Table 1.
Quick snapshot of PCBCL.
| Histological Type | Presentation | Most common involved sites | Behavior | Immunohistochemical | Treatment |
|---|---|---|---|---|---|
| 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 |