Prevalence, clinical features, and survival outcome trends of 627 patients with primary cutaneous lymphoma over 29 years: a retrospective review from single tertiary center in Korea

Sci Rep. 2024 Aug 29;14(1):20118. doi: 10.1038/s41598-024-71210-y.

Abstract

The relative frequency of primary cutaneous lymphoma (PCL) subtypes shows wide variation across different geographical regions. This retrospective study was conducted in a tertiary referral center located in Korea to describe the relative frequency, demographics, survival outcomes, and temporal trend in PCL. A total of 627 PCL cases diagnosed between January 1994 and December 2022 were included. The majority of PCL cases (87.2%) were of T-/NK-cell lineage (CTCL), while the remaining cases (12.8%) were B-cell lineage lymphomas (CBCL). The prevalence of mycosis fungoides (MF) in CTCL increased significantly over time, while other CTCL subtypes, including primary cutaneous extranodal NK/T-cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma (SPTCL), decreased in frequency. Notably, the prevalence of CD4-positive small/medium T-cell lymphoproliferative disorder showed a substantial increase over time. Primary cutaneous marginal zone lymphoma was consistently the commonest CBCL subtype. Survival analysis demonstrated that CTCL had a more favorable 5-year overall survival (OS) than CBCL. OS rate of MF, SPTCL, and primary cutaneous peripheral T-cell lymphoma, NOS improved significantly over time. This study provides comprehensive insights into the dynamic change in the relative frequency and overall survival of PCL subtypes over time.

Keywords: B-cell lineage lymphomas; Incidence; Mycosis fungoides; Primary cutaneous lymphoma; Survival analysis; T-/NK-cell lineage.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Female
  • Humans
  • Lymphoma, B-Cell / epidemiology
  • Lymphoma, B-Cell / mortality
  • Lymphoma, B-Cell / pathology
  • Lymphoma, T-Cell, Cutaneous / epidemiology
  • Lymphoma, T-Cell, Cutaneous / mortality
  • Lymphoma, T-Cell, Cutaneous / pathology
  • Male
  • Middle Aged
  • Prevalence
  • Republic of Korea / epidemiology
  • Retrospective Studies
  • Skin Neoplasms* / epidemiology
  • Skin Neoplasms* / mortality
  • Skin Neoplasms* / pathology
  • Survival Analysis
  • Tertiary Care Centers*
  • Young Adult