Relative frequency and prognosis of primary cutaneous lymphomas included in the 2018 update of the WHO-EORTC classification
| WHO-EORTC Classification 2018 . | Frequency, %* . | 5-y DSS, %* . |
|---|---|---|
| CTCL | ||
| MF | 39 | 88 |
| MF variants | ||
| Folliculotropic MF | 5 | 75 |
| Pagetoid reticulosis | <1 | 100 |
| Granulomatous slack skin | <1 | 100 |
| SS | 2 | 36 |
| Adult T-cell leukemia/lymphoma | <1 | NDA |
| Primary cutaneous CD30+ LPDs | ||
| C-ALCL | 8 | 95 |
| LyP | 12 | 99 |
| Subcutaneous panniculitis-like T-cell lymphoma | 1 | 87 |
| Extranodal NK/T-cell lymphoma, nasal type | <1 | 16 |
| Chronic active EBV infection | <1 | NDA |
| Primary cutaneous peripheral T-cell lymphoma, rare subtypes | ||
| Primary cutaneous γ/δ T-cell lymphoma | <1 | 11 |
| CD8+ AECTCL (provisional) | <1 | 31 |
| Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (provisional) | 6 | 100 |
| Primary cutaneous acral CD8+ T-cell lymphoma (provisional) | <1 | 100 |
| Primary cutaneous peripheral T-cell lymphoma, NOS | 2 | 15 |
| CBCL | ||
| PCMZL | 9 | 99 |
| PCFCL | 12 | 95 |
| PCDLBLC, LT | 4 | 56 |
| EBV+ mucocutaneous ulcer (provisional) | <1 | 100 |
| Intravascular large B-cell lymphoma | <1 | 72 |
| WHO-EORTC Classification 2018 . | Frequency, %* . | 5-y DSS, %* . |
|---|---|---|
| CTCL | ||
| MF | 39 | 88 |
| MF variants | ||
| Folliculotropic MF | 5 | 75 |
| Pagetoid reticulosis | <1 | 100 |
| Granulomatous slack skin | <1 | 100 |
| SS | 2 | 36 |
| Adult T-cell leukemia/lymphoma | <1 | NDA |
| Primary cutaneous CD30+ LPDs | ||
| C-ALCL | 8 | 95 |
| LyP | 12 | 99 |
| Subcutaneous panniculitis-like T-cell lymphoma | 1 | 87 |
| Extranodal NK/T-cell lymphoma, nasal type | <1 | 16 |
| Chronic active EBV infection | <1 | NDA |
| Primary cutaneous peripheral T-cell lymphoma, rare subtypes | ||
| Primary cutaneous γ/δ T-cell lymphoma | <1 | 11 |
| CD8+ AECTCL (provisional) | <1 | 31 |
| Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (provisional) | 6 | 100 |
| Primary cutaneous acral CD8+ T-cell lymphoma (provisional) | <1 | 100 |
| Primary cutaneous peripheral T-cell lymphoma, NOS | 2 | 15 |
| CBCL | ||
| PCMZL | 9 | 99 |
| PCFCL | 12 | 95 |
| PCDLBLC, LT | 4 | 56 |
| EBV+ mucocutaneous ulcer (provisional) | <1 | 100 |
| Intravascular large B-cell lymphoma | <1 | 72 |
CD8+ AECTCL, primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma; DSS, disease-specific survival; NDA, no data available; NOS, not otherwise specified.
Based on data included in Dutch and Austrian cutaneous lymphoma registries between 2002 and 2017.