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517

Case Report

MR Imaging Alan J. Kronthal,1

Sclerosing spectrum

in Sclerosing

Young

S. Kang,

mesenteritis of diseases

mesentery.

Elliot K. Fishman,

is a mare entity causing

Mesenteritis Bronwyn

that lies within

fibrofatty

thickening

We present a case in which characteristic

a

of the

findings

were noted on plain film, small-bowel series, and CT. MR imaging proved helpful in further evaluating the nature of the lesion and delineating vascular involvement. To our knowl-

edge, this is the first description ance of sclerosing

Case

of the MR imaging

appear-

mesenteritis.

Report

A 64-year-old man presented with a long history of abdominal pain and weight loss. CT (Fig. 1A) showed a mesenteric mass of softtissue density that contained calcifications and had a surrounding pattern of radiating spicules. Biopsy of the mass yielded a diagnosis of sclerosing mesenteritis, and the patient was treated conservatively. One year later, CT showed slight enlargement of the mass, and MR imaging

(Signa,

General

Electric,

Milwaukee,

WI,

operating

at 1 .5 T;

Figs. 1 B-i E) was performed to evaluate vascular involvement. The mass had intermediate signal intensity on Ti -weighted images, with scattered foci of lower signal corresponding to areas of calcification. On T2-weighted images, the mass was oflow signal, most compatible with

fibrosis.

Gradient-echo

images

revealed

that

a jejunal

branch

of

the superior mesentenc artery ended abruptly as it entered the mass. Flow within the superior mesentenc vein could not be seen along most of its course, and multiple collateral vessels were seen. Dilated small-bowel loops also were noted, compatible with a partial smallbowel obstruction. Small-bowel series showed angling and kinking of small-bowel loops in the left lower quadrant and luminal narrowing, separation of loops, and irregular fold thickening. Exploratory laparotomy revealed a large hard mass at the mesentenc root with involvement

of the superior

mesentenc

Janet

small-bowel

loops.

Jejunoileal

bypass

M.

and biopsy

C.

Tempany

of the mass

were

Discussion Sclerosing mesententis is one entity within a spectrum of mare diseases that are characterized by fibmofatty thickening of the small-bowel mesentery [1]. The specific diagnosis usually depends on which of the three major pathologic features predominate: fatty degeneration or lipodystrophy, inflammation or panniculitis, or fibrosis or sclerosing (retractile) mesenteritis [2]. The entity has also been called liposclerotic

mesententis and mesentenc Weber-Christian disease [3]. The specific cause of these changes is unknown, although various theories such as ischemia of the mesentery or an autoimmune response have been proposed [3, 4]. Whatever the cause, the final stage appears to be a series of inflammatory changes affecting the mesentery [5]. Subsequent fat necrosis is thought to occur, resulting in intense reactive fibrosis with progressive scarring and retraction. The disease has been described in patients from ages 8 to 80, but it is most common in middle-aged men [1]. Histologically, the lesions are composed of fibrous tissue, lipophages, lymphocytes, plasma cells, and eosinophils [5].

Calcifications

are frequently present, probably as a result of 1. The lesion involves the mesenteric and subfat of the small bowel, often with extension into the

fat necrosis

mucosal

[1

bowel

muscle

and

September 1 0, 1990. of Radiology and Radiological

Science,

The Johns

Hopkins

March 1991 0361-803x/91/1563-051

and Clare

performed, confirming a diagnosis of sclerosing mesenteritis. The patient’s symptoms improved slightly, and no further treatment is planned at this time.

MD 21 205. Address reprint requests to E. K. Fishman. AJR 156:517-519,

E. Kuhlman,

and vein and several

artery

Received July 27, 1 990; accepted after revision , All authors: The Russell H. Morgan Department

Jones,

7 © American Roentgen Ray Society

submucosa.

Medical

The

Institutions,

mucosa,

however,

600 N. Wolfe

me-

St., Baltimore,

KRONTHAL

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518

ET AL.

AJR:156,

March

1991

Fig. 1.-A, Enhanced CT scan shows linear strands radiating from a mesenteric mass (arrow). Calcifications are present within mass. B, Ti-weighted MR image (600/20) shows a mesenteric mass (arrow) of intermediate signal intensity. C, Ti-weighted MR image at level 2 cm supenor to B shows multiple punctate areas of low signal (arrow) corresponding to calcifications.

Stellate radiating folds are also seen. D, T2-weighted

MR image

(2700/80)

shows

mass (arrow) to be of very low signal intensity. E, Gradient-echo MR image (22/13, flip angle = 30#{176}) shows a jejunal branch of superior mesenteric artery (arrow) ending abruptiy as it enters area of fibrosis.

A

B

mains intact [5]. The process can obstruct mesentemic lymphatics, resulting in submucosal edema and lymphangiecta-

sia. Mesenteric rowing [6].

arteries

and veins often display

Iuminal nam-

Abdominal pain is the most frequent symptom, companied by nausea, vomiting, malaise, weight

grade fever, diarrhea,

or constipation

is frequently

[5].

