Epidermotropic Merkel cell carcinoma: A case series with histopathologic examination

doi:10.1016/j.jaad.2009.06.023

Journal of the American Academy of Dermatology

Volume 62, Issue 3, March 2010, Pages 463-468

https://doi.org/10.1016/j.jaad.2009.06.023 Get rights and content

Background

Merkel cell carcinoma (MCC), an aggressive malignancy that has been increasing in incidence, rarely presents with an epidermotropic pattern.

Objective

We conducted an immunohistochemical evaluation of 6 previously unpublished cases of epidermotropic MCC, focusing particularly on the staining characteristics of epithelial membrane antigen and cytokeratin-20 in the hope of providing insight into the mechanism of epidermotropism in MCC.

Methods

This study is a retrospective evaluation using light microscopy and immunohistochemistry.

Results

Forty cases of MCC with pathology at Rhode Island Hospital and the Miriam Hospital in Providence, RI, from 1983 through 2009 were reviewed. Following exclusion criteria, 6 patients (5 men, 1 woman) with a mean age of 82.5 years (range, 72-92) demonstrated epidermotropism. Three of 6 patients had MCC of the eyelid. In cases 1, 3, and 6, the perinuclear dot pattern observed with cytokeratin-20 in the epidermotropic MCC cells was less pronounced than the pattern observed in the dermis, and in all 6 of the tumors, the epidermal staining pattern observed with epithelial membrane antigen was not more or less prominent than the staining observed in the dermis.

Limitations

The small total number of cases of epidermotropic MCC is a limitation.

Conclusion

The data presented reinforce the differential diagnosis of tumors with an epidermotropic growth pattern and the importance of immunohistochemical staining in the histologic workup of such tumors: squamous cell carcinoma in situ, melanoma, mycosis fungoides, eccrine porocarcinoma, sebaceous carcinoma of the eyelid, mammary and extramammary Paget disease, MCC, and epidermotropic metastases. It is notable that 3 of 6 identified tumors were located on the eyelid; further study of epidermotropic MCC may shed more light on this finding, either as an unusual coincidence or a finding with unexplained significance.

Section snippets

Immunohistochemical stains

The immunohistochemical stains on all samples included cytokeratin (CK)-20 dilution: 1:40, clone KS20.8 (Dako North America Inc, Carpenteria, CA); thyroid transcription factor (TTF)-1, dilution: 1:100, clone 8G7G3/1 (Dako North America Inc); epithelial membrane antigen (EMA), dilution: 1:1000, clone E29 (Dako North America Inc); chromogranin, dilution: 1:40, clone LK2H10 (Signet Laboratories, Dedham, MA); and neuron-specific enolase, dilution: 1:100, clone N3 (MIG-N3) (Biogenex Laboratories,

Case reports

The patients were 5 men and one woman whose ages ranged from 72 to 92 years (mean age, 82.5 years). The patients presented with clinical cutaneous changes (Table I) and supportive histopathologic findings (Table II) that led to a diagnosis of epidermotropic MCC.

Immunohistochemical staining

The relative staining patterns of the tumors with each of the immunohistochemical stains, and the percentage of the tumor positively staining, are described in Table II.

CK20 and EMA staining patterns

In cases 1, 3, and 6, the perinuclear dot pattern observed with CK20 in the epidermotropic MCC cells was less pronounced than the pattern observed in the dermis, whereas in the remaining cases there was no significant difference in staining pattern observed. In all 6 tumors, the staining pattern observed with EMA was identical

Discussion

MCC is the second most common cause of nonmelanoma skin cancer death in the United States and has a 5-year overall survival of 75%, 59%, and 25% for local, regional, and distant metastases, respectively. Incidence is estimated at 0.44 cases per 100,000, and is increasing at about 8% per year.1, 2 It is also possible that there is an increase in the incidence of diagnosis as opposed to a true increase in incidence because more is known today about how to diagnose the neoplasm.

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There are more references available in the full text version of this article.

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DermatopathologyEpidermotropic Merkel cell carcinoma: A case series with histopathologic examination