CT and histopathological correlation of congenital cystic pulmonary lesions: a common pathogenesis?

doi:10.1016/j.crad.2008.02.011

Clinical Radiology

Volume 63, Issue 9, September 2008, Pages 995-1005

https://doi.org/10.1016/j.crad.2008.02.011 Get rights and content

Aim

To determine whether similarities exist in both the imaging and histopathological features of congenital cystic lung lesions and whether a more appropriate classification would be to adopt the theory of “malinosculation”.

Material and methods

From the histopathology and computed tomography (CT) database, 24 patients (16 male, median age 3 years) with congenital cystic lung lesions were identified. CT studies were reviewed for site and characteristics of the lesions, parenchymal features, bronchial anatomy, and the presence of a feeding systemic vessel. Individual histopathological parameters were also correlated with CT data.

Results

There were five type 1 congenital cystic adenomatoid malformations (CCAMs), six type 2 CCAMs, one type 4 CCAM, one bronchial atresia, four pleuropulmonary blastomas (PPBs), and seven sequestrations. CCAMs (types 1, 2 and 4), sequestrations and PPBs appeared as cystic lesions, with cyst size less than 2 cm in type 2 CCAMs. Sequestrations were distinguished radiologically from CCAMs by systemic vessels. Reduced pulmonary attenuation was seen in bronchial atresia, type 2 CCAMs and in sequestrations. Histopathology showed an overlap in entities with sequestrations demonstrating CCAM type 2 histology and segmental atresia noted in both type 2 CCAMs and sequestrations. PPBs showed histological and imaging overlap with type 4 CCAMs and were distinguished on histology by the presence of blastematous proliferation.

Conclusions

This study demonstrates overlap in the CT appearances of congenital cystic lesions. The similarity in CT and histopathology findings across the spectrum of developmental lesions supports the hypothesis of a common aetiology.

Introduction

Congenital cystic lesions of the lung comprise a heterogeneous group of bronchopulmonary malformations including congenital cystic adenomatoid malformations (CCAMs), sequestrations, bronchial atresia and bronchogenic cysts. Many lesions are now detected antenatally by prenatal ultrasound, but they can also present in the postnatal period, and more rarely, in later life.

A pathology-based classification system has evolved¹ with controversy as to the degree of overlap between entities as, for example, some sequestrations demonstrate histological features of type 2 CCAMs.2, 3 It has been suggested that a common pathogenetic mechanism is responsible for the development of many of these lesions.¹ This has led to others proposing a more encompassing nomenclature.⁴

There have been few published series on computed tomography (CT) features of congenital cystic lesions, primarily limited to CCAMs.5, 6 Therefore, the aims of this study were to use CT–histopathology correlation to assess the degree of overlap between proposed entities, and to search for CT features that might distinguish such entities preoperatively and, therefore, potentially improve patient management.

Section snippets

Patient selection

Patients who had undergone surgical resection for the final histological diagnosis of congenital cystic lung lesions between 1999 and 2006 were identified from the histopathology database (n = 42). The subset of patients (n = 24) who had also undergone CT of the thorax at our institution prior to surgical resection then formed the basis of the study. CT studies were performed between March 1997 and March 2006. Ethical approval was obtained from the local ethics committee.

Image acquisition

CT studies were obtained on

Results

There were 24 patients (16 male) presenting with congenital cystic lung lesions with a median age of 3 years (interquartile range 2.1–9.5 years) at the time of surgical resection. The median interval between CT study and surgical resection was 79.5 days (interquartile range 20–319 days). Clinical and surgical data for a proportion of these lesions have been published previously.9, 10, 11 Overall, there were five type 1 CCAM, six type 2 CCAM, one type 4 CCAM, one bronchial atresia, four type 1

