Sclerosing Mesenteritis Report of Two Cases M A R T I N RESKE, M.D.,

AND H I D E O N A M I K I ,

M.D.

Department of Pathobgy, The Queen's Medical Center, Honolulu, Hawaii

ABSTRACT

tumor-like lesions composed of fibrous tissue and chronically inflamed adipose tissue have been described under a confusing array of at least a dozen terms. T h e most frequently used terms are "retractile mesenteritis"1'17-18'20-21'26'27'34'37 and "mesenteric panniculitis".10,12'14-24,33 In retractile mesenteritis, extensive proliferation of collagenous fibrous tissue with mild chronic inflammation is found, while the proportions of the two components appear reversed in mesenteric panniculitis, i.e., extensive chronic inflammation and mild fibrous tissue proliferation. Both lesions may be the expression of different stages of a reparative process that occurs in response to mesenteric injuries of various etiologies. We BENIGN MESENTERIC

wish to present two cases which, in our opinion, demonstrate the close relationship of these lesions, and we suggest the unifying term "sclerosing mesenteritis." Report of Two Cases

Case 1: A 40-year-old man experienced a sudden onset of acute, severe, midabdominal pain, more severe on the left, intermittent and cramping in nature and followed by nausea and vomiting. A similar episode had occurred 2 years before; it had spontaneously subsided within 24 hours. On admission, the patient was acutely ill. There was slight abdominal distention, tenderness in the left lower quadrant, and prolonged peristaltic rushes. Abdominal x-rays suggested a partial small-bowel obstruction. T h e Received January 10, 1975; received revised manuleukocyte count increased in the followscript March 10, 1975; accepted for publication March 10, 1975. ing 24 hours from 9,400 to 16,000, with Address reprint requests to Dr. Reske: Buffalo General Hospital, Department of Pathology, 100 75% segmented neutrophils, 3% bands, High Street, Buffalo, New York 14203. 14% lymphocytes, 7% monocytes and 1% 661

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Reske, Martin, and Namiki, Hideo: Sclerosing mesenteritis. Report of two cases. Am J Clin Pathol 64: 661-667, 1975. Two cases of benign tumorlike mesenteric lesions are presented. T h e limited literature on comparable and similar lesions is reviewed, and the histologic findings are correlated. T h e lesions are composed of chronically inflamed adipose and fibrous tissue in various proportions. They probably represent different stages of a reparative process initiated by damage of the mesenteric adipose tissue of various etiologies. Whereas lesions in the younger age groups (mean 39.9 years) are predominantly characterized by fibrosis, those in the older age groups (mean 55.8 years) usually show a chronic inflammatory cell infiltrate rather than fibrosis. More than a dozen terms have been used for these lesions. T h e summarizing term "sclerosing mesenteritis" is proposed. (Key words: Sclerosing mesenteritis.)

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jacent to these islands were small foci of adipose tissue infiltrated by lymphocytes and lipophages (Fig. 2). While thick and dense collagen bundles were laid down in these fibrotic areas, indicating the longstanding nature of the lesion, the mesentery elsewhere was partly replaced by illdefined foci of proliferating fibrocytes embedded in a myxoid matrix suggestive of a recent, active fibroblastic process. T h e fibrotic lesions were confined to the mesentery and did not extend into the intestinal wall. Case 2: A 59-year-old man had had

The mesenteric mass consisted of confluent, large, irregular-shaped foci of fibrosis. Histologically, the fibrotic foci were made u p of irregular, poorly defined streams of spindly and wavy-shaped fibrocytes embedded in an abundant collagenous fibrous stroma richly supplied by ectatic capillary blood vessels (Fig. 3). Although there were scattered small foci of lymphocytic infiltration in the mesenteric fat, there were no aggregates of lipophages or true fatty necrosis to indicate active lipodystrophic lesions. T h e fibrotic lesion was located in the mesen-

