Intralobar Pulmonary Sequestration Showing Increased Serum CA19-9

Abstract

Carbohydrate antigen 19-9 (CA19-9) is a specific tumor marker of the biliary, pancreatic and gastrointestinal tracts. CA19-9 is occasionally elevated in serum in patiens with benign pulmonary diseases such as bronchiectasis, idiopathic interstitial pneumonia or collagen disease-associated pulmonary fibrosis. Intralobar pulmonary sequestration is an uncommon congenital lung anomaly. It is dissociated from the normal tracheobronchial tree and is supplied by an anomalous systemic artery. There have been some reports of elevation of CA19-9 in this lesion. We report a case of intralobar pulmonary sequestration with elevated serum CA19-9 in a 29-year-old man who was diagnosed with bronchiectasia of left lower lung field on general check up. He had no evidence of any malignant disease in pancreatobiliary or gastrointestinal tracts. Elevated serum CA19-9 level might be encountered with benign pulmonary disease such as pulmonary sequestration.

Intralobar Pulmonary Sequestration and Increased Serum CA 19-9

“…Indeed, when recurrent pneumonia is seen in clinical practice, CA 19-9 tests are not requested by physicians if symptoms of malignancy are absent. Furthermore, when an increase in CA 19-9 has been reported in patients with PS, as in our case, no abdominal symptoms are found and in most cases it is an occasional finding [2,3] . CA 19-9 is synthesized by normal bronchial epithelial cells, and is elevated in blood during epithelial cell proliferation and accumulation in PS tissue.…”

“…PS is a rare lung congenital malformation where the absence of a connection with the normal bronchial tree favours infection and can be a cause of recurrent pneumonia. The most common clinical features are fever and cough due to various bacteria and occasionally uncommon pathogens such as Mycobacterium tuberculosis and fungus [1][2][3][4] . The presence of high levels of CA 19-9 is usually considered to strongly suggest malignant pancreatic-biliary or gastrointestinal disease, but this serum marker may also increase in a number of non-malignant diseases [5] .…”

“…However, slight increases in this marker (usually not more than four to five times the normal limit) are reported in several non-malignant diseases such as chronic hepatitis, primary biliary cirrhosis [5,6] and primary sclerosing cholangitis [7] . An increase in CA 19-9 has been rarely reported in some cases of PS [2,3] . We describe the case of a patient with recurrent pulmonary infections associated with an elevated serum CA 19-9 level, who underwent extensive gastrointestinal diagnostic evaluation before intralobar PS was identified.…”

“…Pulmonary sequestration (PS) is a rare congenital abnormality characterized by the presence of non-functioning lung tissue lacking communication with normal bronchial trees and supplied by an anomalous systemic artery instead of a pulmonary arterial branch [1,2] . There are two forms of PS: intralobar and extralobar.…”

Pulmonary sequestration with Aspergillus infection presenting as massive hemoptysis and hemothorax with highly elevated carcinoembryonic antigen in pleural effusion that mimics advanced lung malignancy

“…Given the sensitivity and specificity of CEA in diagnosing malignancy [9], the patient was strongly suspected of having advanced lung cancer at the beginning, and we were only able to rule out this suspicion after surgery. There were some reports of PS patients with elevated serum CEA and carbohydrate antigens 19-9 and 125 (CA199, CA125), and most of the CEA corresponding titers were considerably lower (less than 10 ng/mL), except M Sekiya et al [12], who reported almost five times higher serum CEA levels than normal [4,5,10,11]. Noguchi and Wei Tang separately found elevated serum levels of CEA and CA199 in some patients with allergic bronchopulmonary aspergillosis [12][13][14][15].…”