Walter Reed National Military Medical Center Bethesda, MD, United States
Andrew T. Mertz, MD1, Justin James D. Devera, DO1, Sarah Ordway, MD1, Jeffrey T. Laczek, MD, FACG1, Luz Maria M. Rodriguez, MD, FACS2 1Walter Reed National Military Medical Center, Bethesda, MD; 2National Cancer Institute, Rockville, MD
Introduction: Epstein Barr virus (EBV) infection in immunosuppressed patients is commonly associated with B-cell lymphoproliferative disorders (LPDs). Patients with a B-cell LPD may present with mucocutaneous ulceration in various locations of the gastrointestinal tract that rarely involves the rectum. We present a case of a patient who initially presented with anorectal pain, ultimately found to have an EBV-positive mucocutaneous ulcer.
Case Description/Methods: A 92-year-old man (ECOG status 0) with myasthenia gravis on long-term immunosuppression presented with six-months of progressive fecal incontinence and dyschezia. He also endorsed 20lb unintentional weight loss over the year prior to presentation. Recent cross-sectional imaging of the abdomen was significant for multiple solitary pulmonary nodules. Anoscopy demonstrated a 10 x 3mm excoriated anterolateral lesion extending into the perianal region on the right side. Colonoscopy revealed a circumferential ulcer in the distal rectum near the anal verge. Biopsies of the ulcer edge showed clonal B-cell proliferation with EBV-encoded small RNA (EBER)-positivity, indicating either EBV-induced LPD versus EBV-positive large B-cell lymphoma. Positron emission tomography demonstrated hypermetabolism in the anorectum without other metabolically active foci. Repeat biopsies were obtained from the ulcer base on flexible sigmoidoscopy that were consistent with EBV-positive mucocutaneous ulcer. Concurrently, a brain MRI performed for vertiginous symptoms revealed a synchronous 1.4cm lesion with associated vasogenic edema located in the left frontoparietal vertex. Mycophenolate mofetil was held and weekly rituximab was administered for a total of 4 cycles. The patient achieved complete remission as evidenced by the resolution of both perianal symptoms and the frontoparietal lesion on follow-up head imaging.
Discussion: EBV is a ubiquitous virus with potentially life-threatening oncogenic potential. This case demonstrates the classic association of EBV-associated LPDs in a chronically immunosuppressed patient, although in an uncommon location within the gastrointestinal tract. It is critical to consider the mucocutaneous ulcer phenotype of these LPDs in immunosuppressed patients due to the risk for progression to lymphoma and relative ease of treatment. When treated expeditiously, these lesions are exquisitely responsive to reduction of immunosuppressive therapy in conjunction with targeted monoclonal antibody therapy.
Figure: Figure 1. Retroflexed view of ulcerative lesion located at the anal verge. Figure 2. Axial and coronal PET images demonstrating increased focal metabolic activity in the anorectum.
Andrew Mertz indicated no relevant financial relationships.
Justin James Devera indicated no relevant financial relationships.
Sarah Ordway indicated no relevant financial relationships.
Jeffrey Laczek indicated no relevant financial relationships.
Luz Maria Rodriguez indicated no relevant financial relationships.
Andrew T. Mertz, MD1, Justin James D. Devera, DO1, Sarah Ordway, MD1, Jeffrey T. Laczek, MD, FACG1, Luz Maria M. Rodriguez, MD, FACS2. P2282 - Malignant Rectal Ulcer: An Atypical Presentation of an Omnipresent Virus, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.