TCL-224: Not All Pancreatic Masses Are Pancreatic Adenocarcinomas

Abstract

Introduction T-cell acute lymphoblastic leukemias (T-ALL) and T-cell lymphoblastic lymphomas (T-LBL) are both lymphoblastic neoplasms involving the blood and bone marrow, respectively. T-LBL typically present with lymphadenopathy and/or a mediastinal mass. We describe a case of T-LBL, which presented as a pancreatic malignancy. Case Report A 42-year-old man with no significant past medical history presented with jaundice of 10 days duration. Physical examination was unremarkable, while CT of the abdomen showed a pancreatic head mass 3.2 x 2.9 cm engulfing the superior mesenteric vein with retroperitoneal lymph node enlargement. Significant labs included total bilirubin 16.3 mg/dL, direct bilirubin 9.5 mg/dL, CA19-9 10 U/mL, LDH 161 units/L, and no cytopenia. He underwent an endoscopic ultrasound (EUS)-guided fine needle aspiration (FNA), along with ERCP with distal common bile duct stent placement. Initial pathology was indeterminate. Chest CT revealed a 7.8 x 5.1 cm lobulated heterogeneous anterior mediastinal mass involving the anterior and lateral pericardial surface, thus precluding biopsy due to high risk of vascular injury. Repeat EUS-guided FNA of the pancreatic mass revealed T-LBL. Cerebrospinal fluid (CSF) analysis showed clonal T lymphocytes positive for CD3, CD4, and CD5, and T-cell rearrangement, while bone marrow biopsy revealed no lymphomatous involvement. After the diagnosis of stage IVA extramedullary T-LBL with CNS involvement, he received six cycles of Hyper-CVAD chemotherapy and intrathecal methotrexate. After achieving complete remission, he next underwent autologous stem cell transplant (ASCT) with a conditioning regimen of total body irradiation and etoposide. At five years follow-up, the patient remained in remission but developed worsening cytopenia and a bone marrow biopsy revealed treatment-related myelodysplastic syndrome (T-MDS) with monosomy 7. Subsequently, he underwent a haploidentical allogeneic stem cell transplant, with a follow-up bone marrow biopsy showing no evidence of MDS. Discussion The differential diagnoses of pancreatic masses include pancreatic adenocarcinoma, chronic pancreatitis, neuroendocrine tumor, or lymphoma. To our knowledge, this is possibly the first case of extramedullary T-LBL presenting as a pancreatic mass. Risk-adaptive treatment strategy incorporating hyper-CVAD chemotherapy and early ASCT was effective in achieving long-term remission and is potentially an effective treatment strategy in T-LBL.