2009) 240–243

Clinical Imaging 33 (

Extramedullary plasmacytoma of the pancreas and jejunum

Pramod Gupta⁎, Gregg D. Rice, KiAnne Abraham, Vidisha Ghole, Manoj Ketkar

Radiology Service, Dallas VA Medical Center, VA North Texas health care system, 4500, South Lancaster Road, Dallas, TX 75216, USA

Received 15 October 2008; accepted 1 December 2008

Abstract

Plasmacytomas occurring in the gastrointestinal tract are extremely rare. We report one such case of pancreatic and jejunal plasmacytomain a single patient with known multiple myeloma with emphasis on computed tomographic (CT) findings.Published by Elsevier Inc.

Keywords: Extramedullary plasmacytoma; Plasmacytoma

1. Introduction

Plasma cell neoplasms may present as multiple myeloma,solitary myeloma of the bone, plasma cell leukemia, or asextramedullary plasmacytoma (EMP) [1,2]. Extramedullaryplasmacytoma of the pancreas and gastrointestinal tract,either isolated or in association with multiple myeloma, isextremely rare [3–6]. We report a very rare case of pancreaticand jejunal plasmacytoma in a single patient with knownmultiple myeloma with emphasis on computed tomographic(CT) findings. Such concurrence has not been reportedbefore in radiology literature and, to the best of ourknowledge, CT imaging features of jejunal involvementhave not been described before.

2. Case report

A 59-year-old white male patient with light chainmyeloma (IgA-K type) diagnosed by fine-needle aspirationof sternal mass 1 year ago was admitted with a rapid increasein the size of this mass and excruciating pain for radiationtreatment and pain management. Multiple myeloma surveyradiographs done at the time of initial diagnosis had also

⁎ Corresponding author. 2704, Oates Drive, Plano, TX 75093. Tel.: +1 214857 0185; fax: +1 775 855 4624.

E-mail address: pramodvagisha@hotmail.com (P. Gupta).

0899-7071/09/$ – see front matter. Published by Elsevier Inc.doi:10.1016/j.clinimag.2008.12.006

demonstrated additional bone involvement with lytic lesionsin bilateral distal clavicles, right iliac bone, and rightproximal femur. Serum and urine protein electrophoresisresults were normal; however, he had elevated kappa/lambdaratio in the serum. The patient had refused bone marrowbiopsy due to his inability to lie flat. His serum bilirubin andliver function test results were normal at the time of initialdiagnosis. He was treated with thalidomide and dexametha-sone and did well for 6–7 months with decrease in size of thesternal mass. However, during the last few months beforethis admission he noticed a regrowth of the sternal mass withincreasing pain. Thalidomide was substituted with lenalido-mide a few weeks prior with little response. At the time ofadmission he showed further increase in serum kappa/lambda ratio. Serum total bilirubin was normal (0.7 mg/dl;normal range 0–1 mg/dl) and liver function test results werealso normal. He did not do well during the hospitalizationand after 1 month he was noticed to have jaundice withmarkedly elevated serum total bilirubin of 17.0 mg/dl andalso elevated liver function test results. Ultrasound wasperformed which showed a lobulated hypoechoic avascularmass in the region of the pancreatic head with mild throughtransmission of ultrasound beam (Fig. 1). Dilated commonbile duct and intrahepatic biliary ducts were also noted. TheCT scan showed a homogeneous isodense 4×4-cm mass inthe pancreatic head with enhancement similar to pancreaticparenchyma with mildly dilated pancreatic duct and

Fig. 2. Transverse CT scan images at the level of the pancreatic head without (A) andpancreatic head (arrows) with contrast enhancement similar to the rest of the pancdilatation (arrowheads in C) and biliary ductal dilatation (arrowheads in D), respe

Fig. 1. Transverse ultrasound image of the pancreas shows a hypoechoicmass in the region of the head (arrows) with mild through transmission ofultrasound beam.

