Progression of an untreated pseudotumor

The patient was a fifty-two-year-old male who was diagnosed with factor IX deficiency at the age of two. He developed an abdominal pseudotumour in this twenties. Surgery was considered but not performed for fear of operative complications. Over a five-year period beginning at age 47 years, the pseudotumour rapidly increased in size. The pseudotumour reached a final volume of 21 litres, roughly equivalent in volume and dimensions to a 5 gallon (U.S.) carboy of water. No antibodies to factor IX were detected upon repeated testing. By the end of the fifth year, the patient was suffering from a multitude of compressive complications secondary to the pseudotumour. Figure 1 shows the three-dimensional image reconstruction of the mature pseudotumour. Figure 2 shows the tumour's growth over the five-year preoperative period. To produce these images, semi-automated segmentation and rendering were performed on two systems: General Electric Advantage Workstation v 4.3 (Waukesha, WI, USA) and Osirix Lite 7.0.2 (Geneva, Switzerland). Images were semi-automated segmented using region growing and with manual segmentation on the Advantage Workstation. Pseudotumour volumes were computed and adjusted for slice thicknesses for each of the cases. Volume rendering for display was performed in Osirix. Surgery was eventually attempted due to severe satiety, but proved unsuccessful. The patient eventually succumbed to sepsis. This patient was at the extreme end of the pseudotumour spectrum. Hopefully earlier intervention in the course of pseudotumour development is now generally being considered. Three-dimensionally reconstructed images and corresponding tumour volume calculations may provide a longitudinal perspective of pseudotumour development. These techniques may prove useful in planning the extent and timing of surgical intervention for pseudotumours. MI reviewed the patient's clinical data, wrote the manuscript and analysed radiographic data; PC originated the study, reviewed the manuscript and radiographic data; DW analysed the radiographic data. The authors stated that they had no interests which might be perceived as posing a conflict or bias.