Rare case of Merkel-Cell Carcinoma reported in NEJM

Dr Pedro J. Gomez-Arias and Dr Rafael Salido-Vallejo at Hospital Universitario Reina Sofia, Cordoba, Spain have reported a rare case of Merkel-Cell Carcinoma. The case has appeared in the New England Journal of Medicine.

Merkel cell carcinoma (MCC) is a rare, aggressive form of skin cancer with a high risk of recurrence and metastasis often within two to three years after initial diagnosis. It is 40 times more rare than melanoma and the risk factors for MCC include sun exposure, fair skin, age over 50 and a weakened immune system.

Merkel cell carcinoma (MCC) commonly appears as a painless mass on or just under the skin surface. Appropriate clinical diagnosis is often delayed because of a lack of symptoms. Current treatment consists of wide local excision with adjuvant irradiation. Neck dissection is used for clinically positive nodes, and chemotherapy is given for advanced disease.

An 84-year-old woman was referred to the dermatology department for evaluation of a rapidly enlarging nodule on her right hand that had developed over 1 month. She reported no trauma to the area and had no systemic symptoms, such as fever, night sweats, and weight loss. Physical examination revealed a painless, red, denuded nodule over the base of the fourth finger (Panel A).

No other lesions were identified, and no lymphadenopathy was detected. Analysis of a specimen obtained on punch biopsy revealed nodular infiltration of the dermis (Panel B), and a diagnosis of Merkel-cell carcinoma was made. Merkel-cell carcinoma is an aggressive cutaneous neuroendocrine tumour that is associated with polyomavirus infection, immunosuppression, advanced age, and sun exposure.

Patients typically present with enlarging red or pink nodules in sun-exposed areas. The patient underwent amputation of the finger, and because a biopsy specimen obtained from a sentinel lymph node was positive for malignant cells, axillary lymph node dissection was performed. No evidence of recurrence was found on follow-up at 5 months.