Secretory Carcinoma: A Very Rare Tumor Arising in the Eyelid Skin

Introduction Secretory carcinoma of the skin is a rare tumor characterized by glandular differentiation similar to secretory carcinomas arising in the breast and in the salivary glands. This tumor was initially described in 2009 in the axilla of a 13-year-old girl [ 1 ]. In the skin, less than 35 cases have been documented to date. The most common location seems to be the axilla [ 2 ] (56%), followed by the neck (13%), but other locations such as lip or breast tissue [ 3 ] have been described. In the eyelids, 5 cases [ 3 ], [ 4 ], [ 5 ], [ 6 ], [ 7 ] have to best of our knowledge been reported so far, more commonly affecting women (60%), with a mean age of 51 years (SD 14.8). We hereby document the clinico-pathological findings of such a tumor arising the upper eyelid skin in a 60-year-old woman. The demonstration of a rearrangement the N-terminal domain of ETV6 (E26 Variant Transcription Factor 6) on chromosome 12 and most commonly the C-terminal tyrosinase domain of NTKR3 (Neurotrophic Tyrosine Kinase Receptor 3) on chromosome 15, leading to ETV6-NTKR3 fusion gene is essential to confirm the diagnosis. Publication History Received: 03 November 2025 Accepted: 26 December 2025 Article published online: 05 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 14, 70469 Stuttgart, Germany