De Leonardis Francesco, La Corte Renato, Bruschi Marco, Cavazzini Luigi, Trotta Francesco
Section of Rheumatology, Department of Clinical and Experimental Medicine, University of Ferrara, Azienda Ospedaliera-Universitaria S.Anna, Corso della Giovecca 203, 44100, Ferrara, Italy.
Rheumatol Int. 2009 Jul;29(9):1105-7. doi: 10.1007/s00296-009-0901-z. Epub 2009 Apr 10.
Giant cell tumor of tendon sheath (GCTTS) and Wegener's granulomatosis (WG) are rare conditions both characterized by polyclonal cellular proliferation and multinucleated giant cells formation. Here, we report the case of a 27-year-old Caucasian woman affected by WG who experienced the metachrone appearance of two different GCTTSs at the right hand within a time of 3 years. To our knowledge, the combination of GCTT with WG is exceptional and this could probably be the first case reported. The subsequent appearance of two rare diseases both characterized by giant cell formation apparently points to similarities in their pathogenesis. However, at present no pathogenic relationship between GCTTS and WG is demonstrable and their simultaneous occurrence has to be considered coincidental. Actually, an emerging opinion is to consider GCTTS as a mixed lesion in which both tumoral and non-tumoral inflammatory cells play a central pathogenic action. On this view, the proposed case could support the evidence about the crucial role of a chronic inflammatory injury in enhancing GCTTS appearance.
腱鞘巨细胞瘤（GCTTS）和韦格纳肉芽肿（WG）均为罕见病症，二者均以多克隆细胞增殖和多核巨细胞形成为特征。在此，我们报告一例27岁患韦格纳肉芽肿的白人女性病例，该患者在3年内右手先后出现了两例不同的腱鞘巨细胞瘤。据我们所知，腱鞘巨细胞瘤与韦格纳肉芽肿同时出现的情况极为罕见，这可能是首例报道的病例。两种均以巨细胞形成为特征的罕见疾病先后出现，这显然表明它们在发病机制上存在相似之处。然而，目前尚无证据表明腱鞘巨细胞瘤与韦格纳肉芽肿之间存在致病关系，它们的同时出现只能被视为巧合。实际上，一种新出现的观点认为腱鞘巨细胞瘤是一种混合性病变，其中肿瘤性和非肿瘤性炎性细胞均发挥着核心致病作用。基于这一观点，本病例报告可为慢性炎性损伤在促进腱鞘巨细胞瘤发生过程中起关键作用的证据提供支持。
