Multifocal inflammatory myofibroblastic tumor in the abdomen

Abstract

A17 year old so far healthy young man presented with upper abdominal pain. Imaging with sonography, MRT and PET-CT demonstrated three solid tumors in the duodenal mesentery, pancreatic corpus and tail and on the upper pole of the left kidney. Open biopsy of the mesenterial tumor revealed an inflammatory myofibroblastic tumor which was ALK-1 negative. Chemotherapy with vincristine, actinomycin D and cyclophosphamide was initiated and the imaging control after 4 cycles exhibited morphological and functional regression at all 3 tumor locations. Surgery in curative intention was now performed and the tumors in the mesentery and the kidney were totally resected. The pancreas looked macroscopically normal and only biopsies were taken. Histology showed regressive fibrosis at all sites and no malignancy. The young man  has now  been well for six months and follow-up imaging showed no sign of recurrence so far.