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LYMPHANGIOHEMANGIOMA OF THE ANTERIOR MEDIASTINUM: CASE REPORT AND LITERATURE REVIEW
Peshev Zhivko Vladimirov
Assistant Professor at the Department of General and Clinical Pathology and Forensic Medicine, Medical University, Plovdiv,
Bulgaria
Stefanova Peneva Penka
Professor at Clinic of Pediatric Surgery, St. George University Hospital, Plovdiv, Bulgaria
Bivolarski Iliya Petrov
Assistant at the Department of General and Clinical Pathology and Forensic Medicine, Medical University, Plovdiv, Bulgaria
ABSTRACT
Vascular mediastinal tumors are a very rare disease. Even much more rare are the lymphangiohemangiomas with this localization. Lymphangiohemangioma is a hybrid term for a benign vascular tumor with the presence of lymphatic and venous component, caused by congenital malformation. We report the case of a 4-year old child with spontaneous rupture of such tumor, which led to massive hemorrhage in the mediastinum and the pleural cavity, with lethal end after manifestations of hemorrhagic shock. The diagnosis was made after biopsy and necropsy study of the cystic tumor formation found in the mediastinum. The conducted immunohistochemical study with CD34 h D2-40 Podoplanin showed that the endothelial coating of the lymphangious component had an expression of D2-40 and slightly less distinct expression of CD34 for the endothelial cells of the venous vessels.
Keywords:lymphangioma, hemangioma, lymphangiohemangioma, mediastinum, autopsy
Introduction: Lymphangiohemangiomas are very rarely found. It is even much more rare to find them in the mediastinum. The frequency of vascular tumors in this area is 0.5 - 0.8%, and out of them as lymphangiohemangiomas were only described single cases.[2,7] They are usually congenital and occurring in childhood. They can grow with the growth of the body. They rarely show a clinical picture of pressure on the surrounding tissues and organs.[13]
Autopsy case: We report an autopsy case of a 4-year old child admitted at the Clinic of Pediatric Surgery with evidence for hemorrhagic shock caused by bleeding in the mediastinum. Emergency surgery was performed, which found cystic formation, part of which was removed. During the surgery came the lethal end. The biopsy examination showed cystic dilated cavities coated with endothelium and fibrous walls with sparse smooth muscle fibers infiltrated by small mature lymphocytes. (Fig. 1 and Fig. 2) In the lumen of part of them was found blood and fresh mixed thrombi.
Fig. 1 Lymphangious component HE. x 10 Fig. 2 Venous vessels HE. x 10
The performed immunohistochemical study with CD34 and D2-40 Podoplanin showed that the endothelial coating of the lymphangious component had an expression of D2-40 (Fig. 3) and slightly less distinct expression of CD34 for the endothelial cells of the venous vessels. (Fig. 4)
Fig. 3 D2-40 Podoplanin x 20
After an obduction performed on the soft tissues of the anterior and posterior mediastinum, they were found thoroughly soaked in blood. An open multichamber cystic formation was found with diameter of 3 cm, with 1-2 mm thin walls, with bloody contents. The right lung collapsed towards the hilus and in the pleural cavity was found about 300 ml liquid blood of lighter colour. In the other parenchymal organs there were morphological changes typical for acute posthemorrhagic anemia.