FIG. 1 [upper, left). Irregularly-shaped interlacing bundles of proliferating fibrocytes replacing the mesenteric fat; focal aggregate of lipophages. Hematoxylin and eosin. x90. FIG. 2 (upper, right). Mesenteric adipose tissue infiltrated by lipophages and lymphocytes. Hematoxylin and eosin. x260. FIG. 3 (lower, left). Streams of spindly and wavy-shaped fibrocytes embedded in collagenous fibrous stroma; focal lymphocytic infiltrations. Hematoxylin and eosin. x70. FIG. 4 (lower, right). Focal replacement of longitudinal fibers of intestinal muscularis propria by fibrotic tissue. Hematoxylin and eosin. x25.

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eosinophils. A laparotomy revealed a dull, intermittent pain in the epigastrium cicatricial puckered lesion involving the and right upper quadrant for about 12 antimesenteric margin of the mid-ileum years. He had been treated for possible with constriction of the ileum. T h e serosal gallbladder disease for ten years. In 1972, surfaces showed white, fibrotic streaks. a radiologic diagnosis of a prepyloric ulcer There was an irregular, firm, fibrous had been made and the patient treated nodule in the adventitia at the junction medically. of the adipose mesentery and the smallIn April 1974, the patient experienced bowel wall. This nodular mass was about colicky pain, and again the ulcer was 1.5 X 0.5 cm. in size. A 7-cm. segment found, as well as a hiatus hernia. On of ileum that contained the lesion and laparotomy, an irregular, firm tumor measportions of the mesentery were resected. uring 13 x 7 x 5 cm. was found in the T h e patient has remained symptomless small-bowel mesentery close to the ligafor the last four years. ment of Treitz. At least three loops of Histologically, the sections from the ileum were plicated to the tumor, and a nodular mesenteric lesion showed the branch of the superior mesenteric artery mesentery to be thickened and partly re- was encased within the tumor. A resecplaced by discrete, irregularly shaped tion of the involved small bowel and islands of interlacing bundles of proliferat- mesentery was done. There was no posting fibrocytes (Fig. 1). Within and ad- operative complication.

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tery, and the longitudinal fibers of the intestinal muscularis propria were partly replaced by the fibrous tissue (Fig. 4).

According to Black and co-workers, 4 RM and "multifocal subperitoneal sclerosis" are similar except for the presence of mesenteric and non-mesenteric lesions in the latter; in fact, their case was published twice under these different names. 1,4 "Mesenteric fibromatosis" appeared identical to RM, 6,26 and also showed histologic similarity to "desmoid tumors." MP, or its synonym "liposclerotic mesenteritis," 9 and RM were thought to share similar characteristics. 12,14 RM was interpreted as the result of MP 14,24,33,34 and of "lipogranuloma of mesentery." 38 According to Handelsman and associates,14 MP had been variously designated as RM, "lipogranuloma of mesentery," "retroperitoneal xanthogranuloma," 23 and "isolated lipodystrophy." Aach and colleagues 1 also mentioned similarities between MP and "isolated lipodystrophy." Tedeschi and coworkers 37 believed that the two cases of "Weber-Christian disease" reported by Herrington and colleagues 16 fall into the category of RM. Other authors suggested

initiates an allergic process, resulting in adventitial inflammation and progressively spreading fibrosis.22 T h e terms for the lesions used in the literature may be classified roughly into two groups that reflect the predominance of one of the major histologic components of the lesions: group A includes those characterized by prominent fibroblastic proliferation (RM, multifocal subperitoneal sclerosis, fibromatosis, desmoid tumor), and group B is comprised of lesions characterized by predominant inflammation of the fat with formation of lipogranulomas (MP, lipogranuloma of mesentery, liposclerotic mesenteritis, mesenteric manifestation of Weber-Christian disease, systemic nodular panniculitis, lipodystrophy of adipose areolar tissue in the retroperitoneal space, isolated lipodystrophy, lipodystrophy of mesenteric fat, mesenteric lipodystrophy, and intestinal lipodystrophy). T h e clinical symptoms described for most published cases in both groups were

