Prognostic factors in Merkel cell carcinoma: Analysis of 240 cases

doi:10.1016/j.jaad.2012.09.036

Journal of the American Academy of Dermatology

Volume 68, Issue 3, March 2013, Pages 425-432

Previously presented orally in the Tromovitch Award finalist session at the Annual Meeting of the American College of Mohs Surgery, New York, NY, May 2, 2010.

https://doi.org/10.1016/j.jaad.2012.09.036 Get rights and content

Background

Knowledge regarding behavior of and prognostic factors for Merkel cell carcinoma (MCC) is limited.

Objective

We sought to further understand the characteristics, behavior, prognostic factors, and optimal treatment of MCC.

Methods

A multicenter, retrospective, consecutive study of patients with known primary MCC was completed. Overall survival and survival free of locoregional recurrence were calculated and statistical analysis of characteristics and outcomes was performed.

Results

Among the 240 patients, the mean age at diagnosis was 70.1 years, 168 (70.0%) were male, and the majority was Caucasian. The most common location was head and neck (111, 46.3%). Immunosuppressed patients had significantly worse survival, with an overall 3-year survival of 43.4% compared with 68.1% in immunocompetent patients. In our study, patients with stage II disease had improved overall survival versus those with stage I disease, in a statistically significant manner. Patients with stage III disease had significantly worse survival compared with stage I and with stage II. Primary tumor size did not predict nodal involvement.

Conclusion

The data presented represent one of the largest series of primary MCC in the literature and confirm that MCC of all sizes has metastatic potential, supporting sentinel lymph node biopsy for all primary MCC. Because of the unpredictable natural history of MCC, we recommend individualization of care based on the details of each patient’s tumor and clinical presentation.

Section snippets

Methods

After institutional review board approval, a search was performed of the institution’s medical index for all cases of MCC consulted on at our facilities during January 1981 to December 2008. In each case, a dermatopathologist or pathologist at our institution made or confirmed the diagnosis of MCC based on permanent section biopsy specimen. Patients with MCC of unknown primary were excluded.

A retrospective review of each patient’s medical record was completed. Patient demographics and medical

Results

A total of 240 patients with known primary MCC were identified who met the inclusion criteria as defined above and make up the study population analyzed. Mean age at diagnosis was 70.1 years (SD, 11.7; range, 24-94), and the majority of patients were male (70%) and Caucasian (98.3% of those reported) (Table I). The most common anatomic location of primary MCC in our series was the head and neck at 46.3% (111) (Table II). Mean tumor size at diagnosis was 2.2 cm for all sites. When recorded, the

Discussion

There are few large-scale retrospective studies of MCC in the literature; these data now represent one of the largest series. Similar to the findings of other retrospective studies, our patients were largely older Caucasian men (>65 years of age), with tumors predominately on the head and neck.⁴ Mean tumor size in our patients was slightly larger (2.2 cm) than other reports (<2 cm), possibly because of referral and self-selection bias as Mayo Clinic is a large tertiary care referral center.⁵

Conclusion

Our findings support the current National Comprehensive Cancer Network (NCCN) treatment recommendations for patients with MCC. We note that all recommendations are based on retrospective data and no large-scale prospective data are currently available. As has been demonstrated with other forms of nonmelanoma skin cancer, we found that immunosuppressed patients have a significantly worse course but are unable to conclude that aggressive treatment for this group of patients would impact overall

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MCC is an aggressive tumour with 26% of cases presenting lymph node involvement at diagnosis and 8% with distant metastases. Five-year overall survival rates range between 48% and 63%. Two subsets of MCC have been characterised with distinct molecular pathogenetic pathways: ultraviolet-induced MCC versus virus-positive MCC, which carries a better prognosis. In both subtypes, there are alterations in the retinoblastoma protein and p53 gene structure and function. MCC typically manifests as a red nodule or plaque with fast growth, most commonly on sun exposed areas. Histopathology (small-cell neuroendocrine appearance) and immunohistochemistry (CK20 positivity and TTF-1 negativity) confirm the diagnosis. The current staging systems are the American Joint Committee on Cancer/Union for international Cancer control 8th edition. Baseline whole body imaging is encouraged to rule out regional and distant metastasis.
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Original articlePrognostic factors in Merkel cell carcinoma: Analysis of 240 cases

Background

Objective

Methods

Results

Conclusion

Section snippets

Methods

Results

Discussion

Conclusion

J Am Acad Dermatol

J Am Acad Dermatol

Am J Surg

J Am Acad Dermatol

Clonal integration of a polyomavirus in human Merkel cell carcinoma

Science

Merkel cell carcinoma

J Clin Oncol

Features predicting sentinel lymph node positivity in Merkel cell carcinoma

J Clin Oncol

Original article
Prognostic factors in Merkel cell carcinoma: Analysis of 240 cases