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Extramedullary plasmacytoma of the pancreas and jejunum
Clinical Imaging 33 (2009) 240 – 243
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 plasmacytoma in 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 as extramedullary plasmacytoma (EMP) [1,2]. Extramedullary plasmacytoma of the pancreas and gastrointestinal tract, either isolated or in association with multiple myeloma, is extremely rare [3–6]. We report a very rare case of pancreatic and jejunal plasmacytoma in a single patient with known multiple myeloma with emphasis on computed tomographic (CT) findings. Such concurrence has not been reported before in radiology literature and, to the best of our knowledge, CT imaging features of jejunal involvement have not been described before. 2. Case report A 59-year-old white male patient with light chain myeloma (IgA-K type) diagnosed by fine-needle aspiration of sternal mass 1 year ago was admitted with a rapid increase in the size of this mass and excruciating pain for radiation treatment and pain management. Multiple myeloma survey radiographs done at the time of initial diagnosis had also ⁎ Corresponding author. 2704, Oates Drive, Plano, TX 75093. Tel.: +1 214 857 0185; fax: +1 775 855 4624. E-mail address: [email protected] (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 lesions in bilateral distal clavicles, right iliac bone, and right proximal femur. Serum and urine protein electrophoresis results were normal; however, he had elevated kappa/lambda ratio in the serum. The patient had refused bone marrow biopsy due to his inability to lie flat. His serum bilirubin and liver function test results were normal at the time of initial diagnosis. He was treated with thalidomide and dexamethasone and did well for 6–7 months with decrease in size of the sternal mass. However, during the last few months before this admission he noticed a regrowth of the sternal mass with increasing pain. Thalidomide was substituted with lenalidomide a few weeks prior with little response. At the time of admission 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 were also normal. He did not do well during the hospitalization and after 1 month he was noticed to have jaundice with markedly elevated serum total bilirubin of 17.0 mg/dl and also elevated liver function test results. Ultrasound was performed which showed a lobulated hypoechoic avascular mass in the region of the pancreatic head with mild through transmission of ultrasound beam (Fig. 1). Dilated common bile duct and intrahepatic biliary ducts were also noted. The CT scan showed a homogeneous isodense 4×4-cm mass in the pancreatic head with enhancement similar to pancreatic parenchyma with mildly dilated pancreatic duct and
P. Gupta et al. / Clinical Imaging 33 (2009) 240–243
Fig. 1. Transverse ultrasound image of the pancreas shows a hypoechoic mass in the region of the head (arrows) with mild through transmission of ultrasound beam.
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moderately dilated biliary ducts (Fig. 2). The differential diagnosis included plasmacytoma as the patient had multiple myeloma, primary adenocarcinoma, metastasis, and lymphoma. Incidentally, it was also found that there was multilobulated eccentric nodular wall thickening of an approximately 6-cm-long segment of the mid jejunum with poor contrast enhancement (Fig. 3). There was no evidence of bowel obstruction or abdominal lymphadenopathy. The differential diagnoses for the jejunal lesion considered were plasmacytoma, gastrointestinal stromal tumor, primary adenocarcinoma, lymphoma, and metastases. Endoscopic ultrasound demonstrated a mass in the pancreatic head encasing the common bile duct and invading the portal vein. The fine-needle aspiration showed multiple sheets of atypical plasma cells. The patient was considered inoperable. An ERCP was attempted to place a biliary stent for palliative treatment, but it was unsuccessful. An external–internal biliary drainage catheter was placed via a percutaneous approach. His condition kept deteriorating and 4 weeks later he died of respiratory and cardiac failure.
Fig. 2. Transverse CT scan images at the level of the pancreatic head without (A) and with intravenous contrast (B) show homogeneous isodense solid mass in the pancreatic head (arrows) with contrast enhancement similar to the rest of the pancreas. Slightly higher contrast images (C and D) show mild pancreatic ductal dilatation (arrowheads in C) and biliary ductal dilatation (arrowheads in D), respectively.
