Primary Gastric Hodgkinʼs Lymphoma

Abstract

Introduction: Hodgkin's Lymphoma is a B-Cell lymphoma, that arises from germinal center or post-germinal center B cells. It accounts for 10 % of all lymphomas and approximately 0.6 % of all cancers diagnosed in the developed world annually. The gastrointestinal (GI) tract is the predominant site for extra-nodal involvement in lymphoma, with the stomach being the most common site. The GI tract is more commonly involved as secondary site than a primary site of involvement. We report a case of primary gastric Hodgkin's lymphoma. Case discussion: A 70-year-old woman presented with fever and significant weight loss. Contrast-enhanced computed tomography of the abdomen showed thickened gastric wall along the greater curvature and multiple enlarged peri-gastric lymph nodes. An upper endoscopy revealed an ulcerative lesion in the proximal body (a). Biopsies showed clusters of lymphocytes and occasional atypical cells with an “owl's eye” appearance, resembling Reed-Sternberg cells. Immunohistochemical stains were positive for CD 30, weakly positive for PAX5 and negative for CD 45, confirming Hodgkin's lymphoma. Positron emission tomography showed distant involvement of pulmonary lymph nodes. Patient was initiated on chemotherapy with adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD) for stage IV disease. Stomach is the predominant extra-nodal site involved in lymphoma, usually as a secondary site. Occasionally, non-Hodgkin's lymphoma such as MALToma or diffuse large B cell lymphoma can present as primary gastric lymphoma. However, primary gastric Hodgkin's lymphoma is extremely rare. Diffuse mucosal involvement of the stomach and absence of prominent extra-gastric disease suggest primary gastric Hodgkin's lymphoma. Biopsies may not show the typical Reed-Sternberg cells; immunohistochemical stain is necessary for a definitive diagnosis. Prognosis is largely unknown with no clear guidelines for treatment, because of its rarity. Similar to prior reports, our patient responded favorably to chemotherapy and remains asymptomatic at 10 months. Conclusion: Primary gastric lymphoma can present insidiously without upper GI symptoms and can be easily missed in under-distended stomach on CT; but, attention to surrounding enlarged lymph nodes and history of constitutional symptoms should prompt evaluation with upper endoscopy with biopsies.FigureFigure