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Primary Pancreatic Lymphoma Masquerading as Carcinoma

Primary Pancreatic Lymphoma Masquerading as Carcinoma Hindawi Case Reports in Oncological Medicine Volume 2020, Article ID 5160545, 6 pages https://doi.org/10.1155/2020/5160545 Case Report Nasser A. N. Alzerwi Department of Surgery, College of Medicine, Majmaah University, Ministry of Education, Al-Majmaah City, 11952, P. O. Box 66, Riyadh Region, Saudi Arabia Correspondence should be addressed to Nasser A. N. Alzerwi; n.alzerwi@mu.edu.sa Received 17 January 2020; Accepted 14 February 2020; Published 26 February 2020 Academic Editor: Josep M. Ribera Copyright © 2020 Nasser A. N. Alzerwi. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Adenocarcinoma is the most common primary pancreatic neoplasm type, followed by secondary pancreatic lymphoma and primary pancreatic lymphoma (PPL). PPL is associated with peripancreatic lymphadenopathy and usually presents as a homogenous mass with extrapancreatic invasion into surrounding structures. However, localized involvement of the distal pancreas is uncommon, and diffuse involvement of the pancreas is even rarer. Herein, we present the case of a 53-year-old woman with PPL of the uncinate process with biliary obstruction mimicking pancreatic adenocarcinoma, successfully diagnosed nonoperatively. Abdominal computed tomography showed an ill-defined uncinate process mass, hypodense with mild enhancement (3:9×3:4×3:5cm), infiltrating the second and third parts of the duodenum. Biopsy revealed NHL with no evidence of adenocarcinoma. PPL was diagnosed. She received chemotherapy with a CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) protocol with rituximab, which she tolerated with no clinical or radiological evidence of recurrence at 1-year follow-up. 1. Introduction cutaneous icterus, itching, dark urine, pale stool, and early satiety. She had an unremarkable medical and family history. Primary pancreatic lymphoma (PPL) accounts for approxi- Systemic examination revealed no other symptoms. mately 0.5% of pancreatic neoplasms and <2% of lymphomas Upon examination, the patient looked cachectic, con- [1, 2] and commonly presents as non-Hodgkin lymphoma scious, and alert but dehydrated and deeply jaundiced. Her [3, 4]. Its clinical manifestations and radiological findings vital signs were within normal limits; she was afebrile. mimic those of other pancreatic lesions such as adenocarci- Abdominal examination revealed no scars or prominent noma, neuroendocrine tumors, and chronic pancreatitis [2]. veins; the abdomen was soft and lax with mild tenderness at The rarity, difficulty in access, dissimilar outcomes, treat- the epigastrium, with no palpable mass, organomegaly, or ment, and survival rates of PPL make it a diagnostic and lymphadenopathy. Normal bowel sounds were audible, with therapeutic challenge [3, 4]. Histological diagnosis is essential intact hernial orifices. A digital rectal exam showed unre- [5] as histological subtype is a significant prognostic factor markable results. Laboratory tests revealed direct hyperbiliru- [1, 2, 6, 7]. Herein, we present the case of a 53-year-old woman binemia (Table 1) and anemia. with PPL of the uncinate process with biliary obstruction The clinical impression was obstructive jaundice requir- mimicking pancreatic adenocarcinoma and review the litera- ing further investigation. She was admitted and prescribed ture on PPL. intravenous fluid therapy. Chest X-ray showed unremarkable results; abdominal ultrasonography (US) revealed distended gallbladder with 2. Case Presentation no stones or signs of acute cholecystitis (Courvoisier’s sign). A 53-year-old woman presented to the emergency depart- However, a tortuous cystic duct and dilatation of the intrahe- ment complaining of intermittent sharp epigastric pain patic and extrahepatic biliary ducts and the common bile radiating to the back, associated with nausea, scleral and duct (CBD) (13 mm) (Figures 1 and 2) were observed. 