Format

Send to

Choose Destination
Ann Oncol. 2017 Oct 1;28(10):2517-2525. doi: 10.1093/annonc/mdx352.

Global patterns of care in advanced stage mycosis fungoides/Sezary syndrome: a multicenter retrospective follow-up study from the Cutaneous Lymphoma International Consortium.

Author information

1
Dermatologic Clinic, Department of Medical Sciences.
2
Cancer Epidemiology Unit, Department of Medical Sciences, University of Torino, Torino, Italy.
3
Division of Cancer Medicine, Peter MacCallum Cancer Centre, Melbourne.
4
University of Melbourne, Melbourne, Australia.
5
Division of Hematologic Malignancies, Ohio State University.
6
Department of Medicine, Memorial Sloan-Kettering Cancer Centre, New York.
7
Department of Dermatology and Pathology, MD Anderson Cancer Centre, Houston, USA.
8
Department of Dermatology, Leiden University Medical Centre, Leiden, The Netherlands.
9
Dermatology Department, Hospital St Louis, Paris, France.
10
Department of Dermatology and Pathology, Northwestern University, Chicago, USA.
11
2nd Department of Dermatology and Venereology, Attikon General Hospital, University of Athens, Chaidari, Greece.
12
Department of Dermatology, University of Sao Paulo Medical School, Sao Paulo, Brazil.
13
Beilinson Hospital, Petach Tikva.
14
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
15
Department of Dermatology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
16
Dermatologic Clinic, University of Milano, Milano, Italy.
17
Department of Dermatology, Hospital 12 de Octubre, Madrid, Spain.
18
Dermatologic Clinic, University of Florence, Florence, Italy.
19
Department of Dermatology, Hospital Universitari de Bellvitge, Barcelona, Spain.
20
Dermatologic Clinic, University of Bologna, Bologna.
21
Seragnoli Institute of Haematology, Bologna, Italy.
22
Department of Dermatology, Hospital Clinico, University of Barcelona, Barcelona, Spain.
23
Dermatologic Clinic, University of Vienna Medical School, Vienna, Austria.
24
University Clinic for Dermatology, Venereology, Allergology and Phlebology, Minden, Germany.
25
Cutaneous Lymphoma Service, University Hospital Birmingham, Birmingham.
26
Kings College London, Guys and St Thomas NHS Foundation Trust, London, UK.
27
Department of Dermatology and Pathology, University of Pennsylvania, Philadelphia.
28
Comprehensive Skin Cancer Center, Columbia University Medical Center.
29
Department of Dermatology, Stanford University Medical Centre, USA.

Abstract

Background:

Advanced-stage mycosis fungoides (MF)/Sézary syndrome (SS) patients are weighted by an unfavorable prognosis and share an unmet clinical need of effective treatments. International guidelines are available detailing treatment options for the different stages but without recommending treatments in any particular order due to lack of comparative trials. The aims of this second CLIC study were to retrospectively analyze the pattern of care worldwide for advanced-stage MF/SS patients, the distribution of treatments according to geographical areas (USA versus non-USA), and whether the heterogeneity of approaches has potential impact on survival.

Patients and methods:

This study included 853 patients from 21 specialist centers (14 European, 4 USA, 1 each Australian, Brazilian, and Japanese).

Results:

Heterogeneity of treatment approaches was found, with up to 24 different modalities or combinations used as first-line and 36% of patients receiving four or more treatments. Stage IIB disease was most frequently treated by total-skin-electron-beam radiotherapy, bexarotene and gemcitabine; erythrodermic and SS patients by extracorporeal photochemotherapy, and stage IVA2 by polychemotherapy. Significant differences were found between USA and non-USA centers, with bexarotene, photopheresis and histone deacetylase inhibitors most frequently prescribed for first-line treatment in USA while phototherapy, interferon, chlorambucil and gemcitabine in non-USA centers. These differences did not significantly impact on survival. However, when considering death and therapy change as competing risk events and the impact of first treatment line on both events, both monochemotherapy (SHR = 2.07) and polychemotherapy (SHR = 1.69) showed elevated relative risks.

Conclusion:

This large multicenter retrospective study shows that there exist a large treatment heterogeneity in advanced MF/SS and differences between USA and non-USA centers but these were not related to survival, while our data reveal that chemotherapy as first treatment is associated with a higher risk of death and/or change of therapy and thus other therapeutic options should be preferable as first treatment approach.

KEYWORDS:

CTCL; mycosis fungoides; prognosis; survival; treatment

PMID:
28961843
DOI:
10.1093/annonc/mdx352
[Indexed for MEDLINE]
Free full text

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center