Document Detail


Discriminating Preoperative Features of Pleuropulmonary Blastomas (PPB) from Congenital Cystic Adenomatoid Malformations (CCAM): a Retrospective, Age-Matched Study.
MedLine Citation:
PMID:  21104589     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
BACKGROUND: The etiopathogenesis of pleuropulmonary blastoma (PPB) and its relationship to congenital cystic adenomatoid malformation (CCAM) remain controversial. Our recent analysis indicates that both the co-incidental occurrence and the outcome of PPB among CCAM patients are significant. We report here on our efforts to determine clinical and radiological features discriminating PPB from CCAM preoperatively.
MATERIALS & METHODS: A retrospective analysis of all patients treated for PPB and CCAM between 1981 and 2008 at 2 tertiary academic centers under a centralized, single payer healthcare system was performed (REB#1000013239). Clinical, radiological and demographic data were analyzed. PPB patients were secondarily age matched with CCAM patients (±10% age difference in months), and clinical, radiological and demographic variables were compared. Descriptive statistics and non-parametric analysis were used.
RESULTS: A total of 10 PPB patients was identified. Median age at diagnosis was 24 months; the male to female ratio was 5:5. No PPB patients had an antenatal diagnosis (p<0.01). 9 were symptomatic, with symptoms including dyspnea (7/10), upper respiratory infection (6/10), poor weight gain (3/10), and 1 patient was asymptomatic. 5 of 10 patients had solid parts on CT, of which 4 out of 5 were diagnosed preoperatively as PPB and 1 out of 5 as CCAM. 5 of 10 were predominantly cystic of which none was diagnosed preoperatively as PPB (p=0.0476). Given that most CCAM patients are now diagnosed antenatally, only 5 PPB patients could be age-matched with CCAM patients. In the predominantly cystic PPB patients (n=5), no significant discriminating clinical and radiological features were identifiable when compared preoperatively with age-matched CCAM patients.
CONCLUSION: PPB patients continue to represent a diagnostic challenge. Asymptomatic and predominantly cystic PPB remain indistinguishable from CCAM preoperatively. A high index of suspicion for PPB must be considered in any child presenting with cystic lung lesions beyond early infancy, particularly in a child with poor weight gain.
Authors:
C Oliveira; S Himidan; A C Pastor; A Nasr; D Manson; G Taylor; N L Yanchar; G Brisseau; P C W Kim
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Publication Detail:
Type:  Journal Article     Date:  2010-11-22
Journal Detail:
Title:  European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie     Volume:  21     ISSN:  1439-359X     ISO Abbreviation:  Eur J Pediatr Surg     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-02-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9105263     Medline TA:  Eur J Pediatr Surg     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  2-7     Citation Subset:  IM    
Copyright Information:
© Georg Thieme Verlag KG Stuttgart · New York.
Affiliation:
The Hospital for Sick Children, Division of General and Thoracic Surgery, Toronto, Canada.
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