Mantle cell lymphoma (MCL) tends to disseminate into the gastrointestinal (GI) tract. In fact, many patients with MCL may have asymptomatic disease in the GI tract at the time of diagnosis.1,2 On the other hand, it is quite unusual to find MCL only in the GI tract, without disease involvement in the rest of the body, such as lymph nodes, liver, spleen, bone marrow, or other extranodal sites. Since the first introduction of the concept of mantle-zone lymphoma by Weisenburger et al in 1982,3 there have been only some case reports and small case series published on primary GI MCL.4,5
Primary GI MCL could be a single lesion, multiple lesions in a specific GI segment, or more disseminated GI involvement. The practice pattern is rather diverse among clinicians, ranging widely from surgical resection alone to intensive systemic therapy followed by autologous stem cell transplant (ASCT). Since primary GI MCL is extremely rare, randomized clinical studies are not feasible.
It is unknown whether the clinical outcomes of patients with primary GI MCL are comparable to those with secondary GI MCL (i.e., primary nodal MCL with simultaneous, biopsy-proven GI involvement at diagnosis). We, therefore, reviewed our Mayo Clinic Clinical Database to determine clinical characteristics, treatment patterns, and outcomes of patients with primary GI MCL relative to that of secondary GI MCL.6 We also investigated the predictive role of disease extent on outcomes of patients with primary GI MCL.
Following an extensive review of medical records, we identified 22 patients with primary GI MCL (less than <3% of all MCL) and 79 with secondary GI MCL (~10% of all MCL). All of them had biopsy-proven disease in the GI tract. The majority of patients had an initial GI symptom leading to the diagnosis of GI MCL. Baseline characteristics were generally similar between primary and secondary GI MCL, yet patients with primary GI MCL had fewer elevations in LDH and a trend for lower MCL international prognostic index. These findings suggest a lower disease burden in patients with primary GI MCL.
Overall, patients with primary GI MCL were treated in a less aggressive manner than those with secondary GI MCL. The management also differed among patients with primary GI MCL. Patients with a single lesion were more likely to be observed or treated with surgery or radiation alone, while patients with a greater extent of tumor were more likely to receive intensive systemic therapy and ASCT. Despite these variations, primary and secondary GI MCL had similar survival outcomes, so did cases with different extents of GI involvement in the primary GI MCL group.
Our study on primary GI MCL suggests that for patients with primary GI MCL, less aggressive initial treatment may be reasonable, especially for those with a lower disease burden. Relapses, however, are expected for the majority of patients. We do not know whether more aggressive upfront therapy can lead to better outcomes.
1 Romaguera JE, Medeiros LJ, Hagemeister FB, Fayad LE, Rodriguez MA, Pro B et al. Frequency of gastrointestinal involvement and its clinical significance in mantle cell lymphoma. Cancer 2003; 97: 586–591.
2 Salar A, Juanpere N, Bellosillo B, Domingo-Domenech E, Espinet B, Seoane A et al. Gastrointestinal involvement in mantle cell lymphoma: a prospective clinic, endoscopic, and pathologic study. Am J Surg Pathol 2006; 30: 1274–1280.
3 Weisenburger DD, Kim H, Rappaport H. Mantle-zone lymphoma: a follicular variant of intermediate lymphocytic lymphoma. Cancer 1982; 49: 1429–1438.
4 Abrisqueta P, Scott DW, Slack GW, Steidl C, Mottok A, Gascoyne RD et al. Observation as the initial management strategy in patients with mantle cell lymphoma. Ann Oncol Off J Eur Soc Med Oncol 2017; 28: 2489–2495.
5 Dasappa L, Suresh Babu MC, Sirsath NT, Suresh TM, Govind Babu K, Sathyanarayna V et al. Primary gastrointestinal mantle cell lymphoma: a retrospective study. J Gastrointest Cancer 2014; 45: 481–486.
6 Castellino A, Tun AM, Wang Y, Habermann TM, King RL, Ristow KM et al. Clinical characteristics and outcomes of primary versus secondary gastrointestinal mantle cell lymphoma. Blood Cancer J 2021; 11: 8.