Metachronous colonic mantle cell lymphoma polyposis co-existing with metastatic appendiceal mucinous adenocarcinoma

A 54 year-old man presented recently with a 4 month history of recurring abdominal distention and loss of appetite.His past medical history was notable for low grade mucinous adenocarcinoma of appendiceal origin diagnosed five years prior.This was histopathologically characterized by paucicellular mucin pools flanked by a layer of non-stratified well-differentiated mucinous columnar cells.The tumor was metastatic to the abdominal cavity, omentum, spleen and the serosal surface of the gallbladder.Further, two years preceding the current complaint, he presented with abdominal distention thought to be due to a recurrence of the adenocarcinoma but was instead found to be a lymphoproliferative neoplasm.There were multiple polypoid lesions throughout the colonic mucosa composed of sheets of monotonous, transformed lymphoid cells in a vague nodular pattern with relatively uniform, medium-sized nuclei, mildly irregular nuclear contour and inconspicuous nucleoli.These lymphoproliferative cells displayed strong immunoreactivity to CD20, CD79a, Cyclin D1, BCL2, and CD5 but negative reactivity to CD10, CD3 and CD25 consistent with mantle cell lymphoma (MCL, multiple lymphomatous polyposis).The MCL extended in to the pericolonic adipose tissue as well as regional lymph nodes.His current presentation had clinical and radiological features concerning for pseudomyxoma peritonei and, therefore, he subsequently underwent pelvic exenteration and staging liver biopsy that revealed synchronous metastatic low grade mucinous adenocarcinoma and MCL of the colorectum, pericolonic adipose tissue, liver, prostate, bladder and ureters, as well as MCL involving the regional lymph nodes.Unfortunately, the patient succumbed to post-operative complications after two weeks.(See Figs. 1 and 2.) Synchronous or metachronous gastrointestinal adenocarcinoma, particularly gastric and colorectal, and MCL is an unusually exceptional Human Pathology: Case Reports 10 (2017) 18

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