title: Merkel cell carcinoma of the lower extremity: report of four cases and new considerations.
abstract: 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.
authors.affiliation: Operative Unit of Plastic and Reconstructive Surgery, Hospital of Pisa, Pisa, Italy.
authors.forename: Giordano
authors.initials: G
authors.lastname: Giannotti
authors.forename: Davide
authors.initials: D
authors.lastname: Lazzeri
authors.forename: Paolo
authors.initials: P
authors.lastname: Viacava
authors.forename: Benedetta
authors.initials: B
authors.lastname: Barbieri
authors.forename: Livio
authors.initials: L
authors.lastname: Colizzi
authors.forename: Giovanni
authors.initials: G
authors.lastname: Fanelli
authors.forename: Daniele
authors.initials: D
authors.lastname: Pagnini
authors.forename: Alessandro
authors.initials: A
authors.lastname: Massei
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015266:Carcinoma, Merkel Cell / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D007866:Leg; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000473:pathology / Q000628:therapy