Ann Thorac Surg 2008;85:336-339. doi:10.1016/j.athoracsur.2007.07.033
© 2008 The Society of Thoracic Surgeons
Case Reports
Pulmonary Lymphangioma
Stefan Limmer, MDa,*,
Manuela Krokowski, MDb,
Peter Kujath, MDa
a Department of Surgery, University Hospital of Schleswig-Holstein, Lübeck, Germany
b Department of Pathology, University Hospital of Schleswig-Holstein, Lübeck, Germany
Accepted for publication July 10, 2007.
* Address correspondence to Dr Limmer, Department of Surgery, University Hospital of Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, Lübeck, 23538, Germany (Email: stefan.limmer{at}uk-sh.de).
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Abstract
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We report on the very rare case of a 49-year-old man with a large solitary pulmonary lymphangioma. Rapid growth of the tumor led to dyspnea and pain. A chest roentgenogram and computed tomography scan revealed a large 18 x 12-cm space-occupying cystic lesion in the posterior mediastinum. The tumor was resected by lateral thoracotomy. Histopathology revealed a pulmonary cystic lymphangioma.
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Introduction
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Lymphangioma is a congenital lymphatic malformation that develops from a defective bud formation [1]. A distinction is drawn between pulmonary lymphangioma (local defect), pulmonary lymphangiomatosis (multifocal defects), congenital lymphangiectasia (pathologically dilated lymphatic vessels in their anatomical position), mixed vascular and lymphatic angioma, and combined defects such as lymphangioleiomyomatosis. Histomorphologically, these lesions show multiple cystic cavities lined with endothelium. We report the case of a very rare large solitary cystic pulmonary lymphangioma.
A 49-year-old man was admitted to the thoracic surgery unit of the Schleswig-Holstein University Clinic, Campus Lübeck, for further investigation of a space-occupying lesion in the posterior mediastinum. He had complained for 3 weeks of retrosternal pain, intermittent dyspnea, and right chest pain on deep inspiration. There was no dysphagia. The patient had a smoking history of 25 pack-years.
The physical examination and laboratory variables showed physiologic values within normal reference ranges. His lung function was normal for age, with a forced expiratory volume in 1 second (FEV1) of 3.13l, and FEV1/inspiratory vital capacity of 72%. The blood gas analysis showed normal values (PO
2, 60.8 mm Hg; PCO
2, 35.7 mm Hg).
A computed tomography scan of the chest (Fig 1) showed a hypodense, smoothly marginated, cystic lesion in the posterior mediastinum measuring 18 x 12.5 x 7.3 cm, with a marked space-occupying effect, especially on the left atrium of the heart and the pulmonary vessels. The clinical picture and the roentgenogram findings were suggestive of a bronchogenic cyst.
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Fig 1. Computed tomography scan of the lungs showing a cystic round mass with a uniform density (15 Hounsfield units) in the posterior mediastinum (the line measures 12.37 cm.)
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In view of the size of the space-occupying lesion, thoracotomy was indicated. On Sep 29, 2006, we performed a right anterolateral thoracotomy in left recumbent position using single-lung ventilation without any problem. After opening the chest, we found a large cystic structure measuring about 20 x 20 cm between the hilus of the lung (ventral) and the esophagus (dorsal; Fig 2). The cyst was removed. It was perforated in preparation, resulting in the discharge of a solid gelatinous secretion. The site of origin for the cyst was identified as a peribronchial lymphatic vessel; in addition, the cyst was also connected to the right primary bronchus. After resection of the cyst, the site of junction with the bronchus was sutured. The procedure was ended with the insertion of 2 Bülau drainage tubes.
Histopathologic examination revealed numerous cystic cavities lined with endothelium, without any evidence of ciliated epithelium on the lined sections of the cyst wall (Fig 3). The immunohistochemical expression sample was positive for cluster of differentiation antigen 31 (CD31), lymphatic vessel endothelial receptor 1 (LYVE1), and podoplanin, and was negative for prospero-related homeobox (PROX), CD34, and smooth muscle actin. The histologic diagnosis was ultimately made on the basis of the immunohistochemical sample.
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Fig 3. Photomicrographs show large cystic spaces lined by cytologically inconspicuous predominantly flattened endothelium surrounded by normal lymphatic tissue. (A) Hematoxylin and eosin stain, x2.5 original magnification. (B) Hematoxylin and eosin stain, x40 original magnification. (C) Immunohistochemical expression of cluster of differentiation 31 (CD31) antigen at x10 original magnification. (D) Immunohistochemical expression of lymphatic vessel endothelial receptor 1 (LYVE-1) at x20 original magnification.
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The patient’s postoperative course was without complications. He was discharged home on postoperative day 10 and remains free of symptoms at check-ups.
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Comment
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Lymphangioma is a lymphatic malformation that is less well characterized than other vascular lesions. It consists of abnormally developed and proliferating lymphatic vessels that do not communicate with the rest of the lymphovascular system. If there is a lack of lymphatic drainage, this leads to an increase in the size of the lymphangioma, upon which the condition then becomes clinically apparent. The underlying defect is considered to be an abnormality in lymphangiogenesis [2], but chromosomal anomalies have also been described (eg, in Turner syndrome). Depending on its histomorphologic structure, lymphangioma is classified as a capillary, cavernous, or cystic subtype.
Lymphangioma may occur in any part of the body, the most common sites being in the region of the head, neck, abdomen, and axilla. In 90% of cases, lymphangioma develops in early childhood up to the age of 2; however, thoracic lymphangioma is a rare tumor.
Mediastinal lymphangioma is especially known in children. It accounts for 0.7% to 4.5% [3] of all mediastinal tumors and is found in the upper and anterior mediastinum in particular. Thoracic lymphangioma in adults is usually discovered as a chance finding on a roentgenogram examination or during an investigation of clinical symptoms.
