Case Report Pancreatology 2011;11:553–556 DOI: 10.1159/000332038
Published online: December 28, 2011
B-Cell Lymphoma Presenting as Acute Pancreatitis Edda Federico a Massimo Falconi d Gianni Zuodar a Giovanni Falconieri b Fabio Puglisi c Departments of a Internal Medicine and b Pathology, University Hospital of Udine, and c Department of Medical and Biological Sciences, University of Udine, Udine, and dDepartment of Surgery, Policlinico ‘G.B. Rossi’, Verona, Italy
Key Words Pancreatic lymphoma Acute pancreatitis Pulmonary lymphoma
Abstract Background: Pancreatic lymphoma is uncommon, representing less than 0.5% of pancreatic tumors, with diffuse large B-cell lymphoma being the predominant histotype. Acute pancreatitis associated with pancreatic lymphoma is rare. Case Report: We describe a case of synchronous pancreatic and pulmonary localizations of non-Hodgkin’s lymphoma in a 42-year-old man who presented with acute pancreatitis. Acute pancreatitis resolved after standard treatment with a fasting regimen, gabexate mesilate and parenteral nutrition. However, ultrasound scan and abdominal computed tomography revealed two hypoechogenic areas within the pancreas, and chest X-ray film showed a pulmonary infiltrate in the right basal field. A percutaneous fine-needle aspiration biopsy of the pulmonary infiltrate under computed tomography guidance demonstrated a diffuse infiltration by atypical lymphoid cells positive for leukocyte common antigen, CD20 and CD30. Percutaneous fineneedle aspiration biopsy under ultrasound guidance of the pancreatic mass confirmed the diagnosis of diffuse large B-
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cell lymphoma. The patient was classified as stage IV-A, lowintermediate risk and received 6 cycles of chemotherapy. Conclusion: This is the first case of large B-cell lymphoma presenting with concomitant primary pancreatic and pulmonary involvement. Pancreatic lymphoma is uncommon and represents a rare cause of acute pancreatitis. The discovery of a pancreatic mass needs pathologic diagnosis to distinguish lymphoma from carcinoma or autoimmune pancreatitis. Copyright © 2011 S. Karger AG, Basel and IAP
Non-Hodgkin’s lymphoma (NHL) arises in extranodal sites in more than 50% of patients and the gastrointestinal tract is the most frequent site of involvement, particularly the stomach and the small bowel . However, pancreatic lymphoma is uncommon, representing less than 0.5% of pancreatic tumors. Only 0.2–2% of patients with NHL have pancreatic involvement at presentation, diffuse large B-cell lymphoma being the predominant histotype . Acute pancreatitis associated with pancreatic lymphoma is rare .
Fabio Puglisi, MD PhD Department of Medical and Biological Sciences University of Udine IT–33100 Udine (Italy) Tel. +39 0432 559 309, E-Mail [email protected]
Fig. 1. Chest X-ray showing a pulmonary infiltrate in the right
Fig. 2. Abdominal CT scan showing a mass located in the head of
The present report describes the first documented case of concomitant pancreatic and pulmonary NHL in a 42-year-old man admitted with a diagnosis of acute pancreatitis.
absence of organ failures or local complications. The patient received continuous intravenous infusion with gabexate mesilate at the dosage of 1.5 g/day for 5 days, parenteral nutrition, ranitidine and antibiotics. Within a few days of therapy, the patient’s abdominal pain was resolved and at the end of treatment the serum levels of amylase and lipase normalized. A subsequent thoracic and abdominal computed tomography (CT) scan confirmed the presence of a right pulmonary infiltrate and of a 35-mm mass located in the head of the pancreas (fig. 2). Initially, an echoendoscope-guided fine-needle aspiration of the pancreatic head lesion was performed but the cytological findings were not diagnostic. Next, a percutaneous fine-needle aspiration biopsy (FNAB) of the pulmonary infiltrate was performed under CT guidance. Microscopic examination of formalin-fixed and routinely processed tissue specimens showed a diffuse infiltration by atypical lymphoid cells (fig. 3). A panel of antibodies applied to sections obtained from paraffin tissue was positive for leukocyte common antigen, CD20 and CD30, and negative for wide-spectrum keratins (fig. 4). Histologic examination of bone marrow was negative for tumor infiltrates. One month after the first CT scan, a new abdominal CT scan showed an increase of the pancreatic head lesion (60 ! 55 mm) with involvement of the superior mesenteric vein. Percutaneous FNAB under US guidance of the pancreatic mass confirmed the diagnosis of diffuse large B-cell lymphoma. Total body positron emission tomography (PET)/CT was performed for staging; a pancreatic mass englobing peripancreatic, mesenteric and hepatic lymphadenopathies was detected, constricting the superior mesenteric vein and inferior vena cava (SUVmax = 16.5). A right pulmonary infiltrate was also evident (SUVmax = 16.0). Furthermore, PET/CT disclosed the presence of adenopathies from the right bronchus to the right pulmonary hilus (SUVmax = 13.2). The patient was classified as stage IV-A, based on the Ann Arbor classification, and as low-intermediate risk (score 2), based on the International Prognostic Index for diffuse large B-cell lymphoma. He received 6 cycles of chemotherapy with cyclophospha-