Discussion: The vascular tumors of the mediastinum occur more often in its anterior section and very rarely in the posterior. They include two large groups - hemangiomas and lymphangiomas. Individual authors describe not big series of vascular tumors with this localization. The frequency of the hemangiomas here is about 0.5%, which is 90% of the vascular tumors, whereas the lymphangiomas are much more rare and in the world literature were described only single cases. [5,7,9,10,13] They are mainly found in childhood, up to 10 years of age and rarely with adults. They are well distinguished but not encapsulated. They don't have the characteristic clinical features and for a long time don't manifest any symptoms, until they enlarge their size and press the surrounding tissues and organs. They rarely cause chest pain, constriction, cough. They are usually found by chance. Their diagnosis is difficult and includes imaging with CT and MRI and possibly transthoracic puncture with a needle biopsy.[13] The choice of treatment is their radical surgical removal. Sometimes as intraoperative complication appears massive hemorrhage due to their abundant blood supply and close location to large vessels and their accretion with the surrounding tissues.[2,8]
According to the latest classification of the World Health Organization of 2013, they are regarded as benign mesenchymal tumors originating most probably from vascular malformations. [1,5] They are rarely located in the mediastinum. About 0.8% of them are hemangiomas and about 0.5% are lymphangiomas. In this classification there is no group of "lymphangiohemangiomas". [5] This gives ground to the assertions of some authors to treat them as a separate group of lymphatic and venous malformations by analogy with the arteriovenous malformations. [6]
Their growth has been associated with congenital obstruction or agenesis of the lymph and blood vessels, which may lead to their abnormal expansion and further formation of a tumor. Their tumor nature is disputed by many authors, especially when it regards the lymphangiomas.[1,7,10,11] Their growth is explained with the inability of the lymph vessels to connect with the venous system. The impaired embryonic development of the vessels occurs within the period of 4 - 10 gestation weeks. The embryonic development of the lymphatic system has not been
Fig. 4 CD34 x 20
well clarified. There are two main theories about the origin and development of the lymphatic system. The one examines the origin of the lymphatic vessels as a result of proliferation of the venous vessels with their subsequent branching and formation of a separate network. According to the other theory of self-development, they do not develop out of the venous vessels but independently and simultaneously with the venous vessels from the same slit-shaped spaces coated with endothelium. After their formation, around them gather groups of lymphocytes, which later form the lymph nodes. The second theory has more supporters, and particularly regarding the lymphangiohemangiomas, the theory of proliferation is more trustworthy.^] The presence of lymphatic and venous component can be explained precisely with this close connection during their embryonic development. It can be seen as early as birth and growing with the growth of the body.[1,3] There are single cases reported as secondary -after injury or infections.[2]
Lymphangiohemangiomas are rare congenital, tumor-like anomalies, which in their histological study show the presence of both lymphatic and vascular components. Their mediastinal localization is very rare and only single cases have been reported. They are cystic formations without own capsule and are adjacent to the pericardium or the major blood vessels in the chest cavity.
They are mainly observed in childhood, even though they can grow at a later age. The hybrid term lymphangiohemangioma contains in itself description of a tumor process with growth potential, even though they were found as early as birth and were described as vessel malformations. Their pathogenesis is unclear but most probably is identical to that of all vessel tumors in the area of mediastinum and especially the lymphangiomas. Therefore, the lymphangiohemangiomas can be regarded as a subgroup of the lymphangiomas.[7,9,11]
Their histological study reveals a complex malformation consisting mainly of the lymphangiomatous component and dilated venous vessels intimately associated with it.[7,10] These are dilated endothelial spaces in the wall, where can be found smooth muscle fibers. The combination of dilated lymphatic vessels filled with lymph and wall infiltrated with small mature lymphocytes combined with vessel cavities filled with blood is important.[7,11] The immunohistochemical study allows a relatively precise distinction of the lymphangiomous and venous components of the lymphangiohemangioma. The endothelial coating of the lymphangiomous component has a distinct expression of D2-40 and slightly weaker expression of CD34 for the endothelial cells of the venous vessels.[6]
With regard to differential diagnostics other cystic formations of the anterior mediastinum can be considered - pericardial
cysts, bronchogenic cysts, cystic teratomas and thymomas. Imaging detects the cystic formations but the diagnosis of lymphangiohemangioma can only be determined after a histological study.
A serious but rare complication is spontaneous or traumatic rupture and massive bleeding in the surrounding tissues. Common complications are pressure on the surrounding tissues caused by its outgrowth, clinically manifested by constriction, chest pain, cough.[11]
Conclusion
The question of how much the lymphangiohemangiomas are tumor-like malformation or an hybrid term for a benign vascular tumor with the presence of lymphatic and venous component due to congenital malformation remains controversial. They occur mainly in childhood. Their mediastinal localization is particularly rare and has no clinical manifestations. The correct diagnosis can be only determined after biopsy and immunohistochemical study of material from the vascular lesion. A very rare but life-threatening complication is its spontaneous or posttraumatic rupture with massive bleeding. Surgical removal is difficult due to the rich vascular component and can be further complicated with hemorrhage during the operative intervention.
Reference
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