2 Case Reports in Oncological Medicine Table 1: Laboratory results. Test Result Units WBC 5:9× 10 Cells/L Hgb 9.7 g/dL Platelets 220 × 10 /L INR 0.9 — PT 10.4 sec PTT 39.4 sec AST 146 U/L ALT 230 U/L Figure 2: Transabdominal ultrasonography showing a dilated Alp 358 U/L common bile duct (13 mm) with no visible choledocholithiasis. TB 159.8 μmol/L DB 139.2 μmol/L Alb 33.4 g/L Amylase 22 U/L GGT 466.8 U/L Urea 3.4 mmol/L Creatinine 60.7 μmol/L K 3.8 mmol/L Na 139 mmol/L LDH 128 IU/L CEA 0.8 ng/mL CA 19-9 16 U/mL AFP 2.1 ng/mL Figure 3: Enhanced computer tomography (CT) of the abdomen. WBC: white cell count; Hgb: hemoglobin; INR: international normalized Coronal slice obtained through the second part of the duodenum ratio; PT: prothrombin time; PTT: partial thromboplastin time; AST: (D2; green arrow) shows an uncinate process mass (orange arrow) aspartate aminotransferase; ALT: alanine aminotransferase; Alp: alkaline protruding into the lumen of D2, infiltrating and obstructing the phosphatase; TB: total bilirubin; DB: direct bilirubin; Alb: albumin; GGT: dilated distal common bile duct (yellow arrow). gamma glutamyl transferase; K: potassium; Na: sodium; LDH: lactate dehydrogenase; CEA: carcinoembryonic antigen; CA 19-9: carbohydrate antigen 19-9; AFP: alpha fetoprotein; L: liter; g: gram; dL: deciliter; sec: seconds; U: unit; mmol: millimole; μmol: micromole; IU: international Abdominal computed tomography (CT), performed for unit; ng: nanogram; mL: milliliter. suspected obstructive jaundice due to malignancy, showed findings similar to US findings and an ill-defined uncinate process mass that was hypodense with mild enhancement (3:9× 3:4×3:5cm). The mass had infiltrated the second and third parts of the duodenum with no signs of duodenal obstruction and mass effect on the inferior vena cava (IVC). There was extensive lymphadenopathy (perilesional, retro- peritoneal, mesenteric, and left para-aortic) with no metasta- tic deposits in the liver (Figure 3). Chest CT showed no metastatic lung deposits or medias- tinal lymphadenopathy. Tumor markers were within the normal range (Table 1). Magnetic resonance cholangiopan- creatography (MRCP) showed diffuse intrahepatic biliary duct dilatation, a dilated CBD (13 mm), and a normal pan- creatic duct (Figures 4–7). Duodenoscopy showed mucosal erythema on stenotic and granular major papilla in the periampullary area; thus, Figure 1: Transabdominal ultrasonography showing a distended a biopsy was performed (Figure 8). Endoscopic retrograde gall bladder (102:8× 37:1mm) with a thin wall (3.1 mm) and cholangiopancreatography (ERCP) showed distal CBD and tortuous dilated cystic duct with no intraluminal gallstones or papillary stenosis, which required sphincterotomy and pericholecystic fluid. stenting, and an ampullary mass, which was biopsied (Figure 9). Biopsy revealed NHL with no evidence of adenocarcinoma (Figure 10). Whole-body multislice CT Case Reports in Oncological Medicine 3 Figure 4: Magnetic resonance imaging (MRI) of the abdomen. Figure 7: Magnetic resonance imaging (MRI) of the abdomen. Coronal slice obtained through the second part of the duodenum Axial slice obtained through the distended GB (star) shows the (D2) shows the uncinate process mass (green arrow) obstructing thin gall bladder wall (green arrow) and a thin rim of the distal common bile duct (dotted black arrow). pericholecystic fluid (orange arrow). Figure 5: Magnetic resonance imaging (MRI) of the abdomen. Coronal slice shows the line of demarcation between the distal normal body and tail of the pancreas (orange arrow) and the neoplastic proximal body (green arrow), with multiple mesenteric and peripancreatic neoplastic lymph nodes (black arrowheads). Figure 8: Duodenoscopy shows stenotic major papilla with neoplastic overgrowth (black arrow) protruding into the lumen of the second part of the duodenum (D2). were no clinical or radiological evidence of recurrence after 1 year of follow-up. Written informed consent was obtained from the patient for the publication of this report. The institutional review board of King Fahad Medical City (Riyadh, KSA, Tel: +96612889999 ext. 26913, email: okasu- le@kfmc.med.sa) waived the need for ethical approval on December 05, 2019 (IRB log number: 19-613E). 