Ann Thorac Surg 2009;87:1607-1609. doi:10.1016/j.athoracsur.2008.09.009
© 2009 The Society of Thoracic Surgeons
Case Reports
Macrocystic Lymphatic Malformation in the Pulmonary Parenchyma
Joshua M. Schulman, BAa,
Emily R. Christison-Lagay, MDb,
Harry P.W. Kozakewich, MDa,b,
Phillip M. Boiselle, MDa,c,
Patricia E. Burrows, MDa,b,
Victor L. Fox, MDa,b,
Steven J. Fishman, MDa,b,*
a Harvard Medical School, Boston, Massachusetts
b Departments of Surgery, Pathology, Radiology and Gastroenterology, Children's Hospital Boston, Boston, Massachusetts
c Department of Radiology, Beth Israel Deaconess Medical Center, Boston, Massachusetts
Accepted for publication September 2, 2008.
* Address correspondence to Dr Fishman, 300 Longwood Ave, Boston, MA 02115 (Email: steven.fishman{at}childrens.harvard.edu).
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Abstract
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We present a young girl with a diffuse, macrocystic lymphatic malformation with associated venous dilation involving the left lower pulmonary lobe and mediastinum. Recurrent hemoptysis necessitated left lower lobectomy. This is the first reported case of a macrocystic lymphatic lesion with venous anomalies located within the parenchyma of the lung.
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Introduction
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Congenital anomalies of lymphatic formation comprise a spectrum of disease ranging in presentation from clinically insignificant to life-threatening. Lymphatic malformations may occur either in isolation or in association with other vascular anomalies. The two principle complications of lymphatic malformations are intralesional bleeding and infection, the latter likely related to anomalous flow within affected malformed lymphatic channels. Herein we describe the first reported case of a macrocystic pulmonary, parenchymal, lymphatic malformation with associated venous anomalies in a young girl, which prompted removal of the affected lobe after recurrent episodes of hemoptysis.
A 3-year-old girl underwent excision of a 1.5-cm cystic left cervical mass. Physical examination at the time was notable only for small, cutaneous vascular lesions on the forehead, right malar eminence, and left upper arm. Pathologic assessment of the excised tissue was suggestive of a vascular malformation and prompted further imaging studies of the neck and chest. A T2-weighted magnetic resonance image with gadolinium contrast revealed a vascular malformation involving the parathymic area, superior mediastinum, and left lower lobe, with associated dilated venous structures extending into the parenchyma of the left upper lobe. Because the patient was asymptomatic, it was elected to treat her conservatively with regular radiographic monitoring. At age 9, the patient began to experience recurrent hemoptysis and occasional hematemesis in conjunction with respiratory infections. Bronchoscopy revealed submucosal telangiectasias and dilated lymphatics along the left bronchus. Follow-up endoscopy with concurrent endoscopic ultrasound revealed anomalous submucosal vessels in the distal esophagus and thickening of the esophageal wall. A computed tomographic scan at 10 years of age supported the diagnosis of pulmonary lymphatic malformation of the left lower lobe with extension into the lower mediastinum. In addition, the left inferior pulmonary vein was noted to be grossly dilated and surrounded by the vascular malformation (Fig 1).
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Fig 1. Contrast-enhanced computed tomographic scan demonstrating pulmonary lymphatic malformation affecting the left lower lobe. The malformation encases the left inferior pulmonary vein and is associated with dilation of the vessel (black arrow). Compare with size of normal right inferior pulmonary vein (white arrow).
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Persistent hemoptysis of progressively greater volumes prompted left lower lobectomy at age 11 years. On gross examination, the left lower lobe was enlarged and its surface studded with telangiectatic vessels (Fig 2). Several large, firm masses (measuring up to 5 cm in the greatest dimension) could be identified on palpation. The inferior pulmonary ligament and hilum were encased with the vascular malformation, and the inferior pulmonary vein was massively dilated to a diameter of nearly 3 cm. The left upper lobe had been compressed by the malformation. Pathologic assessment of the resected tissue confirmed the diagnosis of macrocystic lymphatic malformation with associated dilated veins. Histology revealed many channels with thin, predominantly fibrous walls containing blood and hemosiderin-laden macrophages. The endothelium of these channels was immunopositive for the lymphatic marker D2-40 (Fig 3). These histologic findings demonstrated bleeding into the lymphatic malformation, a common phenomenon.
