Résumé
Le dermatofibrosarcome protuberans (DFSP) est un sarcome de bas grade se développant lentement aux dépens de fibroblastes du derme. La principale caractéristique de cette tumeur est son grand potentiel de récidive locale. Nous rapportons le cas d’une patiente âgée de 44 ans présentant un DFSP du sein qui a récidivé à deux reprises. Le bilan d’extension était négatif. Elle a bénéficié d’une exérèse tumorale large. La localisation mammaire d’un DFSP est rare. Le diagnostic est difficile. L’objectif thérapeutique dans la prise en charge de DFSP est d’atteindre une résection chirurgicale la plus complète possible; l’exérèse chirurgicale conventionnelle doit laisser des marges saines de 3 à 5 cm avec l’inconvénient de la perte de substance. La technique chirurgicale de Moh, qui est une technique micrographique contrôlant les marges de résection en peropératoire, peut pallier cet inconvénient. Le risque de récurrence est faible lorsque les marges de résection sont saines. La radiothérapie comme traitement adjuvant est très utile quand les marges ne sont pas suffisantes. La surveillance doit être prolongée.
Abstract
The dermatofibrosarcoma protuberans (DFSP) is a sarcoma of low rank developing slowly from the fibroblasts of the dermis. The main characteristic of this tumour is its big potential for local recurrence. We report the case of a patient aged 44 presenting a DFSP of the breast that relapsed on two occasions. The remaining extension was negative. She benefited from a large timorous excision. The mammary localization of a DFSP is rare. The diagnosis is difficult. The therapeutic objective of DFSP is to reach a possible most complete surgical resection; the conventional surgical excision must let healthy margins (of 3 to 5 cm) with the inconvenience of the substance loss. The technique of Moh, verifying the limits of excision in preoperative, can palliate this inconvenience. The risk of recurrence is low when the resection margins are healthy. Radiotherapy as adjuvant treatment is very useful when the margins are not sufficient. The surveillance must be prolonged.
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Asmouki, H., Soummani, A. & Benelkhaiat, R. Dermatofibrosarcome protuberans récidivant du sein: à propos d’un cas. J Afr Cancer 2, 190–193 (2010). https://doi.org/10.1007/s12558-010-0102-4
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DOI: https://doi.org/10.1007/s12558-010-0102-4