3. Discussion Figure 6: Magnetic resonance imaging (MRI) of the abdomen. The typical age of PPL patients is 35–75 years (mean: 55 Coronal slice shows the uncinate process mass (dotted black years), with a strong male predilection (male : female ratio arrow) and multiple neoplastic peripancreatic lymph nodes (orange arrows). of 7 : 1) [8]. PPL presents with typical symptoms of lym- phoma like fever, chills, and night sweats in only 2% of cases [8]. The clinical presentations of PPL are nonspecific, most was negative for generalized lymphadenopathy, and the mul- commonly abdominal pain followed by abdominal mass tidisciplinary oncology board diagnosed PPL. She received detection, weight loss, jaundice (76%), and acute pancreatitis cyclophosphamide, doxorubicin, vincristine, and prednisone (AP) (18%), which mimics adenocarcinoma. Though PPL is (CHOP) with rituximab with good tolerability, and there rare, due to the differences in the prognosis and treatment 4 Case Reports in Oncological Medicine Figure 9: Duodenoscopy shows the cannulation of the stenotic major papilla (green arrows) and the granular appearance of the neoplastic mass (black arrows). approaches of adenocarcinoma and PPL (surgical resection hematoxylin-eosin staining findings alone may resemble those of a poorly differentiated carcinoma, limiting the value for adenocarcinoma, while chemotherapy for PPL), it is critical to differentiate PPL from adenocarcinoma [1]. of fine needle aspiration cytology (FNAC) as a diagnostic PPL is most commonly located at the head of the pan- technique [11–14]. creas, though it can be found in the body or tail. The tumor The serum CA 19-9 level in PPL patients is usually nor- size ranges from 4 to 17 cm on radiological scans [9]. mal or sometimes slightly elevated in the case of biliary Imaging plays a crucial role in pancreatic mass diagnosis obstruction [15]. In our case, the CA 19-9 level was normal [1]. CT is commonly used in the detection and character- despite the clinical, biochemical, and radiological evidence ization of PPLs appearing as homogenous masses with less of biliary obstruction. contrast enhancement compared with pancreatic adenocar- The management and prognosis of PPL depend on the cinomas [1]. tumor stage and grade. The most common subtype of PPL There are two different morphological patterns of PPL: is the diffuse large B-cell subtype, and long-term remission localized, well-circumscribed tumors and diffuse tumors has been obtained with chemotherapy (CHOP); in the case infiltrating or replacing most of the pancreas [1, 10]. of CD20-positive diffuse large B-cell lymphoma, the addition The radiological criteria for differentiating PPL from of rituximab increases the remission rate [3, 15, 16]. more common pancreatic adenocarcinomas are a bulky PPL patients treated with chemotherapy alone have a bet- localized tumor in the pancreatic head, no significant dilata- ter prognosis than do pancreatic adenocarcinoma patients. tion of the pancreatic duct, enlarged lymph nodes below the PPL patients have a 5-year survival rate of 26%–66% renal vein level, invasive tumor growth, and retroperitoneal [3, 15]. The therapeutic role of radiotherapy in PPL is not yet well defined [3]. Multiple studies have shown a therapeu- or upper abdominal organ infiltration [1]. All of these criteria were fulfilled in our case. tic advantage of adjuvant radiotherapy when combined However, the definitive diagnosis of PPL is impossible with chemotherapy for NHL; however, the