Servicio de Patología, Unidad Médica de Alta Especialidad, Hospital de Pediatría Silvestre Frenk Freund, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Ciudad de México, Mexico.
Servicios de Neumología, Inhaloterapia, Fisiología Pulmonar y Endoscopia, Unidad Médica de Alta Especialidad, Hospital de Pediatría Silvestre Frenk Freund, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Ciudad de México, Mexico.
Ann Diagn Pathol. 2019 Apr;39:78-85. doi: 10.1016/j.anndiagpath.2019.02.005. Epub 2019 Feb 16.
Pulmonary malformations are rare disorders, with cystic and pseudocystic pulmonary malformations (CPPM) the most frequent, and constitute the first cause of lobectomy in children <1 year of age. Morphological overlap of congenital cystic pulmonary lesions might correspond to a spectrum of lesions in which bronchial atresia is a common etiopathogenetic mechanism. We aimed to report the frequency of CPPM resected in a tertiary-level hospital and to evaluate the degree of agreement between presurgical and anatomopathological diagnoses. We studied 44 surgical pieces with a diagnosis of CPPM received at the Pathology Service from 2009 to 2014, resected from 39 patients, 51.3 % males, with a median age of 16.8 months. Up to 69.2% of the patients had adenomatoid malformation of pulmonary airway (AMPA), with type 2 the most frequent (55.5%). Pulmonary sequestration was present in 15.4% of patients; in two cases the diagnosis was an incidental finding during surgery for the repair of a diaphragmatic hernia. Congenital lobar hyperinflation (CLH) occurred in 7.6% cases. Bronchogenic cyst (BC) was present in 7.6% cases. Presurgical and anatomopathological diagnoses in all patients coincided in 71.8% of cases. Kappa coefficient was 0.56 for global concordance in patients with AMPA, and 0.72, 0.64, 0.37 and 0.33 for CLH, BC, and types 1 and 2 AMPA, respectively. This relatively low interobserver agreement could reflect the low reproducibility of diagnoses used in the current nomenclature. Thus, the new nomenclature must be promoted in order to allow for better reproducibility and greater clinico-pathological concordance. The anatomopathological analysis must include the intentional search for bronchial atresia.
肺畸形是罕见的疾病，其中囊性和假囊性肺畸形（CPPM）最为常见，是 1 岁以下儿童行肺叶切除术的首要原因。先天性囊性病损的形态学重叠可能对应于一系列病变，其中支气管闭锁是一种常见的发病机制。我们旨在报告在三级医院切除 CPPM 的频率，并评估术前和解剖病理诊断之间的一致性程度。我们研究了 2009 年至 2014 年病理科收到的 44 例诊断为 CPPM 的手术标本，这些标本来自 39 名患者，其中 51.3%为男性，中位年龄为 16.8 个月。多达 69.2%的患者存在肺气道腺瘤样畸形（AMPA），其中 2 型最常见（55.5%）。肺隔离症存在于 15.4%的患者中；在 2 例患者中，该诊断是在因膈疝修复而进行的手术中偶然发现的。先天性肺过度充气（CLH）在 7.6%的病例中存在。支气管囊肿（BC）存在于 7.6%的病例中。所有患者的术前和解剖病理诊断在 71.8%的病例中相符。在 AMPA 患者中，整体一致性的 Kappa 系数为 0.56，CLH、BC、1 型和 2 型 AMPA 的 Kappa 系数分别为 0.72、0.64、0.37 和 0.33。这种相对较低的观察者间一致性可能反映了当前命名法中使用的诊断方法的低可重复性。因此，必须推广新的命名法，以提高可重复性和更大的临床病理一致性。解剖病理分析必须包括对支气管闭锁的有意搜索。
