Pelvic Lipomatosis With Crossed Renal Ectopia

Pelvic lipomatosis is a feature of the Proteus syndrome (176920). Goswami et al. (1992) described an 18-year-old male with both kidneys located on the right side and with pelvic lipomatosis. Although usually benign, there was a question of neoplastic changes in the lipomatosis of this patient. Goswami et al. (1992) stated that fewer than 100 cases of pelvic lipomatosis had been reported and that only 4 have been in female subjects. A remarkable and probably significant feature of the patient reported by Goswami et al. (1992) was the presence of abnormalities involving chromosome 1 in 37% of metaphases: '...these almost always exhibit a whole chromosome translocation with chromosome 6 (35%), although involvement of chromosome 1 with chromosome 8 is present in 2% of metaphases.'