replication of from imaging alone, and pathologic examination (cytohisto- such superior outcomes in PPL is not as well established pathological confirmation) is imperative [1]. Immunohisto- [17–19]. Few case series studies have shown promising results, Bouvet et al. recommended the routine use of involved-field chemical staining and flow cytometry are essential as Case Reports in Oncological Medicine 5 Conflicts of Interest The author declares that there is no conflict of interest regarding the publication of this article. Acknowledgments The author would like to thank Dr. Sulimal Al-Alshaikh MD, for his help with the radiographs, and Dr. S. Almutairi MD, Dr. Bandar Idrees MD, Dr. Ahmed Alhumaid MD, Dr. A. Alhindi MD, and Editage (https://www.editage.com) for their help in linguistic editing. References [1] E. Zucca, “Extranodal lymphoma: a reappraisal,” Annals of Oncology, vol. 19, pp. iv77–iv80, 2008. [2] J. F. Seymour, “X. Extra-nodal lymphoma in rare localisations: bone, breast and testes,” Hematological Oncology, vol. 31, Supplement 1, pp. 60–63, 2013. [3] M. W. Saif, “Primary pancreatic lymphomas,” Journal of the Pancreas: JOP, vol. 7, no. 3, pp. 262–273, 2006. [4] E. D. Rossi, A. Larghi, E. C. Verna et al., “Endoscopic ultrasound-guided fine-needle aspiration with liquid-based cytologic preparation in the diagnosis of primary pancreatic lymphoma,” Pancreas, vol. 39, no. 8, pp. 1299–1302, 2010. [5] H. Ettahri, M. Elkabous, S. Laanaz, M. Layachi, and L. Laamarti, “Primary pancreatic lymphoma, a case report and review of literature,” Austin Journal of Medical Oncology, vol. 2, article 1018, 2015. [6] E. Zucca, A. Conconi, T. I. Mughal et al., “Patterns of outcome and prognostic factors in primary large-cell lymphoma of the testis in a survey by the International Extranodal Lymphoma Study Group,” Journal of Clinical Oncology, vol. 21, no. 1, pp. 20–27, 2003. Figure 10: Cross-sectional biopsy shows an infiltrate of large [7] J. D. Gundrum, M. A. Mathiason, D. B. Moore, and R. S. Go, atypical pleomorphic lymphoid cells, consistent with large diffuse “Primary testicular diffuse large B-cell lymphoma: a B-cell lymphoma (hematoxylin-eosin). population-based study on the incidence, natural history, and survival comparison with primary nodal counterpart before and after the introduction of rituximab,” Journal of Clinical Oncology, vol. 27, no. 31, pp. 5227–5232, 2009. [8] A. Shnitser, D. Halegoua-DeMarzio, and D. E. Loren, “Pri- radiotherapy after chemotherapy in a survival report of 9 mary pancreatic lymphoma presenting as acute pancreatitis,” out of 11 patients at a median follow-up time of 67 months Gastroenterología y Hepatología, vol. 12, pp. 456–458, 2016. [20]. Behrns et al. reported a mean survival of 13 months, [9] R. Nishimura, T. Takakuwa, Y. Hoshida, M. Tsujimoto, and 22 months, and 26 months, for those who received che- K. Aozasa, “Primary pancreatic lymphoma: clinicopathologi- motherapy alone, radiotherapy alone, and combined che- cal analysis of 19 cases from Japan and review of the litera- moradiotherapy, respectively [21]. The superior outcome ture,” Oncology, vol. 60, no. 4, pp. 322–329, 2001. of combined chemoradiotherapy could be due to the superior [10] H. Nayer, E. G. Weir, S. Sheth, and S. Z. Ali, “Primary pancre- local control effect of radiotherapy, as most therapeutic fail- atic lymphomas,” Cancer, vol. 102, no. 5, pp. 315–321, 2004. ures were local rather than systemic [22, 23]. [11] P. S. Grimison, M. T. Chin, M. L. Harrison, and D. Goldstein, PPL should be considered in the differential diagnosis of “Primary pancreatic lymphoma–pancreatic tumours that are pancreatic head masses, especially when there is extensive potentially curable without resection, a retrospective review lymphadenopathy localized to the peripancreatic region, of four cases,” BMC Cancer, vol. 6, no. 1, article 117, 2006. and should be ruled out with transluminal biopsy to