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Multicenter Study
. 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

P Quaglino  1 , M Maule  2 , H M Prince  3 , P Porcu  4 , S Horwitz  5 , M Duvic  6 , R Talpur  6 , M Vermeer  7 , M Bagot  8 , J Guitart  9 , E Papadavid  10 , J A Sanches  11 , E Hodak  12 , M Sugaya  13 , E Berti  14 , P Ortiz-Romero  15 , N Pimpinelli  16 , O Servitje  17 , A Pileri  18 , P L Zinzani  19 , T Estrach  20 , R Knobler  21 , R Stadler  22 , M T Fierro  23 , S Alberti Violetti  14 , I Amitay-Laish  12 , C Antoniou  10 , C Astrua  23 , S Chaganti  24 , F Child  25 , A Combalia  20 , S Fabbro  4 , P Fava  23 , V Grandi  16 , C Jonak  21 , E Martinez-Escala  9 , M Kheterpal  5 , E J Kim  26 , C McCormack  3 , T Miyagaki  13 , D Miyashiro  11 , S Morris  25 , C Muniesa  17 , V Nikolaou  10 , G Ognibene  27 , F Onida  14 , S Osella-Abate  23 , S Porkert  21 , C Postigo-Llorente  15 , C Ram-Wolff  8 , S Ribero  23 , K Rogers  27 , M Sanlorenzo  23 , R Stranzenbach  22 , N Spaccarelli  26 , A Stevens  24 , D Zugna  2 , A H Rook  26 , L J Geskin  27 , R Willemze  7 , S Whittaker  25 , R Hoppe  28 , J Scarisbrick  24 , Y Kim  28
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Free article
Multicenter Study

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

P Quaglino et al. Ann Oncol. .
Free article

Erratum in

  • Global patterns of care in advanced stage mycosis fungoides/Sezary syndrome: a multicenter retrospective follow-up study from the Cutaneous Lymphoma International Consortium.
    Quaglino P, Maule M, Prince HM, Porcu P, Horwitz S, Duvic M, Talpur R, Vermeer M, Bagot M, Guitart J, Papadavid E, Sanches JA, Hodak E, Sugaya M, Berti E, Ortiz-Romero P, Pimpinelli N, Servitje O, Pileri A, Zinzani PL, Estrach T, Knobler R, Stadler R, Fierro MT, Alberti Violetti S, Amitay-Laish I, Antoniou C, Astrua C, Chaganti S, Child F, Combalia A, Fabbro S, Fava P, Grandi V, Jonak C, Martinez-Escala E, Kheterpal M, Kim EJ, McCormack C, Miyagaki T, Miyashiro D, Morris S, Muniesa C, Nikolaou V, Ognibene G, Onida F, Osella-Abate S, Porkert S, Postigo-Llorente C, Ram-Wolff C, Ribero S, Rogers K, Sanlorenzo M, Stranzenbach R, Spaccarelli N, Stevens A, Zugna D, Rook AH, Geskin LJ, Willemze R, Whittaker S, Hoppe R, Scarisbrick J, Kim Y. Quaglino P, et al. Ann Oncol. 2019 Mar 1;30(3):494. doi: 10.1093/annonc/mdy207. Ann Oncol. 2019. PMID: 29947731 No abstract available.

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.

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