Merkel Cell Carcinoma of the Lower Extremity: Report of Four Cases and New Considerations

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Merkel cell carcinoma (MCC) is a rare neuroendocrine cutaneous malignancy that predominantly arises in the head and neck region. We describe clinical features, diagnosis, and treatment in 4 cases of MCC, presenting an uncommon female predominant occurrence and an unusual primary site: the lower limb.

In all cases diagnosis was established by histopathologic examination. Primary MCC and locally recurrence disease were treated in all patients with a wide surgical excision (3-cm margin) including fascia. Lymphadenectomy was reserved for a patient with clinical evidence of nodal involvement. Both chemotherapy administered in 2 cases and radiotherapy in 1 case produced limited responses.

Early diagnosis is critical because this tumor is aggressive and has a high rate of local recurrence and metastatic spread. However, its nondistinctive appearance frequently delays diagnosis and its rarity avoids an optimal treatment guideline setting.

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