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[肺胚细胞瘤:一例通过产前超声筛查实现早期新生儿诊断的病例]

[Pleuropulmonary blastoma: a case of early neonatal diagnosis through antenatal scan screening].

作者信息

Mechoulan A, Leclair M-D, Yvinec M, Philippe H-J, Winer N

机构信息

Service de gynécologie-obstétrique, Centre pluridisciplinaire de diagnostic prénatal de Nantes, CHU de Nantes, Nantes cedex 01, France.

出版信息

Gynecol Obstet Fertil. 2007 May;35(5):437-41. doi: 10.1016/j.gyobfe.2007.02.014. Epub 2007 Mar 27.

Abstract

We report a case of pleuropulmonary blastoma (PPB) prenatally diagnosed at 32 weeks of gestation. The suspected diagnoses was a congenital cystic adenomatoid malformation of the lung (CCAM). The excision of the malformation was performed through a thoracotomy at 4 months of age. On pathological examination, the presence of focal stromal hypercellularity (cambium layer) in the cyst wall was consistent with both the diagnosis of type IV CCAM or grade I PPB. The presence of nodules of immature cartilage and the positivity of particular immunohistochemical staining (indicate which staining came positive) confirmed the diagnosis of grade I PPB. Pleuropulmonary blastoma is a rare malignant tumour in children, sometimes difficult to differentiate from the type IV CCAM as they share similar circumstances of diagnosis, radiological and macroscopic features. The pathological examination does not always allow to differentiate them clearly, implying the necessity of an extended medical supervision for the children carrying those border lesions. Because of the risks of malignancy and of the risks of septic complications, surgical resection during the first year of life is often advocated. Therefore, prenatal detection is essential to allow adequate early management and complete resection of these lesions.

摘要

我们报告一例在妊娠32周时产前诊断出的胸膜肺母细胞瘤(PPB)。疑似诊断为先天性肺囊性腺瘤样畸形(CCAM)。在患儿4个月大时通过开胸手术切除了畸形病变。病理检查显示,囊肿壁存在局灶性间质细胞增多(生发层),这与IV型CCAM或I级PPB的诊断均相符。未成熟软骨结节的存在以及特定免疫组化染色呈阳性(指明哪种染色呈阳性)确诊为I级PPB。胸膜肺母细胞瘤是儿童罕见的恶性肿瘤,有时难以与IV型CCAM区分,因为它们在诊断情况、影像学和宏观特征方面相似。病理检查并不总能清晰地区分它们,这意味着对患有这些临界病变的儿童需要进行长期的医学监测。由于存在恶变风险和感染并发症风险,通常主张在患儿1岁内进行手术切除。因此,产前检测对于实现这些病变的充分早期管理和完整切除至关重要。

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