Introduction: Mantle cell lymphoma (MCL) is a subtype of non-Hodgkin lymphoma accounting for about 5% of non-Hodgkin lymphomas. Usual presentation is with lymphocytosis or widespread lymphadenopathy, extranodal manifestations involving bone marrow and GI tract are also common. We describe a case of MCL with GI involvement presenting as bilateral eyelid swelling.
Case Description/Methods: An 80-year-old Caucasian male presented to his ophthalmologist for swelling of his bilateral lower lids for the past few months. He denied any visual disturbance or any other associated symptoms. His past medical history was significant for hypertension. He worked as a farmer, with occupational exposure to glyphosate-based insecticides, and smoked a pack of cigarettes per day for 20 years. Physical exam was significant only for bilateral lower eyelid swelling (Figure 1A). Lab work was unremarkable. The ophthalmologist suspected amyloidosis, and a biopsy was sent to diagnose the underlying etiology. He then immediately referred the patient to oncology as biopsy had revealed MCL. GI was consulted to evaluate involvement of the GI tract. EGD showed subtle mucosal irregularity with irregular vascular pattern seen on the fundus and body of the stomach (Figure 1B), the duodenum was unremarkable. Colonoscopy showed normal colon mucosa. Biopsies confirmed mantle cell lymphoma involvement to the stomach (Figure 1C) and the duodenum, with minimal involvement to the colon. There was no evidence of H. pylori. Bone marrow biopsy was also positive for MCL. PET scan showed increased activity in the skin of the nose, enlarged lymph nodes in the mediastinum and inguinal region. It was determined to be stage IV mantle cell lymphoma. Patient was then referred to Radiation Oncology for evaluation of involved site radiation therapy (ISRT) of the eyelids and then targeted therapy with acalabrutinib rather than chemotherapy, given his age.
Discussion: Romaguera et al. described that 88% of patients with MCL have lower GI tract involvement, and 43% have upper GI tract involvement. Similar studies have since reaffirmed this association. Our case demonstrates that GI involvement with MCL can be seen despite normal endoscopic examination or with subtle mucosal changes, so biopsy should be considered. Eyelid swelling or mass is a rare presentation of MCL and a high degree of suspicion is required for diagnosis of MCL with this rare presentation.
