A Case of Idiopathic Sclerosing Mesenteritis with Retroperitoneal Fibrosis

June Ho Bae; Seong Hwan Kim; Sang Bong Ahn; Byoung Kwan Son; Yun Ju Jo; Young Sook Park; Yu Min Jung; Yeon Soo Chang

doi:10.4166/kjg.2011.58.4.221

Journal List > Korean J Gastroenterol > v.58(4) > 1006867

Go to TopGo to Top Go to BottomGo to Bottom

TOOLS

Bae, Kim, Ahn, Son, Jo, Park, Jung, and Chang: A Case of Idiopathic Sclerosing Mesenteritis with Retroperitoneal Fibrosis

Case Report

Korean J Gastroenterol 2011;58(4):221-225.

Published online: 4 October 2011

DOI: https://doi.org/10.4166/kjg.2011.58.4.221

A Case of Idiopathic Sclerosing Mesenteritis with Retroperitoneal Fibrosis

June Ho Bae, Seong Hwan Kim, Sang Bong Ahn, Byoung Kwan Son, Yun Ju Jo, Young Sook Park, Yu Min Jung, Yeon Soo Chang¹

Department of Internal Medicine, Eulji General Hospital, Eulji University College of Medicine, Seoul, Korea

¹Department of Surgery, Eulji General Hospital, Eulji University College of Medicine, Seoul, Korea

Correspondence to: Seong Hwan Kim, Department of Internal Medicine, Eulji General Hospital, Eulji University College of Medicine, 280-1, Hagye-dong, Nowon-gu, Seoul 139-711, Korea. Tel: +82-2-970-8209, Fax: +82-2-970-8621, E-mail: shkim@eulji.ac.kr

Received 6 August 2010 Accepted 24 January 2011

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Sclerosing mesenteritis is a rare inflammatory disease of the bowel mesentery. It produces tumor-like masses of the mesentery composed of varying degrees of fibrosis, chronic inflammation, and fat necrosis. It has been described variously as fibrosing mesenteritis, retractile mesenteritis, mesenteric Weber Christian disease, and systemic nodular panniculitis. The etiology and pathogenesis of the disease are as yet unknown, but autoimmune disorder, previous abdominal surgery, trauma, and ischemia could play a role. The clinical features include abdominal pain, vomiting, diarrhea, and constipation. Occasionally, patients with this condition may present with bowel obstruction. Rarely, It can be associated with other idiopathic inflammatory disorders such as retroperitoneal fibrosis, sclerosing cholangitis, and orbital pseudotumors. We report a case of idiopathic sclerosing mesenteritis with retroperitoneal fibrosis in a 58-year-old man.

Keywords: Sclerosing mesenteritis, Retroperitoneal fibrosis, Tamoxifen

References

1. Phillips RH, Carr RA, Preston R, et al. Sclerosing mesenteritis involving the pancreas: two cases of a rare cause of abdominal mass mimicking malignancy. Eur J Gastroenterol Hepatol. 1999; 11:1323–1329.

2. Lim CS, Singh Ranger G, Tibrewal S, Jani B, Jeddy TA, Lafferty K. Sclerosing mesenteritis presenting with small bowel obstruction and subsequent retroperitoneal fibrosis. Eur J Gastroenterol Hepatol. 2006; 18:1285–1287.

3. Akram S, Pardi DS, Schaffner JA, Smyrk TC. Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients. Clin Gastroenterol Hepatol. 2007; 5:589–596.

4. Park KH, Chang HK, Choi SY, et al. Sclerosing mesenteritis associated with skin panniculitis and pleural thickening. Korean J Med. 1999; 57:103–107.

5. Lee SY, Park DE, Chae KM. Sclerosing mesenteritis. J Korean Surg Soc. 2006; 71:218–221.

6. Kim EG, Kang YW, Yoon SG, Kim HD, Kim KY. Four cases of post-operative sclerosing mesenteritis. J Korean Soc Coloproctol. 2007; 23:374–380.

7. Medina-Franco H, Listinsky C, Mel Wilcox C, Morgan D, Heslin MJ. Concomitant sclerosing mesenteritis and bile duct fibrosis simulating Klatskin's tumor. J Gastrointest Surg. 2001; 5:658–660.

8. Scudiere JR, Shi C, Hruban RH, et al. Sclerosing mesenteritis involving the pancreas: a mimicker of pancreatic cancer. Am J Surg Pathol. 2010; 34:447–453.

