Management of Asymptomatic Lung Lesions: Pediatric Thoracic Surgery Part...
With Dr. Jean Martin · StayCurrentMD
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Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
More about CCAM (congenital cystic adenomatoid malformation)
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What the experts said
Most experts now believe CCAMs do not become pleuropulmonary blastoma (PPB); rather, PPB is a de novo tumor that is cystic and cannot be differentiated from CCAM on imaging.
There are cases of prenatally diagnosed cystic lung lesions that turned out to be PPB after resection.
A Toronto series estimates that approximately 4% of cystic lesions that appear to be CCAM will actually turn out to be pleuropulmonary blastoma.
There is approximately 1% risk of bronchioloalveolar carcinoma arising from CCAM, typically in teenage years or early adulthood.
If thoracoscopic resection is performed, the specimen should be placed in a bag before extraction to avoid tumor spillage in case the lesion is PPB, as there is a risk of recurrence.
Non-communicating extralobar sequestrations have a very low infection rate; hematogenous infection is possible but rare, similar to any other body tissue.
Malignant transformation of extralobar sequestration is extremely rare, with perhaps one or two cases of squamous cell carcinoma described in world literature.
Infection and cancer are not good arguments to resect a non-communicating extralobar sequestration.
The incidence of infection and malignancy in extralobar sequestrations is very low, but has occurred.
Current imaging is not perfect and cannot always definitively diagnose extralobar sequestration or rule out hybrid lesions.
The morbidity of resecting extralobar sequestrations is very low, which favors resection over observation.
There is no role for embolization of extralobar sequestrations; it is much easier to resect them using minimally invasive techniques than to subject an infant or child to embolization.
The differential diagnosis for infradiaphragmatic lesions prenatally includes adrenal hemorrhage and neuroblastoma (including cystic neuroblastoma).
Small infradiaphragmatic lesions can be followed by ultrasound; if they stay the same size or get smaller, they do not require resection.
Adrenal hemorrhage will evolve over time and become recognizable on imaging.
A Children's Oncology Group (COG) study showed it is safe to observe adrenal masses suspicious for neuroblastoma, though they must be watched.
There is no good way to follow pulmonary lesions; CT scan is the best method but induces a certain incidence of malignancy itself.
CT scan cannot differentiate CCAM from pleuropulmonary blastoma, so surveillance imaging will not allow earlier detection of malignancy until stage 2 or 3 disease occurs.
It is almost universal that families cannot be counseled with the word 'cancer' and then choose to watch the lesion.
When deciding whether to operate, the risks of not operating must be weighed against the risks of operating.
There has been very little discussion about the risks of performing lobectomy during the panel.
The risks of pulmonary lobectomy are low, especially in experienced hands, but there are still children who die from pulmonary lobectomy, particularly when done thoracoscopically.
Thoracoscopic lobectomy should not be performed unless the surgeon is experienced; in experienced hands, thoracoscopic lobectomy should have no more morbidity than open lobectomy.
Thoracoscopic lobectomy should only be performed by surgeons with advanced thoracic and minimally invasive skills; in such hands, mortality should be zero.
If you do enough of any operation, you will have significant complications, whether open or thoracoscopic.
If massive bleeding occurs during thoracoscopic lobectomy, the chance of salvaging the situation is probably better if the case is already open rather than thoracoscopic.
Pleuropulmonary blastoma (PPB) can be indistinguishable radiologically from CCAM, but the incidence of CCAM/CPAM is markedly higher than the incidence of PPB; PPBs remain extremely rare.
Peter Kim's paper used the Stalker classification in which one histological category has been termed PPB, but the natural history of that histological finding is unknown.
At a high-volume center, de novo PPB is seen extremely rarely, approximately once every 3-4 years, while approximately 20-25 new cases of CCAM are seen annually.
The incidence of cancer in CCAM remains extremely low and must be balanced against the risk of the operation.
The estimated lifelong risk of infection for observed CCAM is approximately 20-30%, based on institutional data showing 10% infection rate with a mean follow-up of 4 years, tripled to estimate lifetime risk.
The only prospective study that followed asymptomatic CCAM patients long-term was small and showed 18 of 21 asymptomatic patients developed symptomatology during follow-up (average 2 years, up to 13 years).
Before prenatal diagnosis became common, it was not common for patients to present during teenage years or in adult thoracic surgery units with symptomatic infected CCAMs, though it happened from time to time.
In the early career of one surgeon in San Francisco, infected CCAMs were not a rare event and were seen on several occasions in the first few years.
Small asymptomatic extralobar sequestrations are a relatively known finding at autopsy, but asymptomatic CCAMs are essentially nonexistent in autopsy series.
The majority of CCAMs become symptomatic; CCAM is not a normal variant.
At one institution, all prenatally diagnosed lesions are followed with postnatal CT, and those not operated on are followed with chest X-ray and repeat CT.
One surgeon does not advocate non-operative management of all CCAMs; families are counseled about risks including cancer and infection, and many choose resection after hearing these risks.
A balanced approach should be given to families, allowing them to decide, rather than taking an approach where every single CCAM needs an operation.
In a poll, 25% of respondents do not advocate routine resection of asymptomatic CCAMs.
There is a hidden mortality in pediatric surgery because people do not report bad results; the only way to know about them is through lawsuits.
Future participation in the American College of Surgeons NSQIP-Pediatric will provide unbiased, large-scale data on thoracoscopic versus open surgery complications and mortality rates.
Currently there is insufficient data to make fully informed consent regarding CCAM management.
Thoracoscopic lobectomy should not be taken lightly; it requires a major investment in time and learning to become proficient.
Centers that routinely perform thoracoscopic lobectomy and see high volumes of CCAMs can treat them with extremely low morbidity and no mortality.
Infants generally do very well after thoracoscopic lobectomy, typically staying in the hospital for two days before going home.
In a review of the last 100 thoracoscopic lobectomies (not including sequestrations), there were only two transfusions and two prolonged air leaks as complications.
Management decisions for CCAM should be made selectively based on resources, family preferences, patient factors, and location, rather than a dogmatic approach of operating on everyone.