Di Lieto E, Baldi A, Vicidomini G, Di Marino M P, Baldi F
Istituto di Chirurgia Toracica e Metodologia Clinica, Seconda Università degli Studi, Napoli.
Minerva Chir. 1997 Jun;52(6):839-46.
Pulmonary blastoma is a rare tumor of the lung consisting of immature mesenchyma and epithelial structures that can mimic the structure of the embryonic lung. In this article, we report a case of pulmonary blastoma occurred in an adult individual. We review the literature concerning this rare neoplasm that includes less than one hundred cases reported. We also describe the pathologic features of this disease which is microscopically divided in two classes: 1) lesions composed solely of malignant gland of embryonic appearance (WDFA: Well-Differentiated Fetal Adenocarcinomas), and 2) lesions with biphasic appearance, containing both neoplastic glands and either adult sarcomatous or embryonic mesenchyme. The adult pulmonary blastomas are considered as an entity distinct from childhood blastomas. This condition may be frequently asymptomatic; if symptomatic, it show the same clinical picture of a mass in the lung impinging on bronchi or pleura; the most usual symptoms include cough, hemoptysis, and chest pain. Chest radiography typically shows a peripheral or central lung opacity without preference for any lobe. The prognosis is poor, due to the high incidence of metastasis. Histologic class and clinical findings can be of prognostic value in pulmonary blastoma. We believe that an early diagnosis is essential: in our case, this allowed a complete resection of the tumor and the patient is still alive without recurrence 3 years after the surgical treatment.
肺母细胞瘤是一种罕见的肺部肿瘤,由不成熟的间充质和上皮结构组成,可模仿胚胎肺的结构。在本文中,我们报告了一例发生在成年人身上的肺母细胞瘤病例。我们回顾了有关这种罕见肿瘤的文献,其中报道的病例不到100例。我们还描述了这种疾病的病理特征,在显微镜下可分为两类:1)仅由具有胚胎外观的恶性腺体组成的病变(WDFA:高分化胎儿腺癌),以及2)具有双相外观的病变,包含肿瘤性腺体和成人肉瘤样或胚胎间充质。成人肺母细胞瘤被认为是一种与儿童期母细胞瘤不同的实体。这种情况通常可能无症状;如果有症状,则表现为肺部肿块压迫支气管或胸膜的相同临床症状;最常见的症状包括咳嗽、咯血和胸痛。胸部X线摄影通常显示肺外周或中央的不透光区,对任何肺叶无偏好。由于转移发生率高,预后较差。组织学分类和临床发现对肺母细胞瘤的预后可能具有价值。我们认为早期诊断至关重要:在我们的病例中,这使得肿瘤得以完整切除,患者在手术治疗3年后仍然存活且无复发。