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P. Gupta et al. / Clinical Imaging 33 (2009) 240–243
3. Discussion Plasma cell neoplasms may present as multiple myeloma, solitary myeloma of the bone, plasma cell leukemia, or as EMP [1,2]. Extramedullary plasmacytomas are seen and can arise 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 wall thickening 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 the nasal cavity, paranasal sinuses, and upper airways. Involvement of the gastrointestinal tract is reported in approximately 10% of cases, most of which involve the liver and spleen [3–6]. Very few cases of pancreatic and small bowel involvement have been reported. It can occur as primary isolated form or as secondary manifestation of systemic disease. Pancreatic and jejunal involvement in the same patient has never been described before. Most cases of plasma cell infiltrates of the pancreas are microscopic. Well-formed masses are unusual. It may present as focal mass [1,5,6] or as diffuse enlargement of the pancreas [7,8] with or without biliary obstruction. On ultrasound, pancreatic plasmacytoma appears as a multilobulated heterogeneous hypoechoic mass [3,5,6]. The CT features have been described as a focal multilobulated solid hypodense mass with homogeneous intravenous contrast enhancement [1,5,6] or more rarely as diffuse enlargement of the pancreas [7,8]. In our case, enhancement was similar to the rest of the pancreas. Without pathological confirmation, focal mass cannot be reliably differentiated from other pancreatic neoplasms including primary adenocarcinoma, lymphoma, and metastasis. MRI features have been reported only in two patients which included pancreatic enlargement with lobulated contour, signal intensity less than that of the liver on T1, and diffuse increased signal intensity on T2-weighted pulse sequences with heterogeneous enhancement [7,9]. In the gastrointestinal tract, the small bowel is most commonly involved, followed by the stomach, colon, and esophagus [2,4,10]. In the small bowel, the most common site of involvement is the ileum [10]. Jejunal involvement has not been described before in concurrence with pancreatic involvement. On barium studies, imaging features of small bowel involvement simulate that of primary carcinoma or lymphoma [10,11]. There are isolated case reports of patients presenting with small bowel obstruction and one case with ileocolic fistula formation [12]. There are only a few cases reports describing the CT appearance of the small bowel involvement, as most of the cases reported were before 1980. The gastric plasmacytoma has been described on CT as a marked thickening of the gastric wall with poor contrast enhancement, impossible to distinguish from gastric adenocarcinoma or lymphoma [4]. Jejunal involvement has been reported in the pathological literature; however, to the best of our knowledge, the CT appearance of jejunal involvement has not been reported before. In our case, on CT scan an approximately 6-cm-long segment of the mid jejunum was seen involved with multilobulated eccentric nodular wall thickening with poor contrast enhancement, an appearance which is nonspecific and can be seen with gastrointestinal stromal tumor, primary adenocarcinoma, lymphoma, and metastases. There was no bowel obstruction or abdominal lymphadenopathy in our case. EMPs are usually associated with the more aggressive anaplastic form of the disease and have poor prognosis [3].
P. Gupta et al. / Clinical Imaging 33 (2009) 240–243
In conclusion, we present a very rare case of pancreatic and jejunal plasmacytoma in a patient with multiple myeloma and describe the CT imaging features. Myelomatous involvement of the pancreas and gastrointestinal tract is well documented in the pathological literature, but reports of imaging are very few. Radiologist should be aware of this occurrence, particularly when there is history of systemic disease. It may help avoid extensive unnecessary interventions. References [1] Hiller N, Goitein O, Ashkenazi YJ. Plasmacytoma of the pancreas. Isr Med Assoc J 2004;6(11):704–5. [2] Schoretsanitis G, Livingstone JI, el-Japour JN, Watkins N, Wastell C. Duodenal plasmacytoma: a rare extramedullary localization simulating carcinoma of the head of the pancreas. Postgrad Med J 1994;70(823): 378–9. [3] Patlas M, McCready D, Kulkarni S, Dill-Macky MJ. Spectrum of imaging findings in abdominal extraosseous myeloma. AJR Am J Roentgenol 2004;183(4):929–32.