avoid [12] A. Ribeiro, E. Vazquez-Sequeiros, L. M. Wiersema, K. K. unnecessary surgical intervention. However, if adenocarci- Wang, J. E. Clain, and M. J. Wiersema, “EUS-guided fine- noma cannot be safely ruled out, open biopsy with frozen needle aspiration combined with flow cytometry and immu- section should be considered before performing pancreatico- nocytochemistry in the diagnosis of lymphoma,” Gastrointesti- duodenectomy (PD), as most cases are diagnosed post-PD. nal Endoscopy, vol. 53, no. 4, pp. 485–491, 2001. Furthermore, the more common variant (secondary pancre- [13] M. Mehra, A. Tamhane, and M. A. Eloubeidi, “EUS-guided atic lymphoma) should be ruled out before diagnosing PPL. FNA combined with flow cytometry in the diagnoses of 6 Case Reports in Oncological Medicine suspected or recurrent intrathoracic or retroperitoneal lymphoma,” Gastrointestinal Endoscopy, vol. 62, no. 4, pp. 508–513, 2005. [14] X. Du, Y. Zhao, T. Zhang et al., “Primary pancreatic lym- phoma: a clinical quandary of diagnosis and treatment,” Pancreas, vol. 40, no. 1, pp. 30–36, 2011. [15] N. Battula, P. Srinivasan, A. Prachalias, M. Rela, and N. Heaton, “Primary pancreatic lymphoma: diagnostic and therapeutic dilemma,” Pancreas, vol. 33, no. 2, pp. 192–194, [16] Y. Fukita, T. Asaki, S. Adachi, I. Yasuda, M. Toyomizu, and Y. Katakura, “Non-Hodgkin lymphoma mimicking pancreatic adenocarcinoma and peritoneal carcinomatosis,” Journal of Clinical Oncology, vol. 31, no. 21, pp. e373–e376, 2013. [17] T. P. Miller, S. Dahlberg, J. R. Cassady et al., “Chemotherapy alone compared with chemotherapy plus radiotherapy for localized intermediate-and high-grade non-Hodgkin's lym- phoma,” The New England Journal of Medicine, vol. 339, no. 1, pp. 21–26, 1998. [18] J. O. Armitage, “Treatment of non-Hodgkin's lymphoma,” The New England Journal of Medicine, vol. 328, no. 14, pp. 1023– 1030, 1993. [19] R. I. Fisher, E. R. Gaynor, S. Dahlberg et al., “Comparison of a standard regimen (CHOP) with three intensive chemotherapy regimens for advanced non-Hodgkin's lymphoma,” New England Journal of Medicine, vol. 328, no. 14, pp. 1002–1006, [20] M. Bouvet, G. A. Staerkel, F. R. Spitz et al., “Primary pancreatic lymphoma,” Surgery, vol. 123, no. 4, pp. 382–390, 1998. [21] K. E. Behrns, M. G. Sarr, and J. G. Strickler, “Pancreatic lym- phoma: is it a surgical disease?,” Pancreas, vol. 9, no. 5, pp. 662–667, 1994. [22] K. H. Shahar, L. S. Carpenter, J. Jorgensen, L. Truong, K. Baker, and B. S. Teh, “Role of radiation therapy in a patient with primary pancreatic lymphoma,” Clinical Lymphoma and Myeloma, vol. 6, no. 2, pp. 143–145, 2005. [23] P. Mauch, R. Leonard, A. Skarin et al., “Improved survival fol- lowing combined radiation therapy and chemotherapy for unfavorable prognosis stage I-II non-Hodgkin's lymphomas,” Journal of Clinical Oncology, vol. 3, no. 10, pp. 1301–1308, http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Case Reports in Oncological Medicine Hindawi Publishing Corporation

Primary Pancreatic Lymphoma Masquerading as Carcinoma

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Copyright © 2020 Nasser A. N. Alzerwi. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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Hindawi Case Reports in Oncological Medicine Volume 2020, Article ID 5160545, 6 pages https://doi.org/10.1155/2020/5160545 Case Report Nasser A. N. Alzerwi Department of Surgery, College of Medicine, Majmaah University, Ministry of Education, Al-Majmaah City, 11952, P. O. Box 66, Riyadh Region, Saudi Arabia Correspondence should be addressed to Nasser A. N. Alzerwi; n.alzerwi@mu.edu.sa Received 17 January 2020; Accepted 14 February 2020; Published 26 February 2020 Academic Editor: Josep M. Ribera Copyright © 2020 Nasser A. N. Alzerwi. