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Treatment of thoracic lymphangiomatosis
  1. A Y Rostom
  1. Department of Radiotherapy, The Royal Marsden Hospital, Downs Road, Sutton, Surrey SM2 5PT, UK
  1. Dr Rostom email: assem.rostom{at}

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Lymphangiomas are rare benign neoplasms believed to be the result of abnormal development of the lymphatic system. They grow very slowly usually localised to one organ, but occasionally involve several organs in one part of the body. In the chest, involvement of the mediastinum, lung, heart, pleura, pericardium, ribs, and vertebrae may be seen in the same patient. Histologically they are made up of spaces lined with endothelium, varying from capillary size to several centimetres in diameter. They contain clear straw coloured fluid occasionally mixed with blood. The spaces are supported by a stroma of variable thickness containing lymphoid tissue. The aetiology is unknown but believed to be a result of congenital malformation of the lymph vessels. Radiation induced cutanous lymphangioma has been described in a patient with breast cancer following mastectomy and radiotherapy.1

Clinically, lymphangiomas are classified into three types:

Simplex, which is made up of capillary sized thin, walled lymphatic channels. This type usually affects the skin (lymphangioma circumscriptum)
Cystic lymphangioma (or cystic hygroma): this may range in size from a few millimetres to several centimetres, seen in a young age, commonly in the neck or the axilla
Cavernosum: this type is made up of dilated lymphatic channels, often with fibrous adventitial coats. This is the type which usually affects organs in the thorax, abdomen, and bones.

Bill and Sumner2 pointed out that the three types may coexist in the same patient. Reports of single organ involvement have been published relating to nearly all organs of the body except for the CNS, which is devoid of lymphatic channels.

In one large series, lymphangioma of the head and neck accounted for nearly 50% of cases in children, while 10% of cases had visceral disease including the thorax.3 Thoracic lymphangioma has been described in all age groups including infants, neonates, and in the stillborn.4 Presenting symptoms are related to pulmonary and/or mediastinal mass, and signs and symptoms of pleural and/or …

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