PERIRENAL MASS OF LANGERHANS CELL HISTIOCYTOSIS (LETTERER-SIWE DISEASE) AND PERCUTANEOUS NEPHROSTOMY TUBE PLACEMENT

A 15-month-old girl with Langerhans cell histiocytosis (LCH), Letterer -Siwe disease, was referred to our hospital in 1984. Whilst on treatme nt with cytotoxic drugs, a perirenal mass was detected and hydronephro sis became evident when she was 29 months old. Percutaneous nephrostom y tubes were placed in the pelvis, bilaterally and replaced every 6 mo nths. The mass was not completely controlled and chronic pyelonephriti s continued. Biopsy of the mass convoluted kidney hilus revealed histi ocytic invasion. Although multiple organ systems are involved in LCH a nd abdominal malignant tumours may be accompanied by hydronephrosis, t o our knowledge, this is the first case report of abdominal LCH and th e ensuing hydronephrosis. Percutaneous nephrostomy tubes proved useful , but more convenient, less painful and infection-limited approaches n eed to be designed.