Sclerosing mesenteritis: A disease with many names

Surgical Rounds®, October 2007, Volume 0, Issue 0

Raul Mederos, Chief Resident of General Surgery, Department of Surgery; Roxane Scemla, Medical Student IV; Payman Danielpour, Chief Surgical Resident, Department of Surgery; Ana M. Medina, Resident, Department of Pathology; Jamie Barkin, Chief, Division of Gastroenterology; Irwin Willis, Attending Surgeon, Department of Surgery, Mount Sinai Medical Center, Miami Beach, FL

Raul Mederos, MD

Chief Resident of

General Surgery

Department of Surgery

Roxane Scemla, MS

Medical Student IV

Payman Danielpour, MD

Chief Surgical Resident

Department of Surgery

Ana M. Medina, MD

Resident

Department of Pathology

Jamie Barkin, MD

Chief

Division of

Gastroenterology

Irvin Willis, MD

Attending Surgeon

Department of Surgery

Mount Sinai Medical

Center

Miami Beach, FL

Introduction: Sclerosing mesenteritis is a rare benign condition that is found most often in elderly patients. Various terms are used throughout the literature to describe this condition, which may lead to confusion, especially since there is a paucity of data regarding this rare disease.

Results and discussion: This paper reports a case of sclerosing mesenteritis in a patient who underwent invasive procedures before her condition was diagnosed. It also serves to increase awareness of sclerosing mesenteritis in the hopes of preventing patients from undergoing unnecessary invasive procedures.

Conclusion: Treatment for sclerosing mesenteritis should be based on the stage of the disease. Earlier stages may be observed or treated medically; however, when fibrosis becomes extensive, surgical intervention is the treatment of choice.

Sclerosing mesenteritis is an uncommon mesenteric disease, and there are fewer than 200 cases reported in the literature. Although patients most frequently present with an abdominal mass, symptoms related to bowel obstruction, or abdominal pain, many are asymptomatic, and the disease is an incidental finding. Sclerosing mesenteritis may present as a single mass, multiple masses, or as diffuse thickening of the mesentery.

Treatment for sclerosing mesenteritis should be based on the stage of the disease. In the early stage, when fat necrosis predominates, many physicians tend not to treat the condition because the disease process may regress spontaneously. When chronic inflammation is prominent but fibrosis has not yet developed fully, medical treatment with corticosteroids, immunosuppressants, or progesterone may delay or prevent progression of the disease. When fibrosis becomes extensive, especially when sclerosing mesenteritis presents as a large fibrotic mass causing bowel obstruction, surgical intervention is the treatment of choice.1 Patients whose disease is still in the first and second stages usually have a good prognosis.

We report the case of an elderly woman who presented with abdominal pain and weight loss. The patient underwent a laparotomy with biopsy of the mesenteric mass, which showed sclerosing mesenteritis. As awareness of this rare disorder increases, appropriate management options will become more straightforward, preventing unnecessary invasive procedures in some cases.

Case report

An 85-year-old woman presented with a chronic history of weight loss and abdominal pain. Her medical history was significant for arthritis, bronchitis, and hypertension. Clinical examination revealed an anicteric patient in no acute distress; she was alert and oriented. Pertinent findings were positive bowel sounds on auscultation and a slightly distended abdomen. There were no palpable masses or organomegaly, and no abdominal tenderness was elicited.

A small bowel series showed no evidence of a mass defect or obstruction. An axial computed tomography (CT) scan of the abdomen showed a soft tissue mesenteric mass with calcifications and surrounding inflammatory changes (Figure 1). Based on the radiological findings, the differential diagnosis included neuroendocrine tumor, sclerosing mesenteritis, metastatic carcinoma, desmoid-type fibromatosis, or lymphoma.

After further evaluation, it was thought that surgery was required to rule out malignancy. A laparoscopy was attempted, but the mesentery was so infiltrated that it was impossible to obtain a biopsy or find the origin of the primary lesion, and the procedure was converted to open laparotomy. The liver, gallbladder, pancreas, and large bowel were found to be grossly normal; however, a large, hard, infiltrating lesion was identified in the root of the small bowel mesentery. It was determined that resection of the mass would compromise the vascular supply of the small bowel and result in short bowel syndrome.

