Primary Mesenteritis, Mesenteric Fibrosis and Mesenteric Fibromatosis

Path. Res. Pract. 184, 77-85 (1989)

Primary Mesenteritis, Mesenteric Fibrosis and Mesenteric Fibromatosis Report of Four Cases, Pathology, and Classification W. Remmele Institut fOr Pathologie, Dr. Horst-Schmidt-Kliniken, Klinikum der Landeshauptstadt, Wiesbaden, FRG

H. Muller-Lobeck Deutsche Klinik fOr Diagnostik, Wiesbaden, FRG

W. Paulus St. Josefs-Hospital, Chirurgische Klinik, Wiesbaden, FRG

SUMMARY Primary mesenteritis is a rare disease. Two cases and two additional patients with mesenteric fibrosis/fibromatosis are reported. A classification of primary and secondary mesenteritis is suggested in order to replace the confusing variety of terms used for the same disease process. Differential diagnosis of mesenteric fibrosis versus mesenteric fibromatosis may be difficult, and some criteria for the separation of these two entities are discussed. Among the clinical symptoms, transmission ofaortic pulsations to the anterior abdominal wall is a rare but important aid for diagnosis. Associated diseases are malignant lymphoma, colonic adenomatosis (Gardner's syndrome) and retroperitoneal fibrosis. Microscopically, mesenteric lipomatosis and Whipple's disease must be ruled out. Usually, surgical treatment is not necessary. Prednisone and azathioprine may be helpful in severe cases. Fatal outcome of primary mesenteritis is extremely rare.

Introduction Primary (idiopathic) mesenteritis is a rare disease of unknown etiology. Until now, less than 200 cases have been reported. This number does not reflect, however, the true prevalence of the disease since many cases remain unpublished. Numerous terms have been used for the disease such as mesenteric panniculitis, mesenteric lipodystrophy, inflammatory and necrotizing pseudotumor of the mesentery, lipodystrophia mesenterialis, lipogranuloma of mesentery, sclerosing lipogranulomatosis, liposclerotic mesenteritis, primary liposclerosis of the mesentery, sclerosing mesenteritis, retractile mesenteritis or mesen© 1989 by Gustav Fischer Verlag, Stuttgart

teritis retrahens, and multifocal subperitoneal sclerosis (see Ref.) These terms refer either to the gross appearance of the lesion during laparotomy and autopsy or to the microscopic picture. The confusion of nomenclature is further increased by the fact that the terms "mesenteric fibrosis" and "mesenteric fibromatosis" are sometimes used synonymously. The present report deals with the histories and pathological findings in four patients with different mesenteric lesions. Based upon a review of the pertinent literature, the following topics will be briefly discussed: clinical findings, pathology and classification, differential diagnosis, associated diseases, treatment and prognosis of 0344-0338/89/0184-0077$3.50/0

78 . W. Remmele, H. Miiller-Lobeck and W. Paulus

primary mesenteritis, mesenteric fibrosis and mesenteric fibromatosis. Case Reports Clinical histories, gross and microscopic findings of the four patients are summarized in Table 1. The mesenteric lesions presented as a diffuse thickening in case 1, as plaques and nodules in case 2, and as large tumor-like fibrotic masses in cases 3 and 4. Following laparotomy, three patients recovered and remain well. The fourth died from unrelated disease.

Discussion Case 1 was characterized by large numbers of foam cells and by a focal proliferation of fibroblasts, case 2 by relatively few foam cells and a moderate fibrosis, and cases 3 and 4 by a prominent fibrosis with variable content of fibroblasts and fibrocytes. From a morphological point of view, these findings may be interpreted as different stages of one and the same disease starting with acute inflammation of the mesenteric adipose tissue, followed by a stage of progressive fibrosis with persisting inflammation, and terminating in inactive fibrosis. This sequence is obviously true as far as the first two stages are concerned, but the

Table 1. Survey of patients including clinical and pathological findings Case Age, Sex

Clinical History

1 C.S. 65 yrs, F

Recurrent abdominal pain -Laparotomy: Pronounced for 4 years, of increasing thickening of the small inintensity for 1 week. Diag- testine mesentery with yelnosis of cholecystolithiasis lowish-white spots. 1 year prior to admission.