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[4] Yoon SE, Ha HK, Lee YS, Kim PN, Lee MG, Yu E, Auh YH. Upper gastrointestinal series and CT findings of primary gastric plasmacytoma: report of two cases. AJR Am J Roentgenol 1999;173(5):1266–8. [5] Rice NT, Woodring JH, Mostowycz L, Purcell M. Pancreatic plasmacytoma: sonographic and computerized tomographic findings. J Clin Ultrasound 1981;9(1):46–8. [6] Fukuya T, Yoshimitsu K, Kitagawa S, Murakami J, Masuda K, Nakamura K. Plasmacytoma of the pancreatic head. Gastrointest Radiol 1989;14(3):226–8. [7] Pääkkö E, Autio R, Lähde S. Plasmacytoma of the pancreas: CT and MRI appearance. Rofo 1998;169(6):673–5. [8] Scheiman J, Elta G, Francis I. Biliary obstruction secondary to an extramedullary plasmacytoma of the pancreas: confusion with pancreatitis on computed tomography. Pancreas 1987;2(2):237–9. [9] Balliu E, Casas JD, Barluenga E, Guasch I. Multifocal involvement of the pancreas in multiple myeloma: sonographic, CT, and MR imaging findings. AJR Am J Roentgenol 2003;180(2):545–6. [10] Deodhare SG, Pujari BD, Apte PG, Gujar AG. Plasmacytomas of gastrointestinal tract. J Postgrad Med 1975;21(3):145–50. [11] Barzilai A, Toledano C, Arie JB, Gellei B. Plasmacytoma of the gastrointestinal tract. World J Surg 1977;2(1):267–73. [12] Fendel EH, Fazio VW. Extramedullary plasmacytoma of the small intestine: first case report of ileocolic fistula and review of the literature. Dis Colon Rectum 1981;24(8):633–5.
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 plasmacytoma in 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 as extramedullary plasmacytoma (EMP) [1,2]. Extramedullary plasmacytoma of the pancreas and gastrointestinal tract, either isolated or in association with multiple myeloma, is extremely rare [3–6]. We report a very rare case of pancreatic and jejunal plasmacytoma in a single patient with known multiple myeloma with emphasis on computed tomographic (CT) findings. Such concurrence has not been reported before in radiology literature and, to the best of our knowledge, CT imaging features of jejunal involvement have not been described before. 2. Case report A 59-year-old white male patient with light chain myeloma (IgA-K type) diagnosed by fine-needle aspiration of sternal mass 1 year ago was admitted with a rapid increase in the size of this mass and excruciating pain for radiation treatment and pain management. Multiple myeloma survey radiographs done at the time of initial diagnosis had also ⁎ Corresponding author. 2704, Oates Drive, Plano, TX 75093. Tel.: +1 214 857 0185; fax: +1 775 855 4624. E-mail address: [email protected] (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 lesions in bilateral distal clavicles, right iliac bone, and right proximal femur. Serum and urine protein electrophoresis results were normal; however, he had elevated kappa/lambda ratio in the serum. The patient had refused bone marrow biopsy due to his inability to lie flat. His serum bilirubin and liver function test results were normal at the time of initial diagnosis. He was treated with thalidomide and dexamethasone and did well for 6–7 months with decrease in size of the sternal mass. However, during the last few months before this admission he noticed a regrowth of the sternal mass with increasing pain. Thalidomide was substituted with lenalidomide a few weeks prior with little response. At the time of admission 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 were also normal. He did not do well during the hospitalization and after 1 month he was noticed to have jaundice with markedly elevated serum total bilirubin of 17.0 mg/dl and also elevated liver function test results. Ultrasound was performed which showed a lobulated hypoechoic avascular mass in the region of the pancreatic head with mild through transmission of ultrasound beam (Fig. 1). Dilated common bile duct and intrahepatic biliary ducts were also noted. The CT scan showed a homogeneous isodense 4×4-cm mass in the pancreatic head with enhancement similar to pancreatic parenchyma with mildly dilated pancreatic duct and
P. Gupta et al. / Clinical Imaging 33 (2009) 240–243
Fig. 1. Transverse ultrasound image of the pancreas shows a hypoechoic mass in the region of the head (arrows) with mild through transmission of ultrasound beam.
