Aggressive Multiple MyelomaPresenting as Mesenteric PanniculitisJason Goh, Brian Otridge, Hugh Brady, Eamann Breatnach, Peter Dervan, andPadraic MacMathuna, M.D., F.R.C.P.I.Departments of Gastroenterology, Hematology, Nephrology, Radiology, and Histopathology, MaterMisericordiae Hospital and University College, Dublin, Ireland

ABSTRACTMesenteric panniculitis is a rare disease of the bowel mes-entery, characterized by tumor-like infiltration by chronicinflammatory cells, fat necrosis, and fibrosis. Reported casescited clinical presentation ranging from abdominal pain tofever of unknown origin, the majority of which were idio-pathic and associated with a benign prognosis. We report thecase of a 43-yr-old male who presented with malaise, weightloss, microcytic anemia, and a high erythrocyte sedimenta-tion rate. Radiographic and histological investigations re-vealed typical features of mesenteric panniculitis. Initialtreatment with high-dose oral prednisolone led to rapid andcomplete resolution of symptomatology, radiographic, andlaboratory anomalies. Within 6 months, the patient pre-sented again with anemia, renal failure, and hypercalcemia.A diagnosis of IgA kappa chain myeloma was made. De-spite chemotherapy and restoration of normocalcemia, hedied from refractory pulmonary edema. This is the firstreport of a hematological malignancy initially presentingwith features of mesenteric panniculitis culminating in anaggressive course and a fatal outcome. (Am J Gastroenterol2001;96:238–241. © 2001 by Am. Coll. of Gastroenterol-ogy)

INTRODUCTION

Mesenteric panniculitis (MP) is a rare condition character-ized by tumor-like infiltration of the bowel mesentery bychronic inflammatory cells, fat necrosis, and fibrosis (1–3).Since its initial description by Odgenet al. in 1965, confu-sion has arisen with regard to the nomenclature of a clusterof related conditions (namely sclerosing mesenteritis, re-tractile mesenteritis, and mesenteric lipodystrophy) andwhether they represent a single disease entity or differentends of a spectrum (2, 4).

Common clinical presentations include abdominal pain,abdominal mass, and intestinal obstruction, but a third ofreported cases have no intra-abdominal manifestations (1, 2,5). Apart from anecdotal accounts of associations, such asidiopathic peritoneal fibrosis, Weber-Christian disease, hu-man immunodeficiency viral infection, and chronic lympho-cytic leukemia, the vast majority of cases of MP were

idiopathic and occurred in isolation (4–8). Treatment op-tions included immunosuppression with steroids, cyclo-phosphamide, or cyclosporine, and the majority had a be-nign outcome (4, 9, 10).

We present the case of a 43-yr-old man with clinical,radiographic, and histological features of mesenteric pan-niculitis, who responded initially to oral prednisolone. Itsoon emerged that MP was the early presenting feature of anunderlying, aggressive, and ultimately fatal IgA kappa chainmyeloma. This is the first reported case of myeloma pre-senting as mesenteric panniculitis.

CASE REPORT

A 43-yr-old, previously healthy, white male presented withmalaise and weight loss of 2 kg over the preceding two-month period. Systems review was negative, and in partic-ular, there were no symptoms related to the gastrointestinaltract. Clinical examination revealed generalized pallor, mildascites, and evidence of recent weight loss. Abnormal lab-oratory investigations included the following: 1) erythrocytesedimentation rate (ESR) of 160 mm in the first hour, 2)hypochromic microcytic anemia of 7.2 g/dl (normal range13–18), 3) serum iron of 2mmol/L (normal 6–27), 4) serumalbumin of 20 g/L (normal range 34–50), and 5) serumcreatinine of 168mmol/L (normal range 55–118). Ultra-sononographic study of the abdomen revealed ascites. Initialsuspicion of iron deficiency led to a comprehensive gastro-intestinal work-up for occult blood loss. Repeated fecalhemocult tests, gastroscopy, small bowel biopsies, enteros-copy, small bowel barium study, colonoscopy, mesentericangiogram, and Meckle’s isotope scan failed to identify ableeding source.