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Adenocarcinoma is the most common primary pancreatic neoplasm type, followed by secondary pancreatic lymphoma and primary pancreatic lymphoma (PPL). PPL is associated with peripancreatic lymphadenopathy and usually presents as a homogenous mass with extrapancreatic invasion into surrounding structures. However, localized involvement of the distal pancreas is uncommon, and diffuse involvement of the pancreas is even rarer. Herein, we present the case of a 53-year-old woman with PPL of the uncinate process with biliary obstruction mimicking pancreatic adenocarcinoma, successfully diagnosed nonoperatively. Abdominal computed tomography showed an ill-defined uncinate process mass, hypodense with mild enhancement (3:9×3:4×3:5cm), infiltrating the second and third parts of the duodenum. Biopsy revealed NHL with no evidence of adenocarcinoma. PPL was diagnosed. She received chemotherapy with a CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) protocol with rituximab, which she tolerated with no clinical or radiological evidence of recurrence at 1-year follow-up. 1. Introduction cutaneous icterus, itching, dark urine, pale stool, and early satiety. She had an unremarkable medical and family history. Primary pancreatic lymphoma (PPL) accounts for approxi- Systemic examination revealed no other symptoms. mately 0.5% of pancreatic neoplasms and <2% of lymphomas Upon examination, the patient looked cachectic, con- [1, 2] and commonly presents as non-Hodgkin lymphoma scious, and alert but dehydrated and deeply jaundiced. Her [3, 4]. Its clinical manifestations and radiological findings vital signs were within normal limits; she was afebrile. mimic those of other pancreatic lesions such as adenocarci- Abdominal examination revealed no scars or prominent noma, neuroendocrine tumors, and chronic pancreatitis [2]. veins; the abdomen was soft and lax with mild tenderness at The rarity, difficulty in access, dissimilar outcomes, treat- the epigastrium, with no palpable mass, organomegaly, or ment, and survival rates of PPL make it a diagnostic and lymphadenopathy. Normal bowel sounds were audible, with therapeutic challenge [3, 4]. Histological diagnosis is essential intact hernial orifices. A digital rectal exam showed unre- [5] as histological subtype is a significant prognostic factor markable results. Laboratory tests revealed direct hyperbiliru- [1, 2, 6, 7]. Herein, we present the case of a 53-year-old woman binemia (Table 1) and anemia. with PPL of the uncinate process with biliary obstruction The clinical impression was obstructive jaundice requir- mimicking pancreatic adenocarcinoma and review the litera- ing further investigation. She was admitted and prescribed ture on PPL. intravenous fluid therapy. Chest X-ray showed unremarkable results; abdominal ultrasonography (US) revealed distended gallbladder with 2. Case Presentation no stones or signs of acute cholecystitis (Courvoisier’s sign). A 53-year-old woman presented to the emergency depart- However, a tortuous cystic duct and dilatation of the intrahe- ment complaining of intermittent sharp epigastric pain patic and extrahepatic biliary ducts and the common bile radiating to the back, associated with nausea, scleral and duct (CBD) (13 mm) (Figures 1 and 2) were observed. 2 Case Reports in Oncological Medicine Table 1: Laboratory results. Test Result Units WBC 5:9× 10 Cells/L Hgb 9.7 g/dL Platelets 220 × 10 /L INR 0.9 — PT 10.4 sec PTT 39.4 sec AST 146 U/L ALT 230 U/L Figure 2: Transabdominal ultrasonography showing a dilated Alp 358 U/L common bile duct (13 mm) with no visible choledocholithiasis. TB 159.8 μmol/L DB 139.2 μmol/L Alb 33.4 g/L Amylase 22 U/L GGT 466.8 U/L Urea 3.4 mmol/L Creatinine 60.7 μmol/L K 3.8 mmol/L Na 139 mmol/L LDH 128 IU/L CEA 0.8 ng/mL CA 19-9 16 U/mL AFP 2.1 ng/mL Figure 3: Enhanced computer tomography (CT) of the abdomen. WBC: white cell count; Hgb: hemoglobin; INR: international normalized Coronal slice obtained through the second part of the duodenum ratio; PT: prothrombin time; PTT: