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Clinical Trial
. 2012 Jul;19(7):2360-6.
doi: 10.1245/s10434-011-2213-2. Epub 2012 Jan 21.

Merkel cell carcinoma of unknown primary origin

Jeremiah L Deneve  1 , Jane L Messina, Suroosh S Marzban, Ricardo J Gonzalez, Brooke M Walls, Kate J Fisher, Y Ann Chen, C Wayne Cruse, Vernon K Sondak, Jonathan S Zager
Affiliations
Clinical Trial

Merkel cell carcinoma of unknown primary origin

Jeremiah L Deneve et al. Ann Surg Oncol. 2012 Jul.

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.

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Figures

FIG. 1
FIG. 1
Immunohistochemical staining features of all patients with Merkel cell carcinoma of unknown primary origin
FIG. 2
FIG. 2
a Recurrence-free survival (RFS) for all patients treated for Merkel cell carcinoma (MCC) of unknown primary origin. b RFS based on treatment for all patients treated for MCC of unknown primary origin
FIG. 3
FIG. 3
a Overall survival (OS) for all patients treated for Merkel cell carcinoma (MCC) of unknown primary origin. b OS based on treatment for all patients treated for MCC of unknown primary origin
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