Lymphangioma cavernosum of mediastinum in the 16 month old girl

The article describes a sixteen month old girl with a pathological mass in the mediastinum which was first noticed on chest X-ray. The girl was sent to the Childrens Hospital, Zagreb, where further treatment showed a cystic form in the superior part of upper mediastinum It was a cavernous lymfangioma which was removed entirely surgically.REVIEW OF THE PATIENT A sixteen month old girl was sent to the Childrens clinic Zagreb for diagnostic treatment of a mediastinum mass. The patient was born from a second pregnancy in which the mother had an operation to remove a dermoid cyst of the left ovarian. During the pregnancy she rested and took Ritodrin medication. The delivery, new-born and baby period, as well as psychomotoric development was normal. The girl was hospitalised at the childrens department of the hospital in Bjelovar because of an attack of fever seizures. After the X-ray showed a suspect mass lesion of mediastinum (Figure 1) the girl was transferred to the Childrens Hospital Zagreb. When she came to the hospital she had signs of acute infection of the upper respiratory tract, and other clinical findings were within the normal limits. SE 41/80. Clorides in sweat 21.5 mmol/1. IgA, IgG and IgE in order, IgM 3.69 g/L (N= 0.5-2.0). The nasopharinx aspirate - Pneumococcus. Otolaryngological check-up: Otitis media acuta catarrhalis. Rhinitis catarrhalis acuta.
ECG: some sinus tachicardies, otherwise in order. Ultrasound: expansive sharply marginated cystic partially divided formation in the anterior part of right upper mediastinum (Figure 2). Thorax CT: cystic sharply marginated expansive formation, dimensions 58 x 36 x 65 mm in the region of the anterior part of upper mediastinum and partially of the right middle one (Figure 3). After these examinations the girl was transferred to the surgical department where she underwent VMA, alpha-phetoprotein, ferritin and neurone specific enolaze, which were normal. The right thoracotomy showed a cystic formation in the region of the anterior part of the right upper mediastinum. The cyst was entirely removed. PHD: The material is a piece of tissue, 3cm wide at its widest and with some cyctic formation in its centre, whose walls are of connective tissue and surrounded with connection and fat tissue with a number of lymphatic follicles. There is no epithelia. Some deeper cuts have places with endothelia cells which coat the wall of the hollow formation and the finding proved the diagnosis of cavernous lymphangioma (Figures 4A and B). The lung X-ray taken before the patient was released home was normal (Figure 5). The girl is examined by the ultrasound and X-rays and there has been a control mediastinum CT which was normal.
DISCUSSION
Developmental lymphatic thorax disorders include lymphangiectasiae, localised lymphyngioma, diffuse lymphangioma and lymphangioleomioma (1). Lymphangiomas are tumors of the lymph vessels, and are divided into lymphangioma simplex, cavernosum, cysticum (hygroma cysticum) and mixtum (2, 3). Mediastinal lymphangiomas are very rare. They can be found in the upper, front, middle and posterior mediastinum (4). The anterior part of mediastinum also has tymoma, teratoma, lymphoma, hemangioma, parathyroid adenoma, substernal struma of the thyroid gland, bronchogenic cysts, echinococcus cysts, metastases, aortic arch anomalies and aneurysms of the entry aortic arch, Morgan and Larrys hernias. Middle mediastinum is a predilection for tumors and diverticula of the esophagus, neuroenteric cysts, cystic limphangiomas, tuberculosis, sarcoidosis, echinococcus cysts, congenital heart anomalies, aneurysms of the major blood vessels, traumatic diaphragmal hernias. In the posterior mediastinum tumors are most often of neurogenic origin (neurinoma, ganlioneurinoma, neuroblasoma, neurofibroma), pheochromocytomas, enteric cysts, tumors and oesophagus diverticula, thoracal ducts cysts, meningocoele and hernias of the Bochdaleks aperture (5-10). Our patient had a cavernous lymphangioma inside which there was one cystic formation, and after the operation it was thought it was a bronchogenic cyst. Cysts appear in the upper and middle mediastinum (9, 10, 11). A great number of mediastinal tumors do not provoke any disorders and are noticed by chance on X-ray examinations (7). Symptoms that follow the appearance of pathological processes in mediastinum are various. Pain is usually of substernal localisation and sometimes extends towards the shoulder, neck, along the arm and into the back. Breathing can be accompanied with stridor with jugular inhalation. Hoarseness is a sign of left nervus recurens paralysis. Disphagia is a result of tumor compression on the heart and major blood vessels. If there is aorta aneurysm, pulsations can be conveyed to the trachea and then its walls may be pulled in. Horners syndrome is a result of the tumor compression on the exit sympathic fibres (4, 6, 8). As a result of compression, vena cava superior syndrome may even develop (12). The embryonic beginning of the lymphangioma is not clearly understood and it is not known if it is a malformation, benign neoplasm or hamartoma (7, 13). Cavernous lymphangioma can be small and limited, or it can spread diffusely and provoke a macrodactilia. The hystological lymphatic ducts are noticeably widened and can spread into the fat and muscles, as do the so-called infiltrating angiomas. Non-encapsulated extensions complicate local removing (14). A cystic lymphangioma (hygroma cysticum) is differentially diagnostically very similar which can, most often, be found on the neck and submaxillar regions. Isolated cystic lymphangioma of the mediastinum appears very rarely and is most often found as a cervicomediastinal hygroma (15-24). Cavernous lymphangioma can be localised in the eyelid or orbit (25, 26), or on the nose (27). It appears as a bluish formation and grows very slowly. It can appear on the thorax (29), body (29), breasts (30, 31), small intestine (32, 33), as a muscle skeleton angiomathosis (34, 35), on the upper extremities (36), on the scrotum (37). A special and rare form is a lymphangioma in spleen (38). It rarely occurs as isolated cavernous lymphangioma of the mediastinum. Literature describes a 17 month old girl from Taiwan with relapsing respiratory symptoms and in whom a cavernous lymphangioma in the front upper mediastinum was found. Authors from Japan describe the case of a two year old girl and state that there are six described such cases in their literature in all (40). The diagnosis of a mediastinal tumor is made by chest X-ray. A more detailed analysis is made by angiography, scyntigraphy, ultrasound, computer tomography (CT), magnetic resonance (MR). CT and MR give more or less the same information, but in some cases only MR can give information about malignancy or specific diagnosis (48-51). There is also a special MR technic, so called RARE-MR hydrography (rapid acquisition with relaxation enchancement) (52). MR (magnetic resonance) combined with MRA (magnetic resonance angiography) is the diagnostic method of choice in the survey of generalised lymphangiomatosis which extends to more organs (53). Although lymphangiomas are benign tumors, their infiltration tendency complicates removal and influences the post-operative course (54). The first choice for treatment is surgery (55), after careful evaluation of the tumor size, with ultrasound, scanning or MR and orofaringeal endoscopy (22). In refractor cases, there is very often a need for radiotherapy (56). Complete macroscopic resection is possible in 80% of cases. Unfortunately, relapse is very common. Its therapy varies from case to case, with surgical resection, sclerosis and laser therapy (22, 57). Most often the sclerosis is perfomed by belomycin, and recently OK-432 is recommended as the immunostimulator which effects fluid resorbtion (58, 59). In differential diagnostic view all tumor formations which occur in the mediastinum are taken into account, as mentioned before (60, 61). It is important not to mistake lymphangioma with congenital fibrosarcoma which is very similar to it in terms of its clinical, radiographic signs and histologically (62). Although they manifest clinically, anatomic similarities are shown by meningomyelocoele and extradural cysts (63).