Ann Thorac Surg 2009;88:2010-2011. doi:10.1016/j.athoracsur.2009.05.017
© 2009 The Society of Thoracic Surgeons
Case Reports
Intralobar Pulmonary Sequestration Associated With Marked Elevation of Serum Carbohydrate Antigen 19-9
Satoshi Ambiru, MDa,*,
Shunta Nakamura, MDa,
Motoji Fukasawa, MDb,
Osamu Mishima, MDb,
Takeichiro Kuwahara, MDc,
Akihiko Takeshi, MDb
a Department of Surgery, Oami Hospital, Oamishirasato, Japan
b Department of Respiratory Surgery, Kameda General Hospital, Kamogawa, Japan
c Department of Clinical Pathology, Chiba Foundation for Health Promotion and Disease Prevention, Chiba, Japan
Accepted for publication May 5, 2009.
* Address correspondence to Dr Ambiru, Department of Surgery, Oami Hospital, 884-1 Tomita, Oamishirasato, 299-3221, Japan (Email: ambiru-s{at}umin.ac.jp).
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Abstract
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This report describes a 62-year-old man who experienced elevated serum carbohydrate antigen 19-9 (CA19-9) levels (>500 U/mL) for 4 years, and was finally diagnosed with right intralobar pulmonary sequestration. Surgery confirmed the presence an aberrant artery arising from the descending thoracic aorta and entering the right lower lobe basal segment. Immunohistochemistry demonstrated markedly positive staining of CA19-9 in the ciliated cylindrical epithelia, alveoli, and mucus in the cysts. After pulmonary resection, CA19-9 levels decreased to within a normal range. Therefore, the cause of the elevated serum CA19-9 levels in this case was almost certainly due to intralobar pulmonary sequestration.
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Introduction
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Carbohydrate antigen 19-9 (CA19-9) is an important and recognized tumor marker. Elevated serum CA19-9 levels are significant because they may indicate malignancies in the digestive tract, biliary tract, pancreas, or lungs. However, in rare cases, the cause of elevated serum CA19-9 levels is unknown. False-positive CA19-9 results are found in patients with certain digestive system and respiratory diseases [1, 2]. As a further example of the diagnostic pitfalls associated with using CA19-9, this report presents a case of intralobar pulmonary sequestration, which caused elevated serum CA19-9 levels.
Due to elevated serum CA19-9 levels, a 62-year-old man was monitored for 4 years beginning in September 2003. His main complaint was occasional dull abdominal pain. His family doctor suspected hepato-biliary-pancreatic disease and sent him to the Oami Hospital to determine the cause of the elevated serum CA19-9 levels. Laboratory test results were mostly within the normal ranges, but serum CA19-9 levels were consistently elevated (>500 U/mL [normal range <37.0 U/mL]) with no diagnosed cause. The patient had no history of smoking, but a computed tomographic scan of the chest showed a multi-cystic shadow in the right lower region, which was diagnosed as suspected pulmonary inflammation. However, he had received no treatment for pulmonary disease since the suspected condition was not obviously malignant, and he had displayed no respiratory disease symptoms. In December 2007, he was admitted to Kameda General Hospital for further evaluation of the shadow in the right lower lobe. A computed tomographic scan of the chest revealed a multilocular cystic lesion in the right S10 region. A multi-detector computed tomographic angiography demonstrated an anomalous artery arising from the descending thoracic aorta (Fig 1). A right intralobar pulmonary sequestration was diagnosed, and a pulmonary resection was performed. Surgery confirmed the presence of an aberrant artery arising from the thoracic aorta and entering the right lower lobe basal segment. Microscopic findings were consistent with intralobar pulmonary sequestration. Immunohistochemistry demonstrated markedly positive staining of CA19-9 in the ciliated cylindrical epithelia, alveoli, and especially in the mucus in the cysts (Fig 2). After the pulmonary resection, serum CA19-9 levels decreased to within the normal range, and normalized serum CA19-9 levels persisted until the present time.
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Fig 1. (A) Axial computed tomographic chest image shows the multilocular thin-walled cystic lesions in the right lower lobe. (B) Coronal multi-detector computed tomographic angiography shows an aberrant artery extending from the descending thoracic aorta (arrow).
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Fig 2. Immunohistochemical staining of CA19-9 was positive in the ciliated cylindrical epithelia, alveoli, and especially the mucus in the cysts. (A) Magnification, x40. (B) Magnification, x100.
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Comment
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This case report confirms that serum CA19-9 elevation may be found in pulmonary sequestration. Examinations of the patient for a time period of 4 years detected no diseases of the digestive system. After resection of the pulmonary sequestration, the serum CA19-9 levels were normalized. Immunohistochemical staining demonstrated production of CA19-9 in the bronchial and alveolar epithelia of the sequestrated lung. Moreover, mucus in the cysts stained strongly for CA19-9, thus suggesting remarkably high levels of CA19-9 therein. Therefore, it is possible that the elevated serum CA19-9 levels were caused by the pulmonary sequestration. The CA19-9 produced in the epithelia of the sequestration tissue may have become concentrated in the mucus in the cysts, and then transferred into the blood through the injured mucosa of the cyst walls. Other studies have also reported similar elevation of serum CA19-9 associated with pulmonary sequestration [3, 4]. If our suggested mechanism for the elevation of serum CA19-9 is correct, then patients with pulmonary sequestration may actually have elevated serum CA19-9 levels. Carbohydrate antigen 19-9 is a tumor marker that is not specific for cancer; therefore, to avoid potential diagnostic pitfalls, it is important for clinicians to be aware of respiratory diseases associated with elevated serum levels.
Carbohydrate antigen 19-9 secreted by the bronchiolar epithelia may also appear in the serum as a result of airway damage in the lower respiratory tract [5]. In addition, CA19-9 is observed in the normal epithelial lining of the biliary tract, gastric mucosa, pancreatic duct, and bronchial glands [6]. Bronchial mucus contains large amounts of CA19-9, which appears to be produced in the columnar epithelia of respiratory glands, even if the serum CA19-9 levels are normal [6]. These previous studies are consistent with the observed association between pulmonary sequestration and elevated levels of serum CA19-9 in this current case study report.
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References
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- Duffy MJ. CA 19-9 as a maker for gastrointestinal cancers: a review Ann Clin Biochem 1998;35:364-370.[Medline]
- Kodama T, Satoh H, Ishikawa H, Ohtsuka M. Serum levels of CA19-9 in patients with nonmalignant respiratory diseases J Clin Lab Anal 2007;21:103-106.[Medline]
- Shiota Y, Kitade M, Furuya K, Ueda N. A case of intralobar pulmonary sequestration with high serum CA19-9 levels Acta Med Okayama 1988;42:297-300.[Medline]
- Yagyu H, Adachi H, Furukawa K, et al. Intralobar pulmonary sequestration presenting increased serum CA19-9 and CA125 Intern Med 2002;41:875-878.[Medline]
- Mukae H, Hirota M, Kohno S, et al. Elevation of tumour-associated carbohydrate antigens in patients with diffuse panbronchiolitis Am Rev Respir Dis 1993;148:744-751.[Medline]
- Matsuoka Y, Endo K, Kawamura Y, et al. Normal bronchial mucus contains high levels of cancer-associated antigens, CA125, CA19-9, and carcinoembryonic antigen Cancer 1990;65:506-510.[Medline]
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Abstract
Introduction
