1817 Blastic Mantle Cell Lymphoma of the Gastroesophageal Junction: A Unique Presentation

Abstract

INTRODUCTION: Though vast, the medical literature is deficient on reports of gastroesophageal junction (GEJ) involvement of mantle cell lymphoma (MCL). We present a unique case of a 76-year-old male who was found to have a blastic variant of MCL in the GEJ. This case provides additional distinct features to the various clinical presentations associated with this rare proliferative disorder, thereby enhancing the medical literature on MCL. CASE DESCRIPTION/METHODS: A 76-year-old male with a history of prostate and bladder cancer presented with worsening dysphagia, weight loss, and heartburn for over 6 months. He had extensive workup in the past for an ulcerative, gastric/GEJ lesion, found on 4 separate esophagogastroduodenoscopies (EGD) and 2 esophageal ultrasounds (EUS), whose biopsies ranged from mild gastritis to intestinal metaplasia, but were repeatedly negative for malignancy. Physical exam and labs were unremarkable except for anemia. Computerized tomography (CT) scan showed an irregular mass extending from the proximal fundus to the GEJ, and diffuse lymphadenopathy (LAD). EGD on admission showed a large friable, ulcerated lesion with heaped up margins involving the cardia, with extension into the GEJ. GEJ biopsies showed histological and immunohistochemical (IHC) findings consistent with blastic MCL. Further workup of a positron emission tomography (PET) scan showed a fluorodeoxyglucose (FDG) avid mass involving the GEJ and stomach as well as FDG avid regional LAD. A bone marrow biopsy showed minimal involvement (<5%) of CD5+/CD23+ B cells and was negative for both Cyclin D1 and t (11;14). He was started on a chemoimmunotherapy regimen (CITR) with a good response and is currently progression free after 2 years. DISCUSSION: The EGD biopsy was diffusely infiltrated by atypical lymphocytes with prominent nucleoli and IHC stains positive for CD-20, Cyclin D1, BCL-2, and BCL-6, and a Ki-67 proliferative index >90%; all consistent with blastic MCL; a rare and aggressive subtype of MCL. He was not a candidate for stem cell transplant due to his age/comorbidities, so he was started on guideline-based CITR of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone) followed by rituximab maintenance therapy. PET/EGD done 1 year later showed improvement of the LAD and GEJ mass, with negative biopsies for lymphoma. To the best of our knowledge, there have been no reported cases of GEJ involvement of blastic MCL appearing as a large friable lesion, illustrating the unique aspect of this case.