Un myofibroblastome de type mammaire est une tumeur molle rare; les myofibroblastomes extramammaires sont particulièrement rare. Un homme de 78 ans s'est présenté en consultation pour des douleurs pelviennes soulagées par la défécation ou les urines. Le toucher rectal retrouve une masse en avant de la paroi rectale antérieure. L'imagerie par résonance magnétique (IRM) montre une masse de 10 x 6 x 8cm, bien circonscrite et hétérogène, située en arrière de la vessie qu'elle refoule vers l'avant, en avant du recto-sigmoïde. L'immunohistochimie montre des cellules tumorales co-exprimant CD34 et la desmine de façon diffuse, expression de Rb dans la majorité des cellules, expression des récepteurs aux œstrogènes, expression intense et diffuse de la P16, un index de prolifération avec le ki67 estimé à 25%. Il n'y a pas eu de récidive après 8 mois de radiothérapie d'induction suivie de chirurgie. Un myofibroblastome de type mammaire est une tumeur rare et bénigne. La récidive n'est quasiment pas observée après traitement local. Ce cas permet de mettre en avance la possibilité d'utiliser la radiothérapie afin de faciliter la chirurgie.
Objectives:To describe the surgical and prognostic aspects of voluminous gastric stromal tumors at Joliot Curie Cancer Institute.Methods: This was a retrospective study of patients with voluminous stromal tumors from January 2010 to December 2015. Patients had a bulky gastric mass at the clinical examination and CT and had positive CD117 marker. Local stage and extension was evaluated and surgical treatment studied. Prognosis was also analyzed according to surgery and adjuvant treatment.Results: Five large Gastrointestinal Stromal Tumors (GISTs) were found. Most are women with a mean age of 39. The tumor was palpable in all patients. Other symptoms are pain and vomiting. Gastroscopy found a burgeoning tumor in 4 patients with external compression in 1 patient. Biopsy showed stromal tumor with fusiform cells in 3 patients and epithelioid cells in 2 patients. CD117 and CD34 were positive in all patients. Abdominal and thoracic CT showed a sus mesocolic mass voluminous with multi visceral involvement without distant metastasis. Surgery consisted in all cases of total gastrectomy extended to the spleen, the tail of the pancreas, the colon and the liver. One patient died of postoperative peritonitis at day five. There was none other immediate post-operative complications. All patients received adjuvant treatment with Imatinib. After a 23-month follow-up, 1 patient experience important sarcopenia while 1 patient recurred to the liver and brain with resistance to Imatinib and second line Sunitinib and died.
Conclusion:Voluminous gastric GIST in Africa is characterized by young age and involvement of sus mesocolic organs. Surgery with Imatinib as first line adjuvant treatment showed good results.
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