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Merkel Cell Carcinoma of the Eyelid With a Positive Sentinel Node
Arch Ophthalmol. 2002;120:646-648.
INTRODUCTION
Merkel cell carcinoma (MCC) of the eyelid is a rare but aggressive malignancy
that metastasizes early to regional lymph nodes.1
Most clinical series suggest a rate of regional nodal involvement between
21% and 66%.2-4
Early detection of occult regional nodal disease may allow for early institution
of adjuvant therapy. We describe a patient with MCC of the eyelid with clinically
uninvolved nodes who underwent sentinel lymph node (SLN) biopsy soon after
the diagnosis of his primary tumor. An SLN was identified and showed histologic
evidence of MCC. To our knowledge, this is the first reported case of a positive
SLN secondary to MCC of the eyelid.
Report of a Case
A 61-year-old man noted an erythematous lesion on his left upper eyelid
in May 2001. He went to his local ophthalmologist, who excised the lesion
but did not examine it histologically. In June 2001, the lesion recurred.
The recurrent lesion measured approximately 12 mm in diameter. The patient
sought an opinion from an oculoplastic surgeon, who performed a wide local
excision of the lesion with frozen section control of the margins. The histologic
findings were consistent with MCC of the eyelid (Figure 1 A and Figure 1
B). Sentinel lymph node biopsy was scheduled but had to be delayed because
the patient developed acute appendicitis, necessitating an emergent appendectomy.
In July 2001, the patient underwent SLN biopsy using a combination of isosulfan
blue dye and Tc 99m–labeled sulfur colloid. The afferent lymphatics
were identified before surgery using radionucleotide imaging. Intraoperatively,
a combination of radiolabeled sulfur colloid and isosulfan blue dye was used
to identify SLNs. An area of focal radioactive uptake was identified in the
left preauricular (parotid) area using a handheld gamma probe and was marked
on the skin. The corresponding SLN was removed and analyzed histologically
using serial sectioning and immunohistochemical staining. The node was found
to be positive for MCC (Figure 1
C and Figure 1 D). The patient subsequently
underwent a total parotidectomy and completion neck dissection. The parotidectomy
specimen included 1 additional lymph node that was positive for MCC. The node
was located in the deep lobe of the left parotid gland and showed extracapsular
extension.
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A, Histologic section of Merkel cell carcinoma (MCC) of the upper
eyelid (primary lesion) demonstrates a diffuse, poorly cohesive proliferation
of small cells with finely dispersed chromatin, lacking prominent nucleoli,
and without significant intervening stroma (original magnification x4).
B, High-powered view shows round cells with finely distributed chromatin.
Many of the cells display hyperchromatic nuclei consistent with apoptotic
bodies (original magnification x40). C, Histologic section of the sentinel
lymph node shows small foci of round cells similar in appearance to the primary
lesion (original magnification x20). D, High-powered view of the same
node immunostained with antibodies against cytokeratin shows positive cytokeratin
expression supporting the diagnosis of metastatic MCC (original magnification
x40).
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In September 2001, the patient self-referred to the University of Texas
M. D. Anderson Cancer Center (Houston) for further management of his tumor.
At this time, he had a well-healed area of excision in his left upper eyelid,
and the area of the neck dissection and parotidectomy had healed. Findings
on the patient's ophthalmologic examination were essentially normal, with
no clinical evidence of local or regional recurrence of cancer. His systemic
workup, including computed tomography of the head and neck, abdomen, and pelvis,
chest radiography, and magnetic resonance imaging of the brain, was negative
for tumor. A multidisciplinary team, including an ophthalmic surgeon, a head
and neck surgeon, a head and neck medical oncologist, and a head and neck
radiation oncologist examined the patient and recommended that he receive
adjuvant external beam radiation therapy to the eyelid, parotid nodes, and
deeper cervical nodes. The team also recommended that the patient be given
4 courses of chemotherapy with etoposide and cisplatin after the completion
of radiation therapy.
Comment
In patients with MCC, the regional lymph nodes are thought to be the
most common and earliest site of metastasis; thus, adjuvant treatment of the
regional lymph nodes has been advocated by many investigators.3, 5-6
Jean et al7 successfully identified 1 or
more SLNs in 19 of 20 patients with stage I MCC who underwent SLN biopsy at
the time of initial wide local excision. The authors found that 5 (26%) of
the 19 patients in whom SLNs were successfully identified had at least 1 histologically
positive SLN. Other isolated cases of SLN biopsy for MCC have also been reported.8
Merkel cell carcinoma of the eyelid is thought to account for 10% of
all cases of MCC.2 In a review of all previously
reported cases of MCC of the eyelid, Kivela and Tarkkatnen2
concluded that up to two thirds of patients eventually develop regional nodal
involvement. This rate is higher than the rate reported in most single series
of MCC of the eyelid. In the largest single series to date, Peters et al3 reported clinical regional nodal involvement in
3 (21%) of their 14 patients.
Sentinel lymph node biopsy allows for early detection of occult regional
lymph node metastasis and thus, more accurate staging of MCC and the possible
institution of early adjuvant therapy. Although SLN biopsy has recently become
the standard of care for most solid tumors throughout the body, SLN biopsy
in the periocular area remains investigational.9
To our knowledge, there have been only 2 previous reports of application of
SLN biopsy techniques for conjunctival and periocular tumors.10-11
We described successful identification of SLNs in a single patient with a
conjunctival melanoma, using a combination of radiolabeled sulfur colloid
and isosulfan blue dye.10 Wilson et al11 attempted identification of SLNs in 5 patients
with periocular tumors (2 melanomas, 2 meibomian gland carcinomas, and 1 case
of mucoepidermoid carcinoma), using radiolabeled sulfur colloid alone. These
authors reported successful identification of at least 1 SLN in 5 patients,
although the technique used in the latter report is believed by some investigators
not likely to lead to the correct identification of the sentinel nodes.12 Neither of the 2 previous reports of SLN biopsy
for conjunctival and periocular tumors found a positive SLN. To our knowledge,
ours is the first reported case in which an SLN was successfully identified
in a patient with an eyelid tumor and was also found to be histologically
positive.
This case underscores the feasability and potential usefulness of SLN
biopsy as a method for identifying occult metastatic disease from an MCC of
the eyelid. Early detection of micrometastasis in the regional nodes allows
for immediate institution of adjuvant therapy, which may include completion
neck dissection, external beam radiation therapy, and adjuvant chemotherapy.
AUTHOR INFORMATION
Bita Esmaeli, MD;
Aresu Naderi, MD;
Lillie Hidaji, BS;
George Blumenschein, MD;
Victor G. Prieto, MD, PhD
Houston, Tex
Corresponding author and reprints: Bita Esmaeli, MD, Ophthalmology
Section, Department of Plastic Surgery, Box 443, M. D. Anderson Cancer Center,
1515 Holcombe Blvd, Houston, TX 77030 (e-mail: besmaeli{at}mdanderson.org).
REFERENCES
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4. Victor NS, Morton B, Smith JW. Merkel cell cancer: is prophylactic lymph node dissection indicated? Am Surg. 1996;62:879-882.
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cutaneous neuroendocrine (Merkel cell) carcinoma. Int J Radiat Oncol Biol Phys. 1995;31:315-323.
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7. Jean M, Solorzano C, Ross M, et al. Role for sentinel node biopsy in Merkel cell carcinoma patients . Proc Am Soc Clin Oncol. 2001;20:358a.
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12. Esmaeli B. In discussion of: Wilson MW, Fleming JC, Fleming RM, Haik B. Sentinel
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SECTION EDITOR: W. RICHARD GREEN, MD
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