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.
Keywords: extranodal natural killer/T-cell lymphoma; pathology; prognosis; skin; systematic review.
© 2021 Japanese Dermatological Association.