Kaposi's Sarcoma A Lymphologic Perspective

Kaposi’s Sarcoma Restricted to an Immunocompromised District

“…In their report, Kostaki et al [1] , by noticing that KS developed after repeated surgeries in a unique peculiar localized area with a dense lymphatic network, put forward the hypothesis that tissue injuries involving the lymphatic routes could play a central role in the occurrence of KS [1] . A large body of evidence that has been accumulated over the last three decades [3][4][5][6][7][8][9][10] strongly supports this concept, which is much more than a hypothesis. In fact, any persistent hindrance to lymph flow in a given body area impairs local immune surveillance (by disrupting trafficking of the immunocompetent cells) and stimulates vicarious angiogenesis (by promoting development of a collateral lymphatic or blood vascular network in the involved district).…”

“…This is supported by the existence of borderline cases [12] . Clinical observations and experimental investigations have confirmed the parallel course of the lymphatic and immune functions in body regions affected with KS [3][4][5][6][7][8][9] or Stewart-Treves angiosarcoma [13,14] . In this light, the two conditions may only differ in etiology, with human herpes virus 8 (HHV-8) always being causative of KS, while unknown viruses might be responsible for other angiosarcomas (though occasionally the same HHV-8 has been suspected) [12,15] .…”

“…From this lymphological perspective, it has even been assumed that KS and Stewart-Treves angiosarcoma may not be completely different entities, but merely variant expressions of a similar underlying abnormality consisting of impairments in lymph drainage and immune control [6] . This is supported by the existence of borderline cases [12] .…”

“…Bearing in mind that an immunocompromised district may harbor opportunistic and immunity-related disorders -such as infections, tumors and immune reactions [2] -the onset of KS lesions is not at all surprising here, since KS has an infectious etiology (HHV-8), a tumor morphology (angiogenic neoplasm), and an immune pathogenesis (immunosuppression often confined to acral body regions [3][4][5][6][7][8][9][10] ).…”

Enhancement of Classic Kaposi’s Sarcoma Growth after Intralesional Injections of Desferrioxamine

“…It has also been reported that intravenous infusion with DFO could induce pulmonary oedema [14] as well as severe phlebitis and oedema along the tract of the vein [15], suggesting that oedema may be a common denominator in some of the observed side-effects of DFO. Interestingly, antecedent or concurrent lymphoedema is usually noted in classic KS and is thought to participate in the development and/or progression of KS [16]. Oedema caused by DFO is assumed to be related to the up-regulation of the expression of VEGF [17], a growth factor which was originally identified for its vascular permeability properties.…”