Discussion

This study documents CT and histological features of patients presenting with congenital cystic lung lesions over a 9-year period at a tertiary referral centre, and correlates these data with the aims of assessing their inter-relationships. The data show that there is an overlap in the CT and histopathological features in certain non-neoplastic congenital cystic lesions of the lung (type 1 and type 2 CCAMs, sequestrations and bronchial atresia). Type 1 and type 2 CCAMs were primarily

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Quantitative CT imaging analysis to predict pathology features in patients with a congenital pulmonary airway malformation
2022, Journal of Pediatric Surgery
Citation Excerpt :
The use of CT to predict histopathological outcome is increasingly being reported in other diseases and utilizing objective, quantitative scoring methods increase the reliability [27,28]. Previous research on the CT-pathology correlation in CPAM, has primarily focused on the diagnosis of the CPAM subtype [29–35]. In our current study we aimed to investigate if CT- imaging characteristics can be used to predict histopathological features, by using an objective quantitative CT scoring method.
Risk for infection and potential malignant degeneration are the most common arguments for resecting asymptomatic Congenital Pulmonary Airway Malformations (CPAM). We aimed to investigate if CT- imaging characteristics can be used to predict histopathological features, by using an objective quantitative CT scoring method.
Archival CPAM tissue samples were histologically re-assessed and patients who had a pre-operative volumetric CT-scan were included. Lung disease was quantified using the newly-developed congenital lung abnormality quantification(CLAQ) scoring method and obtained percentages were used to predict histopathological signs of inflammation and presence of mucinous proliferation (MP). Because MP is presumed a precursor for mucinous adenocarcinoma in situ (AIS) this method was also used to compare CT-scans of patients with AIS to those with only CPAM.
Thirty-three CPAM patients were included of which 13(39%) had histological signs of inflammation and 8(24%) had a MP. Patients with inflammation had a significantly smaller lesion (14% vs 38%) while those with MP had more extensive disease (54%vs17%). Patients with AIS had a significantly smaller lesion compared to CPAM patients (5%vs29%). Significant predictors for inflammation were smaller lesion size and percentage hypodensity within lesions while a larger lesion size and percentage parenchymal hyperdensity (solid lung tissue components) were predictors for MP as well as AIS.
Smaller CPAM lesions may be more susceptible to inflammation while larger lesions may be associated with the presence of MP. Parenchymal hyperdensity is found as a predictor for MP as well as AIS and should therefore elicit more extensive gross sampling.
Level III.
A clinical guideline for structured assessment of CT-imaging in congenital lung abnormalities
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Furthermore, opposed to homogeneous BC, a heterogeneous mass with central contrast-enhancement is more suggestive for a mediastinal neoplasm [7,8]. BC are commonly fluid filled and as such can objectively be distinguished from a soft tissue mass if the region of interest measurement is lower than the cut-off value of 20 Hounsfield units [23] or by comparing content to fluid in the gall bladder or spinal canal [7,8,20,27]. An internal air-fluid level and enhanced thick cyst wall are signs of infection [4].
To develop a clinical guideline for structured assessment and uniform reporting of congenital lung abnormalities (CLA) on Computed Tomography (CT)-scans.
A systematic literature search was conducted for articles describing CT-scan abnormalities of congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (BPS), congenital lobar emphysema (CLE) and bronchogenic cyst (BC). A structured report using objective features of CLA was developed after consensus between a pediatric pulmonologist, radiologist and surgeon.
Of 1581 articles identified, 158 remained after title-abstract screening by two independent reviewers. After assessing full-texts, we included 28 retrospective cohort-studies.
Air-containing cysts and soft tissue masses are described in both CPAM and BPS while anomalous arterial blood supply is only found in BPS. Perilesional low-attenuation areas, atelectasis and mediastinal shift may be found in all aforementioned abnormalities and can also be seen in CLE as a cause of a hyperinflated lobe. We have developed a structured report, subdivided into five sections: Location & Extent, Airway, Lesion, Vascularization and Surrounding tissue.
