Merkel cell carcinoma of the eyelid and periocular region

Abstract

Merkel cell carcinoma (MCC) is a rare primary cutaneous neuroendocrine carcinoma with a high mortality rate. It typically affects elderly Caucasians, with a slight predilection for males. It is associated with chronic sun exposure and/or immunosuppression. Almost half of all cases occur on the head or neck and an estimated 2.5%–10% arise on the eyelids or periocular skin. It ranks as the 5th most common malignant tumor at these sites, preceded in frequency by basal cell, squamous cell and sebaceous carcinoma, as well as melanoma. Its clinical presentation as a violaceous nodule/plaque lacks specificity, and it can be mistaken for cysts, chalazia or basal cell carcinomas. Sub-specialized histopathological and immunohistochemical evaluations are required for diagnosis. Clinical staging defines the extent of disease and governs management. This includes surgery and adjuvant radiotherapy for localized tumors and of late, immunotherapy for metastatic disease. Significant advances in our understanding of the dual etiopathogenesis (Merkel cell polyomavirus- and Ultraviolet radiation-induced) and the biology of the neoplasm have been achieved in recent years. Issuing from the tumor's known susceptibility to host immunity, a recent therapeutic breakthrough has occurred whereby immune checkpoint inhibition has been shown to mitigate advanced disease. These factors and the increased global incidence of the tumor have brought it to the forefront of medical attention. This review provides a clinically relevant update on MCC, with special reference to cases arising on the eyelid/periocular region.

Unusual Metastasis to Eyelid from Extraocular Merkel Cell Carcinoma

“… 2 It usually appears in the upper eyelid (76%) as a solitary, painless, multilobulated, violaceous (bluish-purple) nodule near the lid margin ( Figure 1 ). 6 Ulcerations, madarosis, abnormal vascular configuration, and local invasion are often associated with MCC of the eyelid. 6 The eyelid mass may be accompanied by early regional lymph node metastasis in the initial presentation of MCC.…”

“… 6 Ulcerations, madarosis, abnormal vascular configuration, and local invasion are often associated with MCC of the eyelid. 6 The eyelid mass may be accompanied by early regional lymph node metastasis in the initial presentation of MCC. MCC of the eyelid may be misdiagnosed as a dermal cyst, chalazion, or hemangioma, and differentiation from other similarly presenting cancerous masses, such as basal cell carcinoma, lymphoma, keratoacanthoma, or metastases, may be difficult in some cases.…”

“…MCC of the eyelid may be misdiagnosed as a dermal cyst, chalazion, or hemangioma, and differentiation from other similarly presenting cancerous masses, such as basal cell carcinoma, lymphoma, keratoacanthoma, or metastases, may be difficult in some cases. 6 Thus, a biopsy for histopathologic evaluation should be performed to confirm the clinical diagnosis.…”

Epidemiology of Merkel Cell Polyomavirus Infection and Merkel Cell Carcinoma

“…MCC most commonly developed on the sun-exposed skin of the head and neck (43–54%), followed by upper limbs and shoulder (24%), lower limbs and hip (15%), trunk (11%), other skin (9.0%), and to a lesser extent on further localizations. An estimated 2.5–10% arise on the eyelids or periocular skin ( Figure 5 ) [ 84 , 85 ]. It was observed that MCC developed slightly more frequently on the left than on the right side of the body [ 86 ].…”

Lower Eyelid Merkel Cell Carcinoma in a Non-Immunocompromised Young Female: A Case Report

“…The 5-year survival rate varies depending on the extent of the disease; for localized disease, it is 51%, whereas for lymph node metastases, it drops to 35%, and for systemic metastases, it further decreases to 14% [ 6 ]. Treatment is based on a combination of surgical resection and radiation therapy, with or without the use of immune checkpoint inhibitors in progressive cases [ 7 ]. MCC is more common in the elderly, with few cases in younger patients associated with immunosuppressive conditions such as HIV/AIDS and solid organ transplantation [ 4 , 8 ].…”