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that MP; "sclerosing lipogranuloma," "systemic nodular panniculitis," and "lipodystrophy of adipose areolar tissue" were merely variants of Weber-Christian disDiscussion ease. 7,12,16,24,30,35 French and associates 11 In the literature, comparable and simi- objected to this concept and chose to follar mesenteric lesions usually have been low the interpretation of Crane and co8 called "retractile mesenteritis" (RM) and workers that the basic defect is prolifera"mesenteric panniculitis" (MP). T h e follow- tion of non-neoplastic adipose cells, which ing terms also have been used: "multi- become altered due to nutritional or other 6 focal subperitoneal sclerosis,"4 "fibroma- disturbances. Clemett and co-workers con31,39 2,29 sidered almost all of the above-mentioned tosis," probably "desmoid tumor," "lipogranuloma of mesentery," 38 "lipo- entities to comprise a single disease, which sclerotic mesenteritis," 9 "sclerosing lipo- should be called "retractile mesenteritis" 17 granuloma," 7 "mesenteric manifestation of as coined by Jura. 3,16 "systemic Weber-Christian disease," Controversial opinions exist about the nodular panniculitis," 35 "lipodystrophy of relationship between RM and retroperiadipose areolar tissue," 30 "isolated lipo- toneal fibrosis.31 However, more recent dystrophy," 8,28 "lipodystrophy of mesen- studies 22 suggest that idiopathic retroperiteric fat,"11 "mesenteric lipodystrophy," 19 toneal fibrosis is a separate entity that and "intestinal lipodystrophy." 25 Despite tends to manifest as a predominantly perithe fact that many authors have recog- aortic, widespread disease associated with nized the similarity of their cases to cases systemic disturbances. T h e underlying abpublished under different names, a de- normality may be damage to the wall tailed histologic comparison is lacking. of the aorta or its major branches that

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Table 1. Summary of Clinical Features

Number of patients Sex distribution Age distribution Mean age Number of patients less than 20 years old Range of duration of symptoms Average duration of symptoms

Group B

32 14 female: 18 male 2-86 years 39.9 years 6 1 week-8 years 15.1 months

116 50 female: 66 male 14-82 years 55.8 years 1 1 week-16 years 13.5 months

taneous adipose tissue. In view of the mentioned systemic variant, some authors 3,16 have chosen the term "mesenteric involvement" or "mesenteric manifestation" of Weber-Christian disease to report cases with exclusive involvement of visceral fat despite a generally benign course. Ogden, 24 however, objected to this concept because of the lack of subcutaneous involvement, despite the pathologic similarity of the diseased adipose tissue in Webera v a i l a b l e l~12>14_16'19-21'24'26-28.30,31,33-35,37-39 Christian disease to that in "mesenteric T h e lesions have been described as firm, panniculitis." T h e interrelationship of grayish, nodular, puckered or lobular, these conditions remains questionable, and poorly delineated masses with pale yellow may give rise to future investigations. areas of fatty necrosis, myxoid areas, and Our second case is of interest since cyst-like structures filled with creamy fluid, there was fibrous-tissue infiltration of the with irregular, reddish-brown plaques at intestinal muscularis propria. In the literathe mesenteric attachment or the anti- ture, this was documented only by Schweitmesenteric surface of the small bowel. zer and Robbins. 29 Except for McGee and The lesions have been associated with ad- associates, 21 who described "impinging hesions, various degrees of intestinal ob- upon the colonic muscularis" and Aach struction, occasional enlarged regional and colleagues, 1 who mentioned "involvelymph nodes, and sclerosis or thrombosis ment of the bowel wall," most authors of mesenteric vessels. They have been described the lesions as confined at the confined to the mesentery of the small border of the small bowel. A peculiar bowel,19,20,26,37 and sometimes, there has invagination of mesenteric fibrous nodules been concomitant involvement of the meso- into the bowel lumen was reported by colon, 1,21,27,34,37 the omentum, 12,17,24 or Black and associates; 4 again, there was no retroperitoneal tissue.14,15,28,30 T h e largest mention of true invasion. fibrous mesenteric mass reported weighed A comparison of histologic descriptions 3,815 Gm. 31 in the published cases of the aforemenExtensive involvement of visceral and tioned groups A and B shows striking subcutaneous fat was described by Stein- similarities. Findings encountered in both berg 35 as "systemic nodular panniculitis," groups include: and it was interpreted as a variant of (a) Scattered aggregates of lymphocytes Weber-Christian disease with a uniformly and plasma cells. 1,3,4,8,9,11,12,14,16 - 21,24 fatal course. Weber-Christian disease, by 26,27,30,33-35,37,38 24 definition, is a relapsing, febrile, nonsuppurative nodular panniculitis of subcu(b) Aggregates of macrophages with