partial thromboplastin time; AST: (D2; green arrow) shows an uncinate process mass (orange arrow) aspartate aminotransferase; ALT: alanine aminotransferase; Alp: alkaline protruding into the lumen of D2, infiltrating and obstructing the phosphatase; TB: total bilirubin; DB: direct bilirubin; Alb: albumin; GGT: dilated distal common bile duct (yellow arrow). gamma glutamyl transferase; K: potassium; Na: sodium; LDH: lactate dehydrogenase; CEA: carcinoembryonic antigen; CA 19-9: carbohydrate antigen 19-9; AFP: alpha fetoprotein; L: liter; g: gram; dL: deciliter; sec: seconds; U: unit; mmol: millimole; μmol: micromole; IU: international Abdominal computed tomography (CT), performed for unit; ng: nanogram; mL: milliliter. suspected obstructive jaundice due to malignancy, showed findings similar to US findings and an ill-defined uncinate process mass that was hypodense with mild enhancement (3:9× 3:4×3:5cm). The mass had infiltrated the second and third parts of the duodenum with no signs of duodenal obstruction and mass effect on the inferior vena cava (IVC). There was extensive lymphadenopathy (perilesional, retro- peritoneal, mesenteric, and left para-aortic) with no metasta- tic deposits in the liver (Figure 3). Chest CT showed no metastatic lung deposits or medias- tinal lymphadenopathy. Tumor markers were within the normal range (Table 1). Magnetic resonance cholangiopan- creatography (MRCP) showed diffuse intrahepatic biliary duct dilatation, a dilated CBD (13 mm), and a normal pan- creatic duct (Figures 4–7). Duodenoscopy showed mucosal erythema on stenotic and granular major papilla in the periampullary area; thus, Figure 1: Transabdominal ultrasonography showing a distended a biopsy was performed (Figure 8). Endoscopic retrograde gall bladder (102:8× 37:1mm) with a thin wall (3.1 mm) and cholangiopancreatography (ERCP) showed distal CBD and tortuous dilated cystic duct with no intraluminal gallstones or papillary stenosis, which required sphincterotomy and pericholecystic fluid. stenting, and an ampullary mass, which was biopsied (Figure 9). Biopsy revealed NHL with no evidence of adenocarcinoma (Figure 10). Whole-body multislice CT Case Reports in Oncological Medicine 3 Figure 4: Magnetic resonance imaging (MRI) of the abdomen. Figure 7: Magnetic resonance imaging (MRI) of the abdomen. Coronal slice obtained through the second part of the duodenum Axial slice obtained through the distended GB (star) shows the (D2) shows the uncinate process mass (green arrow) obstructing thin gall bladder wall (green arrow) and a thin rim of the distal common bile duct (dotted black arrow). pericholecystic fluid (orange arrow). Figure 5: Magnetic resonance imaging (MRI) of the abdomen. Coronal slice shows the line of demarcation between the distal normal body and tail of the pancreas (orange arrow) and the neoplastic proximal body (green arrow), with multiple mesenteric and peripancreatic neoplastic lymph nodes (black arrowheads). Figure 8: Duodenoscopy shows stenotic major papilla with neoplastic overgrowth (black arrow) protruding into the lumen of the second part of the duodenum (D2). were no clinical or radiological evidence of recurrence after 1 year of follow-up. Written informed consent was obtained from the patient for the publication of this report. The institutional review board of King Fahad Medical City (Riyadh, KSA, Tel: +96612889999 ext. 26913, email: okasu- le@kfmc.med.sa) waived the need for ethical approval on December 05, 2019 (IRB log number: 19-613E). 