Open Journal of Ophthalmology, 2013, 3, 16-18
http://dx.doi.org/10.4236/ojoph.2013.31005 Published Online February 2013 (http://www.scirp.org/journal/ojoph)
Merkel Cell Carcinoma of Eyelid: A Rare Clinical
Presentation and Diagnostic Challenge
Manpreet Singh, Swati Singh, Usha Singh*, Zoramthara Zadeng
Department of ophthalmology, Advanced Eye Center, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Received October 27th, 2012; revised November 19th, 2012; accepted November 25th, 2012
ABSTRACT
The purpose of this paper is to report a case of rare localised (limited to eyelid) malignant eyelid tumor in a 70 year old
Indian male. Histopathology confirmed it to be a Merkel cell carcinoma. Surgical excision and eyelid reconstruction
was done successfully. No local or systemic recurrence has been noted at a follow up of 2 years. The well defined na-
ture of a highly malignant tumor, its localised presentation and management is being described in following report.
Keywords: Merkel Cell Carcinoma; Eyelid; Clinical Features; Management
1. Introduction
Merkel cells are cutaneous mechanoreceptors receiving
touch and hair movement sensations. Merkel cell carci-
noma (MCC) of eyelid is a rare aggressive tumor with
less than 100 reported cases worldwide. Its predilection
for elderly males, benign appearance and few typical cli-
nical features can suggest this commonly misdiagnosed
entity [1]. We hereby describe clinical presentation and
management of a localized MCC of eyelid.
2. Case Report
A 70 year old Indian male presented to oculoplastics
clinic with chief complaints of mass overright upper eye-
lid for last 2 months. It was painless and gradually pro-
gressive in nature. There was no history of bleeding from
mass or loss of eyelashes. No history of organ transplant-
tation and immunosuppression. On examination, a soft to
firm non tender, reddish pink mass measuring 20 mm
(horizontally) was present on lateral half of right upper
eyelid. Eyelid margin was involved and mechanical pto-
sis was present. Overlying skin was smooth with small to
medium sized telengiectatic vessels over the surface (Fig-
ure 1). Transillumination test was positive. There was no
regional lymphadenopathy. Left eye and adnexa were es-
sentially within normal limits. Best corrected visual acu-
ity was 20/60 in both eyes attributable to nuclear sclero-
sis of grade 3.
Differential diagnosis of upper eyelid squamous papi-
lloma, acquired capillary hemangioma and Kaposi sar-
coma were kept. Excisional biopsy was performed under
local anesthesia with preservation of overlying skin and
eyelid margin. Mass was sent for histopathology. Report
showed presence of round to oval tumor cells arranged in
sheets having high nuclear: cytoplasmic ratio. Immuno-
histochemistry revealed positivity for CK-20, neuron spe-
cific enolase and epithelial membrane antigen, suggestive
of Merkel cell carcinoma (Figures 2 and 3).
After getting the diagnosis of a malignant tumor of
eyelid, patient was subjected to detailed systemic metas-
tatic workup in the form of abdominal ultrasound, chest
X ray and positron emission tomography. All the inves-
tigations were within normal limits. Patient was taken up
for wide surgical excision (margin 5 mm) and eyelid re-
construction (direct closure) within a week from primary
surgery. Histopathology specimen revealed all margins,
tumor free. Currently, patient is asymptomatic with no
evidence of local or systemic tumor recurrence at 24
months of uneventful follow up (Figure 4).
Figure 1. Photo of right eye upper eyelid (at presentation)
showing a reddish-pink, well defined mass with mechanical
ptosis. On eversion, mass is seen arising from eyelid margin
with telengiectatic vessels over surface (inset).
*Corresponding author.

Merkel Cell Carcinoma of Eyelid: A Rare Clinical Presentation and Diagnostic Challenge 17
Figure 2. Histopathology shows round to oval tumor cells
arranged in diffuse sheets.
Figure 3. Immunohistochemistry shows positivity for cy-
tokeratin 20 & neuron specific enolase (marked with brown
color).
Figure 4. Post-operatively, eyelid showing normal contour
with no evidence of tumor recurrence at 24 months.
3. Discussion
Upto 9% of MCC have been reported to originate from
eyelids. Most commonly it involves upper eyelid arising
near its margin, either sparing or causing partial eyelash
loss. Its clinical benign appearance (overlying smooth
skin, telangiectasia and color) can lead to delay in diag-
nosis and start of appropriate management, timely [2].
Risk factors include male gender, UV-B light exposure,
fair skin, immunosuppression (organ transplant) [3]. Cli-
nical diagnosis of this condition can rarely be made be-
fore histopathology.
On microscopic examination, a highly malignant tu-
mor involving the dermis with subcutaneous extension is
usually seen. It typically shows round to oval tumor cells
with high nuclear: cytoplasmic ratio, scanty cytoplasm,
stippled chromatin and high mitotic figures. Intervening
stroma can have few congested and proliferating blood
vessels. Immunohistochemistry, characterize this tumor
by showing positivity for neuron specific enolase, cy-
tokeratin and epithelial membrane antigen [4].
Differential diagnosis included Kaposi sarcoma which
classically present as indolent erythematous or violace-
ous patch or plaques, acquired capillary hemangioma
which is reddish pedunclated mass with characteristic
histology showing multiple endothelial cells with abun-
dant capillaries. Nearly 66% patients with this tumor had
regional lymph node involvement at the time of diagno-
sis or within 18 months of presentation. Distant metasta-
sis is seen in more than one third of patients spreading to
skin, bone, brain, liver and lung [5].
The National Comprehensive Cancer Network and
American Joint Committee of Cancer staging parameters
have classified MCC into the following stages: stage I
(primary tumor < 2 cm), stage II (primary tumor ≥ 2 cm),
stage III (regional nodal disease), and stage IV (distant
metastasis) [6]. On its basis, the 3-year survival rate of
90%, 70%, 60% & 20% has been described for the above
mentioned stages, respectively [7].
Surgical resection has been described as standard of
care for this tumor. Wide excision (1 to 2 cm) with tumor
free margins shown by frozen section technique been ad-
vised to minimize local recurrence rate. Sentinel lymph
node biopsy can be a helpful tool in determination of
subclinical nodal metastases for evaluating proper extent
of disease [8]. Recurrence rate of 21% has been found by
a case series using wide surgical excision with the long-
est duration of 30 months in one case [9].
Radiotherapy and chemotherapy has been advised in
management of metastatic disease, the former been more
effective. Thirty eight to 68% overall 5-year survival rate
has been described for all sites [10]. However, the overall
prognosis for Merkel cell tumor of eyelid is relatively
poor due to its aggressive nature and potential for local
recurrence. In conclusion, high clinical suspicion of Mer-
kel cell carcinoma should be kept in a benign appearing
reddish-pink eyelid mass lesion in an elderly male. Be-
cause of high malignant nature of MCC, patient should
be kept on close follow up and should be investigated tho-
roughly for metastasis.
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