Masbah Ouafae, Lalya Issam, Mellas Nawfel, Bekkouch Iman, Allaoui Mohamed, Hassouni Khalid, Kebdani Tayeb, Regragui Asmaa, Benjaafar Noureddine, Elgueddari Brahim Khalil
Department of Radiotherapy, National Institute of Oncology, Allal fassi Street, Rabat 10100, Morocco.
J Med Case Rep. 2011 Jun 29;5:249. doi: 10.1186/1752-1947-5-249.
Periductal stromal sarcoma is an extremely rare malignant fibroepithelial tumor of the breast which is characterized by its biphasic histology with benign ductal elements and a sarcomatous stroma made of spindle cells and lacking phyllodes architecture. Its therapeutic management is based on wide surgery with free margins. Adjuvant therapies are not needed. Periductal stromal sarcoma may evolve into a phyllodes tumor with time, as well as a specific soft-tissue sarcoma. To the best of our knowledge, this tumor has never been described in a child.
A 14-year-old Arabic boy was presented to our hospital one year ago with a nodule of the right breast that was gradually increasing in size without signs of inflammation. The histological examination after lumpectomy revealed a periductal stromal sarcoma with free surgical margins. No adjuvant treatment was given. At 50 months of close follow-up, no recurrence was observed.
Periductal stromal sarcoma in a child is a very rare disease which has the same indolent behavior as it does in adults. Therefore, close follow-up is required.
导管周围间质肉瘤是一种极其罕见的乳腺恶性纤维上皮性肿瘤,其组织学特征为双相性,包含良性导管成分以及由梭形细胞构成的肉瘤样间质,且缺乏叶状结构。其治疗方法以切缘阴性的广泛手术为主,无需辅助治疗。随着时间推移,导管周围间质肉瘤可能演变为叶状肿瘤,也可能演变为特定的软组织肉瘤。据我们所知,这种肿瘤从未在儿童中被描述过。
一名14岁的阿拉伯男孩于一年前因右乳出现一个结节且逐渐增大、无炎症迹象而前来我院就诊。肿块切除术后的组织学检查显示为切缘阴性的导管周围间质肉瘤,未给予辅助治疗。在50个月的密切随访中,未观察到复发情况。
儿童导管周围间质肉瘤是一种非常罕见的疾病,其生物学行为与成人相似,因此需要密切随访。