2 W.B. 59 yrs, M Recurrent abdominal pain described as "cramps in the left abdomen following ingestion", followed by pulpy defecation. Fatigue, nausea. Laparotomy 4 years prior to admission: Numerous mesenteric nodules measuring up to a hen's egg size. Pathological diagnosis: Whipple's disease (probable). No beneficial effect of tetracycline treatment. Present admission for further diagnosis. Complaints in the upper 3 R.L. 36 yrs, F and mid-abdomen. Sonography: Large intraperitoneal mass in the left paraortic region. 12 yrs ago appendectomy, 15 yrs ago breast biopsy. Transmission of aortic pulsation to the anterior abdominal wall (initial diagnosis: aortic aneurysm?). 4 R.L. 74 yrs, M

Hepatitis B10 yrs ago progressing to liver cirrhosis. Treatment with prednisone + azathioprine. Iron deficiency anemia. Abdominal pain and nausea 6 mths prior to admission. Sonography: Two tumors in the left lower and right mid-abdomen.

Gross Findings at Laparotomy

Microscopic Findings (Biopsy and/or Autopsy)

Additional findings, Course

Acute mesenteric panPedunculated fibroid niculitis with numerous polyp of the stomach. foam cells, few lymphoUneventful course. cytes, and focal proliferation of fibroblasts (Fig. 1).

Laparotomy: Mesentery rich in adipose tissue, multiple plaques up to a goose's egg size.

Clusters of foam cells. Mod- Uneventful course. erate mesenteric fibrosis (fibrosis representing more than 50% of the total lesion) (Fig. 2).

Laparotomy: Large tumor in the jejunal mesentery, just beneath the Flex. duodeno-jejunalis, fixed to the wall of the jejunum and colon. Resection.

Broad strands of dense con- Uneventful course. Colnective tissue forming a net- onoscopy: No evidence work in the mesenteric adi- of adenomatosis. pose tissue and entrapping islands of adipose tissue (Fig. 3) and lymph nodes. Few lymphocytes. Moderate proliferation of fibroblasts (Fig. 4). Invasion of the M.propria (Fig. 5).

Laparotomy: Two masses with a diameter of approximately 10 em each in the mesentery of the small intestine expanding into the intestinal wall (Fig. 8). Biopsy for frozen section diagnosis.

Dense network of mesenteric fibrosis with encased islands of adipose tissue. Few lymphocytes (Fig. 6 and 7).

Patient died from decompensated liver cirrhosis 10 weeks later. Autopsy: No evidence of colonic adenomatosis.

Mesenteritis and Mesenteric Fibromatosis . 79

question remains open if mesenteric fibrosis as observed in our cases 3 and 4 results from antecedent inflammation or represents primarily chronic fibrosis or even a tumor-like proliferation (fibromatosis).

1 Differential diagnosis: mesenteric fibrosis versus mesenteric fibromatosis: At the first glance, the differenti-

ation of mesenteric fibrosis from mesenteric fibromatosis may appear as a question of purely academic interest. This is not true, however, since fibrosis is a final and inactive

stage of preceding lesions such as trauma, surgical intervention, or irradiation, while fibromatosis is a tumor-like condition with a tendency to invade the surrounding tissue and to recur following surgical excision. The separation of both lesions may be difficult and sometimes impossible ll , and it has been emphasized that the pathology of mesenteric fibromatosis ("fibrous dysplasia of the mesentery") remains vague, poorly defined, and variably interpreted among pathologists33 • One competent author even uses the terms "mesenteric fibrosis" and "sclerosing mesen-

Fig. 1-4. Fig. 1. Case 1 (primary mesenteritis, stage 1 = mesenteric panniculitis). Adipose tissue containing numerous,foam cells, some fibroblasts, and few lymphocytes. Paraffin, H & E, x 350. Fig. 2. Case 2 (primary mesenteritis, stage 2 = transitional stage). Adipose tissue containing a moderate number of foam cells and a moderate fibrosis (more than 50% of the total lesion when examined by low-power). Paraffin, H & E, x 140. Fig. 3. Case 3 (mesenteric fibrosis or fibromatosis). Dense fibrosis of the mesentery entrapping islands of fat cells and blood vessels. Paraffin, H & E, x 35. Fig. 4. Case 3 (mesenteric fibrosis or fibromatosis). Focal proliferation of slender fibrocytes and collagenous fibers. Paraffin, H & E, x 140.

