M Romeu1, J M Foletti2, C Chossegros3, J P Dales4, P Berbis5, B Cribier6, L Guyot2. 1. Service de stomatologie, chirurgie maxillo-faciale et plastique, hôpital Nord, centre hospitalier des Bourrely, 13015 Marseille, France. Electronic address: maxromeu@hotmail.fr. 2. Service de stomatologie, chirurgie maxillo-faciale et plastique, hôpital Nord, centre hospitalier des Bourrely, 13015 Marseille, France. 3. Service de chirurgie maxillo-faciale, hôpital de la Conception, 13005 Marseille, France. 4. Service d'anatomie pathologique, hôpital Nord, centre hospitalier des Bourrely, 13015 Marseille, France. 5. Service dermatologie-vénéréologie, hôpital Nord, centre hospitalier des Bourrely, 13015 Marseille, France. 6. Clinique dermatologique, faculté de médecine, université de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg cedex, France.
Abstract
INTRODUCTION: 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. MATERIALS AND METHODS: 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 Articles published before 2000 were considered outdated and were excluded. RESULTS: 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. DISCUSSION: 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.
INTRODUCTION: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. MATERIALS AND METHODS: 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. RESULTS: 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. DISCUSSION: 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.