9. Chawla S, Yalamarthi S, Shaikh IA, Tagore V, Skaife P. An unusual presentation of sclerosing mesenteritis as pneumoperitoneum: case report with a review of the literature. World J Gastroenterol. 2009; 15:117–120.

10. Daskalogiannaki M, Voloudaki A, Prassopoulos P, et al. CT evaluation of mesenteric panniculitis: prevalence and associated diseases. AJR Am J Roentgenol. 2000; 174:427–431.

11. Kornprat P, Liegl-Atzwanger B, Portugaller H, Bernhardt GA, Mischinger HJ. Sclerosing mesenteritis, a rare cause of a retroperitoneal tumor. Wien Klin Wochenschr. 2010; 122:179–183.

12. Hassan T, Balsitis M, Rawlings D, Shah AA. Sclerosing mesenteritis presenting with complete small bowel obstruction, abdominal mass and hydronephrosis. Ir J Med Sci. 2010. [Epub ahead of print].

13. Edlich RF, Woodard CR, Pine SA, Lin KY. Hazards of powder on surgical and examination gloves: a collective review. J Long Term Eff Med Implants. 2001; 11:15–27.

14. Remmele W, Müller-Lobeck H, Paulus W. Primary mesenteritis, mesenteric fibrosis and mesenteric fibromatosis. Report of four cases, pathology, and classification. Pathol Res Pract. 1988; 184:77–85.

15. Kamisawa T, Nakajima H, Egawa N, Funata N, Tsuruta K, Okamoto A. IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology. 2006; 6:132–137.

16. Kamisawa T, Funata N, Hayashi Y, et al. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut. 2003; 52:683–687.

17. Bush RW, Hammar SP Jr, Rudolph RH. Sclerosing mesenteritis. Response to cyclophosphamide. Arch Intern Med. 1986; 146:503–505.

18. Bala A, Coderre SP, Johnson DR, Nayak V. Treatment of sclerosing mesenteritis with corticosteroids and azathioprine. Can J Gastroenterol. 2001; 15:533–535.

Fig. 1.

Erect abdominal x-ray showed paralytic ileus and fecal stasis.

Fig. 2.

Abdominal CT finding. It revealed mass like enhancement soft tissue lesion (arrow) along the jejunal mesentery, lymph nodes, mesenteric infiltration, and edematous wall thickening of the jejunum.

Fig. 3.

Abdominal CT showed enhancement soft tissue lesion around the abdominal aorta (arrow).

Fig. 4.

Laparoscopy showed severe inflammation and whitish sclerosis of the mesentery with bleeding.

Fig. 5.

(A) Microscopic finding showed irregular fibrosis with myxoid change and expanding into entrapped fat tissue (H&E, ×100). (B) The fibrous tissue showed proliferation of myofibroblastic cells and infiltration of chronic inflammatory cells (H&E, ×200).

Table 1.

Sclerosing Mesenteritis Reported in Korea

Case	Authors	Year of study	Age /Sex	Symptom	Diagnosis	Operation history	Treatment	Prognosis
1	Park KH et al.⁴	1999	53/F	Abdominal pain, nausea, vomiting	Sclerosing mesenteritis	None	Surgical resection+ medical treatment (prednisolone+ cyclophosphoamide)	Improved
2	Lee SY et al.⁵	2006	59/M	Abdominal pain, diarrhea	Sclerosing mesenteritis	None	Surgical resection	Improved
3	Kim EG et al.⁶	2007	58/M	Abdominal pain, nausea, vomiting	Mesenteric panniculitis	Right hemicolectomy	Surgical resection	Improved
4	Kim EG et al.⁶	2007	50/M	Abdominal pain, nausea, vomiting	Sclerosing mesenteritis	Small bowel resection	Surgical resection	Improved
5	Kim EG et al.⁶	2007	60/F	Abdominal pain, nausea, vomiting	Sclerosing mesenteritis	Ileosigmoidostomy	Surgical anastomosis	Improved
6	Kim EG et al.⁶	2007	72/M	Abdominal discomfort, vomiting	Sclerosing mesenteritis	Small bowel resection	Surgical resection	Improved
7	This study	2010	58/M	Abdominal pain	Sclerosing mesenteritis	None	Medical treatment (tamoxifen+ predinisolone)	Improved

M, male; F, female.

TOOLS

Similar articles