Cutaneous B‐cell lymphomas: 2023 update on diagnosis, risk‐stratification, and management

Abstract

Disease Overview Approximately one‐fourth of primary cutaneous lymphomas are B‐cell derived and are generally classified into three distinct subgroups: primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous marginal zone lymphoma (PCMZL), and primary cutaneous diffuse large B‐cell lymphoma, leg type (PCDLBCL, LT). Diagnosis Diagnosis and disease classification is based on histopathologic review and immunohistochemical staining of an appropriate skin biopsy. Pathologic review and an appropriate staging evaluation are necessary to distinguish primary cutaneous B‐cell lymphomas from systemic B‐cell lymphomas with secondary skin involvement. Risk‐Stratification Disease histopathology remains the most important prognostic determinant in primary cutaneous B‐cell lymphomas. Both PCFCL and PCMZL are indolent lymphomas that infrequently disseminate to extracutaneous sites and are associated with 5‐year survival rates that exceed 95%. In contrast, PCDLBCL, LT is an aggressive lymphoma with an inferior prognosis. Risk‐Adapted Therapy PCFCL and PCMZL patients with solitary or relatively few skin lesions may be effectively managed with local radiation therapy. While single‐agent rituximab may be employed for patients with more widespread skin involvement, multiagent chemotherapy is rarely appropriate. In contrast, management of patients with PCDLBCL, LT is comparable to the management of patients with systemic DLBCL.

Adolescent with primary cutaneous follicle center lymphoma treated with rituximab

“…LETTER TO THE EDITOR Primary cutaneous B-cell lymphomas are extra-nodal NHLs that include the subtypes such as primary cutaneous marginal zone B-cell lymphoma, PCFCL, primary cutaneous diffuse large B-cell lymphoma, leg type and Epstein-Barr virus (EBV)-positive mucocutaneous ulcers. [1][2][3] Patients with PCFCL are typically older males with cutaneous disease. PCFCL is very rarely seen in children.…”

“…PCFCL is very rarely seen in children. [3][4][5][6][7][8] PCFCL typically presents as a solitary lesion or several erythematous papules or nodules on the head, neck, and trunk without systemic symptoms or disseminated disease. 3 Survival rates are excellent, greater than 95%, although survival is lower if lesions are present on the lower extremities.…”

“…[3][4][5][6][7][8] PCFCL typically presents as a solitary lesion or several erythematous papules or nodules on the head, neck, and trunk without systemic symptoms or disseminated disease. 3 Survival rates are excellent, greater than 95%, although survival is lower if lesions are present on the lower extremities. 3,9 The differential diagnosis for these lesions may include follicular lymphoma, diffuse large B-cell lymphoma leg type, reactive lymphoid hyperplasia, and primary cutaneous marginal zone lymphoma.…”

“…3 Survival rates are excellent, greater than 95%, although survival is lower if lesions are present on the lower extremities. 3,9 The differential diagnosis for these lesions may include follicular lymphoma, diffuse large B-cell lymphoma leg type, reactive lymphoid hyperplasia, and primary cutaneous marginal zone lymphoma. These entities must be differentiated pathologically after biopsy is obtained.…”

“…While initial response rates are good, relapse rates vary from 30% to as high as 67% based on the study, extent of disease, and treatment choice. 3,9,11,13,15 Fortunately, relapses are generally localized, dissemination is rare, and local recurrences do not usually affect overall survival. Relapses may be treated like the initial management of PCFCL, as they are usually localized to the skin.…”