Primary pancreatic lymphoma in a child presenting with rapidly progressive obstructive jaundice

Primary tumors rarely arise from the pancreas in patients aged <20 years, with a prevalence of approximately 0.46 cases per million and even lower among young children.1Brecht I. Schneider D. Klöppel G. Von Schweinitz D. Barthlen W. Hamre M. Malignant pancreatic tumors in children and young adults: evaluation of 228 patients identified through the Surveillance, Epidemiology, and End Result (SEER) database.Klin Pädiatr. 2011; 223: 341-345Crossref PubMed Scopus (0) Google Scholar Primary pancreatic tumors include pancreatoblastomas, solid pseudopapillary tumors, and neuroendocrine tumors, and metastasis to the pancreas was reported in patients diagnosed with leukemia, lymphoma, Ewing sarcoma, and rhabdomyosarcoma.2Patterson K.N. Trout A.T. Shenoy A. Abu-El-Haija M. Nathan J.D. Solid pancreatic masses in children: a review of current evidence and clinical challenges.Front Pediatr. 2022; 10966943Crossref Scopus (1) Google Scholar Most patients with pancreatic tumors present with abdominal pain, mass, weight loss, or recurrent pancreatitis. Obstructive jaundice is rare, seen in only approximately one-third of adult cases.3Saif M.W. Primary pancreatic lymphomas.JOP. 2006; 7: 262-273PubMed Google Scholar The tumor is commonly found during imaging studies. Herein, we describe the case of a 9-year-old boy who presented with progressive jaundice and later found to have a primary pancreatic lymphoma. A previously healthy 9-year-old boy presented with a 3-week history of progressive jaundice and intermittent abdominal pain. Direct hyperbilirubinemia and high levels of transaminases and alkaline phosphatase were noted. Complete blood count, viral hepatitis panel, serum amylase, lipase, lactate dehydrogenase (201 U/L), and common pediatric-based tumor markers (alpha-fetoprotein, cancer antigen 19-9, and beta-human chorionic gonadotropin) were unremarkable. An upper abdominal ultrasonography revealed a soft tissue density lesion measuring 2.4 × 2.8 cm2 at the head of pancreas and mild dilatation of the bilateral intrahepatic bile ducts and common bile duct. Computed tomography (CT) revealed a 4.2 × 4.1 × 3.6-cm3 faint enhancing solid mass at the head and uncinate process of the pancreas involving the mid to distal common bile duct and the second part of the duodenum, causing dilatation of the proximal common bile duct and intrahepatic bile duct (Fig. 1). The mass caused an encasement of the gastroduodenal artery and induced a pressure effect on the superior mesenteric and proximal portal veins. Exploratory laparotomy was proposed to appropriately gather the tumor's tissue given that ultrasound-guided fine-needle aspiration was very high risk. Intraoperatively, a 4-cm yellowish rubbery mass was found at the head of the pancreas without peritoneal metastasis. Histopathologically, the mass exhibited diffuse proliferation of monotonous, medium-sized lymphoid cells with basophilic cytoplasm and numerous mitoses. Many benign macrophages containing apoptotic debris were also present. The tumor cells were positive for CD20, CD10, BCL6, and c-MYC and had a Ki67 proliferation index of nearly 100 %, consistent with Burkitt lymphoma (Fig. 2). Bone marrow aspiration, chest CT, lumbar puncture, and Gallium scan showed no evidence of metastasis. Finally, the patient was diagnosed with a stage 3 Burkitt lymphoma (standard risk). Four weeks after the chemotherapy regimen (cyclophosphamide, vincristine, prednisolone, intrathecal methotrexate, and cytarabine), CT revealed reduction in the size of the pancreatic mass to 1.5 × 1 cm2. The patient recovered well, with a resolution of jaundice. Six months after the last chemotherapy session, repeated CT and Gallium scan revealed complete tumor resolution. Differentiating the characteristics of pancreatic tumors based on the age group, imaging appearance, and histopathological finding is crucial. Pancreatic lymphoma can occur primarily but more commonly as a metastasis site (i.e., secondary tumor). Primary pancreatic lymphoma occurs in <2 % of all extranodal non-Hodgkin's lymphoma (NHL) and 0.5 % of pancreatic masses.4Nistala S.S. Sawalakhe N.R. Thiruvengadam N.R. Rathi P.M. A rare case of primary pancreatic Burkitt lymphoma in a young Indian male. Case report and review of the literature.JOP. 2009; 10: 686-689PubMed Google Scholar Burkitt lymphoma is the most common type of NHL, with the ileocecal region being the main site of its gastrointestinal involvement.5Athmani I. Le Rouzic M.