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Presented by Ralph Hruban, M.D. and prepared by Zarir E. Karanjawala, M.D., Ph.D.
Case 1: This middle-aged woman with a poorly documented history of surgery for a fibromatosis of the abdominal wall ten years ago developed vague gastrointestinal symptoms.
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1. Question
Week 335: Case 1
This middle-aged woman with a poorly documented history of surgery for a fibromatosis of the abdominal wall ten years ago developed vague gastrointestinal symptoms. An abdominal CT scan revealed an ill-defined mass in the head of the pancreas with extension along the superior mesenteric artery into the mesentery.images/hruban121707_1A.jpg
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images/hruban121707_1c.jpgCorrect
Answer: Sclerosing mesenteritis
Histology: Numerous biopsies were taken of the mass in the pancreas and of the mesentery. They revealed fibrosis, mild chronic inflammation, focal fat necrosis and vascular dilatation.
Discussion: Sclerosing mesenteritis has recently been extensively reviewed by the group from the Mayo Clinic (see reference below). It is a very rare inflammatory disease characterized by inflammation and fibrosis involving the mesentery of the small bowel. Presenting signs and symptoms include abdominal pain, abdominal distention, diarrhea and weight loss. As in this case, one-third of patients with sclerosing mesenteritis have a history of abdominal surgery. Imaging typically reveals a mass at the root of the mesentery, but rare cases involving the pancreas have been described. The histologic features, including fibrosis, chronic inflammation and focal fat necrosis, are non-specific and the diagnosis is essentially a diagnosis of exclusion. Recommended therapies include tamoxifen and steroids. Seventeen percent of patients died of disease in the recent report from the Mayo Clinic.
Reference(s):
– Akram et al. Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients. Clinical Gastroenterology and Hepatology, 2007; 5:589-596.Incorrect
Answer: Sclerosing mesenteritis
Histology: Numerous biopsies were taken of the mass in the pancreas and of the mesentery. They revealed fibrosis, mild chronic inflammation, focal fat necrosis and vascular dilatation.
Discussion: Sclerosing mesenteritis has recently been extensively reviewed by the group from the Mayo Clinic (see reference below). It is a very rare inflammatory disease characterized by inflammation and fibrosis involving the mesentery of the small bowel. Presenting signs and symptoms include abdominal pain, abdominal distention, diarrhea and weight loss. As in this case, one-third of patients with sclerosing mesenteritis have a history of abdominal surgery. Imaging typically reveals a mass at the root of the mesentery, but rare cases involving the pancreas have been described. The histologic features, including fibrosis, chronic inflammation and focal fat necrosis, are non-specific and the diagnosis is essentially a diagnosis of exclusion. Recommended therapies include tamoxifen and steroids. Seventeen percent of patients died of disease in the recent report from the Mayo Clinic.
Reference(s):
– Akram et al. Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients. Clinical Gastroenterology and Hepatology, 2007; 5:589-596.