80 . W. Remmele, H. Miiller-Lobeck and W. Paulus

Fig. 5-8. Fig. 5. Case 3 (mesenteric fibrosis or fibromatosis). Fibrous proliferation invading the m. propria of the small intestine. Paraffin, H & E, x 35. Fig. 6. Case 4 (mesenteric fibrosis or fibromatosis). Dense fibrosis forming a network of collagenous connective tissue and encasing islands of adipose tissue. Paraffin, H & E, x 140. Fig. 7. Case 4 (mesenteric fibrosis or fibromatosis). Focal infiltrates of lymphocytes in the mesenteric adipose tissue. Paraffin, H & E, x 350. Fig. 8. Case 4 (mesenteric fibrosis or fibromatosis). Gross appearance. Discrete large tumor-like mass in the mesentery near the intestinal wall (cross section showing the greyish-white cut surface).

tentls, retractile mesenteritis, sclerosing lipogranulomatosis, and subperitoneal sclerosis" synonymously19. On the background of general pathology of inflammation and the special pathology of fibromatoses as described in different parts of the body, a number of both clinical, gross and microscopic criteria will prove helpful to separate mesenteric fibrosis from fibromatosis (Table 2). The following criteria are of particular value:

Fibromatosis is highly probable if lymphoplasmocytic infiltration is a minor feature or completely absent, if there are no or only few foam cells, if the fibroblasts are of uniform size, often densely packed, and form interlacing bundles of collagenous connective tissue invading the adipose tissue and the muscular coat of the intestinal wall 11, 23, 27, 33, 36. On the other hand, the presence of necroses of the mesenteric adipose tissue, the occurrence of numerous foam cells, lymphocytes, plasma cells, capil-

Mesenteritis and Mesenteric Fibromatosis . 81 Table 2. Differential diagnosis: Mesenteric fibrosis versus mesenteric fibromatosis

Age History Trauma Surgery Irradiation Mesenteric Panniculitis• Pathology Gross Diffuse Single Mass Multiple Masses Pseudocysts Microscopic Fibrosis, Diffuse Fibrosis, Network Fibrosis, Nodular Fibroblasts Number Arrangement

Mesenteric Fibrosis

Mesenteric Fibromatosis

each

each usually > 60 yrs

+ + +

+ + +

+ + + + +

+ + +

+ + +

+ + +

Variable (often low) Variable (rather high) more regular less regular rarely forming forming bundles (fascicles) fascicles -/(+) -/(+) Mitoses Fibrosis, Invasion -/(+) + (+)/+ Fibrosis, Myxoid -/(+) Capillary content (+)/+ -/(+) Lymphocytes +/++ Adipose Tissue, -/+ Necroses -/+ Foam cells -/+ Giant cells -/+ Pseudocysts -/+ Hemorrhages -/+ Siderin -/+ Calcification Associated -/+ Fibromatoses· • • previously confirmed by laparotomy and biopsy. •• e.g. abdominal wall, mediastinum.

laries, hemorrhages, hemosiderin, and occasionally the presence of postnecrotic pseudocysts or cholesterol clefts, strongly speak in favor of a post-inflammatory or posttraumatic mesenteric fibrosis. The previous history, however, is of minor value since both fibroses and fibromatoses may be due to trauma, particularly to surgical trauma. Also the gross appearance does not permit a safe differential diagnosis. Fibromatoses in other organs may present either as a more diffuse or as a nodular lesion, and there is no reason why this should not be true also in mesenteric fibromatosis. On the other hand, acute mesenteric panniculitis may present as a single mass or as multiple masses9,24, and in a number of cases it may be concluded from the description of the microscopic features that transitions between diffuse thickening of the mesen-

tery to circumscribed tumor-like fibrosis were present (e.g. 2,35,42). On the basis of these findings, it appears probable that case 3 of our series belongs to the fibromatosis group. The proper classification of case 4 is dubious due to longstanding treatment with prednisone and azathioprine (see below). It may represent either the final stage of mesenteritis or primary mesenteric fibromatosis.

