Diagnosis and treatment of apocrine carcinoma of the head and neck: A systematic review.

Abstract

Apocrine carcinoma is a rare malignancy of apocrine sweat glands that can arise in the head and neck. These lesions frequently resemble benign adnexal processes, which may contribute to diagnostic delay. Owing to the rarity of this tumor, treatment recommendations are based largely on isolated case reports. A systematic review was performed according to the PRISMA guidelines. PubMed, Embase, and CINAHL databases were queried from inception through March 2025. Studies were included if they reported at least one case of head and neck apocrine carcinoma with demographic, treatment, and outcome data. Risk of bias was assessed using ROBINS-I. Thirty-five studies reporting 46 patients met the inclusion criteria. Mean age was 60.1 years; 73.9% were men. Tumors most commonly arose in the scalp (34.8%) and eyelid (30.4%). Diagnostic delay was reported in 34.8% of the cases. Surgical excision was performed in 93.5% of patients, often requiring complex reconstruction including local tissue methods, skin grafting, and free tissue transfer. Adjuvant radiotherapy was administered in 28.3% and chemotherapy in 17.4% of patients. Recurrence occurred in 41.3% of patients. At the last reported follow-up (mean 55.0 months), 76.1% of patients were alive. Head and neck apocrine carcinoma is an aggressive but poorly recognized neoplasm that carries substantial risk of recurrence. Early biopsy of atypical or persistent eyelid or scalp lesions is critical for diagnosis. Wide local excision remains the primary treatment, and adjuvant radiotherapy or systemic therapy may be considered for high-risk or recurrent disease. Larger multi-institutional datasets are needed to define optimal management.