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more or less similar. The patients had vague, recurrent abdominal pain, partial or complete intestinal obstruction, bouts of diarrhea, a palpable and sometimes pulsatile mass, recurrent low-grade fever, weight loss, nausea, and anorexia. In some cases, no symptoms were present, and the lesions were discovered incidentally during surgery or at autopsy. 11,12,18,20,33 Table 1 summarizes the clinical features of those cases where comprehensive data are

Group A

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foamy cytoplasm.3-8-9-11-14-19-24-30-33-3537,38

(c) Focal fat necrosis.3-9-10-14-17-19'33'35-37-38 (d) Mild focal to extensive fibrosis.1-3-58.9,11,16-21,24,26,27,30,33-35,37

(e) Polymorphonuclears. 14,33-35 - 38 (f) Calcifications.20-24-37 (g) Thrombosis. 18,33 Findings described include:

for group B only

(a) Multinucleated giant cells.3-8'9,12-16-1924,30,33,35

Consistently absent or at least not described in both groups were findings such as enteritis, 34 vasculitis,19-34 and hemosiderin deposits. 4,34 It is felt that the multinucleated giant cells, altered fat cells, cholesterol clefts, "halo effect," and lymphangiectasia confined to the lesions summarized in group B merely represent variants of different stages of a nonspecific reparative process in the mesentery which gradually progresses to fibrosis and scarring. Our first case appears to illustrate a transient stage from a group B lesion to a group A lesion, evidenced by focal infiltrations of lipophages and lymphocytes and by the presence of proliferating fibrocytes in a myxoid matrix. In the second case, the healing process appears completed. There is abundant collagenous scar tissue and no evidence of active lipodystrophic iesions. T h e interval between the mesenteric injury and complete, inactive scarring appears rather variable and inconstant. However, there is some suggestion that the fibrous healing process occurs more frequently and more rapidly in the younger age group (mean 39.9 years), while there is prolonged chronic inflammation of the mesenteric fat with slow fibrous tissue

