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題名 | Dermatofibrosarcoma Protuberans with Fibrosarcomatous Transformation in an Immunosuppressed Renal-transplant Recipient--Case Report and Literature Review=隆突性皮膚纖維肉瘤之纖維肉瘤轉化於免疫抑制腎移植患者--病例報告及文獻回顧 |
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作者 | 張欽富; 顏榮信; 蔡岳錤; 陳彥瑋; 賴志昇; 呂俊德; 陳伊呈; | 書刊名 | 臺灣整形外科醫學會雜誌 |
卷期 | 26:3 2017.09[民106.09] |
頁次 | 頁321-331 |
分類號 | 416.413 |
關鍵詞 | 纖維肉瘤轉化; 免疫抑制; 隆突性皮膚纖維肉瘤; Dermatofibrosarcoma protruberans; Immunosuppression; Fibrosarcomatous transformation; |
語文 | 英文(English) |
中文摘要 | 背景:隆突性皮膚纖維肉瘤為一罕見的纖維母細胞間質性皮膚腫瘤。雖然其具局部侵犯性,並且有高復發率,但是此病灶卻較少遠端轉移。即使隆突性皮膚纖維肉瘤在免疫抑制個體中可能有較高的發生率,但在發生於腎移植後之病例報告仍為罕見。目的及目標:提出一例於接受免疫抑制的腎移植患者發生的隆突性皮膚纖維肉瘤之纖維肉瘤轉化的病例,並且討論修改慢性免疫抑制處方的可能益處。材料及方法:一名三十九歲女性腎移植接受者,患有在腎移植後第七年在左腹股溝上逐漸增大的隆突性皮膚纖維肉瘤。在此提出有關此病例之臨床發現、影像研究、病史、手術方法、病理發現和預後,及關於隆突性皮膚纖維肉瘤發生於腎移植患者之文獻回顧,且介紹最新治療方式及預後。結果:包含本例,目前只有六例關於免疫抑制腎移植患者發生隆突性皮膚纖維肉瘤之病例報告。其中兩例在手術切除腫瘤後有修改免疫抑制劑處方。其中只有本例為大範圍纖維肉瘤轉化之隆突性皮膚纖維肉瘤。在此六例中,皆未使用術前或術後之標靶治療。結論:即使纖維肉瘤轉化之隆突性皮膚纖維肉瘤在接受免疫抑制的移植後患者是罕見的,但臨床醫師須密切注意其較低的無復發生存率及更高的轉移潛力。為了改善結果,可以修改術後免疫抑製劑的處方以及在廣泛切除之前的輔助靶標治療。此外,本例提供隆突性皮膚纖維肉瘤於免疫抑制的個案可能有更高發生率的證據。 |
英文摘要 | Background: Dermatofibrosarcoma Protruberans (DFSP) is a rare fibroblastic mesenchymal skin tumor. While it is characteristically locally invasive, and has a high regional recurrence rate, it also possesses a low potential to metastasize. Even though DFSP may occur at a higher rate in immunosuppressed individuals compared with the immunocompetent population, it has rarely been previously reported in renal transplant recipients. Aim and Objectives: We present the case of DFSP with fibrosarcomatous transformation (DFSP-FS) in an immunosuppressed renal-transplant recipient, and discuss the possible benefits from the modification of a chronic immunosuppressive regimen. Material and Methods: A 39-year-old female renal transplant recipient suffered from a DFSP, which progressively developed on the left groin during the 7th year following the renal transplant. Clinical findings, image studies, medical history, surgical procedures, pathological features and outcomes have all been reported. A literature review with regards to the previous cases of DFSP in renal transplant recipient, the latest treatment modalities, and the outcomes of both classic DFSP and DFSP-FS is also presented. Results: Only 6 cases of DFSP developing in immunosuppressed, renal-transplant recipients have been reported, including those in this case. Modification of immunosuppressants following a wide excision was performed in 2 of the cases. The fibrosarcomatous transformation of DFSP with large tumor size was reported in two cases. Neither adjuvant, nor neo-adjuvant target therapy, had been applied in these six cases. Conclusion: Although DFSP-FS in immunosuppressed post-transplant patients is rare, it is important that all clinicians pay close attention to the lower recurrence-free survival rate, and any higher metastatic potential. In order to improve outcomes, postoperative modification of immunosuppressants, along with neo-adjuvant target therapy prior to wide excision, may be applied. Furthermore, this case provides additional evidence that the incidence of DFSP may be higher in immunosuppressed individuals. |
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