Merkel cell carcinoma: clinicopathologic correlation, management, and follow-up in five patients

To review the clinicopathologic features, management and follow-up in five patients with periocular Merkel cell carcinoma (MCC).

In this case series study we reviewed the clinical records and histopathologic findings of five consecutive patients with MCC, treated and followed between May 1991 and November 1998.

Four patients were female and one was male. Their mean age at the time of surgery was 80 years (range: 69-86 years). Patients presented with a painless, nonulcerated, rapidly growing, solitary reddish or violaceous nodule, ranging in size from 11 to 21 mm, located in the upper eyelid in 4 patients and in the left eyebrow in one patient. Management included excision with frozen section control in three patients, and excision with wide surgical margins in two patients. Histopathologically, all tumors exhibited round cells of intermediate size and scanty cytoplasm, and large, round to oval vesicular nuclei with finely dispersed chromatin and one to three inconspicuous nucleoli. Mitotic figures were numerous. No patient had regional or extraregional metastases at diagnosis. Local recurrence or distant metastases were not detectable during a mean follow-up period of 45 months (range: 32-61 months). This outcome contrasts with the high incidence of local recurrence and early nodal metastasis reported in previous publications.

Early diagnosis and aggressive, histologically controlled surgical treatment of this rare, highly malignant tumor may provide a longer disease-free period. Further investigation is necessary to determine the prognostic factors for recurrence and survival.