Sclerosing mesenteritis affecting the small and the large intestine in a male patient with non-Hodgkin lymphoma: a case presentation and review of the literature

Abstract

IntroductionSclerosing mesenteritis is a rare disease resembling a mesenteric tumour. We present here a case of sclerosing mesenteritis that affected both the large and the small intestine of the patient. Therapeutic and diagnostic issues are discussed.Case presentationA 62-year-old man with a history of non-Hodgkin lymphoma presented with fatigue, a palpable tender abdominal mass and clinical signs of progressing intestinal obstruction. The preoperative evaluation failed to prove recurrence of the lymphoma or any other definite diagnosis. A laparotomy was performed through a midline incision. The mesentery resembled a tumour-like thickened and fibrotic mass. Abundant, rigid intestinal loop adhesions were observed. Diffuse fibrotic infiltration of the ileum and of the sigmoid colon, which obviously affected the intestinal vascular supply, were identified. A right colectomy and partial sigmoidectomy were performed. Pathological evaluation revealed extensive myofibroblastic reaction of the mesentery with accompanying loci of fat necrosis and areas of inflammation. A diffuse fibrotic infiltration that focally showed a ground-glass appearance was observed. The post-operative course was complicated by respiratory insufficiency and infections and the patient died 2 months after the operation.ConclusionSclerosing mesenteritis that affects both the small and the large intestine is extremely rare. The disease is characterized by myofibroblastic reaction, fat necrosis and diffuse fibrosis of the mesentery. Pathological confirmation may be required for definite diagnosis. If the disease is characterized by severe and diffuse fibrosis, then the application of surgical therapy may be problematic.

Mesenteric Panniculitis (MP) in CT – A Predictor of Malignancy?

“…Colorectal, bronchial, prostate and pancreatic cancers were also often associated with MP (• ▶ Table 3). An association of MP with lymphoma, particularly non-Hodgkin lymphoma, has also been described in previous studies [6,9,17,18]. The extent to which MP is a reaction to an already present malignancy or is merely an incidental finding without an association with a tumor disease is unclear.…”

Sclerosing mesenteritis, a rare cause of a retroperitoneal tumor

“…Th e most common site is the small bowel mesentery; in up to 20% the mesocolon is involved, and rare sites are the mesoappendix, the peripancreatic area, the omentum and the pelvis [6,13]. Th e diagnosis of SM is histologically confi rmed by variable degrees of fi brosis, fat necrosis and infl ammation.…”

“…Surgical treatment should be limited to diagnosis or treatment of complications [13], as it may otherwise be problematic due to the diff use fi brotic involvement of major vessels, vascular displacement, encasement or thrombosis seen in over half of patients [6,17]. In our patient, the mass encased the aorta and vena cava, precluding complete resection.…”

“…Corticosteroids are eff ective for initial treatment but their side eff ects limit their utility for long-term treatment. Alternative strategies include experimental use of immunosuppressive and cytotoxic agents such as azathioprine and cyclophosphamide; anti-infl ammatory agents have also been suggested [5,6]. Ginsburg et al [4] reported the experimental use of thalidomide, which has been demonstrated to possess anti-infl ammatory, immunomodulatory and antiangiogenic properties.…”

“…Th e three pathological characteristics of the disorder are chronic nonspecifi c infl ammation, fat necrosis and fi brosis [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18].…”