[Castleman's disease]

Abstract

A 17-year-old girl developed acute colicky periumbilical pain with haematuria. On examination a tightly elastic space-occupying lesion about 10 cm in diameter was palpable around the umbilicus. Erythrocyte sedimentation rate (ESR) was raised to 113 mm in the first hour. There was a microcytic anaemia (8.7 g/dl) and a positive Coombs test. Ultrasonography revealed a periumbilical space-occupying lesion of decreased echogenicity and left hydronephrosis. Computed tomography demonstrated a homogeneous noninfiltrative tumour, about 7 cm in diameter, with increased contrast medium concentration. The tumour, which was covered by peritoneum and well circumscribed, was excised from the base of the mesentery. Histologically it proved to have hyaline-sclerotic changes in the blood vessels and lymphoid and plasma cellular infiltrations, corresponding to a mixed form of Castleman's disease. In a second operation a left nephroureterectomy was performed. The kidney, the regional lymph nodes and the tissue which had caused the stenosis of the ureter all showed the same changes as the tumour. Postoperatively the ESR and the blood count became normal, but the Coombs test remained positive. During a subsequent pregnancy the ESR again rose. The patient has remained symptom-free for 38 months after the second operation.