GRACE S.ROZYCKIMD, MBA
Primary small-bowel tumors are rare, with 5300 cases per year.1In order, the main tumors are adenocarcinoma, carcinoid, lymphoma, and gastrointestinal stromal tumor.1Inflammatory myofibroblastic tumors (IMTs), also known as pseudosarcomatous myofibroblastic proliferation, inflammatory sarcoma, Vanek tumor, plasma cell granuloma, and inflammatory myohistiocytic proliferation, are even rarer.2
An IMT is a rare, benign, neoplastic mass that consists of an inflammatory infiltrate with plasma cells, eosinophils, and lymphocytes. Granulation tissue can also be seen. There is recent evidence that IMTs are more neoplastic than reactive, associated with p22-24 of chromosome 2 with anaplastic lymphoma kinase expression.2This lends favor to IMTs being a neoplasm from the mesoderm due to chromosome defects, in the same group as Ewing sarcoma and alveolar rhabdomyosarcoma.2
These lesions or masses can occur anywhere in the body of young adults and small children and are usually limited to soft tissue and submucosal tissue.2The most commonly reported site is the gastric antrum, with the small bowel being the second most common.3Demirkan et al4described a jejunal IMT in a 2-year-old. Campbell et al3reported a series of patients with IMTs found in the duodenum, ileum, and stomach. A literature search, however, reveals that there are very few IMTs.
The IMTs present as partial or complete small-bowel obstructions, with symptoms such as nausea, vomiting, and pain.1Most small-bowel tumors appear in the fifth to sixth decades of life.1Diagnosis is made from radiological studies revealing a mass, with pathological analysis used to make the final diagnosis. Treatment consists of surgical resection. During an exploratory laparotomy, the pathological findings can vary. Campbell and colleagues described large IMTs that caused intussusceptions and IMTs that were associated with peptic ulcer disease. Removal of the IMT is warranted because of its recurrence and potential malignant behavior.4
Interestingly, the patient we describe is one in a set of identical triplets, and the others have had no abdominal pathological findings to date.
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Correspondence:Raman Kumar, MD, Department of General Surgery, Summa Health System, Akron City Hospital, 525 E Market St, Akron, OH 44304 (firstname.lastname@example.org).
Accepted for Publication:December 31, 2006.
Author Contributions:Study concept and design: Bender. Acquisition of data: Kumar. Analysis and interpretation of data: Kumar. Drafting of the manuscript: Kumar. Critical revision of the manuscript for important intellectual content: Kumar and Bender. Administrative, technical, and material support: Bender.
Financial Disclosure:None reported.
Image of the Month—Diagnosis. Arch Surg. 2008;143(7):712. doi:10.1001/archsurg.143.7.712