CT-imaging findings in CLA are broad and nomenclature is variable. Overlap is seen between and within abnormalities, possibly due to definitions often being based on pathological findings, which is an unsuitable approach for CT imaging. We propose a structured assessment of CLA using objective radiological features and uniform nomenclature to improve reporting.
ACR Appropriateness Criteria® Pneumonia in the Immunocompetent Child
2020, Journal of the American College of Radiology
Citation Excerpt :
Similar to CT chest without IV contrast, CT chest with IV contrast can be used to diagnose anatomical abnormalities in the immunocompetent patient that could predispose children to recurrent localized infections like congenital lobar overinflation [54], foreign bodies that could cause recurrent postobstructive pneumonia [13], and underlying pulmonary disease like bronchopulmonary dysplasia [52]. CT chest with IV contrast provides benefit over CT chest without IV contrast for diagnosing conditions such as bronchial tumors, congenital pulmonary airway malformation [54-58], pulmonary sequestration [21,54,55,59-63], and bronchopulmonary foregut malformations [54,55]. CTA chest with IV contrast is especially helpful for presurgical planning, identifying feeding and draining vessels in patients with suspected pulmonary sequestration, and assessing for a vascular ring that may lead to tracheal narrowing [59,63].
Pneumonia is one of the most common acute infections and the single greatest infectious cause of death in children worldwide. In uncomplicated, community-acquired pneumonia in immunocompetent patients, the diagnosis is clinical and imaging has no role. The first role of imaging is to identify complications associated with pneumonia such as pleural effusion, pulmonary abscess, and bronchopleural fistula. Radiographs are recommended for screening for these complications and ultrasound and CT are recommended for confirmation. The second role of imaging is to identify underlying anatomic conditions that may predispose patients to recurrent pneumonia. CT with intravenously administered contrast is recommended for this evaluation.
The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
Computed tomography features can distinguish type 4 congenital pulmonary airway malformation from other cystic congenital pulmonary airway malformations
2020, European Journal of Radiology
We aimed to identify clinical and computed tomography (CT) features that distinguish among cystic congenital pulmonary airway malformations (CPAMs) and offer new management strategies.
We retrieved data on 145 children (85 male, 60 female; median age, 14 months) with pathologically confirmed cystic CPAMs from 2008 to 2018 and retrospectively analyzed the clinical and CT characteristics.
Of the 145 patients, 54 had type 1, 72 had type 2, and 19 had type 4 cystic CPAMs. Significantly more male patients had type 4 CPAMs. Type 2 CPAMs had a higher frequency of combined malformations and prenatal diagnosis than types 1 and 4. The median diameter of cystic CPAMs was 2.8 cm; that of type 1, 2, and 4 was 4.6, 1.5, and 8.1 cm, respectively. Regression analysis showed that a cyst of >7.9 cm in diameter was likely to be type 4, that of <2.8 cm was likely to be type 2, and that of 2.8–7.9 cm was likely to be type 1. Smaller cysts were more likely to be type 2 and larger cysts were more likely to be type 4. The incidence of pneumonia was higher in type 2 than in types 4 and 1. The frequency of mediastinal shift and pneumothorax was statistically significant, and both were more common in type 4.
A cyst of >7.9 cm in diameter, mediastinal shift, and pneumothorax were the most important characteristics of type 4 CPAMs. CT features can distinguish type 4 CPAM from other cystic CPAMs.
Cystic and pseudocystic pulmonary malformations in children: Clinico-pathological correlation
2019, Annals of Diagnostic Pathology
Citation Excerpt :
Table 3 shows the concordance between the presurgical and the anatomopathological diagnoses. The majority of publications on CPPM are review articles or reports of imaging-clinical correlations, with only a few focused on the presurgical and anatomopathological correlation [1,12,22]. Thus, a major relevance of the present work is that it remedies in part the scarcity of publications on anatomo-clinical correlation in CPPM.
