Primary cutaneous lymphoma: recommendations for clinical trial design and staging update from the ISCL, USCLC, and EORTC

Abstract

The number of patients with primary cutaneous lymphoma (PCL) relative to other non-Hodgkin lymphomas (NHLs) is small and the number of subtypes large. Although clinical trial guidelines have been published for mycosis fungoides/Sézary syndrome (MF/SS), the most common type of PCL, none exist for the other PCLs. In addition, staging in the PCLs has been evolving based on new data on potential prognostic factors, diagnosis, and assessment methods of both skin and extracutaneous disease and a desire to align the latter with the Lugano guidelines for all NHLs. The International Society for Cutaneous Lymphomas (ISCL), the United States Cutaneous Lymphoma Consortium (USCLC), and the Cutaneous Lymphoma Task Force of the European Organization for the Research and Treatment of Cancer (EORTC) now propose updated staging and guidelines for the study design, assessment, endpoints and response criteria in clinical trials for all the PCLs in alignment with that of the Lugano guidelines. These recommendations provide standardized methodology that should facilitate planning and regulatory approval of new treatments for these lymphomas worldwide, encourage cooperative investigator-initiated trials, and help to assess the comparative efficacy of therapeutic agents tested across sites and studies.

Impact of Mogamulizumab in Real-Life Advanced Cutaneous T-Cell Lymphomas: A Multicentric Retrospective Cohort Study

“…Nowadays, flow cytometry tends to be the reference in terms of diagnosis and prognosis. Guidelines have recently been published to harmonize practices and facilitate the conduct of informative and comparable clinical trials [ 20 ]. This will probably incite physicians to perform extensive immunophenotyping at least at diagnosis and at the start of each therapeutic line.…”

Flow cytometry for the assessment of blood tumour burden in cutaneous T‐cell lymphoma: towards a standardized approach

“…12 In recognition of the fact that proportional measures can vary depending on overall leucocyte count, the use of absolute counts was suggested in a 2011 ISCL/United States Cutaneous Lymphoma Consortium (USCLC)/EORTC update, 19 with a subsequent update published in 2021. 20 These definitions are still used in relevant MF/SS treatment guidelines, including those of the EORTC, the European Society for Medical Oncology (ESMO), and the National Comprehensive Cancer Network in the USA. [21][22][23] The 2018 ESMO guidelines recommend peripheral blood FC for all MF stages but state that the test may be more appropriate in patients with suspected SS, while the EORTC recommend FC for measuring blood involvement at all MF stages and in SS, as accurate blood class assignment is necessary for overall disease staging and consequent management.…”

“…18 In 2007, the ISCL/EORTC revised MF/SS staging and classification guidelines defined the following three stages of blood involvement: B 0 , absence of significant blood involvement; B 1 , low blood tumour burden and B 2 , high blood tumour burden (Table 1). 12 In recognition of the fact that proportional measures can vary depending on overall leucocyte count, the use of absolute counts was suggested in a 2011 ISCL/United States Cutaneous Lymphoma Consortium (USCLC)/EORTC update, 19 with a subsequent update published in 2021 20 . These definitions are still used in relevant MF/SS treatment guidelines, including those of the EORTC, the European Society for Medical Oncology (ESMO), and the National Comprehensive Cancer Network in the USA 21–23 .…”

Real‐life experience of using pegylated liposomal doxorubicin in primary cutaneous T‐cell lymphomas

“…Staging and responses were evaluated as per the recommendations of the European Organization for Research and Treatment of Cancer (EORTC), International Society for Cutaneous Lymphomas and US Cutaneous Lymphoma Consortium 4 . Complete response (CR) was defined as total clearance in all compartments and partial response (PR) as 50% improvement in at least one compartment, while progressive disease (PD) was defined as worsening of 25% in at least one compartment, and stable disease (SD) as no significant change (< 50%) in any compartment.…”