2 Classification and staging of mesenteritis: The confusing variety of terms for the different stages of primary mesenteritis demands a unifying concept of nomenclature. We offer a classification which avoids the wide variety of terms and uses only few well-defined terms and stages (Table 3). We think that this classification will make the cases published by different authors better comparable, and this may be of value for future studies on etiology, pathogenesis, treatment, clinical course and prognosis of inflammatory and fibrosing lesions of the mesentery. Originally, we had attempted to label each type and stage of mesenteritis or mesenteric fibrosis by a suffix referring to the gross type (diffuse, nodular, mixed) and to the microscopical stage (acute, subacute = transitional, final), but it appeared doubtful if such a classification would have been generally accepted. 3 Special types of primary mesenteritis: Some special types of primary mesenteritis require separate mentioning: (a) Mesenteric panniculitis with peculiar histological features: While it is stressed by some authors reporting

cases and large series of primary panniculitis that angiitis, granulomas and/or a dense neutrophilic infiltration were absent, some cases have been reported with a microscopic picture different from that in typical mesenteric panniculitis. These cases present a prominent infiltration polymorphonuclear neutrothilic leukocytes 1,38,39,4 , arteriitis and/or phlebitis I, 1 ,38,39, or granulomas 38 • It must be discussed that these cases may represent a special group of primary mesenteritis possibly due to specific agents or as a manifestation of other diseases such as systemic angiitis or pancreatitis. Microscopic evidence of features absent in the usual type of mesenteric panniculitis should therefore be followed by a thorough examination of the patient in order to rule out systemic or organ disease.

bl

(b) Primary mesenteritis associated with Weber-Christian disease: Until 1965, 13 autopsies had been reported in

whom Weber-Christian disease of the subcutaneous adipose tissue had been associated with similar findings in the internal adipose tissue (e.g. mesentery, pericardial and peripancreatic adipose tissue, bone marrow) and sometimes with the occurrence of fat-laden macrophages in the lymph nodes, spleen and liver 9,41. Although WeberChristian disease is characterized primarily by a lymphocytic infiltration, the histological picture may be similar to that of mesenteric panniculitis. Patients in whom mesenteric panniculitis has been observed therefore should be examined for the coexistence of inflammatory changes

82 . W. Remmele, H. Miiller-Lobeck and W. Paulus Table 3. Classification and terminology of mesenteritis. For further explanation see text Gross Type*

Microscopic Pathology

Synonyms, Examples

1.1. Acute stage = stage 1 = mesenteric panniculitis

Type 1: Diffuse mesenteric thickening Type 2: Single discrete tumor-like mass Type 3: Multiple discrete tumor-like masses Type 4: Mixed type: diffuse + either single or multiple mass(es)

PAS negative foam cells, clusters of lymphocytes. Rarely small necroses, pseudocysts, cholesterol clefts. Occasionally mild to moderate fibrosis (less than approximately 25% of total lesion)

Mesenteric lipodystrophy, mesenteric lipogranulomatosis, inflammatory and necrotizing pseudotumor of the mesentery

1.2. Transitional stage = stage 2

see acute stage

Foam cells, lymphocytic infiltrates, occasional pseudocysts and cholesterol clefts. Fibrosis exceeding approximately 25% of total lesion

retractile mesenteritis, sclerosing mesenteritis, sclerosing lipogranulomatosis, multifocal subperitoneal sclerosis

Disease 1.

Primary (Idiopathic) Mesenteritis

see acute stage 1.3. Late (final) stage = stage 3 = retractile mesenteritis

Dense fibrosis, occasional clusters of lymphocytes, varying number of fibroblasts, occasional foam cells

Special types: 1.4. Mesenteric panniculitis with peculiar histological findings 1.5. Primary mesenteritis associated with Weber-Christian disease 1.6. Primary mesenteritis associated with retroperitoneal fibrosis 1.7. Abdominal cocoon (peritonitis fibroplastica encapsulans) 2.

