Focused
Review
© JNCCN—Journal of the National Comprehensive Cancer Network | Volume 12 Number 3 | March 2014
434
Abstract
Merkel cell carcinoma (MCC) is a rare malignancy of the skin,
and prospective randomized clinical studies on management and
treatment are very limited. The NCCN Clinical Practice Guidelines
in Oncology (NCCN Guidelines) for MCC provide up-to-date, best
evidence–based, and consensus-driven management pathways
with the purpose of providing best care and outcomes. Multidisci-
plinary management with consensus treatment recommendations
to individualize patient care within the framework of these guide-
lines is optimal. The University of Michigan multidisciplinary MCC
program uses NCCN Guidelines in the management and treatment
of its patients. This article discusses 4 patient presentations to high-
light the implementation of the NCCN Guidelines for MCC at the
University of Michigan. (J Natl Compr Canc Netw 2014;12:434–441)
Despite its rarity, the increasing incidence and ag-
gressive nature of MCC have brought attention on this
disease and revealed a wide variability in the treatment
and management of patients diagnosed with this malig-
nancy. Patients with MCC may have very different work-
up, treatment, and follow-up depending on the institu-
tion, physician, and specialty providing their care. These
inconsistencies highlight the importance of having best
evidence–based treatment guidelines. Unfortunately,
prospective randomized clinical trials are extremely lim-
ited because of the rarity of the disease, necessitating that
guidelines be based on best available evidence and ex-
pert consensus opinion. Multidisciplinary management
to establish consensus recommendations for individu-
alizing patient care within the framework of evidence-
based guidelines constitutes the optimal treatment model
within the existing limitations. This article discusses
the NCCN Clinical Practice Guidelines in Oncology
(NCCN Guidelines) for MCC, including the importance
of multidisciplinary management, and highlights the im-
plementation of these guidelines in the multidisciplinary
MCC program at the University of Michigan (UM).
Background
MCC is rarely clinically suspected. The most common
presentation is a nondistinctive, rapidly growing, as-
ymptomatic, red-purple–colored nodule.
2
MCC occurs
most frequently on sun-exposed skin of the head and
neck (29.0%–45.3%) and extremities (21.0%–50.0%),
followed by the trunk (4.7%–23.0%) and other sun-pro-
tected areas.
2–5
Immunosuppression from HIV, chronic
lymphocytic leukemia, and certain immunosuppressant
From the Departments of
a
Dermatology,
b
Surgery,
c
Otolaryngology,
d
Radiation Oncology,
e
Internal Medicine, and
f
Pathology, University
of Michigan Health System, Ann Arbor, Michigan.
Submitted September 23, 2013; accepted for publication
December 9, 2013.
The authors have disclosed that they have no fnancial interests,
arrangements, affliations, or commercial interests with the
manufacturers of any products discussed in this article or their
competitors.
Correspondence: Jennifer L. Schwartz, MD, University of Michigan
Health System, 1910 Taubman Center, 1500 East Medical Center
Drive, Ann Arbor, MI 48109-0314.
E-mail: jennschw@med.umich.edu
NCCN Guidelines Implementation in the
Multidisciplinary Merkel Cell Carcinoma
Program at the University of Michigan
Jennifer L. Schwartz, MD
a
; Sandra L. Wong, MD
b
; Scott A. McLean, MD, PhD
c
; James A. Hayman, MD
d
;
Christopher D. Lao, MD, MPH
e
; Jeffrey H. Kozlow, MD
b
; Kelly M. Malloy, MD
c
; Carol R. Bradford, MD
c
;
Marcus L. Frohm, MD
a
; Douglas R. Fullen, MD
a,f
; Lori Lowe, MD
a,f
; and Christopher K. Bichakjian, MD
a
Merkel cell carcinoma (MCC), diagnosed primarily in
the elderly white population, is an uncommon, clinically
aggressive cutaneous malignancy with a high rate of
local, regional, and distant recurrence. During the last
2 decades of the 20th century, the incidence of MCC
more than tripled, a trend that is expected to continue
with an increasingly aging population.
1