Cutaneous malignant lymphomas: update 2006.
Cutaneous lymphomas represent a unique group of lymphomas and are the second most frequent extranodal lymphomas. As with other neoplasias, the pathogenesis is based mainly on a stepwise accumulation of mutations of suppressor genes and oncogenes caused by genetic, environmental or infectious factors. The diagnostic work-up includes clinical, histological, imaging and hematological investigations and in many cases immunohistochemical and molecular biological analyses. The current WHO/EORTC classification of cutaneous lymphomas differentiates "mature T-cell and NK-cell lymphomas", "mature B-cell lymphomas" and "immature hematopoietic malignancies", their variants and subgroups. It is compatible with the WHO classification for neoplasias of the hematopoietic and lymphoid tissue and respects the organ-specific peculiarities of primary cutaneous lymphomas. The assignment of the various types of cutaneous lymphomas into prognostic categories (pre-lymphomatous "abortive" disorders; definite malignant lymphomas of low-grade malignancy; definite malignant lymphomas of high-grade malignancy) provides essential information on the biological behavior and allows an appropriate planning of the therapeutic strategy, which may be topical or systemic and aggressive or non-aggressive. Besides the classical options for therapy, there are new and "experimental" strategies, the efficacy of which has to be studied in clinical trials.
Burg
Günter
G
Dept. of Dermatology, University Hospital, Zürich, Switzerland. burg@derm.unizh.ch
Kempf
Werner
W
Cozzio
Antonio
A
Döbbeling
Udo
U
Feit
Josef
J
Golling
Philippa
P
Michaelis
Sonja
S
Schärer
Leo
L
Nestle
Frank
F
Dummer
Reinhard
R
D003880:Dermatology / Q000639:trends*; D005858:Germany; D006801:Humans; D008223:Lymphoma / Q000175:diagnosis* / Q000628:therapy*; D017410:Practice Guidelines as Topic; D010818:Practice Patterns, Physicians' / Q000639:trends*; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000628:therapy*