Radiologic tional

barium

Small-bowel

palpable

features studies

of sclerosing

often acloss, low-

[1 ]. An abdominal mesenteritis

on conven-

and CT have been previously

series often show separation

mass

described.

of loops with kink-

ing and angulation of the small bowel, suggesting process [5]. The folds may be thickened because

a serosal of either

extension of the mesenteric process into the submucosa or edema from lymphatic obstruction. The colon may occasionally be involved, with narrowing and rigidity; thumbprinting also has been reported [4]. CT shows a mesenteric mass containing a variable proportion of fat and soft tissue, with radiating linear strands; calcifications are occasionally present. Differential diagnosis includes carcinoid, desmoid, and mesenteric lymphoma or carcinomatosis. Carcinoid may evoke a

dense fibrotic tractile

reaction

mesenteritis.

within Elevated

the mesentery, urinary

simulating

5-hydmoxymndoleacetic

me-

acid

levels,

liver

metastases,

or multiplicity

of mesentemic

masses favors the diagnosis of carcinoid [7]. Desmoid tumoms, usually associated with Gardner syndrome, are tumorlike fibmomatoses that tend to occur in injured or surgically traumatized sites [6]. Differentiation from retractile mesentemitis can usually be made by studying the clinical history or finding associated colonic polyposis on gastrointestinal studies [8]. Thickened leaves radiating from the mesenteric moot

have been seen in breast ascites or tumor elsewhere [1 ]. Similarly,

lymphoma

carcinoma [7]. The absence of makes this diagnosis less likely

may involve

the mesentery,

but the

absence of metropemitoneal involvement or nodal involvement elsewhere is unusual. Except for treated lymphoma, the presence of calcifications, as occasionally seen in sclerosing mesenteritis,

would

be distinctly

unusual

in any

of these

pro-

cesses. The MR imaging appearance of sclemosing mesentemitis has not been described before. Two aspects of MR imaging make it a valuable technique for evaluation of this disease. The first is tissue characterization: fibrous tissue containing prepondemantly nonmobile protons with very short T2 has low signal on all sequences. Although masses of fibrous origin, depending on their fatty and vascular content, have recently been shown to exhibit a variety of signal characteristics, low signal

AJR:156,

March

MR OF SCLEROSING

1991

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should suggest mature fibrotic reaction (Quinn presented at the annual meeting of the American Ray Society, May 1990). Therefore, T2-weighted

MESENTERITIS

helpful in confirming a mesentemic cific for the tissue characteristics

SF et al., Roentgen or fat-sup-

appears

to be valuable

pressed pulse sequences may help distinguish benign endstage fibrofatty proliferation from malignant tumors such as lymphoma or metastases. Additional investigation is needed

this disease

to determine whether the fibrotic response to carcinoid shows similar signal characteristics. Second, noninvasive assessment of large to medium-sized vessels is possible with MR imaging. As in this case, routine gradient-echo images or Ti

REFERENCES

-

resection

is necessary

obstruction.

sclerosing treatment

mesenteritis is indicated.

only when complicated

Progressively

enlarging

lesions

has a Bowel

by mechanical and mesentemic

venous thrombosis, both of which were encountered in this case, are rare complications [1]. The few reported cases of severe and progressive disease have been successfully treated

with chemotherapeutic

agents

[3].

Sclerosing mesentemitis is a mare entity affecting the smallbowel mesentery. Although small-bowel series and CT are

2. Thompson sigmoid

GT, Fitzgerald

colon.

Br J Radiol

disease, they are not speof the lesion. MR imaging

for suggesting

and evaluating

vascular

1 . Clemett AR, Tacht DG. The roentgen Radiology 1969:107:787-790

weighted images with presaturation easily depict absence of flow within major mesentenc vessels and presence of collateral pathways. In the vast majority of cases, benign course, and no specific

519

the fibrous

nature of

involvement.

diagnosis

of retractile

EF, Somers 55. Retractile

mesenteritis.

mesenteritis

3. Bush RW, Mammar SP Jr, Rudolph RH. Sclerosing mesenteritis: to cyclophosphamide.

of the

1985:58:266-267

Arch Intern

Med

response

1986:146:503-505

4. Han SY, Koehler RE, Keller FS, Ho KJ, Zomes SL. Retractile mesenteritis involving the colon: pathologic and radiologic correlation. AJR 1986; 147:268-270 5. P#{233}rez-Font#{225}n FJ, Soler

A, Sanchez

J, Iglesias

P, Samjurjo

P. Ruiz

J.

Retractile mesenteritis involving the colon: barium enema, sonographic, and CT findings. AJR 1986:147:937-940 6. Kelly JK, Hwang W-S. Idiopathic retractile (sclerosing) mesenteritis and its differential diagnosis. Am J Surg Pathol 1989;13:513-521 7. Seigel AS, Kuhns LA, Boriaza GS, McCormick TL, Simmons JL. Computed tomography and angiography in ileal carcinoid tumor and retractile mesenteritis. Radiology 1980:134:437-440 8. Menuck LS. Abdominal desmoid masses in Gardner’s syndrome. Gastrointest Radiol 1976:1:81-84