Ann Thorac Surg 1997;64:1476-1478
© 1997 The Society of Thoracic Surgeons
Case Report
Lymphangiohemangioma of the Mediastinum
Marc Riquet, MD,
Josette Brière, MD,
Françoise Le Pimpec-Barthes, MD,
Philippe Puyo, MD
Service de Chirurgie Thoracique and Service d'Anatomo Pathologie, Hôpital Laennec, Paris, France
Accepted for publication June 11, 1997.
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Abstract
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Lymphangiohemangiomas of the mediastinum are exceedingly rare. Clinically they tend to behave like lymphangiomas. We report 3 new cases. All 3 cases showed both lymphatic and vascular components. One case was associated with a left superior vena cava. In both other cases computed tomography with dynamic scans demonstrated the vascular components. Combined lymphatic venous malformation was thus evidenced in the 3 cases and supports that lymphangiohemangiomas are malformative in origin.
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Introduction
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Primary mesenchymal tumors of blood and lymph vessel origin are infrequently encountered in the mediastinum [1]. Tumors of lymph vessels are termed "lymphangiomas," are benign, and are considered to arise from congenital malformation of the lymphatics. There are rare tumors that on histologic examination show vascular and lymphatic elements. Such tumors have been reported as "lymphangiohemangioma" [2, 3]. We observed 3 new cases of such tumors in our Thoracic Surgery Department.
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Case Reports
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Patient 1
A 30-year-old man was admitted in May 1982 because chest x-ray films incidentally demonstrated a superior mediastinal mass predominantly anterior and to the left of the aortic arch. Phlebography demonstrated compression of the left innominate vein and a large venous branch entering the mass downward. At operation a large cystic mass was found and removed. It was crossed downward by a vein originating from the left innominate vein and entering the left auricle (left superior vena cava). The postoperative course was uneventful. Histologic examination revealed a lymphangiohemangioma (Fig 1
). This patient of African origin was lost to follow-up.
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Fig 1. . (Patient 1.) Histology: the tumor is composed of dilated endothelial spaces and smooth muscle cells. Note the combination of lymph-filled and blood-filled spaces (arrow). The same histology was observed in the 3 cases by the same pathologist (Dr Brière). (Hematoxylin and eosin; x100 before 43% reduction.)
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Patient 2
A 15-year-old boy with a history of asthma was admitted in November 1987. Chest x-ray films demonstrated a right superoanterior mediastinal mass. Computed tomographic scan showed a large mass surrounding the vena cava without calcification. Enhanced images demonstrated blood vessels inside the mass. Frozen section of the tumor demonstrated a lymphangioma, which was resected by sternotomy. Numerous venous vessels were connected to the upper part of the vena cava, and the operation was hemorrhagic and require resection of a segment of the phrenic nerve. The postoperative course was uneventful. Microscopic analysis revealed a lymphangiohemangioma. The patient is still doing well without recurrence.
Patient 3
An 18-year-old boy was admitted in July 1992 with the presumed diagnosis of mediastinal lymphangioma. At the age of 16 months, he underwent operation for a lymphangioma of the left axilla. When he was 5 years old, a left superoanterior mediastinal mass was discovered, which progressively increased in size. Computed tomographic scans obtained on admission revealed a mass with few calcifications. Enhanced images (Fig 2) demonstrated blood vessels inside the mass. At operation a large lymphangioma was found and removed. A vein originating from the left innominate vein and entering downward into the mass was cut and sutured. This vein was probably a left superior vena caval remnant. Two venous pedicles located at the level of the pericardium anterior to the left pulmonary artery were ligated. The postoperative course was uneventful. Histologic results were similar to both other cases. This patient is alive and well without recurrence.
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Fig 2. . (Patient 3.) Left-sided lymphangiohemangioma: computed tomographic scan postcontrast images demonstrate blood vessels inside the mass.
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Comment
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"Hemangioma" and "lymphangioma" are generic terms used to describe a variety of vascular and lymphatic lesions with different causes and natural histories. Most of the lesions are hamartomas or tumorlike malformations. They constitute the largest group of tumors or tumorlike malformations in childhood. However, such tumors with similar histologic features may arise later in life [4]. Lymphangiohemangioma or hemangiolymphangioma are combined deformities of blood and lymphatic vessels [5]. This hybrid term implies a potential for growth by cell proliferation [5] and therefore should not be used to describe tumors presenting as congenital vascular anomalies. However, such lymphovenous malformations are already present at birth and in many instances are associated with other vascular malformations, especially of the venous system [6]. In 2 of our patients (patients 1 and 3) such a congenital malformation was evident, and consisted of a left superior vena cava.
Lymphangiohemangiomas tend clinically to behave as lymphangiomas because of their anatomic position. They probably are misdiagnosed as the latter, and this could explain why only 4 previous cases [2, 3, 7] similar to those we observed were found.
In the case reported by Angtuaco and associates [2], computed tomographic scan was helpful in diagnosis because enhancement of the vascular elements within the lesion was demonstrated on the postcontrast images. In Toye and associates' case [3] there was no significant difference despite good opacification of the major intrathoracic arteries and veins, but a subtotal resection had been performed previously, probably interrupting most of the venous pathways. In our patients 2 and 3, venous involvement was perfectly visualized by computed tomographic scan, and in patient 1, computed tomography was not performed but cavography demonstrated blood flow into the tumor, which at operation appeared to be due to a left superior vena cava.
In conclusion, the pathogenesis of hemangiomas and lymphangiomas is not well understood. Lymphangiomas constitute the largest group of tumorlike malformations in childhood. Most often of cervicomediastinal location in the superoanterior mediastinum, they appear to be correlated with anomalies of lymphojugular sacs developing in embryos [8]. They are commonly considered to be of congenital origin for that reason. But most of the reported cases are not well enough documented to explain that origin, and most often histologic examination is not conclusive. Tumors with a predominantly lymphatic nature may be encountered later in life, and many of them are probably of adult onset. Mediastinal lymphangiohemangiomas behave as lymphangiomas, but their association with venous malformations is a sound reason to consider them as a subgroup of lymphangioma, likely of congenital malformative origin.
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Footnotes
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Address reprint requests to Dr Riquet, Service de Chirurgie Thoracique, Hôpital Laennec, 42 rue de Sèvres, 75007 Paris, France.
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References
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- Shields TW, Robinson PG. Mesenchymal tumors of the mediastinum. In: Shields TW, ed. Mediastinal surgery. Philadelphia: Lea and Febiger, 1991:272–88.
- Angtuaco EJC, Jimenez JF, Burrows P, Ferris E. Lymphatic-venous malformation (lymphangiohemangioma) of mediastinum. J Comput Assist Tomogr 1983;7:895–97.[Medline]
- Toye R, Amstrong P, Dacie JL. Lymphangiohaemangioma of the mediastinum. Br J Radiol 1991;64:62–4.[Abstract/Free Full Text]
- Williams HB. Hemangiomas and lymphangiomas. Adv Surg 1981;15:317–49.[Medline]
- Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 1982;69:412–20.[Medline]
- Jackson IT, Carreno R, Potparic Z, Hossain K. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plast Reconstr Surg 1993;91:1216–30.[Medline]
- Balbaa A, Chesterman JT. Neoplasms of vascular origin in the mediastinum. Br J Surg 1957;188:545–55.
- Sabin FR. The lymphatic system in human embryos, with a consideration of the morphology of the system as a whole. Am J Anat 1909;9:43–91.
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Abstract
Introduction
