Bronchioloalveolar Carcinoma in a Child With Congenital Cystic Adenomatoid Malformation*

Kaslovsky, Robert; Purdy, Sheila; Dangman, Barbara C.; McKenna, Barbara J.; Brien, Thomas P.; Ilves, Riivo

doi:10.1378/chest.112.2.548

Cited by 86 publications

(46 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The overwhelming majority of BACs are not associated with congenital lung cysts; the average age at diagnosis of all BACs is 68 years. 72 Twenty-one cases of BAC have been reported in patients with CPAM type 1; 24,50,[73][74][75][76][77][78][79][80][81][82][83][84] in this group the median diagnosis age is 18 years (range 6 months to 75 years) ( Table 2). In most of these cases, the BAC was an incidental finding in a cyst resected for other reasons.…”

Section: Bronchioloalveolar Carcinoma (Bac)mentioning

confidence: 99%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

265

215

View full text Add to dashboard Cite

Summary. Surgery for congenital and early childhood lung cysts is often dictated by symptoms such as respiratory distress, infection or pneumothorax. Asymptomatic cysts present a therapeutic dilemma: surgical intervention and ''conservative'' observation have advocates. The risk of malignancy in such cysts is considered by some an indication for surgical intervention and is reviewed in this paper. Pleuropulmonary blastoma (PPB) is the most frequent malignancy associated with childhood lung cysts. Although rare, PPB occurs predictably in certain clinical and familial situations. This unique biology of PPB can inform the cyst management decision. The earliest manifestation of PPB is a malignant lung cyst in young children, clinically and radiographically indistinguishable from benign congenital lung cysts. Histopathologic examination differentiates cystic PPB from the benign cystic variants. Surgical excision of cystic PPB (with or without chemotherapy) cures approximately 85-90% of children. If not excised, cystic PPB evolves to cystic/solid or solid high-grade sarcoma (cure rate 45-60%) by age 2-6 years. Numerous reports of ''malignancy in a congenital lung cyst'' are now understood as the characteristic progression of cystic PPB. PPB is genetically determined in many cases. Detailed family history may reveal the hallmarks of PPB in the patient or young relatives: a unique constellation of diseases including lung cysts, cystic nephroma, childhood cancers, stromal sex-chord ovarian tumors, seminomas or dysgerminomas, intestinal polyps, thyroid hyperplasias, and hamartomas. Pneumothorax and multifocal/bilateral lung cysts also characterize PPB. These diagnoses predict that a lung cyst is more likely PPB than a benign congenital cyst. Patients fitting this pattern deserve histologic diagnosis. The genetic basis for this heritable syndrome is unknown but is being actively investigated.

show abstract

Section: Bronchioloalveolar Carcinoma (Bac)mentioning

confidence: 99%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

265

215

View full text Add to dashboard Cite

show abstract

“…Synergistic activity between increased ␣ 2 -and ␣ 3 -integrin expression in CCAM compared with BPS in the pathogenesis of these congenital anomalies is also possible and has been shown for other integrin family members (16, 20, 27, 36, 50, 53, 66). Differences in cell adhesion between BPS and CCAM may also contribute to differences in their malignant potential (4,30,64).…”

Section: Discussionmentioning

confidence: 99%

Aberrant cell adhesion molecule expression in human bronchopulmonary sequestration and congenital cystic adenomatoid malformation

Volpe

Chung²,

Ulm³

et al. 2009

American Journal of Physiology-Lung Cellular and Molecular Phys

View full text Add to dashboard Cite

Volpe MV, Chung E, Ulm JP, Gilchrist BF, Ralston S, Wang KT, Nielsen HC. Aberrant cell adhesion molecule expression in human bronchopulmonary sequestration and congenital cystic adenomatoid malformation. Am J Physiol Lung Cell Mol Physiol 297: L143-L152, 2009. First published May 1, 2009 doi:10.1152/ajplung.90618.2008.-In many organs, integrins and cadherins are partly regulated by Hox genes, but their interactions in airway morphogenesis and congenital lung diseases are unknown. We previously showed that the Hox protein HoxB5 is abnormally increased in bronchopulmonary sequestration (BPS) and congenital cystic adenomatoid malformation (CCAM), congenital lung lesions with abnormal airway branching. We now report on ␣ 2-, ␣3-, and ␤1-integrin and E-cadherin expression in normal human lung and in BPS and CCAM tissue previously shown to have abnormal HoxB5 expression and on the relationship of cell adhesion molecule expression to Hoxb5 regulation. ␣ 2-, ␣3-, and ␤ 1 -integrins and E-cadherin expression in normal human lung and BPS and CCAM were evaluated using Western blot and immunohistochemistry. Fetal mouse lung fibroblasts with Hoxb5-specific siRNA downregulation were evaluated for ␣ 2-integrin protein levels by Western blot. Compared with normal human lung, a previously undetected ␣ 2-integrin isoform potentially lacking essential cytoplasmic sequences was significantly increased in BPS and CCAM, and ␣ 2-integrin spatial and cellular expression was more intense. Ecadherin protein levels were also significantly increased, whereas ␣ 3 increased in CCAM compared with canalicular, but not with alveolar, stage lung. ␤ 1-integrin levels were unchanged. We conclude that in BPS and CCAM, altered ␣ 2-integrin cytoplasmic signaling contributes to abnormal cellular behavior in these lung lesions. Aberrant cell adhesion molecule and Hox protein regulation are likely part of the mechanism involved in the development of BPS and CCAM.

show abstract

“…Small CCAMs do not require a change in delivery plans and can be electively evaluated postnatally. Due to the association of CCAM with infection and malignant degeneration [9][10][11], we recommend elective resection even in asymptomatic infants. All infants with a prenatally diagnosed lung lesion should undergo a CT scan evaluation postnatally, in part because small lesions can be missed by plain chest radiography [3,4].…”

Section: Fetal Lung Lesionsmentioning

confidence: 99%

History of Fetal Diagnosis and Therapy: Children’s Hospital of Philadelphia Experience

et al. 2003

View full text Add to dashboard Cite

Since its inception in 1995, the mission of the Center for Fetal Diagnosis and Treatment at the Children’s Hospital of Philadelphia (CHOP) has been to provide comprehensive, multidisciplinary expertise in all facets of prenatal diagnosis, reproductive genetics, and prenatal, perinatal, and postnatal treatment for abnormal fetuses. Most of the authors were trained and/or served on faculty at centers in San Francisco (N.S.A., A.W.F., T.M.C., L.J.H.) or Detroit (A.W.F, M.P.J.). Accurate prenatal detection by serial sonographic study and the development of ultrafast magnetic resonance imaging (MRI) scanners has permitted delineation of the natural history of anatomic malformations. Definition of the pathophysiological features that affect clinical outcome and formulation of management based on prognosis has allowed families to make informed decisions. Outcomes have been improved through carefully planned and coordinated perinatal management. In some cases of life-threatening or severely debilitating defects, prenatal surgery has been offered.

show abstract

Bronchioloalveolar Carcinoma in a Child With Congenital Cystic Adenomatoid Malformation*

Cited by 86 publications

References 9 publications

Pulmonary cysts in early childhood and the risk of malignancy

Pulmonary cysts in early childhood and the risk of malignancy

Aberrant cell adhesion molecule expression in human bronchopulmonary sequestration and congenital cystic adenomatoid malformation

History of Fetal Diagnosis and Therapy: Children’s Hospital of Philadelphia Experience

Contact Info

Product

Resources

About