2676 An Extranodal Presentation of Peripheral T-Cell Lymphoma, Not Otherwise Specified as Primary Gastric Lymphoma, With Coexistence of HTLV-1

Abstract

INTRODUCTION: Peripheral T- Cell Lymphoma, not otherwise specified ( PTCL-NOS) is a subtype of T-cell lymphoma that typically presents as primary nodal lymphoma, however can rarely present as primary extranodal lymphomas with stomach being the most common extranodal site. Here we present a unique case of PTCL-NOS gastric lymphoma with coexistence of HTLV-1, which is usually associated with Adult T-cell lymphoma/leukemia. CASE DESCRIPTION/METHODS: A 58 year old man with known hypertension and a chief complaint of abdominal discomfort, fatigue, occasional black stools and unintentional 10 lb weight loss of 2 months duration was seen in clinic. Vital signs, physical exam were normal except for conjunctival pallor. Labs revealed Hb of 7 gm/dL and elevated LDH. CT scan of the abdomen revealed an 11 × 12 × 12.6 cm poorly defined inseperable mass, in the left upper abdomen (Figure 1). Upper endoscopy (EGD) with Endoscopic ultrasound (EUS) revealed a large, ulcerated, nearly circumferencial, fungating mass that appeared to erode through the cardia of the stomach. Biopsy and immunohistochemical (IHC) staining revealed pleomorphic lymphocyte cells positive for CD2, CD3, CD5, CD4(70%), CD7 (20%), CD8 (30%), CD43 and CD45 (20%) (Figure 2). KI-67 index was 80% suggestive of high grade PTCL-NOS, with necrosis (Figure 3). H. pylori and HTLV-1 antibody were positive. Pretreatment PET scan and bone marrow biopsy did not reveal additional site involvement. He was subsequently started on CHOEP regimen and triple therapy for H.Pylori. Repeat imaging after the 4th cycle revealed an interval decrease in the size of the stomach mass with no new lesions. DISCUSSION: Primary Gastric lymphomas account for 3-4% of all the stomach malignancies, mostly B-cell (85-90%) in origin followed by PTCL-NOS. Biopsy is obtained by EGD in most cases and EUS is being increasingly used to evaluate for locoregional spread. Early diagnosis and initiation of treatment with CHOP/CHOEP regimen is crucial for better outcomes. Pan imaging, FDG PET, bone marrow biopsy are critical for staging, tumour activity and for followup to check for response. Radiation, surgery, bone marrow transplantation are used as adjuvant to chemotheraphy in select patients. Coexistence of HTLV-1 in a PTCL-NOS patient make our case unique and this gives scope for further research to verify if there is any association exists between PTCL-NOS and HTLV-1.