Clinicopathological features of histological transformation from extranodal marginal zone B‐cell lymphoma of mucosa‐associated lymphoid tissue to diffuse large B‐cell lymphoma: an analysis of 467 patients

Abstract

This study analysed incidence, patient outcome, immunophenotype and prognostic factors of histological transformation (HT) from extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) to diffuse large B-cell lymphoma (DLBCL) in 467 patients (median age, 61 years). The primary sites of MALT lymphoma were the stomach (43%), ocular adnexa (25%), lung (8%), systemic (8%) and other tissues (16%). HT occurred in 8% of MALT lymphomas. Risk of HT by 15 years was 5%: 4% in limited-stage diseases (n = 385) and 16% in advanced-stage diseases (n = 56) (P = 0·02). The median time to HT was 48 months (range, 4-139). Five-year progression-free survival (PFS) and overall survival (OS) rates after HT were 80% and 94%, respectively. Immunohistochemical results of DLBCL were as follows: germinal centre B-cell (GCB)/non-GCB, 37%/63%; CD10, 9%; BCL6, 59%; MUM1, 38%; MYC, 42%; BCL2, 35%; Ki67 ≥ 90%, 23%; and CD5, 3%. The majority (75%, 9/12) of GCB-type DLBCLs exhibited CD10(-) , BCL6(+) and MUM1(-) immunophenotypes; the remainder had CD10(+) immunophenotypes. Multivariate analysis revealed that only advanced stage at HT was a significant adverse factor for PFS (P = 0·037). Thus, overall risk of HT was low and prognosis after HT was favourable; however, in advanced-stage cases, risk of HT was relatively high and prognosis was unfavourable.

Composite Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma and Epstein-Barr virus-negative diffuse large B-cell lymphoma in the parotid salivary gland of a patient with Sjögren’s syndrome and rheumatoid arthritis: a case report

“…HGAL, Bcl-6, and MUM1 expression was upregulated in DLBCL compared with MALT lymphoma. The development of Bcl-6 and MUM1 expression in DLBCL is described in histological transformation of MALT lymphoma [23] and does not contradict our original assumption. However, the absence of EBER-ISH and EBV DNA in DLBCL in our patient promotes the notion that the DLBCL was not clonally related to the MALT lymphoma.…”

Gastric MALT lymphoma in a population‐based study in France: clinical features, treatments and survival

“…27 The number of biopsies obtained was indicated in 96% of pathology reports (398/416). The median number of biopsies per patient was 6 (range [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. A second pathology analysis by an expert pathologist before treatment was mentioned in 232 of cases (56%).…”

“…GML is usually a localised disease, and very rarely does it disseminate. Besides dissemination, the main risk in this usually indolent lymphoma is its transformation into the high grade diffuse large B‐cell lymphoma, which is considered very rare (<1%) …”

Successful Treatment of Catastrophic Antiphospholipid Antibody Syndrome Associated with MALT Lymphoma by Autologous Hematopoietic Stem Cell Transplantation

“…Although we were unable to reach a histopathological diagnosis at the progression of lymphoma, histopathological transformation to aggressive lymphoma was suggested because of the progression of lymphoma after chemotherapies with the occurrence of CAPS. Patients with MALT lymphoma generally have an indolent clinical course, with a reported 5-year overall survival (OS) rate of 89% (13). Histopathological transformation to aggressive lymphoma occurs in 8% of MALT lymphoma cases and has been reported as a risk factor for an unfavorable outcome (13,14).…”

“…Patients with MALT lymphoma generally have an indolent clinical course, with a reported 5-year overall survival (OS) rate of 89% (13). Histopathological transformation to aggressive lymphoma occurs in 8% of MALT lymphoma cases and has been reported as a risk factor for an unfavorable outcome (13,14). Patients who are refractory to initial therapy or demonstrate an advanced relapse or transformation to aggressive lymphoma have a shorter time to progression and OS than those who are responsive to therapy and stable.…”