Merkel cell carcinoma: the prognostic implications of an occult primary in stage IIIB (nodal) disease

J Am Acad Dermatol. 2012 Sep;67(3):395-9. doi: 10.1016/j.jaad.2011.09.009. Epub 2011 Oct 26.

Abstract

Background: Merkel cell carcinoma is a highly aggressive cutaneous malignancy with a high rate of lymph node and distant metastatic disease. Approximately one third of patients present with stage IIIB (nodal) disease.

Objective: This cohort study was performed to analyze the outcome of patients with stage IIIB disease with or without an occult primary.

Methods: The details of 91 patients with stage IIIB (nodal) Merkel cell carcinoma treated curatively between 1985 and 2010 at 3 tertiary referral hospitals in Australia were reviewed. Kaplan-Meier plots were used with the primary end point being overall survival. Secondary end points were disease-free survival and relapse-free survival. A multivariate Cox regression analysis was performed for known prognostic factors.

Results: Of 91 patients with stage IIIB (nodal) disease, 36 (40%) had an occult primary. A total of 78 patients (86%) had surgery and 79 patients (87%) had definitive or adjuvant radiotherapy. With a median follow-up of 4.3 years, those with an occult primary did significantly better in terms of overall survival, disease-free survival, and relapse-free survival. On multivariate analysis, occult primary and patient age were the only factors predicting survival with hazard ratios of 0.30 (95% confidence interval 0.13-0.67) and 1.64 (95% confidence interval 1.13-2.38), respectively.

Limitations: This is a retrospective study over several decades with patients treated using various modalities.

Conclusion: This study indicates that for patients with stage IIIB (nodal) Merkel cell carcinoma, the presence of an occult primary confers a significantly better prognosis that may have implications in the future staging and treatment of patients with stage III disease.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Carcinoma, Merkel Cell / mortality*
  • Carcinoma, Merkel Cell / pathology
  • Carcinoma, Merkel Cell / secondary*
  • Carcinoma, Merkel Cell / therapy
  • Combined Modality Therapy
  • Disease-Free Survival
  • Female
  • Humans
  • Lymphatic Metastasis
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Neoplasm Staging
  • Neoplasms, Unknown Primary / epidemiology
  • Neoplasms, Unknown Primary / pathology*
  • Prognosis
  • Retrospective Studies
  • Skin Neoplasms / mortality*
  • Skin Neoplasms / pathology
  • Skin Neoplasms / secondary*
  • Skin Neoplasms / therapy