Inflammatory pseudotumor of the alimentary tract: Clinical and surgical experience☆
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.
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.