Résumé
Le léiomyosarcome de la surrénale (LS) est une tumeur extrêmement rare. Elle dérive des vestiges de la musculeuse des veines surrénaliennes. Elle touche le plus souvent les patients immunodéprimés (sida ou infectés par le virus d’Epstein-Barr). Même dans ces cas, l’incidence est faible. Nous rapportons un cas de LS, chez un patient de 20 ans, qui s’est présenté pour un syndrome occlusif haut par compression duodénale évoluant depuis une semaine. L’imagerie avait évoqué le diagnostic d’une tumeur surrénalienne maligne. Une résection était réalisée avec succès. Les contrôles clinique et radiologique en postopératoire étaient satisfaisants. Le recul est de 55 mois. Le LS est une tumeur agressive. Seule la chirurgie radicale élargie parfois aux organes de voisinage, pour avoir des marges saines, permet de prolonger la survie. La chimiothérapie et la radiothérapie sont indiquées en cas de tumeur non résécable et métastatique.
Abstract
Leiomyosarcoma of the adrenal gland is an extremely rare tumour originating from the central adrenal vein or its tributaries. Patients with human immunodeficiency virus (HIV) and Epstein-Barr infection have a higher incidence of these tumours, but even in those individuals, the overall incidence is very low. In this paper, we report a case of adrenal leiomyosarcoma in a 20-year-old man who presented with a high occlusive syndrome through duodenal compression, developing over the course of one week. The imagery showed a malignant adrenal tumour. A resection was successfully performed. The patient has been in recession for 55 months. Despite the rarity of this tumour, leiomyosarcoma of the adrenal gland has an aggressive behaviour. Adrenalectomy is the primary treatment, while chemotherapy or radiotherapy is not prescribed except in metastatic or bulky and non-operative disease.
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Soufi, M., Mohsine, R., Chenna, M. et al. Leiomyosarcome primitif de la surrénale. J Afr Cancer 1, 168–171 (2009). https://doi.org/10.1007/s12558-009-0032-1
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DOI: https://doi.org/10.1007/s12558-009-0032-1