Secondary Mesenteritis due to defined causes 2.1. Terminology according to identifiable cause

Dependent from cause: Diffuse or nodular mesenteric panniculitis or fibrosis

Examples: glove starch powder mesenteritis, mesenteritis caused by surgical suture material, meconium peritonitis

Special type: 2.2. Sclerosing peritonitis (mesenteritis) due to administration of ~·adrenergic blocking drugs (propranolol, practolol) * According to Kipfer et al. 24 except mixed type. ** Explanation see text.

in other parts of the body including the subcutaneous tissue, and, vice versa, it should be kept in mind that patients with subcutaneous Weber-Christian disease might suffer from similar lesions in other regions of the body and that additional clinical complaints, e.g. pointing to the abdominal organs, might be due to mesenteric panniculitis.

(c) Primary mesenteritis associated with retroperitoneal fibrosis: In some cases of Ormond's disease, extension of

the lesion into the mesentery has been described 8, 32, 45. Conversely, primary mesenteritis may extend into the retroperitoneal space4, 9,24,35, but apparently the histological picture of the retroperitoneal lesion is not completely identical with that usually found in Ormond's disease. We are not aware of cases in whom the association of true primary mesenteritis and true primary Ormond's diseaseeach characterized by its typical microscopical pattern has been certainly proven.

(d) Abdominal cocoon (peritonitis fibroplastica encapsulans): This uncommon lesion has been described by only

few authors. Although primarily a disease of the visceral peritoneum, it may well be included among primary mesenteritis since it is fused to the mesentery. Grossly, it consists of a thick membrane encasing the small bowel. Histology shows proliferating fibrous, dense and loose connective tissue with or without local accumulations of lymphocytes and plasma cells 13, 37. The etiology is unknown. Possibly the abdominal cocoon is due to some unidentified infectious agent. Then it would represent a special type of secondary and not of primary mesenteritis. Secondary mesenteritis, defined by the existence of a well-known etiology also comprises one special type:

(e) Mesenteric fibrosis due to f3-adrenergic blockers (propranolol, practolol): A number of cases has been pub-

lished following the first report by Brown et al. in 19743,10,20,28,32. Mesenteric fibrosis may develop even several months after withdrawal of treatment. It is characterized by a band-like fibrosis beneath the peritoneal surface coiling up the bowel wall in a concertina-like pattern as seen also in the abdominal cocoon37 • Unlike mesenteric

Mesenteritis and Mesenteric Fibromatosis . 83

fibromatosis, this type of mesenteric fibrosis shows no tendency to invade the muscular coat of the bowel wall.

4 Etiology of primary mesenteritis and fibromatosis: "Primary" means that the etiology is unknown. None the less an interesting point has been discussed by Soergel and Hensley38. They mention a disease found in cattle and pigs ("bovine lipomatosis") which may be regarded as a counterpart of human mesenteric panniculitis. In bovine lipomatosis, large masses or nodules of necrotic adipose tissue are observed, predominantly in the abdominal cavity and retroperitoneum 21 ,44. The lesion may produce intestinal stenosis. It is due to disturbed fat metabolism presenting as an increased amount of cholesterol and saturated long-chain fatty acids 21 , possibly caused by an imbalance between the rate of fatty acid transport and increased lipolysis44 • Then the fatty acids and their soaps may crystallize. Comparative biochemical studies have not yet been performed in human mesenteric panniculitis. Vitovic et al. 44 compared the bovine disease to human sclerema neonatorum and adiponecrosis cutis neonatorum. The etiology of mesenteric fibromatosis is still a matter of discussion and of much insecurity. Extra-abdominal fibromatosis has often be found following surgical trauma, other types of injury (e.g. burn scar, fracture, gunshot wound) or irradiation 11. Enzinger and Weiss 11 describe a case of mesenteric fibromatosis one year after surgical removal of a retroperitoneal liposarcoma. Fibro(mato)sis in our case 4 may have been induced by injury of the mesentery due to puncture of ascites, possibly enhanced by irritating substances in the ascitic fluidS. Moreover, hormonal and hereditary factors have been discussed as possible etiologic agents in other than mesenteric fibromatoses l1 ,19. (5) Clinical findings in primary mesenteritis: The clinical complaints in primary mesenteritis are absolutely nonspecific. The most frequent presentation is with no symptoms 24 • A number of patients suffers from abdominal pain and fullness, weight loss, back pain, nausea and other symptoms4, 9, 24. If fibrosis develops or if the lesion extends to the retroperitoneal space, obstruction of viscera and vessels may occur. An interesting symptom rarely described is the transmission of aortic pulsations (with or without aneurysm) to the anterior abdominal wallIS, 17,25,30. It was also present in our case 3. The physician who first saw the patient assumed an aortic aneurysm and therefore referred the patient to the hospital. (6) Associated diseases: A number of cases has been described presenting mesenteric panniculitis and malignant lymphoma. According to Kipfer et al. 6, malignant lymphoma was present in 15% of their 53 cases with mesenteric panniculitis, a percentage by far exceeding the expected incidence. Similar cases have been reported by other authors l8, 30. The true nature of this unusual association remains obscure so far. One might speculate that both lesions are due to an unknown immunological deficiency. The association of mesenteric and retroperitoneal fibrosis