241P. Gupta et al. / Clinical Imaging 33 (2009) 240–243

moderately dilated biliary ducts (Fig. 2). The differentialdiagnosis included plasmacytoma as the patient had multiplemyeloma, primary adenocarcinoma, metastasis, and lym-phoma. Incidentally, it was also found that there wasmultilobulated eccentric nodular wall thickening of anapproximately 6-cm-long segment of the mid jejunum withpoor contrast enhancement (Fig. 3). There was no evidenceof bowel obstruction or abdominal lymphadenopathy. Thedifferential diagnoses for the jejunal lesion considered wereplasmacytoma, gastrointestinal stromal tumor, primaryadenocarcinoma, lymphoma, and metastases. Endoscopicultrasound demonstrated a mass in the pancreatic headencasing the common bile duct and invading the portal vein.The fine-needle aspiration showed multiple sheets ofatypical plasma cells. The patient was considered inoperable.An ERCP was attempted to place a biliary stent for palliativetreatment, but it was unsuccessful. An external–internalbiliary drainage catheter was placed via a percutaneousapproach. His condition kept deteriorating and 4 weeks laterhe died of respiratory and cardiac failure.

with intravenous contrast (B) show homogeneous isodense solid mass in thereas. Slightly higher contrast images (C and D) show mild pancreatic ductalctively.

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3. Discussion

Plasma cell neoplasms may present as multiple myeloma,solitary myeloma of the bone, plasma cell leukemia, or asEMP [1,2]. Extramedullary plasmacytomas are seen and canarise anywhere in the body, although the vast majority of

Fig. 3. Transverse CT scan images of the mid abdomen without (A and B)and with intravenous contrast (C) show multilobular eccentric wallthickening of the mid jejunal loop on the left side of the abdomen (arrows)with no significant contrast enhancement.

cases occur in the head and neck with 80% occurring in thenasal cavity, paranasal sinuses, and upper airways. Involve-ment of the gastrointestinal tract is reported in approximately10% of cases, most of which involve the liver and spleen[3–6]. Very few cases of pancreatic and small bowelinvolvement have been reported. It can occur as primaryisolated form or as secondary manifestation of systemicdisease. Pancreatic and jejunal involvement in the samepatient has never been described before.

Most cases of plasma cell infiltrates of the pancreas aremicroscopic. Well-formed masses are unusual. It maypresent as focal mass [1,5,6] or as diffuse enlargement ofthe pancreas [7,8] with or without biliary obstruction. Onultrasound, pancreatic plasmacytoma appears as a multi-lobulated heterogeneous hypoechoic mass [3,5,6]. The CTfeatures have been described as a focal multilobulated solidhypodense mass with homogeneous intravenous contrastenhancement [1,5,6] or more rarely as diffuse enlargementof the pancreas [7,8]. In our case, enhancement was similarto the rest of the pancreas. Without pathological confirma-tion, focal mass cannot be reliably differentiated from otherpancreatic neoplasms including primary adenocarcinoma,lymphoma, and metastasis. MRI features have beenreported only in two patients which included pancreaticenlargement with lobulated contour, signal intensity lessthan that of the liver on T1, and diffuse increased signalintensity on T2-weighted pulse sequences with heteroge-neous enhancement [7,9].

In the gastrointestinal tract, the small bowel is mostcommonly involved, followed by the stomach, colon, andesophagus [2,4,10]. In the small bowel, the most commonsite of involvement is the ileum [10]. Jejunal involvementhas not been described before in concurrence with pancreaticinvolvement. On barium studies, imaging features of smallbowel involvement simulate that of primary carcinoma orlymphoma [10,11]. There are isolated case reports of patientspresenting with small bowel obstruction and one case withileocolic fistula formation [12]. There are only a few casesreports describing the CT appearance of the small bowelinvolvement, as most of the cases reported were before 1980.The gastric plasmacytoma has been described on CT as amarked thickening of the gastric wall with poor contrastenhancement, impossible to distinguish from gastric adeno-carcinoma or lymphoma [4]. Jejunal involvement has beenreported in the pathological literature; however, to the best ofour knowledge, the CT appearance of jejunal involvementhas not been reported before. In our case, on CT scan anapproximately 6-cm-long segment of the mid jejunum wasseen involved with multilobulated eccentric nodular wallthickening with poor contrast enhancement, an appearancewhich is nonspecific and can be seen with gastrointestinalstromal tumor, primary adenocarcinoma, lymphoma, andmetastases. There was no bowel obstruction or abdominallymphadenopathy in our case. EMPs are usually associatedwith the more aggressive anaplastic form of the disease andhave poor prognosis [3].

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In conclusion, we present a very rare case of pancreaticand jejunal plasmacytoma in a patient with multiplemyeloma and describe the CT imaging features. Myeloma-tous involvement of the pancreas and gastrointestinal tractis well documented in the pathological literature, butreports of imaging are very few. Radiologist should beaware of this occurrence, particularly when there is historyof systemic disease. It may help avoid extensive unneces-sary interventions.

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