LYMPHOMATOUS POLYPOSIS - A NEOPLASM OF EITHER FOLLICULAR MANTLE OR GERMINAL CENTER CELL ORIGIN

Lymphomatous polyposis (LP) is generally thought to be an expression o f non-Hodgkin's lymphoma (NHL) of follicular mantle cell (MC) origin. We report nine patients with LP from more than 3,500 cases of NHL stud ied by the Nebraska Lymphoma Study Group. Our patients differ from tho se reported previously in that LP represented a follicular center cell (FCC) NHL in two of the nine cases, with the remainder consisting of MC NHL. Three patients developed LP during a relapse of previously dia gnosed and treated extraintestinal MC NHL (parotid gland, tonsil, and inguinal lymph node, respectively), whereas the other six patients pre sented with primary LP. In seven of the nine LP cases, a large mass pr edominated among a myriad of small polyps. The FCC cases were confined to the small intestine, whereas the MC cases were either pan-intestin al or colonic on their localization. Two MC cases studied by Southern blotting exhibited rearrangement of the bcl-l locus. Bcl-2 rearrangeme nt was not detected in any of the nine cases when studied by either a polymerase chain reaction-based assay (seven cases) or by Southern blo tting (two cases). To date four patients (three MC, one FCC) have expe rienced recurrent NHL in gastrointestinal sites. With follow-up rangin g from 13 to 147 months, the entire group had a median survival of 41 months (primary MC LP: 13, 13, 41, and 77 months; primary FCC LP:45 an d 147 months; secondary MC LP:17, 41, and 76 months), and only one pat ient has died. We conclude that LP is a rare manifestation of NHL of e ither follicular MC or germinal center cell origin.