Cases reported "Fibromatosis, Abdominal"

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1/3. Mesenteric fibromatosis with involvement of the gastrointestinal tract. A GIST simulator: a study of 25 cases.

    Mesenteric fibromatosis (MF) and gastrointestinal stromal tumors (GISTs) are distinct lesions, but they often are confused with each other. Correct identification is essential because of their vastly different therapeutic and prognostic connotations. We reviewed 25 cases of MF with involvement of the wall of the gastrointestinal tract and found that GIST was the most common misdiagnosis (13 [52%]). MF was characterized by a spatially homogeneous proliferation of wavy spindle cells without atypia, associated with collagen deposition (often of the keloidal type), and an infiltrative border. Most cases displayed prominent muscular arteries and dilated, thin-walled veins. The mitotic count was relatively low, and no atypical mitotic figures were identified. These features are sufficiently characteristic of MF to permit distinction from GIST on the basis of routinely stained sections in the large majority of the cases, but immunohistochemical analysis provides a supporting role in the few equivocal cases.
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keywords = gastrointestinal stromal, stromal
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2/3. Localized intra-abdominal fibromatosis of the small bowel mimicking a gastrointestinal stromal tumor: a case report.

    Intra-abdominal fibromatosis (IAF) is a benign mesenchymal lesion that can occur throughout the gastrointestinal tract. Although rare, it is the most common primary tumor of the mesentery and can develop at any age. We describe a rare case of primary IAF involving the mesentery and small bowel which clinically, macroscopically and histologically mimicked malignant gastrointestinal stromal tumor (GIST). This report highlights the fact that benign IAF can be misdiagnosed as a malignant GIST localized in the mesentery or arising from the intestinal wall. Their diagnostic discrimination is essential because of their very different biological behaviors and the fact that the introduction of effective therapies involving tyrosine kinase inhibitor STI571 (imatinib mesylate) has greatly changed the clinical approach to intra-abdominal stromal spindle cell tumors.
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ranking = 5.0052963970164
keywords = gastrointestinal stromal, stromal
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3/3. Giant mesenteric fibromatosis presenting as small bowel obstruction.

    Mesenteric fibromatosis is a proliferative fibroblastic neoplasia of the small intestine mesentery that may occur as a unique or multiple formation. Mesenteric fibromatosis is a rare, locally aggressive neoplasm and may present with abdominal discomfort, abdominal pain, weight loss, or symptoms of ureteral obstruction, mesenteric ischemia, or intestinal obstruction. It is of the utmost importance to distinguish mesenteric fibromatosis from gastrointestinal stromal tumors. Histopathology accurately differentiates between these two distinct entities. The preferred treatment is local surgical excision with a margin of uninvolved tissue. The involvement of important structures like the superior mesenteric artery and the superior mesenteric vein may pose a challenge during resection, but these tumors can be excised and the vessels repaired primarily.
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ranking = 1
keywords = gastrointestinal stromal, stromal
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