has been already mentioned above. The occurrence of mesenteric fibromatosis (and fibrosis?) in familial colonic adenomatosis is a prominent feature of Gardner's syndrome. Patients with mesenteric fibro(mato)sis should therefore be generally examined towards the possible coexistence of colonic adenomatosis, particularly if they are younger than 40 years. Indeed, most cases of mesenteric fibrosis and fibromatosis in Gardner's syndrome follow surgical resection of the diseased portion of the bowel, but in a smaller number of cases the mesenteric lesion may occur before the onset of polyposis and before any surgical intervention 11 • As a matter of fact, colonoscopic follow-up examinations should be performed particularly in young patients with mesenteric fibrosis/fibromatosis if adenomas have not been found by the first X-ray or colonoscopic examination after the mesenteric lesion has been established.

(7) Pathological differential diagnosis of mesenteric panniculitis: Differential diagnosis of the acute stage concerns mesenteric lipomatosis and Whipple's disease. In our case 1, frozen section diagnosis performed during laparotomy was mesenteric lipomatosis since the surgical biopsy only consisted of normal adipose tissue. The proper final diagnosis was made only after additional tissue had been removed. A sufficient amount of tissue should therefore be removed by the surgeon in each case in order to enable a correct pathological diagnosis. Whipple's disease may easily be distinguished from mesenteric panniculitis by use of PAS staining. In our case 1, another pathologist had discussed Whipple's disease probably because PAS staining had not been performed. Whipple's disease is less probable if the foam cells lie within the adipose tissue instead of the lymph nodes which are primarily concerned in Whipple's disease. The distinction of mesenteric fibrosis or fibromatosis from mesenteric fibroma usually offers no problems since fibromas are well-circumscribed tumors lacking the illdefined margins and the invasion of the adipose tissue by strands of fibrous connective tissue as observed in fibrosis and fibromatosis. 8 Treatment and prognosis of mesenteritis and mesenteric fibrotic lesions: Treatment of primary mesenteric panniculitis has been attempted by surgical intervention, different drugs (antibiotics, steroids, emetine, azathioprine) and by irradiation4,9,24. Since spontaneous regression of the acute early stage of mesenteritis is quite common, the success of treatment is difficult to assess. In two patients described by Durst et a1. 9, steroids proved to be of help. Tytgat et al. 43 observed a dramatic improvement in a patient with severe retractile mesenteritis following the administration of prednisone and azathioprine. Our third patient had been treated for several years with cortisone and azathioprine because of co-existing liver cirrhosis. We do not know if this treatment was successful in the patient's mesenteric lesion since its early stages are unknown. After all, the mesenteric lesion of this patient was characterized by fibrosis with only slight chronic inflammation. In view of the long-standing treatment with prednisone it is unclear if panniculitis had preceded mesenteric fibro(mato)sis.

84 . W. Remmele, H. Miiller-Lobeck and W. Paulus

Surgical intervention should be restricted to those cases in whom spontaneous regression of mesenteritis does not occur and the patient suffers from complaints caused by encasement of vessels or viscera by the fibrotic process. Fatal outcome is an extremely rare event. Only few cases have been reported? and they all belong to the group of mesenteritis with unusual features such as angiitis and pronounced neutrophilic infiltration of the adipose tissue!' 38. In one case, death occurred from cardiogenic shock 12 hrs following laparotomy!, In the second patient, progressive mesenteric fibrosis led to fatal narrowing of the mesenteric veins 38 • Prognosis may further be influenced by associated diseases, particularly by malignant lymphoma.

