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Distinguishing Gastrointestinal Stromal Tumors From Their Mimics: An Update

Abraham, Susan C. MD

Advances in Anatomic Pathology: May 2007 - Volume 14 - Issue 3 - p 178-188
doi: 10.1097/PAP.0b013e318050aa66
Review Articles

Since the discovery of activating KIT mutations in gastrointestinal stromal tumors (GISTs) in 1998 and the subsequent demonstration that some malignant GISTs respond to targeted therapy with imatinib, it has become increasingly important for pathologists to correctly diagnose GISTs and separate them from their potential mimics in the gastrointestinal tract and abdominal cavity. Some mesenchymal tumors, such as leiomyomas of the muscularis mucosae, are easily distinguished from GIST on the basis of their anatomic location and morphologic appearance. Others, such as gastrointestinal schwannomas, can significantly overlap with GIST in their gross appearance and morphology and require a panel of immunostains for correct diagnosis. This article will review the most common mimics of GISTs: desmoid tumors, smooth muscle tumors (leiomyomas and leiomyosarcomas), gastrointestinal schwannomas, inflammatory fibroid polyps, and solitary fibrous tumors. Pertinent differences between each of these tumors and GIST in terms of gross appearance, histologic features, and immunophenotype will be emphasized. It is important to separate GISTs from these potential mimics because their treatment and prognosis can differ markedly.

Division of Anatomic Pathology, Mayo Clinic, Rochester, MN

Reprints: Susan C. Abraham, MD, Department of Pathology, Hilton 11, Mayo Clinic, 200 First Street SW, Rochester, MN 55905 (e-mail:

© 2007 Lippincott Williams & Wilkins, Inc.