Faculty Articles

Merkel Cell Carcinoma a Lethal Polyomavirus Disease

Publication Title

Journal of the American Medical Directors Association

Publisher

Elsevier Inc.

ISSN

1525-8610

Publication Date

3-2012

Keywords

american cancer society, case report, cutaneous malignancy, elderly patients, immunosuppression, lymphatic invasion, malignant skin tumor, mechanoreceptors, mekel cell carcinoma, merkel cell polyomavirus, mcv, neuroendocrine carcinoma, neuroectodermal origin, perineural invasion, polyomavirus, recurrence, skin cancer, sun exposure, type 2 diabetes mellitus, wide excision

Abstract

Introduction: Merkel cell carcinoma (MCC) is a rare cutaneous malignancy that affects mostly elderly Caucasians. It usually develops on sun-exposed skin as a painless, firm, flesh-colored to red or blue lesion. Frequently patients seek advice from their doctor because the lesion grows rapidly. The disease course varies from relatively indolent to highly aggressive and can spread to local, regional and distant sites. The American Cancer Society estimated for 2008 almost 1500 new cases in the USA. Moreover, the mortality rate of MCC at 33% is considerably higher than that of cutaneous melanoma. Case Description: An 86 year old Caucasian male with history of myeloproliferative disorder and type 2 diabetes mellitus, presented for preoperative evaluation of a lesion in his right knee. He first noticed the lesion in May 2008. It was a flesh-colored, small, well-circumscribed, raised nodule on the anterior aspect of the right knee, with no other abnormalities observed. Biopsy of the lesion was positive for Merkel Cell Carcinoma. The tumor was initially 2.5-cm with perineural probably vascular and lymphatic invasion, according to the report. Treatment options were presented, however the patient refused any aggressive intervention, remained asymptomatic for 2 years. In 2010, a small nodule reappeared in the same location. A wide excision was performed in June 2010. Pathology confirmed recurrent Merkel Cell Carcinoma, and the margins were free of tumor. A total body PET Scan was performed with unremarkable findings. Due to the high recurrence rate, he was recommended systemic chemotherapy or local radiotherapy. The patient has yet to decide what would constitute the best option for him. Discussion: MCC is believed to develop from Merkel cells, they possess dense core neuroendocrine granules and neurofilament. Merkel cells are generally thought to function as mechanoreceptor and chemoreceptors, and they are derived from the neuroectoderm. They function as type I mechanoreceptors. Another theory of the origin of MCC is that the cancer arises from pluripotent stem cells that later differentiate in the neuroendocrine direction. This rare carcinoma is typically seen in older individuals, and the median age is 70 years. MCC can also occur in younger individuals who are immunosuppressed. The fact that MCC is more lethal and more common in immunosuppressed patients suggests that other virally induced cancers that have a higher incidence in the immunosuppressed population. Feng et al. applied a technique called digital transcriptome subtraction to perform a search for viral sequences in MCC derived DNA. Their investigation resulted in the discovery of a genome encompassing 5387 base pairs of a new polyomavirus, which they named the Merkel cell polyomavirus (MCV or MCPyV). Hence, Merkel cell polyomavirus appears to be causally linked to the development of MCC. Approximately 80% of MCC has this virus integrated in a monoclonal pattern.

DOI

10.1016/j.jamda.2011.12.040

Volume

13

Issue

3

First Page

B7

Last Page

B7

Disciplines

Medical Specialties | Medicine and Health Sciences | Osteopathic Medicine and Osteopathy

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Peer Reviewed

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