A rare case of atypical chondroid syringoma of the lower eyelid and review of the literature.

A 46-year-old man presented with a painless firm mass in the eyelid margin of the left lower eyelid, which had been present for 9 years. Biopsy nine years previously had not established a diagnosis. We performed excisional biopsy of the lesion and lateral advancement skin flap for reconstruction of the skin defect. Histopathologic and immunohistochemical findings were consistent with atypical chondroid syringoma with eccrine differentiation. The systemic metastatic work-up was negative, and no recurrence or metastasis was present at 30-month follow-up. Chondroid syringoma is a rare skin tumor that occurs, very rarely, in the eyelids, especially the lower eyelid. Only 26 cases of chondroid syringoma in the periorbital area, including our case, have been reported, with various characteristics. Chondroid syringomas are classified as two types, the apocrine type characterized by tubular and cystic branching lumina lined by two layers of epithelial cells, and the eccrine type, which has small tubular lumina lined by a single layer of epithelial cells. The tumor can have benign, atypical, and malignant variants. Our patient had the atypical variant, which is characterized by benign cytological features, as well as the presence of infiltrative margins and/or satellite nodules. Even benign chondroid syringomas, if incompletely excised, can recur with malignant transformation; thus, complete excision and regular follow-up is recommended for all chondroid syringomas.