Pulmonary lymphangioma is extremely rare. According to our literature search, only 17 case reports have been published in the international literature to date. These case reports refer to 9 males and 8 females, with a median age of 36.6 years (range, newborn to 66 years). In a review of more than 20 patients with mediastinal and pulmonary thoracic lymphangioma, Shaffer and colleagues [4] found a slight predominance of women, whereas Wilson and colleagues [5] found only 6 male patients in their review.
The intrapulmonary location of the tumor is variously reported in the literature, but shows a marked predominance of the right lung (n = 13) versus the left (n = 4) [6]. Although Lee and colleagues [7] report on the increased occurrence of lymphangioma in the basal segment of the lung, a review of the literature did not show any predominance versus the other regions of the lung (8 basal, 2 central at the hilus, 7 apical).
The clinical presentation is dependent on the age of the patient and the extent of the tumor. Whereas dyspnea is the predominant presenting symptom in children, it is often a chance radiologic finding in adults and is initially suspected to be a bronchial malignancy. According to the literature, 9 of 17 patients were asymptomatic; in 8 symptomatic patients, the cardinal symptoms were cough and dyspnea. Holden and colleagues [8] reported on a patient with mediastinal and pulmonary lymphangioma who presented with hemoptysis. Kim and colleagues [9] described the treatment of a baby with pneumothorax accompanied by a complete cystic remodeling of the pulmonary parenchyma. In our patient, progressive dyspnea led to further diagnostic investigation and therapy. In no case was the correct diagnosis established before surgery. According to Wada and colleagues [3], the working hypothesis most frequently given was a congenital cystic tumor or bronchogenic cyst. In 5 patients, not even a working hypothesis was presented despite extensive diagnostic procedures [3, 9].
Chest roentgenogram, sonography, thoracic computed tomography (CT), and magnetic resonance imaging (MRI) have proved useful for determining the size and extent of the lesion. The value of diagnostic procedures using high-resolution CT or MRI, or both, has been described for lymphangioma, but it is often not possible to differentiate the lesion from a bronchogenic cyst or tumor. The morphologic findings obtained from CT scans are predominantly described as cystic, clearly marginated, thin-walled space-occupying lesions, although spurs and calcifications are also reported. Imaging procedures cannot offer a sufficient differentiation from non-small cell lung cancer or cystic malformation.
Our patient’s thoracic CT showed a homogeneous cyst (15 Hounsfield units) in the posterior mediastinum without the use of any contrast medium. Ultimately in our case, too, the correct diagnosis was only established after surgical resection and histomorphologic and immunohistochemical examination. It is important here to differentiate a cystic epithelial tumor or a lymphangioleiomyomatosis.
In view of the rarity of the diagnosis, published reports consistently favor surgical tumor resection, both for diagnostic and also for therapeutic reasons. Thoracoscopic resection is described by Hilliard and colleagues [10] as a successful and adequate method in solitary peripheral tumors [6, 10], but the size of the tumor in our case led us to perform a primary thoracotomy.
Although pulmonary lymphangioma is a benign disorder, surgical resection was necessary in half of all published cases simply for reasons of clinical symptoms. To date, no recurrence of pulmonary lymphangioma has been reported, and our patient has also had no symptoms and shows no signs of recurrence 3 months after surgery.
In conclusion, intrapulmonary lymphangioma is an extremely rare lesion. Males and females are equally affected, and the age of patients ranges from the newborn to the mid-60s. Despite extensive invasive and noninvasive diagnostic procedures, a reliable diagnosis is only possible by histopathologic examination. The method of choice is surgical resection, partly also because of the unclear picture regarding the malignancy of the tumor. Patients remain largely free of symptoms on follow-up.
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References
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- Katzenstein AL. Katzenstein and Askin’s surgical pathology of non-neoplastic lung disease: 13 Major Problems in Pathology Series. 4th ed.. Philadelphia, PA: Saunders; 2006.
- Oshikiri T, Morikawa T, Jinushi E, Kawakami Y, Katoh H. Five cases of lymphangioma of the mediastinum in adult Ann Thorac Cardiovasc Surg 2001;7:103-105.[Medline]
- Wada A, Tateishi R, Terazawa T, Matsuda M, Hattori S. Case report: lymphangioma of the lung Arch Pathol 1974;98:211-213.[Medline]
- Shaffer K, Rosado-de-Christenson ML, Patz Jr EF, Young S, Farver CF. Thoracic lymphangioma in adults: CT and MR imaging features Am J Roentgenol 1994;162:238-239.[Medline]
- Wilson C, Askin FB, Heitmiller RF. Solitary pulmonary lymphangioma Ann Thorac Surg 2001;71:1337-1338.[Abstract/Free Full Text]
- Enzinger FM, Weiss SW. Tumors of lymph vesselsIn: Gay SM, editor. Soft Tissue Tumors. St. Louis, MO: Mosby; 1995. pp. 679-700.
- Lee CH, Kim YD, Kim KI, et al. Intrapulmonary cystic lymphangioma in a 2-month-old infant J Korean Med Sci 2004;19:458-461.[Medline]
- Holden WE, Morris JF, Antonovic R, Gill TH, Kessler S. Adult intrapulmonary and mediastinal lymphangioma causing haemoptysis Thorax 1987;42:635-636.[Free Full Text]
- Kim WS, Lee KS, Kim I, et al. Cystic intrapulmonary lymphangioma: HRCT findings Pediatr Radiol 1995;25:206-207.[Medline]
- Hilliard RI, McKendry JB, Phillips MJ. Congenital abnormalities of the lymphatic system: a new clinical classification Pediatrics 1990;86:988-994.[Abstract/Free Full Text]
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Abstract
Introduction
