Recurrent inflammatory pseudotumors in children

Background/Purpose: Inflammatory pulmonary (PPT) and extrapulmonary pseudotumors (EPPT) are uncommon benign tumors, which, in general, do not recur after complete resection. Recurrence rates for both types of pseudotumors are undocumented in a large population of children, and the salient features of potential recurrences are unspecified. Methods: This is a report of 15 children with PPT and EPPT; 3 children had a recurrence. These pseudotumors recurred despite adequate primary resection of all gross disease at first presentation. The literature was reviewed to determine rate of recurrence for PPT and EPPT and also to document features common to recurrent pseudotumors. Results: Overall recurrence rate for pseudotumors was 14%. PPT and EPPT, which were not confined to a single organ, had a high chance of recurrence (46% and 30%, respectively) compared with PPT and EPPT, which were confined to a single organ (1.5% and 8%, respectively). Recurrences have appeared between 3 months and 7 years. Intraabdominal EPPT accounts for more than 75% of the EPPT recurrences. Conclusions: PPT and EPPT recur more frequently than anticipated. All pseudotumors, which on initial presentation extend beyond the confines of a single organ, have a high chance of recurrence despite what appears to be adequate resection. Children with pseudotumors that extend beyond a single organ, require frequent postoperative evaluation for recurrence and may be candidates for chemotherapy or radiotherapy at the time of initial resection.