Syndromic presentation of a pleuropulmonary blastoma associated with congenital cystic adenomatoid malformation. A case report
Section snippets
Case report
This male child was born at 38 weeks of gestation by spontaneous vertex delivery and had respiratory distress at birth. An ultrasound performed at 35 weeks of gestation revealed the presence of right pulmonary cysts. Computerized tomography scan of the thorax after birth revealed multiple large cysts in the right lung and few cysts in the left lung (Fig. 1). He underwent right middle lobectomy on the fifth day of life. The pathology was consistent with CCAM type 4 (Fig. 2A, B).
The baby was
Discussion
Pediatric intrathoracic neoplasms with mixed blastematous and sarcomatous characteristics arising in association with preexisting cystic lung lesions were recognized for the first time as PPB by Manivel et al [5]. Subsequently, single case reports [6] and large series described in detail the clinical and pathological features of PPB [7].
Studies assessing the malignant potential of CCAM have shown that type 4 CCAMs can have focal stromal hypercellularity and subsequently may develop a PPB [8].
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Cited by (22)
Congenital Lung Disease
2019, Kendig's Disorders of the Respiratory Tract in ChildrenAssociation between congenital lung malformations and lung tumors in children and adults: A systematic review
2016, Journal of Thoracic OncologyCitation Excerpt :In either case, prophylactic resection of these cysts might protect the patients from development of PPB or at least provide resection of this malignant lesion at its early stage. In other cases the congenital malformation associated with PPB was more histologically defined as ELS,30,31 CCAM1,32–34 CCAM,6,23,35 congenital pulmonary airway malformation (CPAM) type 4,36,37 BC,38 or congenital lobar emphysema (CLE)39 detected on histological examination of the resection specimen. It has been suggested that prophylactic resection of CPMs does not protect patients from later development of PPB.36
Congenital cystic adenomatoid malformation in 2-year-old girl – case report
2016, Pediatria PolskaRisk-reduction surgery in pediatric surgical oncology: A perspective
2016, Journal of Pediatric SurgeryCitation Excerpt :There is no known genetic predisposition to CPAM, with the exception of type 0 (which has an autosomal-recessive inheritance pattern) [177] and type IV (which is closely related to pleuropulmonary blastoma syndrome [PPB]) [175]. In children, CPAMs may be complicated by infection, pneumothorax, and the development of malignancies, such as bronchoalveolar carcinoma (BAC) [178–180], mucinous adenocarcinoma [181], rhabdomyosarcoma [182,183], PPB, and mucoepidermoid carcinoma [174,184–186]. Previous cases of rhabdomyosarcomas originating in a CPAM are now thought to represent PPB [187,188].
Is radical lobectomy required in congenital cystic adenomatoid malformation?
2012, Journal of Pediatric SurgeryCitation Excerpt :Laberge et al [1] in 2005 in a very well-documented and exhaustive review of literature (until 2003) found 5 published cases of pleuropneumoblastoma, 7 cases of rhabdomyosarcomas, and 8 cases of bronchioloalveolar carcinoma associated with CCAM lesions. Between 2003 and 2009, 2 more cases of pleuropneumoblastoma [8-11] and 2 bronchioloalveolar carcinomas [12,13] were reported. Although the exact pathophysiology remains unclear, between malignant degeneration of a malformation and a common altered genetic susceptibility, surgery and removal of the entire lesion seem to be the more reasonable option for treatment.
Congenital Lung Disease
2012, Kendig and Chernick's Disorders of the Respiratory Tract in Children