Atsunori Baba

CS Karapetis

DJ Gawkrodger

H Brincker

H Umezu

I Le Jeune

J Maeda

K Kaira

K Takanami

Kentaro Mera

L Stegger

LH Brudin

M Chida

Masakazu Yanagi

Mitsuhiro Hisadome

Mitsuyoshi Shimokawa

NM McLoone

PR Cohen

RM Mackie

Shigeto Matsushita

SN Leech

T Kanekura

Takuro Kanekura

Yuko Higashi

Journal of Medical Case Reports

English

PubMed

Crossref

Sarcoid reaction associated with Merkel cell carcinoma revealed by fluorodeoxyglucose positron emission tomography: a case report

Springer - Publisher Connector

CASE REPORT Open Access
Sarcoid reaction associated with Merkel cell
carcinoma revealed by fluorodeoxyglucose
positron emission tomography: a case report
Yuko Higashi1*, Kentaro Mera1, Mitsuyoshi Shimokawa1, Mitsuhiro Hisadome1, Atsunori Baba1, Shigeto Matsushita1,
Masakazu Yanagi2 and Takuro Kanekura1
Abstract
Introduction: Although the association between cancer and sarcoidosis or sarcoid reaction is known, sarcoid
reaction associated with Merkel cell carcinoma is rare.
Case presentation: We report the case of a 57-year-old Japanese woman with Merkel cell carcinoma in the inguinal
area associated with sarcoid reaction. Fluorodeoxyglucose positron emission tomography demonstrated elevated
fluorodeoxyglucose uptake by mediastinal lymph nodes and at the carcinoma site. Histopathologically, the mediastinal
lymph nodes contained no Merkel cell carcinoma components. Sarcoid lesions were identified. Systemic examinations
returned no sarcoidosis-specific findings.
Conclusion: Fluorodeoxyglucose positron emission tomographic scans can be used to assess neoplastic lesions and
depict sarcoidosis. Sarcoid reactions must be considered in the interpretation of fluorodeoxyglucose positron
emission tomographic scans.
Introduction
Sarcoidosis, a common systemic disorder of unknown
etiology, is characterized by the formation of non-caseat-
ing epithelioid cell granulomas. The lungs, lymph nodes,
liver, spleen, skin, eyes, small bones of the hands and feet
and the salivary glands are most often affected [1]. A
diagnosis of sarcoid reaction is made when localized
epithelioid granulomas are found without signs of sys-
temic sarcoidosis. They are attributable to infections, for-
eign materials, gastrointestinal diseases and malignant
tumors [1]. The reported incidence of sarcoid reactions
in carcinoma is 4.4%; in squamous cell carcinoma of the
skin and mucous membranes, it is 13.0% [2]. Merkel cell
carcinoma is a rare and aggressive skin cancer that is
thought to arise from cutaneous Merkel cells which are
neuroendocrine cells [3]. We present the case of a patient
with Merkel cell carcinoma associated with sarcoid
reaction.
Case presentation
A 57-year-old Japanese woman presented to our hospital
with a painless, firm, palpable mass 3 cm in diameter of
five months’ duration in the left inguinal area. She was a
non-smoker and had no particular respiratory symptoms.
The resected inguinal mass was a dermal tumor consisting
of small to intermediate-sized cells with scant cytoplasm
and regular nuclei with dusty chromatin and no nucleoli
(Figure 1). Immunohistochemically, the tumor cells were
positive for cytokeratin 20 and negative for thyroid tran-
scription factor 1. Our diagnosis was Merkel cell carci-
noma. Contrast-enhanced computed tomographic (CT)
scans showed right paratracheal and right tracheobronchial
lymphadenopathies 1 cm in diameter, suggesting meta-
static lesions. No lung parenchymal abnormality was
found. A fluorodeoxyglucose positron emission tomo-
graphic (FDG-PET) study (Figure 2) of her mediastinal
adenopathy revealed increased metabolic activity. The stan-
dardized uptake value was 6.1, corresponding to the nodal
distribution seen on her CT scan. The sentinel lymph node
in her left groin was excised and the mediastinal lymph
nodes were dissected by performing video-assisted thoracic
surgery. Her Merkel cell carcinoma was found to have
* Correspondence: higashiy@m.kufm.kagoshima-u.ac.jp
1Department of Dermatology, Kagoshima University Graduate School of
Medical and Dental Sciences, Kagoshima, Japan
Full list of author information is available at the end of the article
Higashi et al. Journal of Medical Case Reports 2011, 5:282
http://www.jmedicalcasereports.com/content/5/1/282 JOURNAL OF MEDICAL
CASE REPORTS
© 2011 Higashi et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons
Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in
any medium, provided the original work is properly cited.
metastasized to the superficial inguinal lymph node. Patho-
logical examination of the mediastinal lymph node revealed
a non-caseating epithelioid cell granuloma with giant cells.
No tumor cells were identified. These findings were sug-
gestive of sarcoidosis or sarcoid reaction (Figure 3). Her
serum angiotensin-converting enzyme level was 3.5 U/L
(normal 7 U/L to 25 U/L). No ocular manifestations were
observed. Cardiac ultrasonography was within the normal
range without an anomalous cardiac rhythm. The patient
is being followed every three months and is free of relapse
two years after the initial diagnosis.
Conclusion
The association of sarcoid reaction with Merkel cell car-
cinoma is rare. Our literature review uncovered a case
of an 84-year-old woman with Merkel cell carcinoma
and chronic sarcoidosis [4]. Her carcinoma was diag-
nosed nine years after sarcoidosis, and ductal breast car-