Abdominal CT scan showed small bilateral pleural effu-sions, ascites, and grossly abnormal mesentery in theperipancreatic areas, with lymph nodes and mesentericthickening and nonhomogeneous areas of fat interspersedwith soft tissue density—highly suspicious for lymphoma(Fig. 1). An exploratory laparotomy was undertaken on thebasis of the grossly abnormal CT finding. At laparotomy, aninfiltrating mass was seen to be involving the head ofpancreas, gallbladder, and some omentum. There was ex-

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tensive involvement of the retroperitoneum but no lymph-adenopathy was noted. Histology of the mesenteric tissuesampled from the duodenojejunal flexure showed widenededematous septa within the adipose tissue (Fig. 2). The septashowed a moderately dense inflammatory infiltrate withprominent vascularity and fibroblast proliferation. No gran-uloma or malignancy was seen. The appearance was con-sistent with an early inflammatory phase of sclerosing mes-enteritis/mesenteric panniculitis.

Treatment consisted of blood transfusion and oral pred-nisolone starting at a dose of 60 mg daily. This was rapidlyfollowed by return of energy, weight gain, and normaliza-tion of ESR. Most importantly, the previous radiographicabnormalities of the mesentery had completely resolved ona repeat abdominal CT scan performed 2 months later (notshown).

Six months following treatment, the patient presentedagain with profound malaise and arthralgia of both elbows,despite receiving 20 mg of prednisolone. Clinical findingsincluded pallor and peripheral edema. Laboratory investi-

gations revealed a profound microcytic anemia of 5 g/dl,ESR 120 mm in the first hour, C-reactive protein 4 mg/dl(normal,0.6), rouleaux formation on blood film, hyperuri-cemia, renal impairment, and hypoalbuminemia of 17g/L.Nephrotic syndrome was diagnosed on the basis of edema,hypoalbuminemia and proteinuria of 2.23 g/24 h (normal,0.15 g/24 h). The patient became acutely confused with acorrected serum calcium of 4.21 mmol/L (normal range2.2–2.6 mmol/L). Normocalcemia was restored with a com-bination of fluid replacement, corticosteroids, diuresis, cal-citonin, and pamidronate.

Myeloma work-up revealedb2 microglobulin level of 21mg/L (normal 0–2.5), urinary Bence-Jones protein of thekappa type (3.8g/24 h). Serum immunoglobulin A (IgA)was elevated at 908 mg/dl (normal range 71–360), anddiffuse immunoparesis of the other immunoglobulins wasobserved. Serum protein electrophoresis confirmed a mono-clonal band of IgA heavy chain and kappa light chain withparaprotein level measuring 27 g/L. Bone marrow aspiraterevealed infiltration with plasma cells with blast compo-nents, and those cells were found to replace more than 50%of bone marrow on the trephine biopsy (Fig. 3). A percu-taneous renal biopsy showed cast nephropathy consistentwith “myeloma kidney.” Chemotherapy regime comprisingmethylprednisolone and melphalan was commenced. Unfor-tunately, the patient developed gross pulmonary edema re-fractory to intensive treatment, and he died 2 days later. Hisdeath was presumed to be related to cardiomyopathy as aresult of amyloid or myeloma light chain infiltration.

DISCUSSION

No more than 300 case reports of the spectrum of mesentericpathology encompassed by the terms mesenteric panniculi-tis/sclerosing mesenteritis/mesenteric lipodystrophy exist inthe literature (1, 3–5). The present case was classified as MPbecause of the predominance of inflammatory changes over

Figure 1. Abdominal CT scan showing infiltration of the mesenteryand perinephric region with gross mesenteric thickening and non-homogeneous areas of fat interspersed with soft tissue density.

Figure 2. Nonspecific mixed inflammatory infiltrate in mesentericfat (Eosin and Hematoxylin stain, 503 magnification).

Figure 3. Bone marrow aspirate stained with May GrunwaldGeimsa at 12503 magnification showing greater than 50% re-placement of marrow by medium to large cells with very immaturenuclei. The morphology of the cells indicates plasma cells.

239AJG – January, 2001 Multiple Myeloma Presenting as Mesenteric Panniculitis

fat necrosis and fibrosis. Recent excellent comprehensivereviews are available (4–6).

