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      Giant Merkel Cell Carcinoma Masquerading as a Benign Cyst on the Buttock of an African American Man

      case-report

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          Abstract

          We report a case of a 60-year-old African American man who presented with a 4-year history of a previously asymptomatic, recently enlarging nodule on his left buttock, which was initially presumed to be an epidermoid cyst. Physical examination revealed a large, fixed, subcutaneous tumor, and a biopsy revealed merkel cell carcinoma. Immunohistochemical staining was positive for pankeratin, CAM 5.2, synaptophysin, and CD56 and negative for CK7, CK20, TTF-1, chromogranin, CD3, CD20, CD57, MART1, and HMB 45. The patient underwent wide local excision of the lesion with removal of the fascia overlying the gluteus and full body positron emission tomography (PET) and was found to have Stage IIb disease. He subsequently received adjuvant radiotherapy limited to the tumor bed at a dose of 60 gray.

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

          Michèlle C. Heath, Natalia Jaimes, Bianca Durando Lemos … (2008)
          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. We sought to define the clinical characteristics present at diagnosis to identify features that may aid clinicians in recognizing MCC. 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. 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. 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. 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.
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            Merkel cell carcinoma demographics, morphology, and survival based on 3870 cases: a population based study.

            Kristen A. Batich, Noa Sagy, M. F. Montero … (2009)
            Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine tumor arising predominantly on sun-exposed skin of older and usually immunosuppressed individuals. Using data from NCI's SEER (Surveillance, Epidemiology, and End Results) Program from 1973 to 2006, we analyzed the demographics and survival of MCC. SEER had recorded 3870 cases of MCC. The incidence was higher in men (2380 cases, 61.5%) than in women (1490 cases, 38.5%). Most patients were White (94.9%) between 60 and 85 years of age. MCC was rare in Blacks. The most common location was the head and neck. The salivary glands, nasal cavity, lip, lymph nodes, vulva, vagina and esophagus were the most common extracutaneous sites. The 10-year relative survival rate was higher in women than men (64.8% vs. 50.5%, p < 0.001). Patients 50-69 years had the highest 10-year relative survival rate (59.6%). Stage of disease was the best predictor of survival. MCC arises predominantly in the skin of head and neck in White men above 70 years of age. Cases also occurred in extracutaneous sites. Age did not predict survival, yet gender, site and tumor size revealed clear differences. The most significant predictor of survival was tumor stage. Copyright © 2009 John Wiley & Sons A/S.
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              Merkel cell carcinoma: what is it, what will it do and where will it go? What role should the pathologist play in reporting this information?

              Douglas Brown, Bruce Smoller (2009)
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                Author and article information

                Journal
                Journal ID (nlm-ta): Case Rep Oncol Med
                Journal ID (iso-abbrev): Case Rep Oncol Med
                Journal ID (publisher-id): CRIM.ONCMED
                Title: Case Reports in Oncological Medicine
                Publisher: Hindawi Publishing Corporation
                ISSN (Print): 2090-6706
                ISSN (Electronic): 2090-6714
                Publication date (Print): 2011
                Publication date (Electronic): 22 September 2011
                Volume: 2011
                Electronic Location Identifier: 849767
                Affiliations
                Department of Dermatology, College of Medicine, University of Cincinnati, Cincinnati, OH 45267, USA
                Author notes

                Academic Editors: F. F. Attiyeh, J. M. Buchanich, and R. Yamamoto

                Article
                DOI: 10.1155/2011/849767
                PMC ID: 3350009
                PubMed ID: 22606448
                SO-VID: 8c6b2dab-a715-4438-b2b6-24c6b5ec2e89
                Copyright © Copyright © 2011 Marissa J. Perman et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 6 July 2011
                Date accepted : 2 August 2011
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Oncology & Radiotherapy
                Data availability:
                ScienceOpen disciplines: Oncology & Radiotherapy

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