Lymphatic tumors

Abstract

At the Workshop on Vascular Anomalies in Rome in June 1996, the Mulliken and Glowacki classification was accepted for various cutaneous vascular lesions. This classification is based upon clinical, histological and cytological features [9, 10, 30]. According to Enzinger, lymphatic tumors can be classified into benign and malignant lesions [14]. As compared with tumors arising from blood vessels, soft tissue tumors of lymphatic origin are rare; benign lesions include the solitary lymphangioma, lymphangiomatosis, lymphangiomyoma and lymphangiomyomatosis. There is growing evidence to suggest that the majority of these lesions represent hamartomas or developmental lymphangiectasias rather than true neoplasms [11, 40]. Although three types of lymphangioma have been described capillary, cavernous and cystic they all belong to a single group of lesions [40, 50]. They can affect any part of the body, their presentation depending on the surroundings, but a strong predilection for the neck and axilla has been noted [23]. When bones, soft tissues and visceral organs are involved in a diffuse or multifocal manner, the term lymphangiomatosis is used. Like angiomatosis, this extremely rare disease is most often seen in children [14, 46].Clinically and histopathologically, there is considerable overlap between these two entities [18]. Lymph angiomyoma and its more widespread form, which is called lymphangiomyomatosis, are characterized by a proliferation of smooth muscle in the lymphatic structures of the mediastinum, retroperitoneum and the lung. These benign lesions affect only women. The term lymphangiopericytoma has been abandoned [14]. The collective term of angiosarcoma has been suggested instead of lymphangiosarcoma, as these malignant tumors not only are composed of groups of endothelium- lined empty spaces suggesting lymphatics, but also contain areas resembling hemangiosarcoma [11, 12]. They are seen exclusively in patients with longstanding lymph stasis such as is seen after radical mastectomy (postmastectomy lymphangiosarcoma) or in chronic lymphedema of the lower extremities [12]. They are discussed in Chap. 16.