Up to a third of patients had no abdominal symptoms, asin our patient. Because the findings of hypochromic micro-cytic anemia with low serum iron, grossly elevated ESR,and hypoalbuminemia were not symptoms characteristic ofMP, occult gastrointestinal neoplasm was the initial suspi-cion, given the background of weight loss and the malegender. This was excluded upon comprehensive gastroin-testinal work-up. The grossly abnormal appearance of mes-entery on CT led to the diagnosis of MP based on classicalfindings on laparotomy and histology. In a recent retrospec-tive review of 78 cases (on whom data on clinical presen-tation were available) by Emory and colleagues, the mostcommon presenting symptoms were abdominal pain(34.6%), abdominal mass (30.8%), and intestinal obstruc-tion (30.8%) (4). Other presenting features included fever ofunknown origin, ascites, pleural effusion, fatigue, andweight loss (4, 11). The most useful preoperative imagingtechniques are CT and magnetic resonance imaging of theabdomen where hypoechoic mass with fat areas can be seenat the root of the mesentery (11–14). Ultrasonography alonehas a low sensitivity as in our patient (12–14). Laparoscopyor laparotomy remain the mainstay of definitive tissue di-agnosis.

The rapid response to high-dose oral steroids, resulting inalmost complete resolution of symptomatology, laboratoryinflammatory markers, and radiographic abnormalities,provided false reassurance to both clinicians and patient.Indeed, symptomatic response in patients with MP to im-munosuppression using steroids, azathioprine, cyclophos-phamide, and cyclosporine have been well documented(9–11, 15, 16). Surgical resection appears to be of valueonly where features of bowel obstruction are present (15).However, the return of profound anemia and malaise, cou-pled with evidence of hyperuricemia, nephrotic syndrome,renal failure and hypercalcemia, strongly suggested an un-derlying malignant myelomatous process. The eventualcourse proved rapidly fatal despite chemotherapy and cor-rection of hypercalcemia.

An association between MP and hematological malig-nancy is rare. The closest report was MP occurring in apatient with a history of chronic lymphocytic leukemia(CLL) associated with vincristine toxicity occurring 9 yrpreviously (4). Such an association was remote at best,given the temporal relationship between CLL and MP in thecase cited. Only 2 cases of MP occurring in Hodgkin’sdisease and large cell lymphoma, respectively, have beenreported (4). The fibrosis occurring with lymphoma must bedistinguished from MP.

The differential diagnosis of an intra-abdominal benigninflammatory pseudotumor (plasma cell granuloma) is im-portant in so far as the latter can present with anemia andgrossly elevated erythrocyte sedimentation rate (17). How-ever, the expected plasma cell infiltrate and fibrosis wereabsent in our patient.

In retrospect, we speculate that a more aggressive initialevaluation might have disclosed the multiple myeloma attime of his presentation with mesenteric panniculitis. How-ever, the combination of weight loss and microcytic anemiain a middle-aged man prompted an exhaustive search for anoccult gastrointestinal neoplasm. The emergence of radio-logical and histological evidence of MP did not further raisethe possibility of an underlying myeloma.

The institution of steroid therapy would have providedtransient symptomatic relief for multiple myeloma. It isunclear if earlier detection and initiation of chemotherapy atinitial presentation would have altered the outcome, giventhe aggressive nature of the myeloma.

Most cases of mesenteric panniculitis occurde novo,andoutcome varies between spontaneous resolution, response toimmunosuppression and persistent symptoms. This is thefirst report of mesenteric panniculitis presenting as an earlyfeature of an aggressive IgA myeloma. Unexplained ane-mia, grossly elevated ESR, and hypoalbuminemia are notcompatible with a benign outcome in mesenteric pannicu-litis and should alert the clinician to seek an alternative,perhaps more sinister, explanation.

Reprint requests and correspondence:Padraic MacMathunaM.D., F.R.C.P.I., Department of Gastroenterology, GI Unit, MaterMisericordiae Hospital, Eccles’s Street, Dublin 7, Ireland.

Received Jan. 12, 1999; accepted Apr. 1, 1999.

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