Type I pleuropulmonary blastoma: Pathology and biology study of 51 cases from the International Pleuropulmonary Blastoma Registry
Type I pleuropulmonary blastoma: Pathology and biology study of 51 cases from the International Pleuropulmonary Blastoma Registry
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DOI：
10.1097/pas.0b013e3181484165
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发表时间：
2008-02-01
影响因子：
5.6
通讯作者：
Dehner, Louis P.
中科院分区：
文献类型：
--
作者：
Hill, Dana Ashlev;Jarenzbowski, Jason A.;Dehner, Louis P.
胸膜肺母细胞瘤（PPB）是一种发生于儿童早期的肺部恶性肿瘤。这种疾病的早期形式;囊性I型PPB，由于其与一些发育性肺囊肿相似，因此在临床和病理上可能具有欺骗性。本研究回顾了51例I型PPB和6例来自PPB患儿亲属的肺囊肿。I型PPB是一种精致的多房囊肿，在良性上皮表面下有数量不等的原始间充质细胞。横纹肌母细胞和软骨结节分别见于49%和40%的病例。与年龄较大的患者组相比，从出生到2个月大的最年轻患者亚组中的肿瘤在组成和细胞结构上更均匀。早期肿瘤在正常发育的肺和肿瘤之间有一个微妙的过渡;表现出温和的间质细胞均匀地扩张肺泡隔。包括囊壁坏死在内的假定退行性变化是常见的。这种现象可以解释在一些I型PPBs中观察到的可变的且有时稀疏的肿瘤细胞结构。在生物学层面上，这一过程支持并非所有第一类PPB都注定会发展为第二类或第三类PPB的概念。控制进展和消退之间平衡的因素可能在预测肿瘤行为和确定哪些患者将从辅助化疗中获益方面很重要。与此同时，认识到这种病变是一种恶性肿瘤的潜力，而不是一个发育性囊性畸形是至关重要的，使孩子可以得到完整的切除和适当的后续护理。
Pleuropulmonary blastoma (PPB) is a malignant neoplasm of the lung that presents in early childhood. The early form of the disease; cystic type I PPB, can be clinically and pathologically deceptive because of its resemblance to some developmental lung cysts. This study reviews 51 cases of type I PPB and 6 lung cysts from relatives of children with PPB. Type I PPB is a delicate multilocular cyst with variable numbers of primitive mesenchymal cells beneath a benign epithelial surface. Rhabdomyoblasts and cartilage nodules are seen in 49% and 40% of cases, respectively. Tumors in the youngest subset of patients, from birth to 2 months of age, are more uniform in composition and cellularity compared with those in older Groups. Early tumors have a subtle transition between normal developing lung and tumor; showing bland interstitial mesenchymal cells uniformly expanding the alveolar septa. Presumed regressive changes including cyst wall necrosis are common. This phenomenon may explain the variable and sometimes sparse tumor cellularity seen in some type I PPBs. On a biologic level, this process supports the concept that not all type I PPBs are fated to progress to a type II or III PPB. Factors that control the balance between progression and regression may be important in predicting tumor behavior and determining which patients will benefit from adjuvant chemotherapy. In the meantime, recognition of this lesion as a neoplasm with malignant potential rather than a developmental cystic malformation is vital so the child can receive complete excision and appropriate follow-up care.