Don't Blame It on the Scar Tissue

Abstract

Question: A 61-year-old white woman was evaluated for complaints of intermittent attacks of a sharp, severe, periumbilical abdominal pain for 15 years with recent worsening in severity and nocturnal episodes. Her symptoms were partially alleviated by bowel movements and no worsening factors were identified. Her past medical history was significant for a total hysterectomy and adhesiolysis. Physical examination revealed prior surgical incisions over the abdominal wall and otherwise benign on examination. She denied fever, nausea, vomiting, painful adenopathy, weight loss, night sweats, recent infectious exposures, or foreign travels. Laboratory studies, including a complete blood cell count with white blood cell differential, renal profile, hepatic transaminases, erythrocyte sedimentation rate, serum electrophoresis, and 5-hydroxyindoleacetic acid levels, were negative or within normal values. Esophagogastroduodenoscopy and colonoscopy were unremarkable. A tuberculin purified protein derivative test was negative as was computed tomography (CT) of the chest. CT enterography (Figure A) showed enlarged mesenteric lymph nodes in the right mid abdomen (arrows). Capsule endoscopy and upper and lower double balloon enteroscopy with complete visualization of the entire small bowel lumen were unremarkable. Because of persistent symptoms, follow-up CT enterography was performed 8 weeks later and showed interval increase in the size of the conglomerate lymph nodes masses in the right side of the mesenteric fat, as well as cicatricial changes involving loops of bowel adjacent to the mesenteric masses. CT-guided fine-needle aspiration biopsy was performed. What is the diagnosis? Look on page 914 for the answer and see the Gastroenterology web site () for more information on submitting your favorite image to Clinical Challenges and Images in GI. Fine-needle aspiration showed fat necrosis (arrow) with surrounding fibroblastic proliferation, dense fibrosis and admixed inflammatory infiltrate (Figure B, medium power). A high power image (Figure C) demonstrated inflammatory cells consisting of mononuclear leukocytes, histiocytes, and scattered plasma cells with background of bland fibroblasts and fibrosis. There was no evidence of malignant cells, lymphoma, or carcinoma. These findings were most consistent with a diagnosis of sclerosing mesenteritis (SM). First described in the 1920s, SM is a rare, idiopathic, nonmalignant inflammatory disease that generally affects the small bowel mesentery.1Akram S. Pardi D.S. Schaffner J.A. et al.Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients.Clin Gastroenterol Hepatol. 2007; 5: 589-596Abstract Full Text Full Text PDF PubMed Scopus (210) Google Scholar The etiology is unknown and has been linked with a medical history of abdominal surgeries, trauma, paraneoplastic syndrome, and autoimmunity.2Emory T.S. Monihan J.M. Carr N.J. et al.Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity?.Am J Surg Pathol. 1997; 21: 392-398Crossref PubMed Scopus (274) Google Scholar SM is part of a spectrum of an inflammatory process of the mesentery that may manifest at different stages, including fat necrosis (lipodystrophy), chronic tissue inflammation (mesenteric panniculitis), and fibrosis (SM).2Emory T.S. Monihan J.M. Carr N.J. et al.Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity?.Am J Surg Pathol. 1997; 21: 392-398Crossref PubMed Scopus (274) Google Scholar Affecting men more than women (2:1) in the fifth to seventh decades of life, its nonspecific presenting symptoms represent a diagnostic challenge for physicians.3Avelino-Silva V.I. Leal F.E. Coelho-Netto C. et al.Sclerosing mesenteritis as an unusual cause of fever of unknown origin: a case report and review.Clinics (Sao Paulo). 2012; 67: 293-295Crossref PubMed Scopus (7) Google Scholar Diagnosing SM includes the use of laboratory and imaging tests, with CT of the abdomen and pelvis being the most sensitive and useful, showing a soft tissue mass in the small bowel mesentery. However, the gold standard is biopsy.2Emory T.S. Monihan J.M. Carr N.J. et al.Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity?.Am J Surg Pathol. 1997; 21: 392-398Crossref PubMed Scopus (274) Google Scholar When concerning signs are present (eg, weight loss, anemia or night sweats), further workup is recommended to rule out a lymphoproliferative disorder.1Akram S. Pardi D.S. Schaffner J.A. et al.Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients.Clin Gastroenterol Hepatol. 2007; 5: 589-596Abstract Full Text Full Text PDF PubMed Scopus (210) Google Scholar Thirty percent of patients may present with intra-abdominal malignancies, the majority being lymphoma.1Akram S. Pardi D.S. Schaffner J.A. et al.Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients.Clin Gastroenterol Hepatol. 2007; 5: 589-596Abstract Full Text Full Text PDF PubMed Scopus (210) Google Scholar Treatment is offered to symptomatic patients. A regimen of tamoxifen and glucocorticoids has been described, as well as use of other anti-inflammatory, immunomodulatory, and antifibrotic agents; bowel obstruction is treated with surgery.1Akram S. Pardi D.S. Schaffner J.A. et al.Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients.Clin Gastroenterol Hepatol. 2007; 5: 589-596Abstract Full Text Full Text PDF PubMed Scopus (210) Google Scholar The patient was placed on a prednisone taper and started on tamoxifen 10 mg twice daily. Within 3 weeks, the intermittent abdominal pain attacks resolved completely and she continues to remain asymptomatic.