Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type

Comments:
Introduction: Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT), is an aggressive lymphoma that makes up 4% of all cutaneous lymphomas and about 20% of primary cutaneous B-cell lymphomas.

It presents as rapidly enlarging, multiple red or purplish tumors on one or both lower legs in elderly females (median age 78 years). Less commonly, the lesions consist of patches or plaques that may undergo necrosis or ulceration. About 20% of cases involve non-leg sites, such as trunk, upper limbs, head and neck. Between 5-20% of cases have multifocal disease. Spread to extracutaneous sites is frequent (17-47%), including lymph nodes, viscera, and the CNS.

The treatment is similar to that for systemic diffuse large B-cell lymphoma and consists of anti-CD20 antibody Rituximab + CHOP-like regimen. Relapses occur in 55-69% of cases. With the addition of rituximab, the 5-yr disease-specific survival rate has increased from 50% to about 70%.

Image Source: Dumont M, Battistella M, Ram-Wolff C, Bagot M, de Masson A. Diagnosis and Treatment of Primary Cutaneous B-Cell Lymphomas: State of the Art and Perspectives. Cancers. 2020; 12(6):1497. Cropped from the original image and used under Creative Commons Attribution License.