色素沉著絨毛結節性滑膜炎(pigmented villonodular synovitis, PVNS)是一種臨床上少見，關節腔、 滑囊、或腱鞘滑膜高度增生的疾病。經常侵犯膝關節、髖關節，但也可發生於踝關節等部位。色素沉著 絨毛結節性滑膜炎(PVNS)有兩種表現形式：局部型和彌漫型。雖然彌漫型色素沉著絨毛結節性滑膜炎鮮 少致命或轉移，但卻難以完全切除且復發率高。目前的實證醫學證據支持絨毛結節性滑膜炎的腫瘤起源， 其中機轉包含CSF-1基因的過度表現，近年已開始研究新式標靶藥物對絨毛結節性滑膜炎的療效，其中 部分藥物具有潛力，令人期待。但仍需更多研究探討新式標靶藥物的確切療效。本文概述色素沉著絨毛 結節性滑膜炎的現況和未來的治療趨勢。

Pigmented villonodular synovitis (PVNS) is a rare disorder occurring in synovium, bursa or tendon sheath. It is highly proliferative and usually involves the knee and hip joints, it can also occur in ankle and other sites. It has two types, i.e., localized or diffuse. Although diffuse PVNS is rarely lethal and rarely metastases, it is difficult to completely resect and has a high recurrence rate. Evidences now suggest a neoplastic origin for PVNS, including CSF-1 gene overexpression. With this discovery, targeted medical therapies have been developed. As novel methods of treatment for pigmented villonodular synovitis, some of them have promising results. More researches are required to understand the definite efficacy of such new targeted agents. In this article, we reviewed current and future treatments of pigmented villonodular synovitis.