Primary pancreatic lymphoma presenting as acute pancreatitis

Primary pancreatic lymphoma presenting as acute pancreatitis

Case Report Pancreatology 2003;3:403–405 DOI: 10.1159/000073656 Published online: September 24, 2003 Primary Pancreatic Lymphoma Presenting as Acute...

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Case Report Pancreatology 2003;3:403–405 DOI: 10.1159/000073656

Published online: September 24, 2003

Primary Pancreatic Lymphoma Presenting as Acute Pancreatitis Deepa Reddy Vivek Gumaste

Vladimir Benisovich

Department of Medicine, Mount Sinai Services at Elmhurst, New York, N.Y., USA

Key Words Pancreatic lymphoma W Abdominal pain W Jaundice W Acute pancreatitis

Abstract Primary pancreatic lymphoma is a rare disease and usually presents with nonspecific symptoms like abdominal pain, weight loss, or jaundice. Here we report a case of primary pancreatic lymphoma presenting as acute pancreatitis. Copyright © 2003 S. Karger AG, Basel and IAP

Introduction

Primary pancreatic lymphoma (PPL) is a rare disease that constitutes less than 5% of all pancreatic malignancies and accounts for less than 3% of the non-Hodgkin’s lymphomas [1, 2]. Secondary involvement of the pancreas in non-Hodgkin’s lymphoma is much more commoner, being seen in about one third of the cases [3]. PPLs usually present with abdominal pain, jaundice, or weight loss. Acute pancreatitis can be a presenting feature, but is extremely uncommon [4]. Here we report a 32-year-old male who was admitted with acute pancreatitis and was subsequently found to have a PPL.

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Case Report A 32-year-old male from Ecuador, living in the USA for 4 years with no significant past medical history, presented with abdominal pain for 36 h. The pain had started in the right lower quadrant, but now involved the entire abdomen. He described the pain as ‘rocklike’ and constant in nature. The pain was worse with ingestion of food or liquids. He denied having similar complaints in the past. He had no symptoms of nausea, vomiting, diarrhea, blood per rectum, fevers, or any weight loss. On physical examination, the patient was lying comfortably in bed and was not in any distress. The blood pressure was 130/77 mm Hg, the pulse 80/min, the temperature around 38 ° C, and the respiratory rate was 18/min. He was not jaundiced and did not have any evidence of lymphadenopathy. The abdominal examination was significant for periumbilical and right upper quadrant tenderness, with some voluntary guarding. The bowel sounds were normal. There was no hepatosplenomegaly. The rest of his physical examination was normal. The patient’s laboratory measurements revealed the following values: leukocyte count 5.8 ! 10/l with 89% neutrophils, hemoglobin 13.8 g/day, creatinine 0.9 mg/dl, amylase 733 (normal range 0– 130) U/l, lipase 1,942 (normal range 13–104) U/l, aspartate aminotransferase 166 (normal range 2–40) U/l, alanine aminotransferase 106 (normal range 2–50) U/l, lactate dehydrogenase 299 (normal range 90–225) U/l, alkaline phosphatase 162 (normal range 30–115) U/l, albumin 4 g/dl, total bilirubin 1.8 mg/dl, direct bilirubin 0.3 mg/ dl, prothrombin time 12.7 s, INR 1.05 IU, and partial thromboplastin time 27 s. A chest X-ray was normal. Ultrasound showed no gallstones, but the pancreas was poorly visualized. The patient was diagnosed as having acute pancreatitis and was treated with bowel rest and pain control. He improved clinically and was started on clear liquids on the 6th hospital day. However, his

Vivek V. Gumaste, MD Chief, Division of Gastroenterology, Mount Sinai Services at Elmhurst 79-01 Broadway New York, NY 11373 (USA) E-Mail [email protected]

2 Fig. 1. a Initial CT scan showing a mass in the region of the pancreas measuring 6 ! 8 cm. b CT scan after therapy showing complete disappearance of the mass. Fig. 2. Pancreatic biopsy specimen showing diffuse involvement by small- and medium-sized cells with hyperchromatic nuclei. Immunohistochemical stains confirmed a B cell lymphoma.

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abdominal pain recurred and a CT scan was done. Abdominal CT (with contrast) revealed a 6 ! 8-cm pancreatic mass (fig. 1a). No lymphadenopathy was noted. Subsequently, he underwent a CT scan guided biopsy which revealed a fragment of tissue diffusely involved by small to medium size lymphocytes (fig. 2) positive for CD45, CD20, and CD79a and negative for CD3 and CD4. The tissue was also positive for CD10 and kappa light chains and negative for CD5, bc1, and lambda light chain. The lymphoma was classified according to the WHO/REAL classification as a B cell follicular lymphoma, grade II (follicular center lymphoma, follicular, mixed). CT scan of the chest and bone marrow biopsy were negative. The gallium scan showed markedly increased uptake only in the region of the pancreas, corresponding to the mass. The lymphoma was staged as 1E, A. His hospital course was complicated by an episode of cholangitis, but attempts to place a biliary stent were unsuccessful. The patient was treated with chemotherapy consisting of cyclophosphamide, doxorubicin, Oncovin (vincristine), and prednisone (CHOP). While on chemotherapy, serial CT scans demonstrated a gradual decrease in size of the pancreatic mass. The patient received a total of eight cycles of CHOP. By the end of his chemotherapy, CT scan (fig. 1b) and gallium scan became completely normal. He was then referred for radiation therapy and received 45 cGy to the involved field. He remains in complete remission 3 years after completion of treatment.

