Retroperitoneal Fibrosis

Abstract

Since Ormond (22) in 1949 first described 2 patients with a chronic inflammatory disease characterized by fibrosis in the retroperitoneal area, 37 similar cases have been documented. The condition was originally called periureteral fibrosis because the major symptoms resulted from ureteral obstruction. A greater knowledge of the structural changes involved indicates that the designation retroperitoneal fibrosis, although broader, is also more accurate. In the majority of the cases the diagnosis was made by surgical exploration and microscopic examination of excised tissue. In 1957 Talbot and Mahoney (30) presented an excellent review of 16 of these cases with full postoperative follow-up. Two necropsies confirmed the basic nature of the disease. These authors added one case of their own. In 1958 Hackett (9) reported 2 cases and reviewed 22. Other examples have been well described (1, 3–8, 12, 13, 15–21, 24, 26–29). Case Reports Case I: F. O., a 57-year-old white man, had been treated since 1935 for pulmonary tuberculosis. He also had an enlarging thyroid. In May 1950 he began to experience upper abdominal discomfort and lost 8 pounds. He was admitted to Bellevue Hospital, where he lost 10 additional pounds, and in July of 1950 an exploratory laparotomy revealed a retroperitoneal mass which was thought to be an inoperable malignant tumor. Biopsy, however, was interpreted as showing chronic inflammation. There were marked fibrosis of the retroperitoneal fat and infiltration with lymphocytes, plasma cells, and eosinophils. Because of weakness and dyspnea, with abdominal swelling and edema, the patient was admitted to the New York Hospital in October 1951. Urea nitrogen levels in the blood, urinalysis, and an excretory pyelogram were normal. In November a peritoneal tap yielded fluid containing cells rated cytologically as Papanicolaou Class V. The patient grew progressively worse and required frequent pericardial taps and diuresis before death. Autopsy revealed a large mass in the retroperitoneal area, extending from deep in the pelvis up about the aorta into the chest and to the heart, involving the epicardium, particularly the atrioventricular groove. The “tumerous tissue” also encased the coronary arteries. The adventitia of the thoracic aorta was completely replaced by collagenous tissue which extended to and partially replaced the pleura as well. In the lungs and kidneys this unusual fibrotic process followed the course of the arteries and arterioles from the aorta. The collagenous tissue spread out and involved the branches of the celiac axis. In addition to the scirrhous fibrosis, there were many infiltrating lymphocytes and foamy or lipid-laden macrophages. Some cells with large, dark nuclei and vacuolated cytoplasm were interpreted as representing primitive fat cells, and the final postmortem diagnosis was fibroliposarcoma.