Frequency and prognosis of main types of primary cutaneous lymphomas according to the 2018 update of the WHO-EORTC classification

Revised WHO classification 2018	Frequency (%)*	Five-year DSS (%)
Cutaneous T cell and NK cell lymphomas
Mycosis fungoides	39	89
Mycosis fungoides variants and subtypes
Folliculotropic mycosis fungoides	5	79
Pagetoid reticulosis	<1	100
Granulomatous slack skin	<1	100
Sézary syndrome	2	30
Adult T cell leukemia/lymphoma	<1	NDA
Primary cutaneous CD30+ lymphoproliferative disorders
Primary cutaneous anaplastic large cell lymphoma	10	95
Lymphomatoid papulosis	15	100
Subcutaneous panniculitis-like T cell lymphoma	1	87
Extranodal NK/T cell lymphoma, nasal type	<1	NR
Primary cutaneous peripheral T cell lymphoma, rare subtypes
Primary cutaneous gamma-delta T cell lymphoma	<1	NR
Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma (provisional)	<1	12
Primary cutaneous CD4+ small/medium-sized pleomorphic T cell lymphoproliferative disorder (provisional)	3	100
Primary cutaneous acral CD8+ T cell lymphoma (provisional)	<1	100
Primary cutaneous peripheral T cell lymphoma, not otherwise specified	2	15
Cutaneous B cell lymphomas
Primary cutaneous marginal zone B cell lymphoma	7	99
Primary cutaneous follicle center lymphoma	12	95
Primary cutaneous diffuse large B cell lymphoma, leg type	4	52

WHO: World Health Organization; EORTC: European Organization for Research and Treatment of Cancer; DSS: disease-specific survival; NK: natural killer; NDA: no data available; NR: not reached.
* Relative frequency and disease-specific five-year-survival.

Adapted from: Willemze R, Cerroni L, Kempf W, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood 2019; 133:1703.

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Frequency and prognosis of main types of primary cutaneous lymphomas according to the 2018 update of the WHO-EORTC classification