Inflammatory pseudotumor of the alimentary tract: Clinical and surgical experience

Presented at the 31st Annual Meeting of the American Pediatric Surgical Association, Orlando, Florida, May 25-29, 2000.
https://doi.org/10.1053/jpsu.2001.20045Get rights and content

Abstract

Background/Purpose: Initially described in 1937, inflammatory pseudotumor (IPT) inflammatory myofibroblastic tumor (IMT) or plasma cell granulomas are synonymous for an inflammatory solid tumor that contains spindle cells, myofibroblasts, plasma cells, and histocytes. Common sites of presentation include lung, mesentary, liver, and spleen; intestinal presentations are rare, and the etiology remains obscure. This report details the clinical and surgical experiences in 4 children with alimentary tract IPT at a single institution. Methods: A retrospective chart review was conducted of pediatric patients with the pathologic diagnosis of IPT. Results: Between 1990 and 1999, 4 patients (4 girls, ages 5 to 15 years) were identified with gastrointestinal tract origins of IPT. Symptoms at presentation included anemia (n = 4), intermittent abdominal pain (n = 3), fever (n = 3), weight loss (n = 2), diarrhea (n = 2), dysphagia (n = 1). Two patients had comorbid conditions of juvenile rheumatoid arthritis and mature B cell lymphoma. Three of 4 patients had elevated sedimentation rates. The sites of origin were the gastroesophageal junction, the colon, the rectum, and the appendix, with the referral diagnosis achalasia, perforated appendix, inflammatory bowel disease, and recurrent lymphoma, respectively. All were treated with aggressive surgical resection, and 3 girls have had no recurrences since the initial surgery. One patient had 3 recurrences within 8 months of presentation; she remains disease free 8 years later. Conclusions: IPT, although rare in the gastrointestinal tract, mimics more common problems. Successful surgical management is possible even in cases of multiple recurrences. J Pediatr Surg 36:169-173. Copyright © 2001 by W.B. Saunders Company.

Section snippets

Materials and methods

A retrospective chart review of children with the pathologic diagnosis of inflammatory pseudotumor affecting the alimentary tract was carried out at the J.W. Riley Hospital in Indianapolis, Indiana from 1980 to 1999. A review of the pediatric and adult literature concerning ITP also was undertaken.

Case 1

A 5-year-old girl with juvenile rheumatoid arthritis was evaluated for progressive dysphagia. The workup included a barium swallow that was interpreted as esophageal achalasia, and a subsequent manometric study was confirmatory of that diagnosis. At laparotomy for an esophageal myomectomy, a large submucosal mass involving the gastroesophageal junction was encountered and biopsied. The surgery was terminated for determination of the final pathology, which was consistent with IPT.

Results

Between 1990 and 1999, 4 patients were identified with alimentary tract IPT at our institution. All of the patients were girls and ranged in age from 5 to 15 years. Symptoms at initial presentation included anemia (n = 4), intermittent abdominal pain (n = 3), fever (n = 3), weight loss (n = 2), diarrhea (n = 2), and dysphagia (n = 1). Table 1 details the referral diagnosis and subsequent surgical interventions in this group in which half of the patients had ongoing comorbid conditions.

. Summary

Discussion

Inflammatory pseudotumors have been identified in virtually every organ system in both children and adults, but are most commonly reported in the lungs.1, 3 Microscopically, this solid tumor consists of inflammatory plasma cells, histiocytes, and lymphocytes in a matrix of spindle-shaped myofibroblasts. Because these lesions can be locally invasive, they are most commonly confused with sarcomas. Microscopically, there is a lack of mitosis and nuclear atypia, which are present in sarcomas. The

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Address reprint requests to Karen W. West, MD, J.W. Riley Hospital for Children, 702 Barnhill Dr, RI 2500, Indianapolis, IN 46202.

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