A biopsy of the retractile mesentery was obtained, and frozen-section pathology revealed benign tissue. The histologic sections showed fat necrosis with extensive sclerosing fibrosis, focal areas of calcification, and chronic granulomatous inflammation (Figure 2). The chronic inflammation consisted of a lymphoplasmacytic infiltrate with occasional lymphoid aggregates. These findings were consistent with sclerosing mesenteritis. The tissue cultures did not grow any organisms, such as mycobacteria. At a follow-up appointment 2 weeks postoperatively, the patient was doing well.

Discussion

Sclerosing mesenteritis is a chronic, nonneoplastic, fibrotic condition that involves the adipose tissue of the mesentery. A variety of terms are used throughout the literature to describe this condition, including mesenteric panniculitis and retractile, liposclerotic, or xanthogranulomatous mesenteritis. In some cases, the term selected depends on the stage at which the disease is diagnosed.

Examinations for sclerosing mesenteritis using CT scanning suggest a prevalence of approximately 0.6%.2 The condition has a peak incidence between the sixth and seventh decades of life and has been associated with several disease processes, such as malignancy, pancreatitis, infection, trauma, and surgery. Studies have shown that up to 69% of sclerosing mesenteritis cases have been linked to malignancy, particularly lymphoma.3 Ege and colleagues reported a rare case of mesenteric panniculitis associated with tuberculosis lymphadenitis.4 Our patient did not have a medical history or laboratory findings that would indicate a malignant process or tuberculosis lymphadenitis.

Although its etiology remains unknown, sclerotic mesenteritis has been recognized to progress through three stages. The first stage is mesenteric lipodystrophy, which involves degeneration of the mesenteric fat. Histologically, sheets of foamy microphages along with lymphocytic infiltrates are seen in place of mesenteric fat. The second stage is known as mesenteric panniculitis and is distinguished by mesenteric inflammatory changes. Histological analysis reveals plasma cells, foamy microphages, and foreign body giant cells. The final stage is sclerosing mesenteritis, also known as retractile mesenteritis. This term was given to describe the scarring and retraction of the mesentery secondary to collagen deposition. Pathology reveals hyalinization, calcifications, and chronic inflammation, which was observed in our patient.5

The definitive diagnosis of sclerosing mesenteritis is made through histological analysis obtained laparoscopically or through open laparotomy. Pancreatitis, inflammatory bowel disease, extra-abdominal fat necrosis (Weber-Christian disease), and malignancy must be ruled out before sclerosing mesenteritis can be diagnosed. In rare cases, lymphomas may display similar histologic characteristics, which is why radiological findings have become increasingly important in identifying sclerosing mesenteritis.6

It has been suggested that CT scanning shows certain criteria relevant to this disorder. These include the appearance of a solitary soft tissue mass in the mesentery of the small bowel, entrapment of the superior mesenteric artery with no vascular involvement, and no invasion of adjacent small bowel loops. Studies show that a hyperattenuated stripe surrounding the mass is visible in up to 50% of patients with sclerosing mesenteritis. A hypodense fatty halo, know as a "fat-ring sign," may help clinicians distinguish lymphoma from sclerosing mesenteritis. The fat-ring sign was observed on nearly 79.5% of CT scans of patients with sclerosing mesenteritis (Figure 3).2

The integration of positron emission tomography (PET) with CT scanning plays a critical role in assessing sclerosing mesenteritis. PET studies are highly accurate in excluding tumoral involvement of the mesentery by showing no 2-fluoro-2-deoxy-D-glucose (FDG) uptake.3

Although patients with mesenteric panniculitis seem to have a good prognosis and their condition may regress spontaneously,7,8 the outcome for patients with sclerosing mesenteritis appears less favorable. Some patients have shown improvement after being treated with cyclophosphamide,9 corticosteroids with or without colchicine,10,11 azathioprine,12,13 or tamoxifen.14 Surgical treatment is usually not indicated for sclerosing mesenteritis, except for frozen-section biopsy. Surgery may be required, however, to remove the mesenteric mass in cases of bowel obstruction, arterial occlusion, and symptomatology refractory to medical treatment.