-A. Valduga J. Mansuy L. Contet A. Galloy M.-A. et al.Pediatric pancreatic Burkitt lymphoma with obstructive jaundice: case report of a six-year-old child.Arch Clin Cases. 2017; 4Crossref Google Scholar Pediatric case reports of primary pancreatic Burkitt lymphomas are sparse.4Nistala S.S. Sawalakhe N.R. Thiruvengadam N.R. Rathi P.M. A rare case of primary pancreatic Burkitt lymphoma in a young Indian male. Case report and review of the literature.JOP. 2009; 10: 686-689PubMed Google Scholar Primary pancreatic lymphoma is an extranodal lymphoma arising in the pancreas, with the bulk of the disease localized to this site. Among the histologic variants of NHL, large-cell and Burkitt lymphomas are the most common types in children. Burkitt lymphoma, a type of NHL, is characterized by translocation and deregulation of the c-myc gene on chromosome 8. Most patients with Burkitt lymphoma present with large abdominal mass, frequently involving the ileocecal region. Pancreatic involvement is extremely rare and may be a sign of a preterminal disease. Clinically apparent obstructive jaundice is rare because it is seen in only approximately one-third of adult cases.3Saif M.W. Primary pancreatic lymphomas.JOP. 2006; 7: 262-273PubMed Google Scholar However, a recent study reported that children were more likely to present with jaundice (62 % vs. 40 % in adults).6Facchinelli D. Boninsegna E. Visco C. Tecchio C. Primary pancreatic lymphoma: recommendations for diagnosis and management.J Blood Med. 2021; 12: 257-267Crossref PubMed Scopus (5) Google Scholar CT findings of pancreatic lymphoma include a well-circumscribed, homogeneous, sometimes bulky infiltrating mass with mild contrast enhancement. The tumor is commonly located at the pancreatic head (61.9 % vs. 63.8 % in adults)6Facchinelli D. Boninsegna E. Visco C. Tecchio C. Primary pancreatic lymphoma: recommendations for diagnosis and management.J Blood Med. 2021; 12: 257-267Crossref PubMed Scopus (5) Google Scholar; however, any location is possible. Diffuse pancreatic enlargement, multiple noncontiguous masses, or large lymph nodal masses may be also present. After collecting appropriate histopathological sampling, the mainstay treatment for primary lymphoma is chemotherapy. Primary surgery is reserved for treatment failure or diagnostic uncertainty. Chemotherapy in primary pancreatic lymphoma results in a high complete response rate (93.3 %) and provides a long disease-specific survival, with a median follow-up of 56 months. Multiple chemotherapy regimens, including cyclophosphamide, doxorubicin, vincristine, and prednisolone with the addition of rituximab, improve the response rates for diffuse large B-cell lymphoma.2Patterson K.N. Trout A.T. Shenoy A. Abu-El-Haija M. Nathan J.D. Solid pancreatic masses in children: a review of current evidence and clinical challenges.Front Pediatr. 2022; 10966943Crossref Scopus (1) Google Scholar Overall, most children with primary pancreatic tumors have a better prognosis than adults, with a disease relapse rate of 6.7 % vs. 63.8 % in adults.6Facchinelli D. Boninsegna E. Visco C. Tecchio C. Primary pancreatic lymphoma: recommendations for diagnosis and management.J Blood Med. 2021; 12: 257-267Crossref PubMed Scopus (5) Google Scholar Primary pancreatic lymphoma is rare but has an excellent prognosis in children. This presented case highlights that an atypical etiology such as various pancreatic tumors should always be included in the differential diagnosis of progressive jaundice in pediatric patients.