Pulmonary malformations are rare disorders, with cystic and pseudocystic pulmonary malformations (CPPM) the most frequent, and constitute the first cause of lobectomy in children <1 year of age. Morphological overlap of congenital cystic pulmonary lesions might correspond to a spectrum of lesions in which bronchial atresia is a common etiopathogenetic mechanism. We aimed to report the frequency of CPPM resected in a tertiary-level hospital and to evaluate the degree of agreement between presurgical and anatomopathological diagnoses. We studied 44 surgical pieces with a diagnosis of CPPM received at the Pathology Service from 2009 to 2014, resected from 39 patients, 51.3 % males, with a median age of 16.8 months. Up to 69.2% of the patients had adenomatoid malformation of pulmonary airway (AMPA), with type 2 the most frequent (55.5%). Pulmonary sequestration was present in 15.4% of patients; in two cases the diagnosis was an incidental finding during surgery for the repair of a diaphragmatic hernia. Congenital lobar hyperinflation (CLH) occurred in 7.6% cases. Bronchogenic cyst (BC) was present in 7.6% cases. Presurgical and anatomopathological diagnoses in all patients coincided in 71.8% of cases. Kappa coefficient was 0.56 for global concordance in patients with AMPA, and 0.72, 0.64, 0.37 and 0.33 for CLH, BC, and types 1 and 2 AMPA, respectively. This relatively low interobserver agreement could reflect the low reproducibility of diagnoses used in the current nomenclature. Thus, the new nomenclature must be promoted in order to allow for better reproducibility and greater clinico-pathological concordance. The anatomopathological analysis must include the intentional search for bronchial atresia.
Congenital Lung Disease
2019, Kendig's Disorders of the Respiratory Tract in Children
This chapter discusses the spectrum of congenital lung disease from the upper airway down to the lung parenchyma and microvasculature, and associated relevant malformations in the chest wall and mediastinum, and systemically. A systematic way of describing an individual malformation is proposed, using clear words to delineate the components of the malformation before planning treatment. Congenital lung disease may present in utero right up to old age. Many large malformations diagnosed antenatally largely regress in the third trimester of pregnancy, and are only detectable postnatally on computed tomography (CT) scanning. Management of asymptomatic congenital cystic malformations is controversial; many remain symptom free for a long time, whereas others become the seat of infection or malignancy, or result in other complications, such as air embolism. Histological overlap between what were once thought of as discrete entities, such as congenital cystic adenomatoid malformation and sequestration, are common, and attempting to determine histology from clinical images is fraught with difficulty. Newer imaging techniques, such as magnetic resonance and CT angiography, are increasingly used to image congenital lung malformations, with conventional angiography reserved for situations when therapeutic embolization of the malformation is being considered. Advances in surgical techniques, in particular for congenital diaphragmatic hernia, mean that there are more survivors into childhood and adult life. The optimal follow-up for patients with congenital lung malformations, whether surgically treated or not, remains to be determined.

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CT and histopathological correlation of congenital cystic pulmonary lesions: a common pathogenesis?

Aim

Material and methods

Results

Conclusions

Introduction

Section snippets

Patient selection

Image acquisition

Results

Discussion

Semin Pediatr Surg

Hum Pathol

Ann Thorac Surg

Ann Thorac Surg

Hum Pathol

J Pediatr Surg

Congenital cystic adenomatoid malformation connected to an extralobar pulmonary sequestration in the contralateral cyst: common origin?

J Pediatr Surg

Congenital lung disease. A plea for clear thinking and clear nomenclature

Pediatr Pulmonol

Congenital cystic adenomatoid malformation in adults: CT findings

J Comput Assist Tomogr

Congenital cystic adenomatoid malformation of the lung: CT–pathologic correlation

AJR Am J Roentgenol

An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation

Am J Surg Pathol

A cautionary note about congenital cystic adenomatoid malformation (CCAM) type 4

Am J Surg Pathol