Relative frequency and disease-specific 5-year survival of 1905 primary cutaneous lymphomas classified according to the WHO-EORTC classification
WHO-EORTC classification . | No. . | Frequency, %* . | Disease-specific 5-year survival, % . |
|---|---|---|---|
| Cutaneous T-cell lymphoma | |||
| Indolent clinical behavior | |||
| Mycosis fungoides | 800 | 44 | 88 |
| Folliculotropic MF | 86 | 4 | 80 |
| Pagetoid reticulosis | 14 | < 1 | 100 |
| Granulomatous slack skin | 4 | < 1 | 100 |
| Primary cutaneous anaplastic large cell lymphoma | 146 | 8 | 95 |
| Lymphomatoid papulosis | 236 | 12 | 100 |
| Subcutaneous panniculitis-like T-cell lymphoma | 18 | 1 | 82 |
| Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma† | 39 | 2 | 75 |
| Aggressive clinical behavior | |||
| Sézary syndrome | 52 | 3 | 24 |
| Primary cutaneous NK/T-cell lymphoma, nasal-type | 7 | < 1 | NR |
| Primary cutaneous aggressive CD8+ T-cell lymphoma† | 14 | < 1 | 18 |
| Primary cutaneous γ/δ T-cell lymphoma† | 13 | < 1 | NR |
| Primary cutaneous peripheral T-cell lymphoma, unspecified‡ | 47 | 2 | 16 |
| Cutaneous B-cell lymphoma | |||
| Indolent clinical behavior | |||
| Primary cutaneous marginal zone B-cell lymphoma | 127 | 7 | 99 |
| Primary cutaneous follicle center lymphoma | 207 | 11 | 95 |
| Intermediate clinical behavior | |||
| Primary cutaneous diffuse large B-cell lymphoma, leg type | 85 | 4 | 55 |
| Primary cutaneous diffuse large B-cell lymphoma, other | 4 | < 1 | 50 |
| Primary cutaneous intravascular large B-cell lymphoma | 6 | < 1 | 65 |
WHO-EORTC classification . | No. . | Frequency, %* . | Disease-specific 5-year survival, % . |
|---|---|---|---|
| Cutaneous T-cell lymphoma | |||
| Indolent clinical behavior | |||
| Mycosis fungoides | 800 | 44 | 88 |
| Folliculotropic MF | 86 | 4 | 80 |
| Pagetoid reticulosis | 14 | < 1 | 100 |
| Granulomatous slack skin | 4 | < 1 | 100 |
| Primary cutaneous anaplastic large cell lymphoma | 146 | 8 | 95 |
| Lymphomatoid papulosis | 236 | 12 | 100 |
| Subcutaneous panniculitis-like T-cell lymphoma | 18 | 1 | 82 |
| Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma† | 39 | 2 | 75 |
| Aggressive clinical behavior | |||
| Sézary syndrome | 52 | 3 | 24 |
| Primary cutaneous NK/T-cell lymphoma, nasal-type | 7 | < 1 | NR |
| Primary cutaneous aggressive CD8+ T-cell lymphoma† | 14 | < 1 | 18 |
| Primary cutaneous γ/δ T-cell lymphoma† | 13 | < 1 | NR |
| Primary cutaneous peripheral T-cell lymphoma, unspecified‡ | 47 | 2 | 16 |
| Cutaneous B-cell lymphoma | |||
| Indolent clinical behavior | |||
| Primary cutaneous marginal zone B-cell lymphoma | 127 | 7 | 99 |
| Primary cutaneous follicle center lymphoma | 207 | 11 | 95 |
| Intermediate clinical behavior | |||
| Primary cutaneous diffuse large B-cell lymphoma, leg type | 85 | 4 | 55 |
| Primary cutaneous diffuse large B-cell lymphoma, other | 4 | < 1 | 50 |
| Primary cutaneous intravascular large B-cell lymphoma | 6 | < 1 | 65 |
NR indicates not reached.
Data are based on 1905 patients with a primary cutaneous lymphoma registered at the Dutch and Austrian Cutaneous Lymphoma Group between 1986 and 2002
Primary cutaneous peripheral T-cell lymphoma, unspecified excluding the three provisional entities indicated with a double dagger (‡)