241
moderately dilated biliary ducts (Fig. 2). The differential diagnosis included plasmacytoma as the patient had multiple myeloma, primary adenocarcinoma, metastasis, and lymphoma. Incidentally, it was also found that there was multilobulated eccentric nodular wall thickening of an approximately 6-cm-long segment of the mid jejunum with poor contrast enhancement (Fig. 3). There was no evidence of bowel obstruction or abdominal lymphadenopathy. The differential diagnoses for the jejunal lesion considered were plasmacytoma, gastrointestinal stromal tumor, primary adenocarcinoma, lymphoma, and metastases. Endoscopic ultrasound demonstrated a mass in the pancreatic head encasing the common bile duct and invading the portal vein. The fine-needle aspiration showed multiple sheets of atypical plasma cells. The patient was considered inoperable. An ERCP was attempted to place a biliary stent for palliative treatment, but it was unsuccessful. An external–internal biliary drainage catheter was placed via a percutaneous approach. His condition kept deteriorating and 4 weeks later he died of respiratory and cardiac failure.
Fig. 2. Transverse CT scan images at the level of the pancreatic head without (A) and with intravenous contrast (B) show homogeneous isodense solid mass in the pancreatic head (arrows) with contrast enhancement similar to the rest of the pancreas. Slightly higher contrast images (C and D) show mild pancreatic ductal dilatation (arrowheads in C) and biliary ductal dilatation (arrowheads in D), respectively.
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P. Gupta et al. / Clinical Imaging 33 (2009) 240–243
3. Discussion Plasma cell neoplasms may present as multiple myeloma, solitary myeloma of the bone, plasma cell leukemia, or as EMP [1,2]. Extramedullary plasmacytomas are seen and can arise 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 wall thickening 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 the nasal cavity, paranasal sinuses, and upper airways. Involvement of the gastrointestinal tract is reported in approximately 10% of cases, most of which involve the liver and spleen [3–6]. Very few cases of pancreatic and small bowel involvement have been reported. It can occur as primary isolated form or as secondary manifestation of systemic disease. Pancreatic and jejunal involvement in the same patient has never been described before. Most cases of plasma cell infiltrates of the pancreas are microscopic. Well-formed masses are unusual. It may present as focal mass [1,5,6] or as diffuse enlargement of the pancreas [7,8] with or without biliary obstruction. On ultrasound, pancreatic plasmacytoma appears as a multilobulated heterogeneous hypoechoic mass [3,5,6]. The CT features have been described as a focal multilobulated solid hypodense mass with homogeneous intravenous contrast enhancement [1,5,6] or more rarely as diffuse enlargement of the pancreas [7,8]. In our case, enhancement was similar to the rest of the pancreas. Without pathological confirmation, focal mass cannot be reliably differentiated from other pancreatic neoplasms including primary adenocarcinoma, lymphoma, and metastasis. MRI features have been reported only in two patients which included pancreatic enlargement with lobulated contour, signal intensity less than that of the liver on T1, and diffuse increased signal intensity on T2-weighted pulse sequences with heterogeneous enhancement [7,9]. In the gastrointestinal tract, the small bowel is most commonly involved, followed by the stomach, colon, and esophagus [2,4,10]. In the small bowel, the most common site of involvement is the ileum [10]. Jejunal involvement has not been described before in concurrence with pancreatic involvement. On barium studies, imaging features of small bowel involvement simulate that of primary carcinoma or lymphoma [10,11]. There are isolated case reports of patients presenting with small bowel obstruction and one case with ileocolic fistula formation [12]. There are only a few cases reports describing the CT appearance of the small bowel involvement, as most of the cases reported were before 1980. The gastric plasmacytoma has been described on CT as a marked thickening of the gastric wall with poor contrast enhancement, impossible to distinguish from gastric adenocarcinoma or lymphoma [4]. Jejunal involvement has been reported in the pathological literature; however, to the best of our knowledge, the CT appearance of jejunal involvement has not been reported before. In our case, on CT scan an approximately 6-cm-long segment of the mid jejunum was seen involved with multilobulated eccentric nodular wall thickening with poor contrast enhancement, an appearance which is nonspecific and can be seen with gastrointestinal stromal tumor, primary adenocarcinoma, lymphoma, and metastases. There was no bowel obstruction or abdominal lymphadenopathy in our case. EMPs are usually associated with the more aggressive anaplastic form of the disease and have poor prognosis [3].