Case Report A 42-year-old man presented to the emergency room because of constant and intense epigastric pain. Laboratory investigations showed an increased serum level of total amylase (152 U/l, normal range 10–100 U/l). Ultrasound (US) scan of the abdomen revealed two hypoechogenic areas, measuring 35 and 20 mm, within the pancreatic head and the head/body of the pancreas, respectively. There was no evidence of stones in the gallbladder and the common bile duct. An acute pancreatitis was initially suspected and the patient was admitted to the hospital. Past medical history was unremarkable; the patient denied alcohol consumption. Usual medications included omeprazole for chronic gastritis. Family history was positive for systemic lupus erythematosus. Physical examination showed epigastric tenderness. Further laboratory tests disclosed a mild increase of the erythrocyte sedimentation rate (19 mm/h), C-reactive protein (10.7 mg/l, normal range 0.0–5.0 mg/l), alanine aminotransferase (44 U/ml, normal range 5–40 U/ml) and CA-19.9 (64.2 U/ml, normal value !31 U/ml, pathologic value 1 60 U/ml). The remaining parameters were all in the normal range. Among the tests of autoimmunity, a positivity for antinuclear antibodies (1: 640) was found. A plain chest X-ray film revealed the presence of a 55-mm pulmonary infiltrate in the right basal field (fig. 1). Three days after the admission, because of a significant increase of serum amylase and lipase levels (793 and 1,779 U/l, respectively), a diagnosis of mild acute pancreatitis was made in the
Federico /Falconi /Zuodar /Falconieri / Puglisi
Fig. 3. A pulmonary infiltrate featuring large atypical cells with a
Fig. 4. Tumor cells show strong immunoreactivity to leukocyte
polygonal outline and hyperchromatic nuclei often with smudged chromatin. HE. !40.
mide, doxorubicin, vincristine, prednisone and rituximab (RCHOP regimen). A follow-up total body PET/CT scan performed after 2 cycles revealed residual peripancreatic (20 mm), mesenteric and pulmonary adenophathies which were deemed consistent with adequate antitumoral therapy response.
Pancreatic B-cell lymphoma is rare (about 0.2–2% of patients with NHL) and its diagnostic assessment may be problematic without the aid of histologic examination inasmuch as patients’ symptoms, laboratory and imaging findings are non-specific . Clinical presentation may include abdominal pain, weight loss, nausea and vomiting. Fever and night sweats, often seen in lymphoma, are uncommon. Pancreatic involvement by NHL may rarely manifest as an acute pancreatitis. To date, only 9 cases of pancreatic B-cell lymphoma presenting as acute pancreatitis have been described in the literature [4, 5]. Jaundice, abdominal mass, diarrhea, gastric outlet obstruction, and small bowel obstruction have also been reported at presentation [6, 7]. Laboratory findings are also non-diagnostic and may reflect abnormality of pancreatic and liver function. Lactate dehydrogenase may be elevated in up to 50% of cases and CA-19-9 may occasionally be increased. Our patient had a family history for autoimmune disease and an antinuclear antibody titer was positive.
Once the main causes of acute pancreatitis such as bile stones, alcohol consumption and hypertriglyceridemia were ruled out, the clinical presentation could still suggest autoimmune pancreatitis. In fact, autoimmune chronic pancreatitis with a mass formation within one or two segments of the pancreas has been reported as a potential primary neoplasm mimicker [8, 9]. CT imaging in pancreatic lymphoma also lacks specificity merely revealing a large head mass with or without associated lymphadenopathies. Less common presentations include masses in the body or tail, or rarely, a diffuse involvement of the gland. Extrapancreatic spread is common and the tumor may encroach on the mesenteric vessels . The rapid growth of the bulky tumor, as in the case herein described, is reported as a peculiarity of the pancreatic lymphoma. FNAB can be performed either percutaneously (under US or CT) or under endoscopic US guidance, the latter being preferred in order to avoid possible peritoneal seeding. In any case, cytological examination is mandatory in order to distinguish carcinoma from lymphoma. Immunophenotyping may assist in the differential diagnosis. In the present case, the patient initially underwent echoendoscope-guided FNAB, but the diagnosis was inconclusive. High-grade pancreatic lymphoma generally responds well to chemotherapy. Anthracycline-based chemotherapy is the standard treatment for NHL, and includes 6–8 cycles of R-CHOP . Our patient showed a good re-
Unusual Sites of B-Cell Lymphoma
sponse following the first two R-CHOP courses and completed 6 cycles. Furthermore, he was eligible for peripheral blood cell autotransplantation. To the best of our knowledge, this is the first case of large B-cell lymphoma presenting with synchronous pancreatic and pulmonary localizations. Although there was a presence of a bulky pulmonary infiltrate, the patient did not complain of any respiratory symptoms, whereas pancreatic involvement determined the clinical
presentation and led to the diagnosis. Acute pancreatitis resolved after standard treatment with fasting regimen, gabexate mesilate and parenteral nutrition. In conclusion, pancreatic lymphoma, with or without other localization, is a rare cause of acute pancreatitis. While acute inflammation of the gland may be resolved with the standard treatment, the presence of a pancreatic mass needs pathologic diagnosis to distinguish lymphoma from carcinoma or autoimmune pancreatitis.
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