Collection
CCAM (congenital Cystic Adenomatoid Malformation)
Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
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Postnatal Management of Lung Lesions Part III: Pediatric Thoracic Surgery...
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Course directors Drs Alan Flake, Steve Rothenberg and Todd Ponsky, along with additional faculty including Drs Jean Martin Laberge, Ramesh Santhanakrishnan, Giovanna Riccipetitoni, Mark Wulkan, Alexander Razumovskiy, Starla Martinez, Marcel
video27:56 · Sep 2018
Management of Asymptomatic Lung Lesions: Pediatric Thoracic Surgery Part...
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Course directors Drs Alan Flake, Steve Rothenberg and Todd Ponsky, along with additional faculty including Drs Jean Martin Laberge, Ramesh Santhanakrishnan, Giovanna Riccipetitoni, Mark Wulkan, Alexander Razumovskiy, Starla Martinez, Marcel
video20:11 · Jan 2019
Fetal Interventions Part II: Lung Lesions
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Dr. Alan Flake leads a discussion in fetal interventions for prenatally-diagnosed lung lesions.
video16:30 · Jan 2019
Lung Lesions: Fetal Interventions Parts I+II
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Dr. Alan Flake leads a discussion in fetal interventions for prenatally-diagnosed lung lesions.
video30:56 · Jan 2019
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CCAM (Congenital Cystic Adenomatoid Malformation) is a lung abnormality that doctors now usually find before birth through ultrasound [e884-c7, e1088-c22]. Most of these lung lesions do not need any treatment before the baby is born, and very few need immediate attention at delivery [e884-c8, e1088-c23]. Many large lesions that look worrisome on prenatal scans can actually shrink during pregnancy or cause no problems after birth [e884-c11, e1088-c27]. However, true CCAMs don't completely disappear—they may get smaller but remain visible on CT scans after birth [e884-c13, e1088-c29]. Doctors estimate that if a CCAM is left alone, there's about a 20-30% chance it could become infected during the child's lifetime . There's also a very small risk (about 1%) of a type of lung cancer developing in the teenage years or early adulthood [e418-c4, e883-c4]. A rare concern is that about 4% of cystic lung lesions that look like CCAM might actually be a different tumor called pleuropulmonary blastoma, which can't be told apart on scans [e418-c3, e883-c3]. Because of these risks, many surgeons recommend removing the affected part of the lung, though some families choose careful monitoring instead after discussing all the risks and benefits with their surgical team [e883-c38, e883-c39].
CCAM (Congenital Cystic Adenomatoid Malformation) is a lung abnormality that doctors now usually find before birth through ultrasound [e884-c7, e1088-c22]. Most of these lung lesions do not need any treatment before the baby is born, and very few need immediate attention at delivery [e884-c8, e1088-c23]. Many large lesions that look worrisome on prenatal scans can actually shrink during pregnancy or cause no problems after birth [e884-c11, e1088-c27]. However, true CCAMs don't completely disappear—they may get smaller but remain visible on CT scans after birth [e884-c13, e1088-c29]. Doctors estimate that if a CCAM is left alone, there's about a 20-30% chance it could become infected during the child's lifetime . There's also a very small risk (about 1%) of a type of lung cancer developing in the teenage years or early adulthood [e418-c4, e883-c4]. A rare concern is that about 4% of cystic lung lesions that look like CCAM might actually be a different tumor called pleuropulmonary blastoma, which can't be told apart on scans [e418-c3, e883-c3]. Because of these risks, many surgeons recommend removing the affected part of the lung, though some families choose careful monitoring instead after discussing all the risks and benefits with their surgical team [e883-c38, e883-c39].
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Management of Asymptomatic Lung Lesions: Pediatric Thoracic Surgery Part...
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.
clinicalJean Martin0:31 ↗
There are cases of prenatally diagnosed cystic lung lesions that turned out to be PPB after resection.
clinicalJean Martin0:51 ↗
A Toronto series estimates that approximately 4% of cystic lesions that appear to be CCAM will actually turn out to be pleuropulmonary blastoma.
epidemiologicalJean Martin1:24 ↗
There is approximately 1% risk of bronchioloalveolar carcinoma arising from CCAM, typically in teenage years or early adulthood.
epidemiologicalJean Martin1:34 ↗
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.
clinicalJean Martin2:11 ↗
Non-communicating extralobar sequestrations have a very low infection rate; hematogenous infection is possible but rare, similar to any other body tissue.
clinicalJean Martin2:39 ↗
Malignant transformation of extralobar sequestration is extremely rare, with perhaps one or two cases of squamous cell carcinoma described in world literature.
epidemiologicalJean Martin3:00 ↗
Infection and cancer are not good arguments to resect a non-communicating extralobar sequestration.
opinionJean Martin3:11 ↗
The incidence of infection and malignancy in extralobar sequestrations is very low, but has occurred.
clinicalSteve3:44 ↗
Current imaging is not perfect and cannot always definitively diagnose extralobar sequestration or rule out hybrid lesions.
clinicalSteve3:51 ↗
The morbidity of resecting extralobar sequestrations is very low, which favors resection over observation.
opinionSteve4:03 ↗
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.
opinionSteve4:49 ↗
The differential diagnosis for infradiaphragmatic lesions prenatally includes adrenal hemorrhage and neuroblastoma (including cystic neuroblastoma).
clinicalAlan5:18 ↗
Small infradiaphragmatic lesions can be followed by ultrasound; if they stay the same size or get smaller, they do not require resection.
clinicalAlan5:24 ↗
Adrenal hemorrhage will evolve over time and become recognizable on imaging.
clinicalAlan5:37 ↗
A Children's Oncology Group (COG) study showed it is safe to observe adrenal masses suspicious for neuroblastoma, though they must be watched.
clinicalJean Martin5:52 ↗
There is no good way to follow pulmonary lesions; CT scan is the best method but induces a certain incidence of malignancy itself.
clinicalAlan6:17 ↗
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.
clinicalAlan6:32 ↗
It is almost universal that families cannot be counseled with the word 'cancer' and then choose to watch the lesion.
opinion6:56 ↗
When deciding whether to operate, the risks of not operating must be weighed against the risks of operating.
opinionJack7:10 ↗
There has been very little discussion about the risks of performing lobectomy during the panel.
opinionJack7:23 ↗
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.
clinicalJack7:29 ↗
Thoracoscopic lobectomy should not be performed unless the surgeon is experienced; in experienced hands, thoracoscopic lobectomy should have no more morbidity than open lobectomy.
opinionSteve8:08 ↗
Thoracoscopic lobectomy should only be performed by surgeons with advanced thoracic and minimally invasive skills; in such hands, mortality should be zero.
opinionSteve8:26 ↗
If you do enough of any operation, you will have significant complications, whether open or thoracoscopic.
opinionJack8:40 ↗
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.
opinionJack9:04 ↗
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.
epidemiologicalJack9:47 ↗
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.
clinicalJack10:06 ↗
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.
epidemiologicalJack10:28 ↗
The incidence of cancer in CCAM remains extremely low and must be balanced against the risk of the operation.
opinionJack10:44 ↗
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