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Comprehensive studies of primary cutaneous extranodal natural killer/T‐cell lymphoma (PCENKTL) are scarce. The objectives of this study are to describe PCENKTL in terms of its clinical features, histopathology, immunophenotypes, and prognosis, and to analyze factors affecting patient survival outcomes. We searched four databases and include studies with extractable data. We also searched the Asan Medical Center database for cases of PCENKTL. We include a total of 289 patients. The mean age at diagnosis was 52.8 years and the female to male ratio was 1:1.2. The most common clinical morphology was a subcutaneous nodule, followed by ulceration. About half of the patients presented with disseminated skin lesions. The median overall survival was 12.0 months and the 5‐year survival rate was 22.0%. There was no correlation between the clinical morphology or the histopathological features of the skin lesions with the patient outcomes. Advanced TNM stage, a disseminated skin lesion, tumor location on the leg or trunk, the presence of B symptoms, and a high International Prognostic Index score were associated with a worse prognosis, and chemoradiotherapy was associated with a better survival outcome as compared with chemotherapy alone in univariable analyses. In multivariable analyses, only advanced TNM stage and tumor location on the leg were associated with a worse prognosis. In conclusion, PCENKTL is an aggressive cutaneous lymphoma and its prognosis is associated with TNM stage and tumor location. [ABSTRACT FROM AUTHOR]

Lymphomatoid papulosis (LyP) is a self‐limiting cutaneous T‐cell lymphoproliferative disorder that may progress into malignant lymphoma. Most of the previously reported associated lymphomas are primary cutaneous anaplastic large‐cell lymphoma and mycosis fungoides with a low mortality rate. We report a case of primary cutaneous peripheral T‐cell lymphoma, not otherwise specified (pcPTCL‐NOS), associated with LyP after long‐term follow up. The patient was a 79‐year old Japanese man followed up for 9 years. He suddenly developed a 3‐cm ulcerated lesion on his forehead, which was diagnosed as an exacerbation of LyP. The lesion regressed after conservative treatment, but the patient soon developed multifocal pcPTCL‐NOS. Thereafter, the patient developed pneumonia and cerebral infarction and died within a few months of the onset of malignant lymphoma. Aggressive cutaneous lymphoma may develop in LyP patients. The present case re‐emphasizes the need for careful follow up of patients with persistent LyP. [ABSTRACT FROM AUTHOR]

Types of cutaneous lymphoma ( CL) and their incidences may vary among geographic areas or ethnic groups. The present study aimed to investigate the incidences of various CL in Japan, using epidemiological data from a nationwide registration system for CL. Between 2007 and 2011, 1733 new patients with CL were registered from over 600 dermatological institutes in Japan. The 1733 patients registered included 1485 (85.7%) patients with mature T- and natural killer ( NK)-cell neoplasms, 224 (12.9%) with B-cell neoplasms and 24 (1.4%) with blastic plasmacytoid dendritic cell neoplasm. Mycosis fungoides ( MF) is the most common CL subtype in the present study (750 patients, 43.3%). The proportion of MF patients with early-stage disease was 73%, similar to that of previous studies from other cohorts. The incidence rates of adult T-cell leukemia/lymphoma and extranodal NK/T-cell lymphoma, nasal type were 16.7% and 2.0%, respectively, which may account for the higher incidence of mature T- and NK-cell neoplasms in Japan, as compared with that in the USA and Europe. A male predominance was observed in most types of CL, except for several CL subtypes such as subcutaneous panniculitis-like T-cell lymphoma. [ABSTRACT FROM AUTHOR]

The first Japanese edition of guidelines for management of cutaneous lymphoma was published jointly in 2009 by the Japanese Dermatological Association ( JDA) and the Japanese Skin Cancer Society ( JSCS) - Lymphoma Study Group; the guidelines were revised in 2011, and published in English in 2013. The JDA/ JSCS guidelines are unique because they describe information and management practices for lymphomas specific to Asia, such as adult T-cell leukemia/lymphoma and extranodal natural killer/ T-cell lymphoma, nasal type. In the present article, we have highlighted the essential points of management for cutaneous lymphomas in Asia. In order to complement the guidelines, we have added further information and our clinical experience of some currently available agents for cutaneous lymphomas in Japan. [ABSTRACT FROM AUTHOR]