An Unusual Cause of Epigastric Pain

Abstract

Question: A 64-year-old woman was hospitalized because of epigastric pain, back soreness, and poor appetite for 3 months. Her past and family histories were unremarkable. Physical examination showed epigastric tenderness without rebound pain and right axillary lymph node enlargement. Initial laboratory examination showed abnormalities as follows: Leukocyte count, 3.75 × 103/μL; hemoglobin level, 9.2 g/dL; platelet count, 148 × 103/μL; and aspartate aminotransferase, 92 U/L. Abdominal computed tomography demonstrated no significant changes. Upper gastrointestinal endoscopy identified elevated lesions with central ulcer on the greater curvature of stomach (Figure A) and in the duodenal bulb (Figure B). Magnified endoscopic observation with narrow band imaging was performed for the gastric lesion and revealed that it was surrounded by an elevation of gastric mucosa with normal gastric pits, indicating a submucosal tumor (Figure C). What is the most likely diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Excisional biopsies taken from gastric and duodenal lesions showed anaplastic large cell lymphoma (ALCL) characterized by the presence of large, anaplastic lymphoma cells around blood vessels (Figure D). Immunohistochemically, the tumor cells were positive for CD30 (Figure E) and Ki-67, but negative for ALK (anaplastic lymphoma kinase; Figure F), CD20, CD79a, and epithelial membrane antigen. The whole-body dual-modality 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography images showed multiple increase of FDG uptake around the pons, cerebellum, C-, T-, L-spines, pelvic bones, humeral and femoral bones, mandibular bones, mediastinum, left supraclavicle, axillary, and gastrointestinal and abdominal lymph nodes (Figure G). CD30-positive ALCL is a distinctive malignant large-cell lymphoma of T-cell lineage, often presenting in lymph node or extranodal sites, such as the skin (the most common site), bone, lung, spleen, and gastrointestinal tract.1Lu Y. Zhao X. Wang E. et al.ALK-negative anaplastic large cell lymphoma with extensive peripheral blood and bone marrow involvements manifested as “leukemic phase”.Leuk Res. 2010; 34: 475-482Crossref PubMed Scopus (19) Google Scholar, 2Ross C.W. Hanson C.A. Schnitzer B. CD30 (Ki-1)-positive, anaplastic large cell lymphoma mimicking gastrointestinal carcinoma.Cancer. 1992; 70: 2517-2523Crossref PubMed Scopus (30) Google Scholar Our patient showed an extremely rare manifestation of ALK-negative ALCL with extensive involvement of multiple organs. The treatment regimens for primary systemic ALCL are the same as those used for diffuse large B-cell lymphoma.3Armitage J.O. Mauch P.M. Harris N.L. et al.DeVita Jr, V.T. Hellman S. Rosenberg S.A. Cancer: principles and practice of oncology. 6th ed. Lippincott, Philadelphia2001: 2297-2298Google Scholar Without receiving chemotherapy, our patient died of respiratory failure 1 month later.