Maffucci综合征及伴发病变的影像学表现(附1例报告并文献复习)

Imaging Findings of Maffucci Syndrome with Associated Lesions(1 Case Report and Literatures Review)

目的提高对Maffucci综合征及其伴发病变的临床和影像学表现的认识。资料与方法回顾性分析1例Maffucci综合征及其伴发病变的临床和影像学资料并作文献复习。结果 Maffucci综合征可同时伴发卵巢粒层细胞瘤和甲状腺结节性疾病。本病的内生性软骨瘤分布广泛,各部位病变影像表现可不一致;其伴发的卵巢粒层细胞瘤影像表现与同一部位和组织学类型的肿瘤基本相同,且合并瘤内出血。结论 Maffucci综合征诊断确立后,有必要对患者的其他重要器官进行影像学检查及随访,以减少漏诊或早期发现伴发病变。

Objective To improve the understanding of clinical and imaging findings of Maffucci syndrome with associated lesions.Materials and Methods The clinical and imaging findings of one case of Maffucci syndrome with associated lesions were retrospectively analyzed and literature review was done.Results Maffucci syndrome could accompany with ovarian granulosa cell tumor and thyroid nodular lesion.The widely distributed endogenous chondroma at different regions had diverse imaging manifestations;the concomitant ovarian granulosa cell tumor and the tumor with the similar histological type at the same position had basically similar imaging findings,which was accompanied with intratumoral hemorrhage.Conclusion After establishing the diagnosis of Maffucci syndrome,the imaging examination of other important organs and follow-up are necessary to reduce the missed diagnosis and discover the associated lesions at early stage.