Lymphangioma

•lymphangioma is a benign tumor arising from lymphatic endothelial cells

•lymphangioma arises as either a congenital malformation (i.e., hamartoma) or following failure of primitive lymphatic sacs to achieve venous communication

•lymphangioma often presents as a large, spongy, and poorly circumscribed mass which may be associated with lymph oozing from the surface or single to multiple fistulae

•treatment: surgical resection with wide margins (as local tumor recurrence is common due to infiltrative growth and poor margins), marsupialization, and radiation therapy

Lymphangiosarcoma

•lymphangiosarcoma is a very rare tumor arising from lymphatic endothelial cells

•lymphangiosarcomas are composed of lymphatic vessels forming capillary, cavernous, or cystic spaces

•lymphangiosarcomas are associated with or sequelae to chronic lymphatic obstruction

•sites: subcutaneous ± liver, pericardium and nasopharynx have been reported

•gross appearance: soft, cyst-like, and edematous ± invasive

•clinical signs are usually associated with extensive edema and drainage of lymph through the skin or mass

•moderate to high metastatic potential

•lymphangiosarcoma is differentiated from lymphangioma by pleomorphism, mitotic activity, and lack of dermal collagen support

•prognosis: unknown but aggressive surgical treatment recommended and possibly responsive to radiation therapy