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, the most common type of PCL, none exist for the other PCLs. In addition, staging of 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 LymphomaConsortium (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.

Figure 1.

Regional areas of the body for assessment of total body skin lesions. Body region percent BSA for mSWAT determination by Lund and Browder. A, B, and C designate the body regions of head, thigh, and leg, respectively, for which they provided adjustments in BSA for children aged 1 to 5 years.

Figure 2.

Revised nodal drainage areas for determination of classification of skin involvement in non-MF/non-SS PCLs. The nodal regions (based on the Ann Arbor classification) and their boundaries are as follows: head and neck (HN), inferior borders = clavicles anterior and T1 spinous process posterior; left upper arm (LUA), superior border = glenohumeral joint (exclusive of axilla), inferior border = ulnar/radial/humeral (elbow) joint; left lower arm and hand (LLAH), superior border = ulnar/radial/humeral (elbow) joint; right upper arm (RUA), superior border = glenohumeral joint (exclusive of axilla), inferior border = ulnar/radial/humeral (elbow) joint; right lower arm and hand (RLAH), superior border = ulnar/radial/humeral (elbow) joint; chest (C), superior border = superior border clavicles, inferior border = inferior margin rib cage, lateral borders = midaxillary lines and glenohumeral joints (inclusive of axilla); abdomen/genital (AG), superior border = inferior margin rib cage, inferior border = inguinal folds and anterior perineum; upper back (UB), superior border = T1 spinous process, inferior border = inferior margin rib cage, lateral borders = midaxillary lines; lower back/buttocks (LBB), superior border = inferior margin rib cage, inferior border = inferior gluteal fold and anterior perineum (inclusive of perineum), lateral borders = midaxillary lines; left upper leg (LUL), superior border = inguinal fold and gluteal folds, inferior border = midpatella anterior and mid–popliteal fossa posterior; left lower leg and foot (LLLF), superior border = midpatella anterior and mid–popliteal fossa posterior; right upper leg (RUL), superior border = inguinal fold and gluteal folds, inferior border = midpatella anterior and mid–popliteal fossa posterior; right lower leg and foot (RLLF), superior border = midpatella anterior and mid–popliteal fossa posterior.