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Topic: Neuroendocrine Tumors

Merkel cell carcinoma responsive to Etoposide: a case report and brief
literature review

Chiara Ancona , Andrea Caff , Giovanni Manfredi Assanto , Stefano Cordio 3
2
1
1
1 Department of Medical Oncology, Policlinico P. Giaccone, University of Palermo, Via del Vespro 129, 90127 Palermo, Italy.
2 Endocrinology, Department of Clinical and Experimental Medicine, Garibaldi-Nesima Medical Center, University of Catania, Via Palermo 636, 95122 Catania, Italy.
3 Department of Oncology, Garibaldi-Nesima Medical Center, University of Catania, Via Palermo 636, 95122 Catania, Italy.
Correspondence to: Dr. Andrea Caff, Endocrinology, Department of Clinical and Experimental Medicine, Garibaldi-Nesima Medical Center,
University of Catania, Via Palermo 636, 95122 Catania, Italy. E-mail: caff.andrea@gmail.com

A B S T R AC T
Merkel cell carcinoma (MCC), first described in 1972, is an aggressive primary cutaneous carcinoma able to incorporate both
epithelial and neuroendocrine features. MCC mainly appears in individuals in their eighth decade and it is related to a high
mortality rate. The etiology of this rare disease is not well-understood but ultraviolet radiation exposure, immune suppression,
and aging have a consistent role in its pathogenesis. Usually, clinical lesions appear as asymptomatic coloured dermal nodules.
The tumour can involve lymph nodes but further evaluation with imaging is recommended. The common approach for localized
disease is surgical. This work reports a case of an 86-year-old man with locally advanced MCC where, based on clinical
experience, oral mono-chemotherapy with single-agent etoposide was chosen as first-line therapy. A complete objective response
was achieved in 2 months.

Key words: Merkel cell carcinoma; neuroendocrine; chemotherapy; etoposide

INTRODUCTION latter, many reports described a strong correlation between
infection and carcinogenesis, although the presence of the
Merkel cell carcinoma (MCC) is a rare, aggressive, virus itself is not sufficient to induce MCC.
neuroendocrine carcinoma of the skin that originates from
Merkel cells of the dermoepidermal junctions, although Clinically, the lesion appears as a fast-growing, painless,
some recent work proposes pluripotent dermal stem cells solitary dermal nodule, firm, non-tender, coloured from
to be origin of this neoplasm. [1] red to violet; rarely does it present as an ulceration.

The annual incidence is 0.6 per 100,000 persons but Skin of the face, arms and lower limbs are the most
[2]
is apparently increasing in the last years thanks to more common sites of localization whereas the trunk and oral
accurate diagnostic pathology techniques, an aging and genital mucosa are rare. [2]
population, increased sun exposure, and improved registry
tools. Typical clinical features are summarized in the acronym
“AEIOU” proposed by Heath et al. : asymptomatic,
[5]
MCC has a high mortality rate, the overall 5-year survival expanding rapidly, immunosuppression, older than age 50
rates ranging from 30% to 64%. [3] and ultraviolet-exposed site.

Males are more often affected than females, the median The approach to disease management includes

age at diagnosis being 76 years. It is extremely rare in with a complete physical examination followed by
[2]
children, with only a few cases reported in literature. imaging. Treatment strategies are best considered in
a multidisciplinary board consultation. The surgical
Ultraviolet radiation exposure, chronic immune suppression approach, when negative margins are possible and the
(especially from chronic lymphocytic leukemia, human disease is not disseminated, should be the first choice
immunodeficiency virus, and prior solid organ transplant) followed, when the risk assessment contemplates it or
and the Merkel cell polyomavirus are the main risk factors
involved in the tumour pathogenesis. Concerning the This is an open access article distributed under the terms of the Creative
[4]
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others to remix, tweak, and build upon the work non-commercially, as long as
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How to cite this article: Ancona C, Caff A, Assanto GM, Cordio S.
Merkel cell carcinoma responsive to Etoposide: a case report and
DOI: brief literature review. J Cancer Metasta Treat 2016;2:310-2.
10.20517/2394-4722.2016.46
Received: 28-07-2016; Accepted: 01-08-2016.
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©2016 Journal of Cancer Metastasis and Treatment ¦ Published by OAE Publishing Inc.