Pulmonary sequestration is classified in two types, intralobar pulmonary sequestration in which normal pulmonary tissue and visceral pleura is shared, and extralobar pulmonary sequestration where pulmonary tissue is surrounded with the pleura of the lesion itself1
. In intralobar pulmonary sequestration, some cases features with elevated CA19-9 level alone or together with the elevation of other tumor marker such as CEA or CA1255
, which is also reported in extralobar pulmonary sequestration8
. In the cases of elevated CA19-9 in patients with pulmonary sequestration, no abdominal symptoms were found and most cases were accidently identified by blood test for diagnosis of pulmonary sequestration or prior to surgery5
. Although the exact cause for the condition is not yet, main mechanism is thought to be the effect of chronic inflammatory process of epithelial cells, which is based on the histologic findings of abundancy of mucus, identification of tumor marker response, and no signs for infection in the patients with remarkable elevation of CA19-9 after several years, who had normal level of CA19-9 at the time of initial identification of emphysematous lesion before5
. According to other literature, tumor markers are reported to be synthesized in normal bronchial epithelial cells, and elevated in the blood by productive process of epithelial cells and accumulation in the pulmonary sequestration9
. Some other reports claims that the proliferation of bronchial epithelial cells is the cause for the elevation of tumor marker by chronic inflammatory process by infection of pathogenic organisms such as Aspergillus or Mycobacterium10
. However, in this case, although histologic confirmation of tumor markers through postoperative immunohistochemical staining or identification of infection of other organism in tissue was not available, tumor markers is thought to be elevated in the repetitive process of injury and recovery as chronic inflammatory process.
Over 50% of the patients with pulmonary sequestration, cough, sputum, and frequent pulmonary infection are typically accompanied, but no symptoms could be found in many cases2
. Although there still exists controversy whether to perform surgery or not in asymptomatic young patients as in this case, embolization of abnormal artery or active surgical intervention is required because serious condition could possibly happen such as hemoptysis or hemothorax, followed by rapid aggravation of disease course in the pulmonary sequestration12
. In the cases with elevation of tumor markers like CA19-9 without any other gastrointestinal diseases, identification of any hidden pulmonary diseases is required as fatal situation such as hemorrhage due to pulmonary sequestration could occur if unnoticed. And also, if ignorant of the association with this kind of benign pulmonary diseases, unnecessary examination for gastrointestinal diseases could be implemented by misidentification of gastrointestinal malignancy. Regarding pulmonary disease, such as bronchiectasis, one case was reported related to elevated CA19-9 in Korea14
Decreased survival rate was reported in the group with higher CA19-9 level in idiopathic interstitial pneumonia and collagen vascular disease associated lung diseases, and the level of CA19-9 was expected to be related to disease activity in these condition4
. However, further study on the association of survival rate with the tumor marker in pulmonary sequestration is required, as the disease was not included on this study. Furthermore, additional study on the association of the change of tumor marker level with diseases activity or prognosis in pulmonary sequestration is also required. Here we report a novel case of pulmonary sequestration showing increased CA19-9 with literature review, which has not yet been reported in Korea to our best knowledge.