References 1 Andersen JA, Rasmussen NR, Pedersen JK (1982) Mesenteric panniculitis: a fatal case. Am J Gastroenterol 77: 523-525 2 Bendon JA, Poleynard GD, Bordin GM (1979) Fibrosing mesenteritis simulating pelvic carcinomatosis. Gastrointest Radiol4: 195-197 3 Brown P, Baddeley H, Read AE, Davies JD, McGarry J (1974) Sclerosing peritonitis, an unusual reaction to a ~-adrener­ gic-blocking drug (practolol). Lancet II: 1477-1481 4 Biiltmann B, Merkle P, Walter G, Haferkamp (1981) Die sklerosierende Mesenteritis. Bericht iiber drei Patienten und Literaturiibersicht. Z Gastroenterologie 19: 655-662 S Buhac I (1981) Biopsy of the peritoneum in decompensated cirrhosis of the liver. Am J Gastroenterol 76: 32-34 6 Crane JT, Aguilar MJ, Grimes OF (1955) Isolated lipodystrOfhy, a form of mesenteric tumor. Am J Surg 90: 169-179 DeCastro JA, Calem WS, Papadakis L (1967) Liposclerotic mesenteritis. Arch Surg 94: 26-29 8 Dolle W (1972) Die retroperitoneale Fibrose (Ormond'sche Krankheit). Erg Inn Med Kinderheilk 32: 1-30 9 Durst AL, Freund H, Rosenmann E, Birnbaum D (1977) Mesenteric panniculitis: Review of the literature and presentation of cases. Surgery 81: 203-211 10 Eltringham WK, Espiner HJ, Windsor CWO, Griffiths DA et al. (1977) Sclerosing peritonitis due to practolol: a report on 9 cases and their surgical management. Br J Surg 64: 229-235 11 Enzinger FM, Weiss SW (1983) Soft Tissue Tumors. Mosby, St. Louis-Toronto-London 12 Erskine JM (1964) Idiopathic fibrosis of the small bowel mesentery (primary lip'osclerosis). Am J Surg 180: 57-63 13 Flex G (1965) Uber Peritonitis fibroplastica incapsulata. Zbl Chir 90: 156-163 14 French BE, Bale GF, Winborn WB (1966) Lipodystrophy of mesenteric fat. Surg Gynecol Obstet 142: 1046-1052 IS Gaylis H, Isaacson C, Decker GAG (1973) Mesenteric panniculitis presenting as abdominal aortic aneurysm. Surgery 74: 626-630 16 Godquin B, Saout R, Loupias B, Blanchard J (1971) Pseudotumeur inflammatoire et necrotique du mesentere. Nosologie des panniculites mesenteriques. J Chir (Paris) 104: 167-176 17 Grossman LA, Kaplan HJ, Preuss HJ, HerringtonJL (1963) Mesenteric manifestation of Weber-Christian disease. JAMA 183: 318 18 Guldholt I (1981) Lipodystrophia mesenterialis. Ugeskr Laeger 143: 421