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proliferation in older patients (mean age 55.8 years). Scarring associated with retraction and intestinal obstruction occurs only in a limited number of lesions. We therefore propose the more general term "sclerosing mesenteritis," which expresses the inflammatory and fibrotic phase of mesenteric response to injury. This term, like "retractile mesenteritis," is still a part of Jura's original terminology. 17 As to etiologic considerations, various speculations have been proposed, although many investigators have concluded that the etiology remains obscure.2'14-19-21'26-34 Numerous possible etiologic factors for the development of these lesions have been implemented. 1 - 2 - 5 - 11 - 13 - 14 ' 17 ' 18 - 25 - 29 - 32 - 34 - 37 They include blunt or surgical trauma, thermal or chemical injury, phlebitis or vasculitis, abnormal fat cell metabolism, and avitaminosis. Also, the process may be initiated by a coexisting surgical illness such as hemorrhage, Gardner's syndrome, or leakage of bile, urine, pancreatic secretions, chylous fluid, or intestinal contents. Finally, various infectious diseases, including Whipple's disease, as well as autoimmune and collagen diseases or drug hypersensitivity reactions may contribute to the development of the lesions. An occasional association with malignant lymphoma has been reported.14-19-24 Fatty necrosis and fibrosis have been described to follow injections of various substances. 35 Lesions resembling RM were reproduced experimentally by intraperitoneal injections of E. coli, as well as Mycobacterium tuberculosis,1* and by surgical trauma to the mesentery. 36 T h e diversity of implemented etiologic factors appears to support the conclusion that the lesions are merely nonspecific reactions to any single specific mesenteric injury. The reaction, in the form of either protracted panniculitis with resulting fibrosis or predominant fibrosis, may be influenced by the age of the patient. T h e lesions usually are localized and selflimited. The diagnosis cannot be established without exploratory laparotomy and

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(b) Altered fat cells.8-11-19-25-28 (c) Cholesterol clefts.3-8-11-12-16-19 (d) "Halo effect"—lipid-laden macrophages, central aggregates of lymphocytes and interposed normal adipose tissue.8-24 (e) Lymphangiectasia. 7,24,33

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References 1. Aach RD, Kahn LJ, Freeh RS: Obstruction of the small intestine due to retractile mesenteritis. Gastroenterology 54:594-598, 1968 2. Ackermann LV: Tumors of the retroperitoneum, mesentery and peritoneum, Atlas of T u m o r Pathology. Section 6, Fascicle 2 3 - 2 4 . Armed Forces Institute of Pathology, 1954 3. Arnold HA, Bainborough AR: Weber-Christian disease with visceral involvement. Can Med Assoc J 89:1138-1142, 1963 4. Black W, Nelson D, Walker W: Multifocal subperitoneal sclerosis. Surgery 63:706-710,1968 5. Carillo FJ, Ruzicka FF, Clemett AR: Value of angiography in the diagnosis of rectractile mesenteritis. Am J Roentgenol Radium T h e r Nucl Med 115:396-398, 1972 6. Clemett AR, Tracht DG: T h e roentgen diagnosis of retractile mesenteritis. Am J Roentgenol Radium Ther Nucl Med 107:787-790, 1969 7. Coppridge WM, Roberts LC, Hughes J: Sclerosing lipogranuloma. South Med J 48:827-833, 1955 8. Crane J T , Aguilar MJ, Grimes OF: Isolated lipodystrophy, a form of mesenteric tumor. Am J Surg 90:169-179, 1955 9. DeCastro JA, Calem WS, Papadakis L: Liposclerotic mesenteritis. Arch Surg 94:26-29, 1967 10. Decker GAG, Gaylis H: Mesenteric panniculitis simulating an abdominal aortic aneurysm. S Afr Med J 46:750, 1972 11. French WE, Bale GF, Winborn WB: Lipodystrophy of mesenteric fat. Surg Gynecol Obstet 122:1046-1052, 1966 12. Grossman LA, Kaplan HJ, Preuss HJ, et al: Mesenteric panniculitis. JAMA 183:318-323, 1963 13. Handelsman JC, Ravitch MM: Chylous cysts of the mesentery in children. Ann Surg 140: 185-193, 1954 14. Handelsman JC, Shelley WM: Mesenteric panniculitis. Arch Surg 91:842-850, 1965

