Trichoépithéliome desmoplastique : étude anatomoclinique et comparaison avec le carcinome basocellulaire sclérodermiforme

Abstract

Desmoplastic trichoepithelioma (DTE) is an uncommon form of adnexal tumour that was described for the first time as a separate clinicopathological entity in 1976. We carried out a retrospective histopathological study of a large series of cases of DTE in order to better characterise this tumour and compare it with sclerodermiform basal cell carcinoma (BCC), which is in fact the most common as well as the most complex type of differential diagnosis. We included in this study all cases of DTE diagnosed between 1979 and 2001 at our dermatopathology laboratory. The clinical features were taken from the patient files. Diagnosis was confirmed by two different examiners and all microscopic elements were reviewed. The same clinical and demographic data were collected for cases of BCC diagnosed over the same period. We included 68 cases of DTE in 67 patients in our study, of whom 83.5% were women, and the mean age was 42.8 years. Lesions were found primarily on the head (98.5% of cases), mainly on the cheeks (29.2% of cases) and forehead (23.1% of cases). The diagnosis was only made by the clinician in four cases; in 38 cases the diagnosis made was BCC. In all cases, histological examination revealed thin lines of basaloid epithelial cells associated with small keratinising cysts. A common finding was granulomas with foreign bodies and calcifications. In six cases (8.8%), a tumour combining DTE with an intradermal naevus was observed. Mean clinical follow-up of 8 years (1 to 23 years) in 29 patients showed absence of relapse or metastasis. Over the same period, 662 cases of BCC were recorded in 499 patients, 58.9% of whom were women. The mean age was 65.6 years. The principal location was the nose (34% of cases). No cases of associated naevus were recorded. DTE is a firm plaque-like lesion, flesh coloured or yellowish, and generally with a depressed centre; it is seen primarily in middle-aged women and occurs principally on the cheeks and forehead. There is a significant association with naevus, a singular feature among adnexal tumours. On average, it is 10 times less common than BCC. The size of our study groups shows for the first time distinguishing features with regard to BCC, for which the age of onset is far higher and predominance among women less marked. Further, the sites are different, with BCC being seen predominantly on the nose while DTE is seen mainly on the cheeks. A suggested clinical diagnosis is thus possible.