3. Discussion Figure 6: Magnetic resonance imaging (MRI) of the abdomen. The typical age of PPL patients is 35–75 years (mean: 55 Coronal slice shows the uncinate process mass (dotted black years), with a strong male predilection (male : female ratio arrow) and multiple neoplastic peripancreatic lymph nodes (orange arrows). of 7 : 1) [8]. PPL presents with typical symptoms of lym- phoma like fever, chills, and night sweats in only 2% of cases [8]. The clinical presentations of PPL are nonspecific, most was negative for generalized lymphadenopathy, and the mul- commonly abdominal pain followed by abdominal mass tidisciplinary oncology board diagnosed PPL. She received detection, weight loss, jaundice (76%), and acute pancreatitis cyclophosphamide, doxorubicin, vincristine, and prednisone (AP) (18%), which mimics adenocarcinoma. Though PPL is (CHOP) with rituximab with good tolerability, and there rare, due to the differences in the prognosis and treatment 4 Case Reports in Oncological Medicine Figure 9: Duodenoscopy shows the cannulation of the stenotic major papilla (green arrows) and the granular appearance of the neoplastic mass (black arrows). approaches of adenocarcinoma and PPL (surgical resection hematoxylin-eosin staining findings alone may resemble those of a poorly differentiated carcinoma, limiting the value for adenocarcinoma, while chemotherapy for PPL), it is critical to differentiate PPL from adenocarcinoma [1]. of fine needle aspiration cytology (FNAC) as a diagnostic PPL is most commonly located at the head of the pan- technique [11–14]. creas, though it can be found in the body or tail. The tumor The serum CA 19-9 level in PPL patients is usually nor- size ranges from 4 to 17 cm on radiological scans [9]. mal or sometimes slightly elevated in the case of biliary Imaging plays a crucial role in pancreatic mass diagnosis obstruction [15]. In our case, the CA 19-9 level was normal [1]. CT is commonly used in the detection and character- despite the clinical, biochemical, and radiological evidence ization of PPLs appearing as homogenous masses with less of biliary obstruction. contrast enhancement compared with pancreatic adenocar- The management and prognosis of PPL depend on the cinomas [1]. tumor stage and grade. The most common subtype of PPL There are two different morphological patterns of PPL: is the diffuse large B-cell subtype, and long-term remission localized, well-circumscribed tumors and diffuse tumors has been obtained with chemotherapy (CHOP); in the case infiltrating or replacing most of the pancreas [1, 10]. of CD20-positive diffuse large B-cell lymphoma, the addition The radiological criteria for differentiating PPL from of rituximab increases the remission rate [3, 15, 16]. more common pancreatic adenocarcinomas are a bulky PPL patients treated with chemotherapy alone have a bet- localized tumor in the pancreatic head, no significant dilata- ter prognosis than do pancreatic adenocarcinoma patients. tion of the pancreatic duct, enlarged lymph nodes below the PPL patients have a 5-year survival rate of 26%–66% renal vein level, invasive tumor growth, and retroperitoneal [3, 15]. The therapeutic role of radiotherapy in PPL is not yet well defined [3]. Multiple studies have shown a therapeu- or upper abdominal organ infiltration [1]. All of these criteria were fulfilled in our case. tic advantage of adjuvant radiotherapy when combined However, the definitive diagnosis of PPL is impossible with chemotherapy for NHL; however, the replication of from imaging alone, and pathologic examination (cytohisto- such superior outcomes in PPL is not as well established pathological confirmation) is imperative [1]. Immunohisto- [17–19]. Few case series studies have shown promising results, Bouvet et al. recommended the routine use of involved-field chemical staining and flow cytometry are essential as Case Reports in Oncological Medicine 5 Conflicts of Interest The author declares that there is no conflict of interest regarding the publication of this article. Acknowledgments The author would like to thank Dr. Sulimal Al-Alshaikh MD, for his help with the radiographs, and Dr. S. Almutairi MD, Dr. Bandar Idrees MD, Dr. Ahmed Alhumaid MD, Dr. A. 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Published: Feb 26, 2020

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