MAMMO-RENAL AND ACRO-MAMMO-RENAL SYNDROMES - A NOSOLOGIC APPROACH, SYNOPSIS AND UPDATE

Mammo-renal syndromes cover a heterogeneous spectrum of complex congen ital anomalies - regarding formation, number and location - concomitan tly affecting the breasts/nipples and the kidneys/urinary collecting s ystem. A nosologic classification of these entities should include the following subsets: (1) ''pure mammo-renal syndromes'', which occur as sporadic conditions, characterized by the involvement of mammary tiss ue and kidney alone; (2) mammo-renal syndromes associated with chromos omal disorders; (3) mammo-renal syndromes associated with dysmorphic g enetic syndromes; (4) acro-mammo-renal syndromes, characterized by an additional coexistence of acral (limb) defects. This classificative ap proach would be a ''starting point'' for the correct arrangement and i n-depth investigation of these embryogenetic disorders which are poorl y considered in dermatological literature. Therefore, the evidence of inborn anomalies pertaining to the breast/nipple, or pectoralis major muscle, or an acral location of Becker's naevus with ipsilateral polyt helia, should always alert the physician who should then perform a ren al ultrasound examination to quickly rule out the occurrence of hidden , concomitant nephrouropathies. Although the exact embryogenetic mecha nisms are far from clear, mammo-renal and acro-mammo-renal syndromes r epresent distinct poly topic congenital malformations which reflect di sturbances in those specific developmental fields involved (nipple/kid ney/skin/limb buds).