Background: A wide variety of tumors can affect the nail unit, with some commonly mistaken as inflammatory or infectious diseases. Obtaining an optimal sample for histopathologic evaluation requires understanding of nail unit anatomy as well as the histopathology of the suspected nail tumor. Summary: This review discusses clinical and histopathologic features of a subset of benign and malignant nail tumors, including subungual melanoma, nail unit squamous cell carcinoma in situ, nail unit squamous cell carcinoma, onychomatricoma, onychopapilloma, onychocytic matricoma, and onychocytic carcinoma. Optimal surgical sampling techniques for each lesion are presented. Key Messages: In summary, the clinical and histopathologic features of several common and rare nail unit tumors and the optimal sampling techniques for each lesion are presented in this manuscript. Knowledge of the clinical and histopathologic features of these tumors informs the surgical approach to them and will produce the best patient outcomes.

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