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Fig 2. Appearance of malformation after resection. Studding of the visceral pleural surface is visible, as is the abnormal architecture of the underlying pulmonary parenchyma.
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Fig 3. (A) Resected pulmonary tissue showing irregular blood-containing vascular channels within the parenchyma. (Hematoxylin & eosin; x40). (B) Magnification of these channels reveals thin fibrous walls and luminal hemosiderin-laden macrophages, a finding present in lymphatic but not venous malformations. (Hematoxylin & eosin; x400). (C) Channel endothelium stains for a lymphatic endothelial marker. (D2-40; x400).
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On physical examination 2 months after lobectomy, the patient's breath sounds were excellent, and a chest roentgenogram showed improved volume of the residual left upper lobe. Five years after lobectomy, the patient remained asymptomatic and had achieved national ranking as a biathlete.
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Comment
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Historically termed "lymphangioma" or "cystic hygroma," space-occupying slow-flow vascular anomalies of the lymphatic system consist of localized or diffuse malformations of lymphatic channels that are better characterized as microcystic, macrocystic, or mixed. They commonly demonstrate associated enlargement of their surrounding veins, along with anomalous vessels in the septae between cysts; these vessels may bleed into the lymphatic cysts.
Pulmonary lymphatic anomalies previously described in the literature are diffuse, nonspace occupying, reticular lesions often termed "lymphangiomatosis" or "lymphangiectasia." These patients may have isolated pulmonary involvement or additional anomalous lymphatics in other organs [1]. Nonpulmonary lymphatic malformations in these patients include abdominal and skeletal lesions [2, 3]. Age at presentation ranges from early infancy to late adulthood, and symptoms at the time of presentation include dyspnea, hemoptysis, and respiratory infection [4–7]. Pleural effusions, both chylous and nonchylous, are frequently present.
Space-occupying lymphatic malformative masses, whether macrocystic or microcystic, are not rare in the mediastinum or chest wall. However, we believe that they have not previously been described within the pulmonary parenchyma, although the absence of a systematic nosology by which to characterize lymphatic and venous malformations complicates the search for similar lesions.
Lymphatic malformations are developmental morphologic anomalies, and treatment is limited to surgical resection or occasionally intralesional sclerotherapy. In the present case, intimate association with the pulmonary venous system could have rendered sclerotherapy to be of high risk for systemic embolization, and furthermore, sclerosing of the lymphatic malformation would not have adequately addressed bleeding from the anomalous vessels. Thus, surgical resection was the only option to address the recurrent large-volume hemoptysis.
Although rare, lymphatic malformation should be considered in the differential diagnosis of a multi-cystic pulmonary parenchymal mass, especially in the presence of other vascular anomalies. Lobectomy, when feasible, may be effective in controlling symptoms.
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References
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- Faul JL, Berry GJ, Colby TV, et al. Thoracic lymphangiomas, lymphangiectasias, lymphangiomatosis, and lymphatic dysplasia syndrome Am J Respir Crit Care Med 2000;161:1037-1046.[Free Full Text]
- Hilliard RI, McKendry JBJ, Phillips MJ. Congenital abnormalities of the lymphatic system: a new clinical classification Pediatrics 1990;86:988-994.[Abstract/Free Full Text]
- Alvarez OA, Kjellin I, Zuppan CW. Thoracic lymphangiomatosis in a child J Pediatr Hematol Oncol 2004;26:136-141.[Medline]
- Wagenaar SS, Swierenga J, Wagenvoort CA. Late presentation of primary pulmonary lymphangiectasis Thorax 1978;33:791-795.[Abstract/Free Full Text]
- Holden WE, Morris JF, Antonovic R, et al. Adult intrapulmonary and mediastinal lymphangioma causing haemoptysis Thorax 1987;42:635-636.[Free Full Text]
- Drut R, Mosca HH. Intrapulmonary cystic lymphangioma Pediatr Pulmonol 1996;22:204-206.[Medline]
- 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]
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142(3):
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Abstract
Introduction
