Recurrent Pigmented Villonodular Synovitis with Distant Metastases: Case Report and Review of the Literature on the Efficacy of Targeted Therapies
TY Lee, ACL Chan, RKC Ngan
Hong Kong J Radiol 2016;19:43-8
DOI: 10.12809/hkjr1615343
Pigmented villonodular synovitis (PVNS), also known as tenosynovial giant cell tumour, diffuse type, is an uncommon pathological disease of the synovium and tendon sheaths. In most cases the disease is benign but rarely, it presents with aggressive and infiltrative features. We report a case of recurrent PVNS with bone and pleural metastases. Surgical resection remains the mainstay of treatment. For cases that are not amenable to surgery, novel targeted therapeutic agents have demonstrated favourable treatment response in some case reports and early-phase clinical trials. The macrophage colony-stimulating factor receptor inhibition pathway is hypothesised to be responsible for the potential therapeutic effect of targeted therapies. Due to the rarity of the disease, however, the definitive role of targeted therapeutic agents is still not well defined. Further molecular studies and clinical trials are needed to better understand the molecular basis and clinical efficacy of these targeted therapeutic agents.
中文摘要
復發性色素沉著絨毛結節性滑膜炎伴遠處轉移：病例報告和針對標靶治療功效的文獻回顧
李天恩、陳澤林、顏繼昌
色素沉著絨毛結節性滑膜炎亦稱為瀰漫型腱鞘巨細胞瘤，是滑膜和腱鞘的一種罕見病患。此病多為良性，但在罕見情況下可呈現為具入侵性和浸潤性。本文報告一宗復發性色素沉著絨毛結節性滑膜炎伴骨、胸膜轉移的病例。手術切除為色素沉著絨毛結節性滑膜炎的主要治療方法。至於不適合手術的患者，一些病例報告和早期臨床試驗證明新型標靶治療藥物有良好的治療效果。標靶治療有效的原因可能是透過抑制巨噬細胞集落刺激因子受體。然而，由於此病罕見，標靶治療藥物的實際臨床應用仍有待證實。需進一步的分子研究和臨床試驗，以明確地掌握標靶治療的分子基礎和臨床療效。