cinoma was diagnosed six months before Merkel cell
carcinoma. As the pathogenesis of sarcoidosis involves
immune dysfunction [4], her two different malignant
tumors may be attributable to disturbances in the
immune system, which play an important role in
immune surveillance. A longitudinal study adjusted for
age, sex and smoking history showed a significantly
higher incidence of cancer in patients with sarcoidosis
than in the general population [5]. Sarcoidosis can
develop after cancer, and in some patients there is an
association between the administration of anti-neoplastic
drugs and the appearance of sarcoidosis [6-8]. Although
our patient manifested sarcoid reaction rather than sar-
coidosis, it remains unknown whether sarcoid reaction
in the presence of malignancy is different from systemic
sarcoidosis involvement in the etiology of cancer.
Figure 1 Photomicrograph showing small to intermediate-sized
cells with scant cytoplasm and regular nuclei with dusty
chromatin. No nucleoli are visible (hematoxylin and eosin stain;
original magnification, ×400).
Figure 2 Fluorodeoxyglucose (FDG) positron emission
tomographic scan showing areas of FDG accumulation in the
mediastinum and left inguinal region.
Figure 3 Photomicrograph showing the pathological findings of
non-caseating epithelioid cell granulomas with giant cells (arrow)
(hematoxylin and eosin stain; original magnification, × 100).
Higashi et al. Journal of Medical Case Reports 2011, 5:282
http://www.jmedicalcasereports.com/content/5/1/282
Page 2 of 3
Merkel cell carcinoma, composed of small, round blue
cells, should be distinguished from small cell lung carci-
noma, which has a similar pathological appearance.
Their differentiation is possible because antibodies to
cytokeratin 20 and thyroid transcription factor 1 are
specific to Merkel cell carcinoma and small cell lung
carcinoma, respectively [9].
In our patient, the immunohistochemical findings
regarding the inguinal mass and the absence of tumor
cells in the mediastinal lymph node led to a diagnosis of
Merkel cell carcinoma arising in the inguinal region. Her
sarcoid reaction was mediastinal. Although sarcoid reac-
tions are most commonly observed in cancer-draining
lymph nodes, they can occur in non-regional tissues
[2,6]. They are thought to be attributable to soluble anti-
genic or granulomagenic factors derived from tumor cells
and include antigen-antibody complexes, peptides and
modified tumor cells [2]. Our observation that the sar-
coid reaction in our patient occurred at distant tissue
sites supports the involvement of soluble factors.
FDG-PET, widely used to assess neoplastic lesions,
depicts the glucose avidity of tissues. Although FDG-
PET images reflect the different utilization of glucose by
normal and malignant tissues, they fail to differentiate
malignancy from inflammation reliably. Increased glu-
cose uptake has been reported in patients with benign
disorders [10,11], and elevated FDG uptake has been
observed in patients with sarcoidosis. Brudin et al. [12]
proposed that FDG-PET images reflect disease activity
and the distribution of sarcoidosis. In cancer patients
with sarcoidosis [13,14] or sarcoid reaction [12], FDG-
PET has shown lesions that mimicked lymph node
metastases. Kaira et al. [16] reported that the use of
L-[3-18F]-a-methyltyrosine PET (18F-FMT PET) in com-
bination with FDG-PET can distinguish sarcoidosis from
malignancy. Sarcoid reaction must be considered in the
evaluation of cancer metastasis, and the acquisition of
18F-FMT PET scans is desirable.
Consent
Written informed consent was obtained from the patient
for publication of this case report and any accompany-
ing images. A copy of the written consent is available
for review by the Editor-in-Chief of this journal.
Author details
1Department of Dermatology, Kagoshima University Graduate School of
Medical and Dental Sciences, Kagoshima, Japan. 2Surgical Oncology,
Kagoshima University Graduate School of Medical and Dental Sciences,
Kagoshima, Japan.
Authors’ contributions
YH drafted the manuscript and reviewed the literature. KM, MS, MH and AB
obtained informed consent from the patient, assisted in data collection and
coordinated and helped to draft the manuscript. SM and MY carried out the
patient’s surgery and revised the manuscript. TK was responsible for the
diagnosis, patient management and review. All authors read and approved
the final manuscript.
Competing interests
The authors declare that they have no competing interests.
Received: 30 September 2010 Accepted: 5 July 2011
Published: 5 July 2011
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doi:10.1186/1752-1947-5-282
Cite this article as: Higashi et al.: Sarcoid reaction associated with
Merkel cell carcinoma revealed by fluorodeoxyglucose positron
emission tomography: a case report. Journal of Medical Case Reports 2011
5:282.
Higashi et al. Journal of Medical Case Reports 2011, 5:282
http://www.jmedicalcasereports.com/content/5/1/282
Page 3 of 3


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Increased glucose uptake by keratoacanthoma detected by positron emission tomography. Clin Exp Dermatol

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Sarcoid reaction associated with Merkel cell carcinoma revealed by fluorodeoxyglucose positron emission tomography: a case report

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