P. Gupta et al. / Clinical Imaging 33 (2009) 240–243
In conclusion, we present a very rare case of pancreatic and jejunal plasmacytoma in a patient with multiple myeloma and describe the CT imaging features. Myelomatous involvement of the pancreas and gastrointestinal tract is well documented in the pathological literature, but reports of imaging are very few. Radiologist should be aware of this occurrence, particularly when there is history of systemic disease. It may help avoid extensive unnecessary interventions. References [1] Hiller N, Goitein O, Ashkenazi YJ. Plasmacytoma of the pancreas. Isr Med Assoc J 2004;6(11):704–5. [2] Schoretsanitis G, Livingstone JI, el-Japour JN, Watkins N, Wastell C. Duodenal plasmacytoma: a rare extramedullary localization simulating carcinoma of the head of the pancreas. Postgrad Med J 1994;70(823): 378–9. [3] Patlas M, McCready D, Kulkarni S, Dill-Macky MJ. Spectrum of imaging findings in abdominal extraosseous myeloma. AJR Am J Roentgenol 2004;183(4):929–32.
243
[4] Yoon SE, Ha HK, Lee YS, Kim PN, Lee MG, Yu E, Auh YH. Upper gastrointestinal series and CT findings of primary gastric plasmacytoma: report of two cases. AJR Am J Roentgenol 1999;173(5):1266–8. [5] Rice NT, Woodring JH, Mostowycz L, Purcell M. Pancreatic plasmacytoma: sonographic and computerized tomographic findings. J Clin Ultrasound 1981;9(1):46–8. [6] Fukuya T, Yoshimitsu K, Kitagawa S, Murakami J, Masuda K, Nakamura K. Plasmacytoma of the pancreatic head. Gastrointest Radiol 1989;14(3):226–8. [7] Pääkkö E, Autio R, Lähde S. Plasmacytoma of the pancreas: CT and MRI appearance. Rofo 1998;169(6):673–5. [8] Scheiman J, Elta G, Francis I. Biliary obstruction secondary to an extramedullary plasmacytoma of the pancreas: confusion with pancreatitis on computed tomography. Pancreas 1987;2(2):237–9. [9] Balliu E, Casas JD, Barluenga E, Guasch I. Multifocal involvement of the pancreas in multiple myeloma: sonographic, CT, and MR imaging findings. AJR Am J Roentgenol 2003;180(2):545–6. [10] Deodhare SG, Pujari BD, Apte PG, Gujar AG. Plasmacytomas of gastrointestinal tract. J Postgrad Med 1975;21(3):145–50. [11] Barzilai A, Toledano C, Arie JB, Gellei B. Plasmacytoma of the gastrointestinal tract. World J Surg 1977;2(1):267–73. [12] Fendel EH, Fazio VW. Extramedullary plasmacytoma of the small intestine: first case report of ileocolic fistula and review of the literature. Dis Colon Rectum 1981;24(8):633–5.