These three new cases and the review of 43 cases reported in the literature call attention to the clinical features of carcinomas arising in the context of rhinophyma, raising further concerns about the possible association between these two entities. The need for histologic examination of all surgically removed tissue in patients with rhinophyma is highlighted. Several macroscopic changes, including ulceration, drainage, and a rapid growth pattern, should alarm the physician and should be considered as suspicious of a malignant degeneration. Unexpected clinical modifications of a preexisting long-lasting silent rhinophyma could indicate the possibility of hidden malignancy rather than a rhinophyma itself. Although evidence of an association between the two entities remains inconclusive, half of the malignancies reported in our review were incidental findings associated with rhinophyma. Thus, since rhinophyma should not be considered solely a cosmetic problem, we recommend that all specimens be reviewed by a pathologist and if malignancy is diagnosed, re-excision with clear margins should be achieved when necessary with periodic follow-up.
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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