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极为罕见,需引起注意:胸膜肺母细胞瘤酷似先天性肺气道畸形。

It's Rare So Be Aware: Pleuropulmonary Blastoma Mimicking Congenital Pulmonary Airway Malformation.

作者信息

Haider Fayza, Al Saad Khulood, Al-Hashimi Fatima, Al-Hashimi Hakima

机构信息

Pediatric Surgery Unit, Department of Surgery, Salmaniya Medical Complex, Manama, Bahrain.

Pediatric Department, Salmaniya Medical Complex, Manama, Bahrain.

出版信息

Thorac Cardiovasc Surg Rep. 2017 Jan;6(1):e10-e14. doi: 10.1055/s-0037-1598625.

Abstract

Pleuropulmonary blastoma (PPB) is a rare aggressive malignant tumor of infancy and early childhood. The tumor arises in the lung and pleura and is regarded as a pulmonary dysontogenetic or embryonic neoplasm. Four types are defined in literature. Type I PPB is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced with a poor prognosis. We report this case to increase awareness about this entity so that the pediatricians, pediatric surgeons, radiologist, and pathologist recognize it early.

摘要

肺胚细胞瘤(PPB)是一种罕见的婴幼儿侵袭性恶性肿瘤。该肿瘤起源于肺和胸膜,被认为是一种肺发育异常或胚胎性肿瘤。文献中定义了四种类型。I型PPB是一种罕见的婴儿期囊性肺肿瘤,其特征为有细微的恶性变化且预后良好。I型PPB复发后通常病情进展且预后不良。我们报告此病例以提高对该疾病的认识,以便儿科医生、小儿外科医生、放射科医生和病理科医生能够早期识别它。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9880/5388606/027f90485f8f/10-1055-s-0037-1598625-i160249crt-1.jpg

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