PCMZL |
Red-violaceous small solitary or multiple papules or nodules and rarely plaques |
Trunk, arms or head |
Indolent |
CD20 +, CD79a +, BCL2 +, CD5-, CD10-, BCL 6-, MUM 1 - |
Radiotherapy or surgical excision, topical drugs, intralesional therapies, immunochemotherapy |
PCFCL |
Solitary or grouped erythematous or erythemato-violaceous papules, plaques, and/or nodules |
Trunk, head or neck |
Indolent |
CD20+, CD79a+, CD5-, CD10+/-, BCL 6+, BCL2-, MUM-1/IRF-4 negative |
Radiotherapy or surgical excision, topical drugs, intralesional therapies, immunochemotherapy |
PCDLBCL |
Erythemato-cyanotic plaques and/or nodules with rapid growth |
Legs |
Aggressive |
CD20+, CD79a+, BCL2+, CD10-, BCL 6+/-, FOX-P1 and MUM-1/IRF-4 positive |
Local radiotherapy, R-CHOP, pegylated liposomal doxorubicin, monoclonal antibodies |
IVBCL |
Violaceous patches and plaque, painful blue-red nodules, ulcerated tumors or telangiectasic skin lesions |
SNC, lungs, and skin |
Aggressive |
CD20 +, BCL 2+, IRF4/MUM-1 + (MIB-1/Ki 67++) |
Chemotherapy in combination with rituximab |
EBV-MCU |
Solitary, sharply demarcated ulcerating lesion |
Oropharyngeal mucosa, skin and gastrointestinal tract |
Indolent |
Variable expression of CD20; CD19+, CD79a +, CD10-, CD30+, BCL2+, PAX 5+, BCL 6 -, MUM-1/IRF-4+ |
Local radiotherapy or surgical excision, systemic chemotherapy, rituximab |