Table 3: Summary of etiopathogenesis and characteristic clinical features of primary cutaneous B-cell lymphoma variants.
|
|
PCDLBCL-LT |
PCFCL |
PCMZL |
|
Epidemiology
|
- 20% of all primary B-cell lymphomas - seventh decade - female predominance [11] |
- 60% of all primary B-cell lymphomas - fifth decade - male predominance[3] |
- fifth and sixth decades - male predominance [12] |
|
Pathogenesis |
- no identifiable risk factors or hereditary tendencies [13] - MYC, BCL6, IGH, MALT1, CDKN2A/2B, PDL1/2, FOXP1, PAX5, PIM1, MYD88, CARD11, CD79B, and TNFAIP3/A20 mutations [10, 13] - Borrelia burgdorferi [6] |
||
|
Clinical |
- violaceous papules, plaques, or nodule(s) +/- ulceration - multifocal - 80% leg involvement - aggressive - high relapse rate, in general - 60% extracutaneous dissemination [1,3,11] |
- violaceous papules, plaques, or nodule(s) +/- ulceration - 75% solitary - head/scalp > trunk - 5% on the legs - indolent - 10% extracutaneous dissemination [1,3] |
- violaceous papules, plaques, or nodule(s) +/- ulceration - 50% solitary - trunk > arms - indolent - higher relapse rate if multiple skin lesions present - extracutaneous dissemination rare [1,3,12] |
|
First-line therapy [4] |
Polychemotherapy |
Radiotherapy, surgery |
Radiotherapy, surgery |
|
Additional therapies [4] |
- Radiotherapy, surgery - Monoclonal antibodies - Lenalidomide - Ibrutinib |
Polychemotherapy |
Polychemotherapy |
|
Prognosis |
5-year overall survival, 50-60% [1] |
5-year overall survival, 95% [1] |
5-year overall survival, 97% [8] |
PCDLBCL-LT: primary cutaneous diffuse large B cell lymphoma- leg type; PCFCL: primary cutaneous follicle center lymphoma; PCMZL: primary cutaneous marginal zone lymphoma; MYC: proto-oncogene; BCL6: B-cell lymphoma 6; IGH: Immunoglobulin heavy locus; MALT1: Mucosa-associated lymphoid tissue lymphoma translocation protein 1; CDKN2A/2B: cyclin-dependent kinase inhibitor 2A/2B, PDL1/2: programmed deathâ1/2; FOXP1: Forkhead box P; PAX5: Paired Box 5; PIM1: proto-oncogene and serine/threonine kinase; MYD88: Myeloid differentiation primary response protein; CARD11: Caspase recruitment domain-containing protein 11; CD79B: Cluster of differentiation 79B; TNFAIP3/A20: Tumor necrosis factor, alpha-induced protein 3 or A20; Polychemotherapy, e.g. rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone.