与实验病理学杂志》2015年第4期413-417,共5页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨小儿肺囊性病变（cysticlungdiseases，CLD）的形态学特征及临床干预时机。方法对125例CLD行常规HE及免疫组化染色，并复习相关文献。结果125例CLD中男性75例，女性50例，男女比为1．5：1。年龄0—11．5岁，平均23．0个月，中位年龄15个月，小于1岁者60例（48．0％）。眼观：50例肺组织局部可见单房或多房囊肿，囊肿直径0．5～8．0cm，囊腔与支气管不相通；18例肺切面呈蜂窝状，囊腔直径0．1-2．0cm。26例肺组织实变，囊腔不明显。21例肺组织见较厚脓肿囊壁，内壁粗糙，可见脓苔。7例肺气肿肺组织按之有捻发音，切面见微囊。2例见肺组织内囊实性肿物，切面灰白色，鱼肉状。125例CLD中先天性肺支气管发育异常相关的囊肿共94例（75．2％），其中先天性肺气道畸形（congenial pulmonary air-way malformation, CPAM）59例（47．2％），其中1型29例（49．2％），2型18例（30．5％），4型12例（20．3％）；肺隔离症26例（20．8％），叶内型15例（57．7％），叶外型11例（42．3％），叶外型肺隔离症中5例合并CPAM2型；支气管源性囊肿8例（6．4％）；肠源性囊肿1例（0．8％）。获得性病变31例（24．8％），其中感染后肺脓肿21例，1例为真菌性脓肿；肺气肿改变7例；胸膜肺母细胞瘤共3例（Ⅰ型1例，Ⅱ型2例）。结论小儿CLD种类较多，结合影像学特征可明确诊断，选择合适的手术时机，可取得良好的治疗效果。Purpose To investigate the histopathological features of cystic lung diseases （ CLD）, and to discuss the timing of clinical interventions. Methods HE and immunohistoehemical staining were performed and reviewed in 125 cases of CLD. Results 125 ca- ses of CLD aged from birth to I 1 years and 6 month, with an average age of 23.0 months, median age 15 months, of which 60 cases were less than 1 year （48. 0% ）. 75 cases were male and 50 cases female, with male to female ratio of 1.5 ： 1. Grossly, 50 cases showed single or multiple cysts with the size 0. 5 - 8.0 cm in diameter, which did not communicate with bronchial cavity. 18 cases showed honeycomb cysts with the diameter of 0. 1 - 2.0 cm. 26 cases were solid lesions without visible cysts. 21 cases were observed lung abscess with thick and rough wall and pus inside. 7 cases of emphysema showed microcysts with crepitation. 2 cases were identi- fied cystic and solid masses, with fish-fresh like cut surface. Histopathologically, 94 cases （75.2%） were related to congenital bron- ehopulmonary dysplasia in 125 cases of CLD, in which there were 59 patients （47. 2% ） of congenial pulmonary airway malformation （ CPAM）, including 29 cases of type 1 （49. 2% ）, 18 cases of type 2 （30. 5% ）, and 12 cases of type 4 （20. 3% ）, there were 26 ea- ses （20. 8% ） of pulmonary sequestration, including 15 cases of intralobar type （57.7%） and 11 of extralobar cases （42. 3% ）, 5 ca- ses were complicated with CPAM type 2, 8 cases were bronchial cyst （6.4%） and 1 case of enteric cyst （0. 8% ）. Acquired lesions were detected in 31 cases （24. 8% ） , including 21 cases of infected lung abscess, 1 case of fungal abscess. 7 cases of emphysema, and 3 cases of pleuralpulmonary blastoma （ type Ⅰ 1 case and type Ⅱ 2 cases）. Conclusion Pediatric CLD is characterized as com- plexed categories. The prognosis depends on correct pathological diagnosis, combined with imaging evaluation and appropriate timing of surgery.

关 键 词：肺囊肿 胸膜肺母细胞瘤 先天性肺气道畸形 肺隔离症 支气管源性囊肿 

分 类 号：R734.2[医药卫生—肿瘤]