Merkel cell carcinoma of the eyelid management and prognosis

Herbert, Helen M., Sun, Michelle T., Selva, Dinesh, Fernando, Bertie, Saleh, George M., Beaconsfield, Michele, Collin, Richard, Uddin, Jimmy, Meligonis, George, Leatherbarrow, Brian, Ataullah, Sajid, Irion, Lucianne, McLean, Chris J., Huilgol, Shyamala C., Davis, Garry and Sullivan, Timothy J. (2014) Merkel cell carcinoma of the eyelid management and prognosis. JAMA Ophthalmology, 132 2: 197-204. doi:10.1001/jamaophthalmol.2013.6077

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Author Herbert, Helen M.
Sun, Michelle T.
Selva, Dinesh
Fernando, Bertie
Saleh, George M.
Beaconsfield, Michele
Collin, Richard
Uddin, Jimmy
Meligonis, George
Leatherbarrow, Brian
Ataullah, Sajid
Irion, Lucianne
McLean, Chris J.
Huilgol, Shyamala C.
Davis, Garry
Sullivan, Timothy J.
Title Merkel cell carcinoma of the eyelid management and prognosis
Journal name JAMA Ophthalmology   Check publisher's open access policy
ISSN 2168-6165
Publication date 2014-02
Year available 2014
Sub-type Article (original research)
DOI 10.1001/jamaophthalmol.2013.6077
Open Access Status File (Publisher version)
Volume 132
Issue 2
Start page 197
End page 204
Total pages 8
Place of publication Chicago, United States
Publisher American Medical Association
Collection year 2015
Language eng
Formatted abstract
Importance:  The literature on Merkel cell carcinoma (MCC) of the eyelid remains scarce, and there has yet to be a study using the most up-to-date TNM staging system for this rare but aggressive tumor.

Objective:  To analyze the TNM stage, management, and outcomes of patients with MCC of the eyelid.

Design, Setting, and Participants:  Retrospective case series of 21 patients from 5 tertiary referral centers in the United Kingdom and Australia with primary MCC of the eyelid presenting at a median age of 77 years, with median follow-up of 54 months. Tumors were staged according to the American Joint Committee on Cancer, 7th edition, TNM criteria for eyelid carcinoma and MCC.

Main Outcomes and Measures:  TNM stage, treatment modalities, and clinical outcome.

Results:  The eyelid carcinoma TNM stages were T2aN0M0 for 5 patients, T2bN0M0 for 7 patients, T3aN0M0 for 4 patients, T3bN0M0 for 3 patients, T2bN1M0 for 1 patient, and T3aN1M0 for 1 patient. The MCC TNM stages were T1N0M0 for 12 patients, T2N0M0 for 7 patients, T1N1M0 for 1 patient, and T2N1M0 for 1 patient. One patient had a sentinel lymph node biopsy, and 8 patients underwent head/neck imaging. Eighteen patients underwent a wide local excision, 12 with a paraffin section and 6 with a frozen section. Two patients underwent Mohs surgery, 1 of whom required an orbital exenteration. Twelve patients (57%) received adjuvant radiotherapy, and 2 patients received chemotherapy. The local recurrence rate was 10%, the regional nodal recurrence rate was 10%, and the distant metastatic recurrence rate was 19%. The lowest T category tumor metastasizing to both regional nodes and distant locations was a T2a (eyelid TNM)/T1 (Merkel TNM) tumor measuring 8 mm. Two patients with T3a (eyelid TNM)/T2 (Merkel TNM) tumors died of metastatic MCC.

Conclusions and Relevance:  The majority of patients with MCC of the eyelid present with localized eyelid disease of T category T2 (eyelid TNM)/T1 (Merkel TNM). A wide local excision with margin control remains the mainstay of treatment, whereas the use of radiotherapy is institution specific. Tumors with a low T category are associated with regional nodal and distant metastatic disease. It may therefore be reasonable to consider a sentinel lymph node biopsy or strict regional lymph node surveillance for all MCCs of the eyelid, regardless of T category or size.

Merkel cell carcinoma (MCC) is an aggressive skin tumor that was first described in 1972 and that has since been increasing in incidence.1,2 Established risk factors for MCC include immunosuppression and extensive sun exposure, and Merkel cell polyomavirus has recently been identified in 80% of MCCs.3- 5 The estimated mortality rate for all patients with MCC is 25% to 35%.6 Involvement of the eyelid occurs in 10% of cases, with an annual incidence of approximately 0.23 cases per 100 000 persons.7,8 There are no randomized clinical treatment trials determining the efficacy of treatment, and management is stage dependent.

Historically, a number of staging systems were used for patients with MCC.9,10 These have since been consolidated into the 2010 TNM staging system supported by both the American Joint Committee on Cancer (AJCC) and the International Union for Cancer Control (Table 1).11,12 In addition, in recognition of the fairly unique structure of the eyelid, the AJCC TNM staging for eyelid carcinoma, 7th edition, was established to provide a standardized approach to staging disease in this region (Table 2). However, to the best of our knowledge, there are no studies of MCC of the eyelid that have used either staging system. Given the few published series available to help guide us in the management of MCC of the eyelid, we present our experience with 21 cases of primary MCC of the eyelid using both the eyelid and MCC AJCC TNM staging systems.
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Document type: Journal Article
Sub-type: Article (original research)
Collections: Non HERDC
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