Conclusion

Treatment for sclerosing mesenteritis should be based on the stage of the disease at diagnosis. Earlier stages may be observed or treated medically; however, when fibrosis becomes extensive, surgical intervention is the treatment of choice.

Acknowledgment

We thank Steven Leighton, MD, Mount Sinai Medical Center, Department of Radiology, for his assistance with reading the CT scans.

Disclosure

The authors have no relationship with any commercial entity that might represent a conflict of interest with the content of this article and attest that the data meet the requirements for informed consent and for the Institutional Review Boards.

References

  1. Parra-Davila E, McKenney MG, Sleeman D, et al. Mesenteric panniculitis: case report and literature review. Am Surg. 1998;64 (8):768-771.
  2. Daskalogiannaki M, Voloudaki A, Prassopoulos P, et al. CT evaluation of mesenteric panniculitis: prevalence and associated diseases. AJR Am J Roentgenol. 2000;174(2):427-431.
  3. Zissin R, Metser U, Hain D, et al. Mesenteric panniculitis in oncologic patients: PET-CT findings. Br J Radiol. 2006;79(937): 37-43.
  4. Ege G, Akman H, Cakiroglu G. Mesenteric panniculitis associated with abdominal tuberculous lymphadenitis: a case report and review of the literature. Br J Radiol. 2002;75(892):378-380.
  5. Patel N, Saleeb SF, Teplick SK. General case of the day. Mesenteric panniculitis with extensive inflammatory involvement of the peritoneum and intraperitoneal structures. Radiographics. 1999;19(4):1083-1085.
  6. Lawler LP, McCarthy DM, Fishman EK, et al. Sclerosing mesenteritis: depiction by multidetector CT and three-dimensional volume rendering. AJR Am J Roentgenol. 2002;178(1):97-99.
  7. Runyon BA, Sich J. Diseases of the mesentery and omentum. In: Feldman M, Friedman LS, Sleisenger MH, eds. Gastrointestinal and Liver Disease. 7th ed. Philadelphia, Pa: Saunders, 2002:2366-2367.
  8. Beauchamp RD, Peeler MO. Diseases of the mesentery and omentum. In: Yamada T, Alpers DH, Lane L, et al, eds. Textbook of Gastroenterology. 3rd ed. Philadelphia, Pa: Lippincott Williams and Wilkins; 1999:2376-2377.
  9. Bush RW, Hammar SP Jr, Rudolph RH. Sclerosing mesenteritis. Response to cyclophosphamide. Arch Intern Med. 1986; 146(3):503-505.
  10. Horing E, Hingerl T, Hens K, et al. Protein-losing enteropathy: first manifestation of sclerosing mesenteritis. Eur J Gastroenterol Hepatol. 1995;7(5):481-483.
  11. Genereau T, Bellin MF, Wechsler B, et al. Demonstration of efficacy of combining corticosteroids and colchicine in two patients with idiopathic sclerosing mesenteritis. Dig Dis Sci. 1996; 41(4):684-688.
  12. Bala A, Coderre SP, Johnson DR, et al. Treatment of sclerosing mesenteritis with corticosteroids and azathioprine. Can J Gastroenterol. 2001;15(8):533-535.
  13. Tytgat GN, Roozendaal K, Winter W, et al. Successful treatment of a patient with retractile mesenteritis with prednisone and azathioprine. Gastroenterology. 1980;79(2):352-356.
  14. Venkataramani A, Behling CA, Lyche KD. Sclerosing mesenteritis: an unusual cause of abdominal pain in an HIV-positive patient. Am J Gastroenterol. 1997;92(6):1059-1060.