Splenic inflammatory myofibroblastic tumor (inflammatory pseudotumor): A clinicopathologic and immunophenotypic study of 12 cases

T. S. Neuhauser, G. A. Derringer, L. D.R. Thompson, Julie Fanburg-Smith, N. S.I. Aguilera, J. Andriko, W. S. Chu, S. L. Abbondanzo

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100 Scopus citations

Abstract

Context. - Inflammatory pseudotumor is an uncommon and enigmatic lesion. The spindle cells found in this tumor have features of myofibroblasts. Because of the indefinite relationship of these lesions with inflammatory fibrosarcoma and their indefinite biologic behavior, inflammatory pseudotumor is currently classified as inflammatory myofibroblastic tumor (IMT). To date, only case reports or small series have been published on these tumors, which are primary in the spleen. Design. - In this study, we describe the clinical, morphologic, and immunophenotypic findings of 12 cases of splenic IMT and examine their relationship to Epstein-Barr virus (EBV). Results. - The patients included 8 women and 3 men, ranging from 19 to 77 years of age (mean, 53 years; median, 60 years). Demographic data were unavailable for 1 patient. Patients generally presented with abdominal pain (n = 5) and fever (n = 4). Associated lesions included renal cell carcinoma (n = 2), colonic adenocarcinoma (n = 1), and cholecystitis (n = 1). All tumors were composed of a bland spindle cell proliferation in association with a variable mixed inflammatory component. There were 2 growth patterns, namely, a cellular spindle cell pattern and a hypocellular fibrous pattern. An immunohistochemical panel confirmed the myofibroblastic nature of the spindle cells. The spindle cells of 2 cases were immunoreactive for EBV latent membrane protein 1, whereas 6 of 10 cases were positive for EBV-encoded RNA using in situ hybridization. Follow-up was available for 8 patients; 6 were alive with no evidence of recurrence and 2 were dead of other causes. Conclusion. - Splenic IMTs are uncommon lesions that can be distinguished from other conditions using a combination of clinical, histologic, and immunophenotypic findings. Epstein-Barr virus may play a role in the pathogenesis of splenic IMT, and there may be an association of splenic IMT with concomitant disease or malignancy. Most splenic IMTs have an excellent long-term prognosis.

Original languageEnglish (US)
Pages (from-to)379-385
Number of pages7
JournalArchives of Pathology and Laboratory Medicine
Volume125
Issue number3
StatePublished - Mar 22 2001

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine
  • Medical Laboratory Technology

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    Neuhauser, T. S., Derringer, G. A., Thompson, L. D. R., Fanburg-Smith, J., Aguilera, N. S. I., Andriko, J., Chu, W. S., & Abbondanzo, S. L. (2001). Splenic inflammatory myofibroblastic tumor (inflammatory pseudotumor): A clinicopathologic and immunophenotypic study of 12 cases. Archives of Pathology and Laboratory Medicine, 125(3), 379-385.