Sujay Alvencar, MD1, Erik Holzwanger, MD2, Rohit Dhingra, MD2, Aaron Dickstein, MD2; 1Tufts Medical Center, Boston, MA; 2Tufts University Medical Center, Boston, MA
Introduction: Mantle cell lymphoma (MCL) is a mature B-cell non-Hodgkin lymphoma (NHL) comprising 2-8% of NHLs. MCL tends to present at an advanced stage and has a predilection for the gastrointestinal tract. Rarely, it can present as multiple lymphomatous polyposis (MLP), which typically involves multiple polypoid lesions in the large intestine. We describe an unusual case of MLP presenting as acute appendicitis with colonoscopic findings of multiple large masses throughout the colon.
Methods: A 58-year-old man with no significant medical history presented with acute, severe right lower quadrant abdominal pain and fevers after a road trip. He had normal vitals aside from the fever of 39.1. Labs were largely normal aside from a white blood cell (WBC) count of 11.3 K/L. He underwent CT imaging which revealed an abnormal appendix with irregular wall thickening as well as other abnormal colonic lesions. He had mesenteric lymphadenopathy as well. We proceeded with a colonoscopy to obtain tissue and found multiple mass lesions throughout the colon, the largest being 10 cm and involving the proximal right colon. In addition, he had an abnormal appearing appendiceal orifice and multiple sub-centimeter polyps throughout the colon. The appearance was atypical for adenocarcinoma. Multiple biopsies were taken for tissue diagnosis. Surprisingly, all samples showed mantle cell lymphoma with immunohistochemical staining positive for CD20, BCL-2, CD5, cyclin D1. Ki-67 was 50%. Further cytogenetic testing was positive for the t(11;14)(q13;32) translocation. Bone marrow biopsy did not show MCL involvement. A PET scan showed uptake in the appendix, small bowel, sigmoid colon and mesenteric lymph nodes with no uptake above the diaphragm. He is tentatively planned for chemotherapy induction and autologous stem cell transplant with maintenance rituximab. Discussion: MLP is a rare entity that usually presents with small polypoid lesions within the gastrointestinal tract. Interestingly, this patient had multiple, large lesions throughout the large intestine including the appendix. In his case, this appendiceal involvement led to acute inflammation which brought him to medical attention. With his lack of comorbidities, he is a good candidate for aggressive chemotherapy though the average median survival for this disease is three years. Again, this case highlights the aggressive nature of MLP and the possibility of it masquerading as acute appendicitis.
Image A: Abnormal appendiceal orfice with edema and nodularity. Image B: Fungating, partially obstructing 10 cm mass involving approximately 50% of the luminal circumference in the distal cecum extending to the ascending colon. Image C: Polypoid, non-obstructing 3 cm mass found in distal ascending colon. Image D: A 10 mm erythematous, pedunculated polyp found in the sigmoid colon adjacent to the sigmoid mass.
Disclosures: Sujay Alvencar indicated no relevant financial relationships. Erik Holzwanger indicated no relevant financial relationships. Rohit Dhingra indicated no relevant financial relationships. Aaron Dickstein indicated no relevant financial relationships.