Survival in patients with primary dermatofibrosarcoma protuberans: National Cancer Database analysis

J Am Acad Dermatol. 2018 Jun;78(6):1125-1134. doi: 10.1016/j.jaad.2017.11.030. Epub 2017 Nov 23.

Abstract

Background: The predictors of mortality, second surgery, and postoperative radiation therapy for treating dermatofibrosarcoma protuberans (DFSP) are not well described.

Objective: We sought to determine the impact of patient demographics, tumor characteristics, and treatment site and modality on survival after primary DFSP.

Methods: A retrospective analysis of data from the National Cancer Database was performed for patients diagnosed with DFSP during 2003-2012.

Results: A total of 5249 cases were identified. Of these, 3.1% of patients died during an average of 51.4 months of follow-up. After adjusting for relevant factors, lack of insurance, Medicaid and Medicare insurance, anaplastic histology, and positive postoperative margins all predicted mortality, while treatment at an Integrated Network Cancer Program predicted survival (P < .05). Higher odds of postoperative radiation therapy were directly associated with large tumor size, anaplastic and poorly differentiated histology, and positive postoperative margins and inversely associated with treatment at high volume facilities, and non-head and neck tumors. Higher second surgery rates were associated with Hispanic ethnicity, and lower rates were associated with female sex.

Limitations: Survival data was not cancer-specific.

Conclusion: Better understanding of factors affecting survival outcomes might help improve management of DFSP and delineate other potential causes of increased morbidity and mortality.

Keywords: National Cancer Database; cutaneous oncology; dermatofibrosarcoma protuberans; population-based; postoperative radiation therapy; second surgery rates.

MeSH terms

  • Adult
  • Age Factors
  • Aged
  • Cause of Death*
  • Cohort Studies
  • Combined Modality Therapy
  • Dermatofibrosarcoma / mortality*
  • Dermatofibrosarcoma / pathology*
  • Dermatofibrosarcoma / therapy
  • Disease-Free Survival
  • Female
  • Humans
  • Logistic Models
  • Male
  • Middle Aged
  • Mohs Surgery / methods
  • Mohs Surgery / mortality
  • Multivariate Analysis
  • Neoplasm Invasiveness
  • Neoplasm Staging
  • Prognosis
  • Proportional Hazards Models
  • Radiotherapy, Adjuvant
  • Registries*
  • Retrospective Studies
  • Risk Assessment
  • Sex Factors
  • Skin Neoplasms / mortality*
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / therapy
  • Survival Analysis
  • Treatment Outcome
  • United States