Primary Cutaneous Lymphoma: Recommendations for Clinical Trial Design and Staging Update from the ISCL, USCLC, and EORTC

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.

Mycosis Fungoides with Dermal Mucin Deposition: An Unusual Variant of the Most Challenging Primary Cutaneous Lymphoma

Mycosis fungoides (MF) is defined as an epidermotropic primary cutaneous T-cell lymphoma composed of small-to-medium-sized T lymphocytes with cerebriform nuclei and with a T-helper phenotype. LeBoit first described an unusual variant of MF with dermal acid mucin deposition. Such a variant was still considered in the list of clinicopathological variants of MF by Cerroni and colleagues. We herein report a case of patch-stage MF with abundant papillary dermal mucin deposition in a clinical setting of an erythematous patch on the lower abdomen and thigh.

Systemic chemotherapy for primary cutaneous b-cell lymphoma, leg-type: a case report

Primary cutaneous lymphomas are a group of extranodal B-cell non-Hodgkins lymphomas. Primary cutaneous diffuse B-large cell lymphoma, leg-type, is an extremely rare and aggressive variant of primary cutaneous lymphoma. Due to the contradictory nature, poor prognosis, and high frequency of recurrence of this disease, the treatment of patients is multidisciplinary, based on an accurate histological and immunohistochemical classification as well as risk factor assessment. In this report, we present a clinical case of primary cutaneous B-large cell skin lymphoma, leg-type, with a positive response to chemotherapy. Clinical case. A clinical observation of chemotherapy of a patient with primary B-large cell skin lymphoma, leg-type, is presented. The patient underwent 6 R-CHOP chemotherapy courses and achieved an uncertain complete disease remission. Conclusion. The results obtained confirm the limited literature data on the need for timely administration of systemic chemotherapy in the management of this pathology.