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Clinical characteristics of Merkel cell carcinoma at diagnosis in 195 patients: the AEIOU features

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Background

Merkel cell carcinoma (MCC) is an aggressive skin cancer with a mortality of 33%. Advanced disease at diagnosis is a poor prognostic factor, suggesting that earlier detection may improve outcome. No systematic analysis has been published to define the clinical features that are characteristic of MCC.

Objective

We sought to define the clinical characteristics present at diagnosis to identify features that may aid clinicians in recognizing MCC.

Methods

We conducted a cohort study of 195 patients given the diagnosis of MCC between 1980 and 2007. Data were collected prospectively in the majority of cases, and medical records were reviewed.

Results

An important finding was that 88% of MCCs were asymptomatic (nontender) despite rapid growth in the prior 3 months (63% of lesions) and being red or pink (56%). A majority of MCC lesions (56%) were presumed at biopsy to be benign, with a cyst/acneiform lesion being the single most common diagnosis (32%) given. The median delay from lesion appearance to biopsy was 3 months (range 1-54 months), and median tumor diameter was 1.8 cm. Similar to earlier studies, 81% of primary MCCs occurred on ultraviolet-exposed sites, and our cohort was elderly (90% >50 years), predominantly white (98%), and often profoundly immune suppressed (7.8%). An additional novel finding was that chronic lymphocytic leukemia was more than 30-fold overrepresented among patients with MCC.

Limitations

The study was limited to patients seen at a tertiary care center. Complete clinical data could not be obtained on all patients. This study could not assess the specificity of the clinical characteristics of MCC.

Conclusions

To our knowledge, this study is the first to define clinical features that may serve as clues in the diagnosis of MCC. The most significant features can be summarized in an acronym: AEIOU (asymptomatic/lack of tenderness, expanding rapidly, immune suppression, older than 50 years, and ultraviolet-exposed site on a person with fair skin). In our series, 89% of primary MCCs had 3 or more of these findings. Although MCC is uncommon, when present in combination, these features may indicate a concerning process that would warrant biopsy. In particular, a lesion that is red and expanding rapidly yet asymptomatic should be of concern.

Section snippets

Methods

Institutional review board approval was obtained from each institution. Tumor registry data and prospective patient identification (beginning in 2003) were used to identify 195 patients from 3 medical centers in Boston, Mass (Dana Farber Cancer Institute, Brigham and Women's Hospital, and Massachusetts General Hospital) and two medical centers in Seattle, Wash (Seattle Cancer Care Alliance and University of Washington Medical Center). The study included patients with a pathologic diagnosis of

Patient characteristics

As shown in Table I, the median age at diagnosis was 69 years, with 90% of patients being older than 50 years. There was a slight male predominance with a ratio of 1.4:1 (58.5% male and 41.5% female). The vast majority of patients were white (98%), with only 4 patients being nonwhite (3 Asian and 1 black). Profound immunosuppression including HIV (3 patients), CLL (8 patients), or solid organ transplantation (4 patients) was noted in 7.8% of patients. The mean age of presentation of the

Discussion

This study of 195 patients was conducted to identify key features of MCC that may aid clinicians in recognizing when a biopsy may be warranted. The basic demographic profile of our cohort is similar to that described in other studies: mostly elderly, white, and with a slight male predominance.

The anatomic distribution of the tumors seen in our study further supports sun exposure as a risk factor for the development of MCC, consistent with prior studies. Agelli and Clegg3 used SEER registry data

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  • Cited by (698)

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    Dr Jaimes is currently affiliated with Dermatology, Universidad Pontificia Bolivariana, Medellín, Colombia. Dr Mostaghimi is currently affiliated with Beth Israel Deaconess Medical Center, Boston, Mass.

    Supported by Harvard/National Cancer Institute Skin Cancer Specialized Program of Research Excellence (SPORE), University of Washington Merkel Cell Carcinoma Research Fund, National Institutes of Health K02-AR050993, and American Cancer Society Jerry Wachter Fund.

    Conflicts of interest: None declared.

    Selected and limited preliminary descriptive results presented in abstract form at the 2007 Annual Meeting of the Society for Investigative Dermatology, in Los Angeles, California, on May 12, 2007.

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