Ann Thorac Surg 2001;71:1337-1338
© 2001 The Society of Thoracic Surgeons
Case report
Solitary pulmonary lymphangioma
Chad Wilsona,b,
Frederic B. Askin, MDa,b,
Richard F. Heitmiller, MDa,b
a Department of Surgery, Johns Hopkins Hospital, Baltimore, Maryland, USA
b Department of Pathology, Johns Hopkins Hospital, Baltimore, Maryland, USA
Accepted for publication September 19, 2000.
Address reprint request to Dr Heitmiller, Department of Surgery, Johns Hopkins Hospital, Osler 624, 600 Wolfe St, Baltimore, MD 21287-5674
e-mail: rheitmil{at}jhu.edu
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Abstract
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Lymphangioma is an abnormal collection of lymphatics that are developmentally isolated from the normal lymphatic system. Lymphangioma rarely presents as a solitary pulmonary lesion. We present a case of solitary pulmonary lymphangioma and review the literature on its pathogenesis, clinical features, and radiographic findings.
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Introduction
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Lymphangioma is an abnormal collection of lymphatics that are isolated from the normal lymphatic system. It results from an abnormality in developmental lymphangiogenesis. Only rarely does lymphangioma present as a solitary pulmonary "coin lesion." We present a case of solitary pulmonary lymphangioma and review the literature on its pathogenesis, clinical features, and radiographic findings.
We treated a 51-year-old white man who fell, sustaining blunt left chest wall trauma. An emergency room evaluation included PA and lateral chest and selective left rib films. No rib fractures, pneumothorax, or pleural fluid was identified. However, a 1.8-cm peripheral right lung mass was identified, which was interpreted as suspicious for a nonsmall cell tumor (Fig 1). Aside from the left chest trauma, the patient was asymptomatic. Medical history was remarkable for a 60 pack-year smoking history and gastroesophageal reflux. A chest x-ray 16 years earlier was normal. Computed tomography (CT) of the chest and abdomen showed a solitary 1.8-cm peripheral, noncalcified lesion in the right upper lobe anterior segment (Fig 2) without hilar or mediastinal adenopathy. Pulmonary function included a forced expiratory volume in 1 second of 3.8 L, and a single breath deffusing capacity for carbon monoxide (DLCO) of 75% predicted levels. We chose to resect the lesion because of concern that it might be a primary lung cancer. A right lateral thoracotomy was used. A solitary lesion was identified in the upper lobe, anterior segment. The lesion was removed in a wedge fashion. Intraoperative pathologic evaluation found a benign lesion that initially was thought to be a mucocoele. Final pathology results showed it to be a benign mass consisting of contiguous, interconnecting lymphatic spaces (Fig 3). The surgical margins were negative. The diagnosis of pulmonary lymphangioma was made by one of the authors (F.A.), who has described this entity previously [1]. The postoperative course was uncomplicated. The patient is asymptomatic and without recurrent lung nodules 20 months postoperatively.
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Fig 2. Chest computed tomography through the level of the lesion demonstrating a peripheral, noncalcified lesion in the anterior segment of the upper lobe.
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Fig 3. Photomicrograph of the solitary pulmonary lymphangioma showing a benign collection of interconnecting lymphatics. (Hematoxylin and eosin stains. Original magnification: left, x 40; right, x 160.)
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Comment
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Lymphangioma is an uncommon lesion that rarely results in a solitary pulmonary lesion. The term lymphangioma refers to a benign collection of contiguous, intercommunicating lymphatics that do not communicate with the normal lymphatic system (Fig 2). As lymph is secreted into this sequestered space, the lesion grows. This lesion is thought to be the result of a developmental abnormality in lymphangiogenesis. Hilliard and colleagues [2] emphasized that lymphangioma is part of a spectrum of developmental lymphatic disorders that share a common pathogenesis, including lymphangioma (localized lesions), lymphangiomatosis (multifocal lesions), lymphangiectasis (dilated lymphatics in their normal location), mixed vascular lymphatic angiomas, and combinations of lymphatic and other tissues (including the syndrome lymphangiomyomatosis). Recent immunohistochemical data confirmed a common origin of these separate syndromes [3]. All of these abnormalities are thought to be neoplastic, but clinically they can exhibit a wide range of benign or malignant characteristics.
Lymphangiomas can occur in any region of the body in which there is lymphatic drainage; however, they are most common in the head and neck, abdomen, and axilla. The single most common site of occurrence is in the neck, where the lesion is referred to as a cystic hygroma. In the chest, lymphangiomas are most commonly found in the mediastinum, where they account for 0.7% to 4.5% of all mediastinal tumors [4]. Localized pulmonary lymphangiomas have been described but are rare [5, 6]. They have been found in patients over a wide age range (6 months to 67 years). In a review of 20 patients with thoracic lymphangioma (including mediastinal), Shafer and associates [4] noted a slight predominance in women. In our review, all adult patients with intrapulmonary lymphangioma were men, including our patient. Symptoms varied widely depending on patient age and extent of disease. In neonates and infants, cystic lymphangioma often present with pneumothorax and respiratory distress. Holden and colleagues [7] treated a patient with both intrapulmonary and mediastinal lymphangioma who presented with hemoptysis. Most adult patients, however, present with an asymptomatic lung lesion suspicious for primary lung tumor. The CT and magnetic resonance imaging findings of lymphangioma have been described, but these findings do not appear to be sufficiently characteristic to differentiate lymphangioma from nonsmall cell lung cancer reliably. The most common CT appearance of lymphangioma is that of a cystic mass with smooth margins; however, spiculations and calcification have also been noted.
The natural history of intrapulmonary lymphangioma is not known. Surgical resection should be considered in light of the neoplastic nature of these lesions and the difficulty in clinically differentiating them from primary lung cancer. The available data support the use of limited (wedge) lung resection. Minimally invasive techniques have been used successfully [6]. No recurrence of completely resected intrapulmonary lymphangioma has been reported.
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Acknowledgments
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This work was supported by the Lawrence R. Katz Thoracic Internship Award.
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References
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Langston C., Askin F.B. Pulmonary disorders in the neonate, infant, and child. In: Thurlbeck W.M., Churg A.M., eds. Pathology of the lung, 2nd ed. New York: Thieme Medical Publishers, 1995:151-194.
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Hilliard R.I., Mckendry J.B.J., Phillips M.J. Congenital abnormalities of the lymphatic system: a new clinical classification. Pediatrics 1990;86:988-994.[Abstract/Free Full Text]
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Brown M., Pysher T., Coffin C.M. Lymphangioma and congenital pulmonary lymphangiectasis: a histologic, immunohistochemical, and clinicopathologic comparison. Mod Pathol 1999;12:569-575.[Medline]
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Shafer K., Rosado-de-Christenson M.L., Patz E.F., Young S., Farver C.F. Thoracic lymphangioma in adults: CT and MR imaging features. AJR Am J Roentgenol 1994;162:283-289.[Abstract/Free Full Text]
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Takahara T., Morisaki Y., Torigoe T., Sano S., Nakamura E., Fujino K., et al. Intrapulmonary cystic lymphangioma: report of a case. Surg Today 1998;28:1310-1312.[Medline]
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Holden W.E., Morris J.F., Antonovic R., Gill T.H., Kessler S. Adult intrapulmonary and mediastinal lymphangioma causing hemoptysis. Thorax 1987;42:635-636.[Free Full Text]
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Abstract
Introduction
