Virginia Tech Carilion School of Medicine Roanoke, VA, United States
Yazan Y. Alshawkani, BS1, Michael S. Stump, MD2, Manoj Kumar, MD1, Douglas J. Grider, MD2 1Virginia Tech Carilion School of Medicine, Roanoke, VA; 2Carilion Clinic, Roanoke, VA
Introduction: Lymphomas of the gastrointestinal tract (GIT) commonly arise from disseminated nodal disease. In contrast, primary GIT lymphomas are rare, nearly all being non-Hodgkin’s lymphomas. Hence, a primary GIT Hodgkin lymphoma is extremely rare. Presented is primary gastric mixed-cellularity classical Hodgkin Lymphoma with similar clinical and radiological features to other common gastric disease entities. Thus, ancillary immunohistochemical stains, along with a high index of suspicion, are requisite to make the diagnosis.
Case Description/Methods: An 84-year-old female with unremarkable medical, social, and family history presented with 2 months of progressively worsening diffuse abdominal pain, 15-pound weight loss, fatigue, non-bloody diarrhea, and recent dyspnea on exertion. She had no fevers/chills, chest pain, hematemesis, or palpable lump. Physical exam revealed pallor, diffuse abdominal tenderness with distension, and decreased air entry in the left lung base but no palpable mass or node. Initial labs revealed anemia (Hgb 9.8), hyponatremia (130), and hypoalbuminemia (2.7). Abdominal CT scan was suspicious for primary malignancy of the stomach or pancreas with extensive local metastatic disease, peritoneal spread, and extensive lymphadenopathy. EGD revealed a 6 cm non-bleeding ulcerated gastric mucosal tumor with a necrotic base in the proximal gastric body. Histopathology demonstrated mixed inflammatory infiltrate (small lymphocytes, neutrophils, eosinophils) and large Hodgkin’s cells, including rare Reed-Sternberg forms, that stained positive for CD15, CD30, CD45, CD79A, PAX-5, BCL6 and EBV virus. The malignant cells showed high Ki-67 proliferative index (~100%). Negative keratin CAM5.2 and CK7 stains helped exclude primary gastric carcinoma. Negative CD20 stain helped exclude large B-cell lymphoma. Staging chest CT showed multiple pulmonary nodules and necrotic mediastinal lymph nodes. Patient ultimately declined chemotherapy for her unresectable metastatic disease and was transferred to hospice care.
Discussion: Although primary GIT lymphomas are rare and most frequently of the non-Hodgkin’s variety, clinicians and pathologists should maintain a high index of suspicion for primary GIT Hodgkin lymphoma. The poor prognosis of this disease is often attributed to the difficulties in achieving the diagnosis. Careful examination of biopsies followed by appropriate immunohistochemistry, despite the clinical and radiologic findings, can help reduce delays in diagnosis and treatment.
Figure: Mixed inflammatory infiltrate with central Reed-Sternberg Cell
Yazan Alshawkani indicated no relevant financial relationships.
Michael Stump indicated no relevant financial relationships.
Manoj Kumar indicated no relevant financial relationships.
Douglas Grider indicated no relevant financial relationships.
Yazan Y. Alshawkani, BS1, Michael S. Stump, MD2, Manoj Kumar, MD1, Douglas J. Grider, MD2. P2063 - Primary Gastric Hodgkin’s Disease: A Very Rare Entity, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.