Multiple cutaneous metastases: a rare and late sequelae of lacrimal gland adenoid cystic carcinoma

Abstract

Primary cutaneous adenoid cystic carcinoma (ACC) is an uncommon malignancy with approximately 60 cases reported in the literature. Metastatic ACC to the skin is even less frequent with eight previously published cases, including only one arising from the lacrimal gland. We report a case of a 61-year-old male, who developed multiple cutaneous metastases to the scalp and back from a lacrimal gland ACC 21 years after the initial diagnosis. Initially, in the absence of the past clinical history, the cutaneous lesions were interpreted as primary ACC of the skin. Histopathologically, the metastatic lesions were poorly circumscribed dermal and subcutaneous nodules that were composed of islands of basaloid epithelial cells with ducts and cribriform pattern, mucinous stroma and prominent perineural invasion. Histomorphologically, the cutaneous tumors were similar to the primary ACC. The cutaneous metastases were preceded by tumor dissemination to the lung and other visceral organs, and ultimately led to patient's demise. This report illustrates the importance of juditious clinico-pathologic correlation and raises awareness regarding prolonged latency of cutaneous metastases in patients with visceral ACC.