Abstract

Introduction: Hidradenocarcinoma is a very rare malignant tumor of the sweat glands. Diagnosis is based essentially on histological and immunohistochemical studies. Standard treatment is surgery combined with radiotherapy if there is a prognostic factor for local recurrence. We report a case treated in our department. Observation: A 24-year-old female patient with no specific pathological history consulted the Joliot Curie Institute of the Dantec Hospital for a forehead nodule that appeared in 2012 and was painless, evolving towards February 2021 into two right temporo-maxillary masses progressively increasing in volume. Examination revealed two tumors of stony consistency, with smooth, ulcerated surface, infiltrated center, raised borders, budding, not bleeding on contact, painless, the largest measuring approximately 16cm, located at the temporofrontal and right parotid level with extension to the right auricle associated with homolateral periorbital edema and right retroauricular and submental adenopathies, the largest measuring 3x2cm. Parotid skin biopsy revealed nodular tumour masses in the deep dermis, composed of a proliferation of medium- to large-sized epithelial cells, sometimes clear with central nuclei and numerous atypia and abnormal mitoses. Epidermoid differentiation can be seen in places. The stroma is fibrino-hyaline. The appearance was suggestive of a hidradenocarcinoma. The cervical CT scan revealed an inhomogeneous tissue process, strongly enhanced after well-limited contrast injection, in close contact with the right parotid gland. Similar homolateral parietofrontal lesions were noted, as well as a spiky homolateral frontoparietal appearance with cortical irregularity. The extension work-up was negative. Conclusion: Hidradenocarcinoma is a very rare malignant tumour with a high risk of locoregional and distant recurrence, mainly in lymph nodes. Its prognosis is poor.

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