Merkel Cell Carcinoma of the Upper Eyelid: When Reconstruction Becomes a Challenge

Merkel cell carcinoma (MCC) is a rare and aggressive tumor arising from Merkel cells located in the basal layer of the epidermis and in the hair follicle. MCC presents primarily in Caucasians (98.3%) with a median age of 69 years. Ultraviolet radiation exposure and immunosuppression appear to play a role in the development of this cancer [1]. MCCs display an aggressive clinical course with a high rate of local recurrence (26%–60%), regional lymph node invasion (45%–91%), and metastasis to the lung, liver, brain, bone, and skin (18%–52%). Eyelid MCCs account for 5%–10% of all MCCs [2]. Although clinical management is often discussed, few cases involving the eyelid location have been described. For anatomical reasons and due to the rarity of the tumor, standard site-specific treatment regimens have not been defined [3]. We present a case of Merkel cell carcinoma of the eyelid that was treated with the aim of achieving surgical eradication of the tumor, as well as satisfactory aesthetic and functional outcomes. A 75-year-old Caucasian female was referred to our clinic 1 month after presentation of a rapidly growing lesion of the right upper eyelid. Clinical examination showed a solitary, asymptomatic, 1 cm diameter, pink-red vascularized cutaneous nodule of the middle third of the right upper eyelid (Fig. 1). Histological examination of an incision biopsy established the diagnosis of MCC. Clinically, there was no evidence of lymph node involvement in the head, neck, or axillae. The preoperative staging of MCC included a whole-body computed tomography (CT) spiral scan with contrast and positron emission tomography (F-fluorodeoxyglucose positron emission tomography/CT). Screening did not reveal any metastases, classifying the patient in IB stage (T1cN0-M0). Under general anesthesia, we performed a wide excision of the whole upper right eyelid containing the lesion with 1 cm free margins and preserving the upper part of the lid levator muscle. Intraoperative sections showed no tumor presence in the edges. A full thickness fasciocutaneous Fricke flap from the temporal region was raised, and passed subcutaneously, after de-epithelialisation, to reconstruct the upper lid (Fig. 2). The pedicle, based on peripheral branches of the superficial temporal artery, had previously been identified by ultrasonography about 1 cm from the lateral orbit rim. In order to preserve palpebral functionality, the internal lining (i.e., fascia) of the flap was anchored to the levator muscle. Conjunctival reconstruction using a mucosal graft taken from the lower lip was also necessary for the internal lining. Histologic examination showed a MCC involving the skin and the conjunctiva of the upper lid positive to CK-20 and synaptophysin, with complete excision. The patient underwent adjuvant radiotherapy. At the 1 year follow-up, the patient was free from disease and continued to demonstrate a good functional and cosmetic outcome (Figs. 3, 4). MCC is a rare skin cancer with a mortality rate higher than melanoma. In its early stage, the neoplasm does not demonstrate specific clinic characteristics. Given the rarity of the tumor and the possibility of misdiagnosis, a high index of suspicion is necessary, especially in immunosuppressed patients. The