[Malignant cutaneous adnexal neoplasms of the face and scalp: Diagnostic and therapeutic update]

Malignant cutaneous adnexal neoplasms are rare and have been characterized only recently. They can occur at any age but preferentially in elderly. There are 3 of them: trichoblastic carcinoma, trichilemmal carcinoma and malignant pilomatricoma. The aim of our study was to make a diagnostic and therapeutic update about these tumors when located at the face or at the scalp.A bibliographic research was made on PubMed using following keywords: appendage skin carcinoma AND pathology AND/OR therapeutic. Articles published before 2000 were considered outdated and were excluded.Twenty-five articles met the inclusion criteria. Clinical presentation was non-specific. Histological examination only allowed for diagnosis. Lesions were locally or loco-regionally aggressive. Lymphatic or hematogenous metastasis were reported. No consensus about treatment was found. When surgery was used, it consisted in resection with safety margins ranging from 0.5 to 3cm depending on the teams. In case of metastasis, treatment consisted in chemo- and/or radiotherapy. A quarterly medical monitoring was recommended.Malignant cutaneous adnexal tumors are rare. There is nowadays no treatment consensus. An initial staging by mean of a head and neck, chest, abdominal and pelvic CT-scan is mandatory. Treatment has to be decided in a multidisciplinary cancer committee. In the absence of metastasis, the reference treatment is surgical resection, possibly by Mohs micrographic technique, with large safety margins. In case of metastasis or if the loco-regional extension does not allow for a complete excision, chemotherapy and/or radiotherapy may be proposed. A close monitoring is essential.