The clinical spectrum of lymphangiomas and lymphangiomatosis of the spleen

Abstract

Lymphangiomas of the spleen are infrequent neoplasms of the spleen with clinical manifestations ranging from insignificant incidental findings to large, symptomatic cystic masses requiring surgical intervention. An associated syndrome may be lymphangiomatosis, in which the lymphangiomatous process involves other sites or organs, such as the liver, spleen, axilla, and mediastinum. Vasoformative neoplasms (hemangioma, lymphangioma) are relatively uncommon tumors or hamartomas of the spleen. Although well reported in the literature, their surgical significance is not well recognized. These lesions present a broad spectrum of pathologic findings of varied surgical importance. As incidental findings, they may be mistakenly considered as a valid indication for splenectomy. On the contrary, more extensive involvement of the spleen may cause symptomatic splenomegaly, which is a valid indication for splenectomy. When the spleen is diffusely involved (lymphangiomatosis), it may be part of a syndrome of generalized lymphangiomatosis involving structures and organs other than the spleen. This syndrome should be considered in obscure cases of splenomegaly in which the usual hematologic causes have been ruled out.