°

19 Hajdu SI (1979) Pathology of Soft Tissue Tumors. Lea & Febiger, Philadelphia 20 Harry RF (1978) Sclerosing peritonitis and propranolol. Arch Intern Med 138: 1424-1426 21 Hoflund S, Holmberg J, Nihlen H (1953) On the aetiology, pathogenesis and clinical picture of fat necrosis in cattle. Proc. 15th Internatl Veterinary Congr, vol 1, p 632 (cited by Soergel and Hensley) 22 Jura V (1924) Sulla mesenterite retrattile e slerosante. Policlinico (Prat) 31: 575 (cited by Tytgat et al.) 23 Kim D-H, Goldsmith HS, Quan SH, Huvos AG (1971) Intraabdominal desmoid tumor. Cancer 27: 1041-1045 24 Kipfer RE, Moertel CG, Dahlin DC (1974) Mesenteric lipodystrophy. Ann Intern Med 80: 582-588 2S Lawrence MS, Stanford W, Sheets RF (1966) Retractile mesenteritis with transmitted pulsations. AmJ Med 41: 847-848 26 Leger F, Roberti A, Deloche de Noyelle A, Lemaigre G (1967) Panniculite mesenterique variete de fibro-sclerose retroperitoneale. Presse med 75: 75-79 27 Mackenzie DH (1970) The Differential Diagnosis of Fibroblastic Disorders. Blackwell, Oxford-Edinburgh 28 Marshall AJ, Baddeley H, Barritt DW, Davies JD, Lee REJ, Low-Beer TS, Read AE (1977) Practolol peritonitis, A study of 16 cases and a survey of small bowel function in patients taking ~ adrenergic blockers. Quart J Med, NS, 181: 135-149 29 Mitchinson MJ (1965) Systemic idiopathic fibrosis and systemic Weber-Christian disease. J clin Path 18: 645-648 30 Ogden WW, Bradburn DM, Rives JD (1965) Mesenteric panniculitis: review of 27 cases. Ann Surg 161: 864-873 31 Pallette EM, Pallette EC, Harrington RW (1967) Sclerosing lipogranulomatosis: its several abdominal syndromes. Arch Surg 94: 803-810 32 Remmele W (1984) Peritonitis, Mesenteritis. In: Remmele W (Ed) Pathologie, vol 2, 548-560. Springer, Berlin-Heidelberg-New York-Tokyo 33 Richards RC, Rogers SW, Gardner EJ (1981) Spontaneous mesenteric fibromatosis in Gardner's syndrome. Cancer 47: 597-601 34 Roberts FM, Nielson OF (1964) Retractile mesenteritis. Am J Digest Dis 9: 231-235 3S Rogers CE, Demetrakopoulos MS, Hyamns V (1961) Isolated lipodystrophy affecting the mesentery, the retroperitoneal area and the small intestine. Ann Surg 153: 277-282 36 Simpson RD, Harrison EG Jr, Mayo CW (1964) Mesenteric fibromatosis in familial polyposis. A variant of Gardner's syndrome. Cancer 17: 526-534 37 Sieck JO, Cowgill R, Larkworthy W (1983) Peritoneal encapsulation and abdominal cocoon. Case reports and a review of the literature. Gastroenterology 84: 1597-1601 38 Soergel KH, Hensley GT (1966) Fatal mesenteric panniculitis. Gastroenterology 51: 529-536 39 Soumerai S, Kirkland WG, McDonnell WV, Schantz A (1976) Nodular mesenteritis: report of a case simulating carcinoma of the sigmoid colon and analysis of its histologic profile. Dis Col Rect 19: 448-452 40 Spark RB, Yakovac WC, Wagget J (1971) Retractile sclerosin~ mesenteritis. Clin Pediat (Philadelphia) 10: 119-122 1 Steinberg MD (1953) Systemic nodular panniculitis. Am J Pathol29: 1059-1081 42 Tedeschi CG, Botta GC (1962) Retractile mesenteritis. N EnO J Med 266: 1035-1040 Tytgat GN, Roozendaal K, Winter W, Esseveld MR (1980) Successful treatment of a patient with retractile mesenteritis with prednisone and azathioprine. Gastroenterology 79: 352-356 44 Vitovec J, Proks C, Valvoda V (1975) Lipomatosis (fat necrosis) in cattle and pigs. J Comp Pathol 85: 53-59

Mesenteritis and Mesenteric Fibromatosis . 85 45 Wagenknecht LV (1978) Retroperitoneale Fibrosen. Symptomatik·Diagnostik-Therapie-Prognose. Thieme, Stuttgart 46 Weeks LE, Block MA, Hathaway JC, Rinaldo JA (1963) Lipogranuloma of mesentery producing abdominal mass. Arch Surg 86: 615-620

47 Yannopoulos K, Stout AP (1963) Primary solid tumors of the mesentery. Cancer 16: 914-927

Received October 8, 1987 . Accepted March 7, 1988

Key Words: Mesenteritis - Mesenteric fibrosis - Mesenteric fibromatosis - Weber-Christian disenase - Panniculitis Professor Dr. W. Remmele, Institut fur Pathologie, Ludwig-Erhard-StraBe 100, D-6200 Wiesbaden, FRG