Melanomas

Annals of Surgical Oncology

, Volume 19, Issue 7, pp 2360-2366

First online:

Merkel Cell Carcinoma of Unknown Primary Origin

  • Jeremiah L. DeneveAffiliated withDepartment of Cutaneous Oncology, Moffitt Cancer Center
  • , Jane L. MessinaAffiliated withDepartment of Cutaneous Oncology, Moffitt Cancer CenterDepartments of Anatomic Pathology and Cell Biology and Dermatology, University of South Florida
  • , Suroosh S. MarzbanAffiliated withDepartment of Cutaneous Oncology, Moffitt Cancer Center
  • , Ricardo J. GonzalezAffiliated withDepartment of Cutaneous Oncology, Moffitt Cancer CenterDepartments of Oncologic Sciences and Surgery, University of South Florida
  • , Brooke M. WallsAffiliated withDepartment of Cutaneous Oncology, Moffitt Cancer Center
  • , Kate J. FisherAffiliated withDepartment of Biostatistics, Moffitt Cancer Center
  • , Y. Ann ChenAffiliated withDepartment of Biostatistics, Moffitt Cancer Center
  • , C. Wayne CruseAffiliated withDepartment of Cutaneous Oncology, Moffitt Cancer CenterDepartments of Oncologic Sciences and Surgery, University of South Florida
  • , Vernon K. SondakAffiliated withDepartment of Cutaneous Oncology, Moffitt Cancer CenterDepartments of Oncologic Sciences and Surgery, University of South Florida
    • , Jonathan S. ZagerAffiliated withDepartment of Cutaneous Oncology, Moffitt Cancer CenterDepartments of Oncologic Sciences and Surgery, University of South Florida Email author 

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Abstract

Background

Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin. MCC from an unknown primary origin (MCCUP) can present a diagnostic and therapeutic challenge. We describe our single-institution experience with the diagnosis and management of MCCUP presenting as metastases to lymph nodes.

Methods

After institutional review board approval, our institutional database spanning the years 1998–2010 was queried for patients with MCCUP. Clinicopathologic variables and outcomes were assessed.

Results

From a database of 321 patients with MCC, 38 (12%) were identified as having nodal MCCUP. Median age was 67 years, and 79% were men. Nodal basins involved at presentation were cervical (58%), axillary/epitrochlear (21%), or inguinal/iliac (21%). CK20 staining was positive in 93% of tumors tested, and all were negative for thyroid transcription factor-1. Twenty-nine patients (76%) underwent complete regional lymph node dissection (LND): 3 had LND alone, ten had LND and adjuvant radiotherapy, and 16 underwent LND followed by chemoradiotherapy. Definitive chemoradiotherapy without surgery was provided to six patients (16%), while radiotherapy alone was provided to three (8%). Recurrence was observed in 34% of patients. Median recurrence-free survival was 35 months. Ten patients (26%) died, five of disease and five of other causes. The median overall survival was 104 months.

Conclusions

Nodal MCCUP is a rare disease affecting primarily elderly white men. Recurrence is observed in approximately one-third of patients, with a 104 month median overall survival after a multimodal treatment approach consisting of surgery along with adjuvant chemotherapy and radiotherapy in the majority of patients.