LYMPHANGIOSARCOMA OF THE PUBIC REGION - A RARE COMPLICATION ARISING IN CONGENITAL NONHEREDITARY LYMPHEDEMA

Lymphangiosarcoma is a rare, aggressive, vascular neoplasm arising in chronic congenital or acquired lymphedema. Although it is most frequen tly associated with post-mastectomy lymphedema (Stewart-Treves's syndr ome), lymphangiosarcoma can exceptionally arise in congenital heredita ry lymphedema (Milroy's syndrome and Meige's syndrome) and non-heredit ary lymphedema (congenital, praecox or forme tarde lymphedemas). We re port a case of lymphangiosarcoma of the pubic region, supported by imm unohistochemical studies, in a 42-year-old woman affected by congenita l, nonhereditary lymphedema of the left genital region and homolateral lower limb. In addition, molecular analysis demonstrated the absence of Kaposi's sarcoma-associated Herpes virus (KSHV) DNA sequences in tu mour lesions. To our knowledge, this is the first case of lymphangiosa rcoma associated with congenital non-hereditary lymphedema confined to the pubic region. The literature concerning the cases of lymphangiosa rcoma arising in congenital hereditary and non-hereditary lymphedema i s reviewed. Moreover, we emphasized the importance of regular clinical controls in all patients affected by chronic lymphedema, In fact, alt hough the prognosis of this neoplasm is very poor, a prompt diagnosis and a rapid, ablative surgery associated with radiation therapy can in crease the possibility of survival of these patients.