Abstract

Chondroid syringoma is a rare benign, skin appendageal tumor, also known as mixed tumors of the skin. The clinical presentation of chondroid syringoma is non-specific, and the diagnosis is made histopathologically. A case of a 74-year-old man presented with an asymptomatic 1.3-cm-sized, firm, skin-colored, subcutaneous nodule of 8 years’ duration on the superior helical rim of the left ear. Stretching of the skin over the tumor showed the “tent sign” with many facets and angles, a pathognomonic sign for pilomatricoma. The nodule was totally excised under the impression of pilomatricoma. The histopathologic findings showed the characteristic features of chondroid syringoma. Chondroid syringoma arising in the helix is extremely rare; only four cases have been reported in the literature. This case represented the fifth documented case of chondroid syringoma occurring in the helix with clinical presentation mimicking pilomatricoma and the first case involving a male patient. A review of the relevant literature is also presented.