Ayadi-Kaddour A, Chaabouni N, Smati B, Mehouachi R, Djilani H, El Mezni F
Service d'Anatomie Pathologique, Hôpital Abderrahmen Mami, Ariana, Tunisia.
Rev Mal Respir. 2008 Jan;25(1):82-6. doi: 10.1016/s0761-8425(08)70472-8.
Fibromatosis are uncommon connective tissue tumours arising from musculo-aponeurotic tissue and characterised by spindle cell fibroblastic and myofibroblastic proliferation. The exact aetiology is unknown but several factors are considered to be positively correlated with their development and growth (genetic and hormonal factors and trauma). Although they are considered histologically benign they behave aggressively locally and relapse repeatedly after surgical excision. Mediastinal localisation is very rare. We describe a case of de novo fibromatosis of the posterior mediastinum in a 61 year old man with no history of thoracotomy or trauma. Although mediastinal fibromatosis is very uncommon physicians should be aware of this disease in order to ensure appropriate surgical treatment.
纤维瘤病是一种罕见的结缔组织肿瘤,起源于肌-腱膜组织,其特征是梭形细胞成纤维细胞和肌成纤维细胞增生。确切病因尚不清楚,但有几个因素被认为与其发生和生长呈正相关(遗传和激素因素以及创伤)。尽管它们在组织学上被认为是良性的,但在局部具有侵袭性,手术切除后会反复复发。纵隔定位非常罕见。我们描述了一例61岁男性后纵隔原发性纤维瘤病,该患者无开胸手术或创伤史。尽管纵隔纤维瘤病非常罕见,但医生应了解这种疾病,以确保进行适当的手术治疗。