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Discussion

PPL is an extremely rare disease affecting mostly elderly patients. Although the average age of onset is 60 years [5–7], it has been diagnosed in patients as young as 23 years. There appears to be no sex predilection [5–7]. The patients usually present with nonspecific symptoms like abdominal pain, jaundice, and weight loss. In one study [5], abdominal pain was the commonest symptom, occurring in over 80% of the patients, while in another report [2], weight loss was the commonest manifestation. Jaundice is found in about 30% of the patients with PPL [5, 6, 8]. As opposed to this, more than 50% of the patients with adenocarcinomas have jaundice. Although patients with PPL may have raised amylase levels [1, 5], acute pancreatitis as a presenting feature is rare. Only 7 cases have been reported in the literature so far [4]. The presence of an abdominal mass is a striking feature of PPL. More than 50% of the patients have a palpable abdominal mass [1, 2, 5]. In contrast, less than 20% of the patients with an adenocarcinoma of the pancreas have a palpable abdominal mass [9]. A shorter duration of symptoms is also suggestive of PPL. The mean duration of symptoms with PPL is 6–8 weeks [2, 10, 11], whereas

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with adenocarcinoma of the pancreas the mean duration is 4 months. The size of the mass on a CT scan may provide some clue to the diagnosis. Ward et al. [12] found that 60% of the adenocarcinomas are !6 cm in diameter and rarely exceed 10 cm in size. Lymphomas tend to be bulkier, with 8 of 9 patients in one report [2] having a size equal to or greater than 6 cm. Another study [1] corroborated this finding, with 70% of their patients having a mass size 16 cm. In addition, 3 of 7 patients in the same study [1] had lesions 110 cm. Our patient had a mass which measured 6 ! 8 cm in size. Even though the histopathological diagnosis is ultimately required to differentiate a PPL from an adenocarcinoma, certain features such as the duration of symptoms and the size of the mass on a CT scan should increase the index of suspicion. The diagnosis may be established either with percutaneous needle biopsy or by open laparotomy. One study [2] reported excellent results with percutaneous needle biopsy, helping to confirm the diagnosis in 4 of 4 patients in whom it was attempted. Results from other reviews have not been as encouraging [2, 6]. In fact, laparotomy and open biopsy were the methods of choice to establish the diagnosis in most other reports [1, 5, 6]. However, with refinement in immunohistochemical techniques, the yield

of the percutaneous route may increase and should be the initial approach. A positive result may obviate the need for surgery, especially in those patients in whom chemotherapy or radiation alone is thought to suffice. Laparotomy should be reserved for those patients in whom percutaneous methods fail to make a diagnosis or in whom surgery is considered a therapeutic alternative. The standard treatment for pancreatic lymphomas has been chemotherapy with or without radiation [13]. Acceptable results have been reported with chemotherapy [2] and chemo-radiation [10]. Therefore, we decided to treat our patient with chemotherapy and radiation. Some authors suggest that inclusion of surgical resection as a part of the treatment may result in better remission and cure rates [13]. Although this inference is based on anecdotal reports and retrospective studies implicit with a selection bias, surgical resection may be considered when planning the treatment, especially for a patient with an early-stage pancreatic lymphoma [13]. Patients with a pancreatic lymphoma have a far better prognosis than those with an adenocarcinoma with survival rates ranging from 2 to 6.5 years [1, 2]. Long-term follow-up of patients with a pancreatic lymphoma is necessary, as recurrences may occur even 18 years after complete remission [14].

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Primary Pancreatic Lymphoma Presenting as Acute Pancreatitis

5 Behrns KE, Sarr MG, Strickler JG: Pancreatic lymphoma: Is it a surgical disease? Pancreas 1994;9:662–667. 6 Mansour GM, Cucchiaro G, Niotis MT, Fetter BF, Moore J, Rice RR, Branum GD, Meyers WC: Surgical management of pancreatic lymphoma. Arch Surg 1989;124:1287–1289. 7 Nishimura R, Takakuwa T, Hoshida Y, Tsujimoto M, Aozasa K: Primary pancreatic lymphoma. Oncology 2001;60:322–329. 8 Islam S, Callery MP: Primary pancreatic lymphoma – a diagnosis to remember. Surgery 2001;129:380–383. 9 DiMagno EP: Pancreatic adenocarcinoma; in Yamada T, Alpers DH, Powell DW, Silverstein FE (eds): Textbook of Gastroenterology. Philadelphia, Lippincott, 1995, pp 2113–2130. 10 Ezzat A, Jamshed A, Khafaga Y, Rahal M, Linjawi T, Martin J, Taha I: Primary pancreatic non-Hodgkin’s lymphoma. J Clin Gastroenterol 1996;23:109–112.

11 Cappell MS, Yao F, Cho KC, Axiotis CA: Lymphoma predominantly involving the pancreas. Dig Dis Sci 1989;34:942–947. 12 Ward EN, Stephens DM, Sheedy PF: Computer tomographic characteristics of pancreatic carcinoma: An analysis of cases. Radiographics 1984;3:547–563. 13 Koniaris LG, Lillemoe KD, Yeo CJ, Abrams RA, Coleman J, Nakeeb A, Pitt H, Cameron JL: Is there a role for surgical resection in the treatment of early-stage pancreatic lymphoma? J Am Coll Surg 2000;190:319–330. 14 Popescu RA, Wotherspoon AC, Cunningham D: Local recurrence of a primary pancreatic lymphoma 18 years after complete remission. Hematol Oncol 1998;16:29–32.

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