Abstract Purpose Apocrine hidradenocarcinoma has been named with different terms as apocrine carcinoma, malignant clear cell hidradenoma, nodular hidradenocarcinoma, eccrine acrospiroma, and some others. This wide terminology, together with the rarity of the tumour and variability of the cells that compose it, make it difficult to diagnose. Methods Description of a case. Results 81 years‐old female, with a history of pterygium, subject to enucleation of the eye globe after a preoperative diagnosis of squamous cell carcinoma affecting ocular adnexa, this tumor infiltrated bulbar conjunctiva and cornea. Conclusion Malignant tumours of sweat glands are rare. Among these, hidradenocarcinoma has been described in multiple cutaneous locations and exceptionally in the eye. The frequency is not easy to determine because the terminology that has been used makes difficult to compare the data. In the eye, it may originate from glands of the eyelid or from lacrimal gland. The immunohistochemical study is not important for diagnosis. The neoplastic cells express low molecular weight cytokeratins, and cytokeratin 19. EMA and CEA are expressed in the luminal edge of glandular structures. It is an aggressive tumour, which can widely metastasize and can be cause of death.
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