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Journal of Pathology and Disease Biology | Volume 2

September 06-07, 2018 | Edinburgh, Scotland

Pathology and Surgical Pathology

International Conference on

Merkel cell carcinoma of unknown primary site: A case presentation and review of the literature

Erisa Kola

and

Mehdi Alimehmeti

University Hospital Center, Albania

erkel cell carcinoma (MCC) is a rare and highly aggressive

neuroendocrine tumor of the skin. MCC was described

first by Toker in 1972 as trabecular carcinoma of the dermis with

high lymphaticmetastatic risk. The incidenceof this rare tumor is

increasing rapidly; the American Cancer Society estimates 1500

new cases in the USA. Based on case reports, the risk factors

include: ultraviolet exposure, Merkel Cell polyomavirus DNA

infection, immunosuppression (HIV-patients, post transplant

pts under immunosuppresive therapy).

MCC is characterized by a high incidence of early locoregional

relapse and distant metastases. The clinical and pathologic

diagnosis of MCC can be challenging, especially when it

presents as nodal metastasis. As a “small round blue cell

tumor,” it can be difficult to differentiate from other small cell

neoplasms of different primary origin. Diagnosis is based on

typical histology representation on hematoxylin-eosin stained

slides along with the results of immunohistochemistry. The

tumour expresses both epithelial and neuroendocrine markers,

so exhibits both epithelial and neuroendocrine differentiation.

Despite aggressive multimodality treatment, Merkel Cell

Carcinoma outcome is primarily based on the stage of disease at

presentation, with both increasing tumor size and lymph node

positivity being associated with a worse prognosis. Moreover,

the mortality rate of MCC is considerably higher than that of

cutaneous melanoma.

Although it has been identified in various anatomical sites, LN

metastatic MCC in the absence of a primary site is extremely

rare and for this reason there is no standard approach to its

management.

We report a case of a 39 year-old male, diagnosed with HIV

infection 8 years ago, who presented to the hospital with an

enlarged lymphnode in the left inguinal area, which revealed

to be metastatic Merkel Cell Carcinoma in the absence of a

primary skin lesion.