Lymphangiosarcoma is a rare, aggressive,
vascular neoplasm arising in chronic congenital or acquired
lymphedema. Although it is most frequently associated with
post-mastectomy lymphedema (Stewart-Treves's syndrome),
lymphangiosarcoma can exceptionally arise in
congenital hereditary lymphedema (Milroy's syndrome and Meige's syndrome) and non-
hereditary lymphedema (congenital, praecox or forme tarde
lymphedemas). We report a case of
lymphangiosarcoma of the pubic region, supported by immunohistochemical studies, in a 42-year-old woman affected by congenital, non-
hereditary 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 tumour lesions. To our knowledge, this is the first case of
lymphangiosarcoma associated with congenital non-
hereditary lymphedema confined to the pubic region. The literature concerning the cases of
lymphangiosarcoma arising in congenital hereditary and non-
hereditary lymphedema is reviewed. Moreover, we emphasized the importance of regular clinical controls in all patients affected by chronic
lymphedema. In fact, although the prognosis of this
neoplasm is very poor, a prompt diagnosis and a rapid, ablative surgery associated with
radiation therapy can increase the possibility of survival of these patients.