Plasmablastic lymphoma of the rectum: case report of a rare location and literature review

Abstract

Plasmablastic lymphoma (PBL) is an aggressive and rare variant of diffuse large B-cell lymphoma, which is thought to occur in immunocompromised individuals, specifically HIV-positive patients. We report the case of a 27-year-old Saudi male with PBL. The patient had a low CD4 count at presentation, however, he was HIV negative at the time of diagnosis; also Human herpesvirus-8 was negative on immunohistochemical stain, but Epstein–Barr virus showed expression in scattered cells through the utilization of EBV-EBER.

Plasmablastic lymphoma in an HIV-negative patient with complete remission after chemotherapy with DA-EPOCH

“…In images such as computed tomography or nuclear magnetic resonance, most cases of lymphoma of the gastrointestinal tract usually present as a concentric mural mass that infiltrates adjacent tissues. It may or may not cause luminal stenosis, as well as the formation of fistulas [2,6]. In the present case, there is evidence of wall thickening with the involvement of the ischiorectal fossa, levator ani muscle, obturator internus, seminal vesicles, and the formation of a complex anorectal fistula.…”

“…Isolated cases of plasmablastic lymphoma have been reported in the rectum with a presentation similar to that of the case presented, such as a rectal mass with signs of local inflammation. The mass tends to be painful on palpation, friable to the touch and protrudes through the anal canal with local invasion of surrounding tissues, which can be accompanied by rectal bleeding and pain on defecation [5][6][7][8]. Only scarce cases of plasmablastic lymphoma of the rectum have been reported in HIVnegative patients.…”

“…Only scarce cases of plasmablastic lymphoma of the rectum have been reported in HIVnegative patients. Table 1 summarizes the clinical features of plasmablastic lymphoma of the rectum in HIV-negative patients [6,[8][9][10][11].…”

“…Among the prognostic factors, complete remission after chemotherapy, Ki-67 expression greater than 80%, and male sex stand out, with response to chemotherapy being the most associated with total survival [6][7][8].…”

“…There is currently no standard handling for PL. However, several organizations recommend CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)chemotherapy schemes and more intense chemotherapy schemes such as EPOCH (infusion of etoposide, vincristine, doxorubicin + bolus of cyclophosphamide and prednisone), CODOX-M/IVAC (cyclophosphamide, vincristine, doxorubicin, methotrexate altering with ifosfamide, etoposide and cytarabine), and hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone) [6][7][8].…”