15. Harbrecht PJ: Variants of retroperitoneal fibrosis. Ann Surg 165:388-401, 1967 16. Herrington JL, Edwards WH, Grossman LA: Mesenteric manifestations of Weber-Christian disease. Ann Surg 154:949-955, 1961 17. J u r a V: Sulla mesenterite retrattile e sclerosante. Policlinico (Prat) 31:575-581, 1924 18. Jura V: La mesenterite retrattile. Policlinico (Chir) 34:535-556 and 566-599, 1927 19. Kipfer RE, Moertel CG, Dahlin DC: Mesenteric lipodystrophy. Ann Intern Med 80:582-588, 1974 20. Lawrence MS, Stanford W, Sheets RF: Retractile mesenteritis with transmitted pulsations. Am J Med 41:847-848, 1966 21. McGee HJ, Traylor FA: Retractile (sclerosing) mesenteritis. Cancer Seminar (Penrose Cancer Hosp) 3:203-205, 1965 22. Mitchinson MJ: T h e pathology of idiopathic retroperitoneal fibrosis. J Clin Pathol 2 3 : 6 8 1 689, 1970 23. Oberling C: Retroperitoneal xanthogranuloma. Am J Cancer 23:477-489, 1935 24. Ogden WW, Bradburn DM, Rives J D : Mesenteric panniculitis. Ann Surg 161:864-873, 1965 25. Pemberton JdeJ, Comfort MW, Fair E, et al: Intestinal lipodystrophy (Whipple's disease). Surg Gynecol Obstet 8 5 : 8 5 - 9 1 , 1947 26. Phintuyothin P, Surabhakdi M, Kanchanakul K, et al: Retractile mesenteritis. J Med Assoc Thai 56:485-490, 1973 27. Roberts EM, Nielsen OF: Retractile mesenteritis. Am J Dig Dis 9:231-235, 1964 28. Rogers CE, Demetrakopoulos MS, Hyamns V: Isolated lipodystrophy affecting the mesentery, the retroperitoneal area and the small intestine. Ann Surg 153:277-282, 1961 29. Schweitzer RJ, Robbins GF: Desmoid tumor of multicentric origin. Arch Surg 80:489-494, 1960 30. Shafiroff BGP, Kau QY: Lipodystrophy of adipose areolar tissue in the retroperitoneal space. Surgery 59:696-702, 1966 31. Simpson RD, Harrison EG, MayoCW: Mesenteric fibromatosis in familial polyposis, a variant of Gardner's syndrome. Cancer 17:526-534, 1964 32. Smetana HF, Bernhard W: Sclerosing lipogranuloma. Arch Pathol 50:296-325, 1950 33. Soergel KH, Hensley GT: Fatal mesenteric panniculitis. Gastroenterology 51:529-536, 1966 34. Spark RB, Yakovac WC, Wagget J: Retractile sclerosing mesenteritis. Clin Pediatr 10:119122, 1971 35. Steinberg B: Systemic nodular panniculitis. Am J Pathol 29:1059-1081, 1953 36. Tedeschi CG: La neoformazione di tessuto muscolaye liscio mella rigenerazione posttraumatica del omento tipografia immacolata concezione. Modena Boll Soc Med Chir Modena p 1-15, 1932 37. Tedeschi CG, Botta GC: Retractile mesenteritis. N Engl J Med 266:1035-1040, 1962 38. Weeks LE, Block MA, Hathaway JC, et al: Lipogranuloma of mesentery producing abdominal mass. Arch Surg 86:615-620, 1963 39. Yannopoulos K, Stout AP: Primary solid tumors of the mesentery. Cancer 16:914-927, 1963

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biopsy, 5,20 although a radiologic diagnosis of RM may be possible. 8,26 However, angiography appears to be of little value, 5 and a laboratory work-up is not helpful.12 Electron microscopy also fails to reveal specific changes." There is general agreement that surgery should be limited to diagnostic biopsies5,6,9,12,14 except in complicated cases with mechanical obstruction. 1,14 Radiation treatment is without effect,11 and spontaneous recovery is likely.12,20,27 Only one fatal case has been reported; in that case, only the visceral fat was diseased. 33 In contrast, fatal outcomes were reported in all cases of patients who had systemic involvement of the adipose tissue (systemic nodular panniculitis). 35

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