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Ann Thorac Surg 1997;63:1169-1171
© 1997 The Society of Thoracic Surgeons

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Case Report

Extralobar Pulmonary Sequestration Presenting as a Mediastinal Malignancy

Medical Service, Portland Veterans Affairs Medical Center, Portland, Oregon

Accepted for publication November 11, 1996.

	Abstract

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Pulmonary sequestration is an uncommon congenital anomaly usually diagnosed in childhood. It frequently presents as a lower lobe mass with symptoms from vascular shunting, anatomic impingements, or associated anatomic defects. This case report describes an adult with asymptomatic extralobar sequestration involving the mediastinum and left upper lobe. The unusual location and radiographic appearance, suggestive of malignancy, led to prompt surgical exploration. We review the literature on this topic, focusing on anatomic variability and difficulties with preoperative diagnosis.

	Introduction

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A 25-year-old man with regular cigarette and alcohol use presented with a mediastinal mass on the chest roentgenogram (Fig 1) during evaluation for an alcohol rehabilitation program. History and physical examination were unremarkable. Computed tomographic scan demonstrated a soft tissue mass in the left paratracheal area (Fig 2) that extended to the aortopulmonary window. The mass also involved the left upper lobe adjacent to the aortic arch (Fig 3). Needle aspiration of the paratracheal mass was nondiagnostic. Fiberoptic bronchoscopy was normal. Because the radiographic appearance suggested malignancy, an excisional biopsy via Chamberlain procedure was performed. A smooth-walled, maroon soft tissue mass was found applied to the aortic arch and superior mediastinum. The incision was extended an additional 3 cm, which revealed a vessel originating from the main pulmonary artery, and a small bronchus originating from the trachea. The abnormal bronchus was stapled. Discrete venous drainage was not identified. The adjacent anterior and middle mediastinum were normal to palpation, so further exploration was not undertaken. Microscopic examination of the soft tissue mass, which measured 5 cm by 5 cm by 2 cm, revealed lung parenchyma encased within pleura, characteristic of extralobar sequestration (Fig 4). Repeat computed tomographic scan done 4 months later showed no change in mediastinal structures. The patient remains asymptomatic after 12 months.

View larger version (138K): [in this window] [in a new window]   Fig 1. . Posteroanterior view of the chest roentgenogram reveals a mass (arrow) silhouetting the aortic arch.

View larger version (82K): [in this window] [in a new window]   Fig 2. . Computed tomographic scan demonstrating right paratracheal mass (arrow).

View larger version (78K): [in this window] [in a new window]   Fig 3. . Computed tomographic scan showing caudal extension of the paratracheal mass, and mass involving the left upper lobe (arrow).

View larger version (158K): [in this window] [in a new window]   Fig 4. . Photomicrograph showing bronchiole with cilia, cartilage, and alveolar tissue filled with inflammatory cells and debris. (Hematoxylin and eosin stain; x10 before 50% reduction.)

	Comment

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Extralobar sequestration occurs in the left hemithorax 80% of the time. It occurs most commonly between the lower lobe and diaphragm, but intradiaphragmatic, pericardial, and retroperitoneal locations have been reported [2]. Although there may be attachment of the sequestration to the mediastinum or diaphragm, upper mediastinal locations are exceedingly rare. Only 2 cases have been reported in the anterior mediastinal area [3, 4]. In Savic and associates' review of 133 cases of extralobar sequestration [5], only 2 occurred within the left mediastinum, and none were reported adjacent to the left upper lobe. Associated congenital abnormalities are common, occurring in up to 65% of cases. They include diaphragmatic hernias (the most common), pectus excavatum, foregut duplications, and congenital heart defects [6]. With extralobar sequestration, the blood supply is arterial in 95% of cases (85% are aortic in origin), but 5% may arise from the pulmonary circulation, as was true in this case. The venous drainage is almost always to the systemic veins. Nearly two thirds of cases are symptomatic, and diagnosed within 6 months of age. Symptoms are caused by left-to-right shunting, anatomic impingements, or associated anatomic defects, and include dyspnea, cyanosis, difficulty feeding, and failure to thrive. Only 10% remain asymptomatic and persist to adulthood [2].

Physicians emphasize the importance of preoperative diagnosis in pulmonary sequestration, because there is risk of uncontrolled hemorrhage if inadvertent injury of the blood supply occurs. However, establishing a preoperative diagnosis is difficult. In one series, intralobar sequestration was suspected or diagnosed preoperatively in only 47 of 100 cases, and extralobar sequestration was diagnosed preoperatively in only 6 (9%) of 66 cases [5]. Aortic arteriography is considered the diagnostic gold standard for sequestrations. Typically, arteriography shows an anomalous systemic arterial supply (50% arise from the thoracic aorta, and 30% from the abdominal aorta) and anomalous venous return. However, in one recent series, arteriography was diagnostic in only 28 of 40 cases [1]. Pulmonary angiography, magnetic resonance imaging, computed tomographic scanning, bronchography, and ultrasonography have all been used in selected cases to confirm preoperative diagnosis.

Treatment of pulmonary sequestration is by surgical resection, especially in symptomatic cases. Preoperative embolization therapy may reduce operative blood loss [2]. Some authors argue that asymptomatic cases can be followed up expectantly. However, at least 1 published case exists of fatal hemoptysis due to hemorrhage from an intralobar sequestration diagnosed several years previously [7].

In summary, we present the case of an adult with extralobar pulmonary sequestration occurring in an unusual location that had associated mediastinal abnormalities resembling malignancy. This led to rapid operative intervention rather than additional studies that might have rendered the diagnosis. This case demonstrates that pulmonary sequestration belongs in the differential diagnosis for asymptomatic mediastinal masses with adjacent lung involvement seen on chest roentgenograms, even in adults.

	Footnotes

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Address reprint requests to Dr Holden, Pulmonary Disease Section (111D), Portland Veterans Affairs Medical Center, PO Box 1034, Portland, OR 97207 (e-mail: holden.william_e{at}portland.va.gov).

	References

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1. Sauvanet A, Regnard JF, Calanducci F, Rojas-Miranda A, Dartevelle P, Levasseur P. Pulmonary sequestration. Surgical aspects based on 61 cases. Rev Pneumol Clin 1991;47:126–32.[Medline]
2. Felker RE, Tonkin ILD. Imaging of pulmonary sequestration. Am J Roentgenol 1990;154:241–9.[Free Full Text]
3. Williams AO, Enumah FI. Extralobar pulmonary sequestration. Thorax 1968;23:220–3.
4. Ke FJ, Chang SC, Su WJ, Perng RP. Extralobar pulmonary sequestration presenting as an anterior mediastinal tumor in an adult. Chest 1993;104:303–4.[Medline]
5. Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R. Lung sequestration: report of seven cases and review of 540 published cases. Thorax 1979;34:96–101.[Abstract/Free Full Text]
6. Stocker JT. Sequestrations of the lung. Semin Diagn Pathol 1986;3:106–21.[Medline]
7. Rubin EM, Garcia H, Horowitz MD, Guerra JJ. Fatal massive hemoptysis secondary to intralobar sequestration. Chest 1994;106:954–5.[Medline]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Pasala S. Ravichandran William E. Holden Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Sippel, J. M. Articles by Holden, W. E. Search for Related Content PubMed PubMed Citation Articles by Sippel, J. M. Articles by Holden, W. E.