Primary cutaneous marginal zone B-cell lymphoma: response to treatment and disease-free survival in a series of 137 patients

Abstract

Background: Primary cutaneous marginal zone B-cell lymphomas are low-grade lymphomas running an indolent course. Skin relapses have been frequently reported but little information about disease-free survival (DFS) is available.

Objective: We sought to evaluate relapse rate and DFS in patients with primary cutaneous marginal zone B-cell lymphomas.

Methods: Clinical features, European Organization for Research and Treatment of Cancer/International Society for Cutaneous Lymphomas stage, light chain restriction, clonality, treatments, skin relapses, DFS, stage progression, extracutaneous disease, and outcome are analyzed in a series of 137 patients.

Results: Patients were classified as solitary lesion (T1) (n = 70; 51%), regional skin involvement (T2) (n = 40; 29%), and generalized skin lesions (T3) (n = 27; 20%). Surgical excision, local radiotherapy, or a combination were the initial treatment in 118 patients (86%). In 121 of 137 patients (88%) a complete remission was observed after initial treatment, including 99 of 106 patients (93%) with solitary or localized disease and 22 of 31 patients (71%) with multifocal lesions. Cutaneous relapses were observed in 53 patients (44%). Median DFS was 47 months. Patients with multifocal lesions or T3 disease showed higher relapse rate and shorter DFS. No significant differences were observed between surgery and radiotherapy, but surgery alone was associated with more recurrences at initial site. Overall survival at 5 and 10 years was 93%. Six patients (4%) developed extracutaneous disease during follow-up.

Limitations: This was a case series retrospective study.

Conclusion: Our results support long-term follow-up in patients with primary cutaneous marginal zone B-cell lymphomas. Disseminated skin lesions have higher relapse rate and shorter DFS suggesting further investigation on systemic therapies in such a group of patients.

Keywords: B-cell lymphoma; CR; DFS; EORTC; European Organization for Research and Treatment of Cancer; ISCL; IV; International Society for Cutaneous Lymphomas; PCMZBCL; PR; WHO; World Health Organization; complete remission; disease-free survival; extracutaneous disease; intravenous; partial remission; primary cutaneous marginal zone B-cell lymphoma; skin relapses; treatment.