Recurrent Dermatofibrosarcoma Protuberans of the Scalp Followed by Brain Metastasis-A Case Report with Literature Review

復發於頭皮之隆凸性皮膚纖維肉瘤併腦部轉移－病例報告及文獻回顧

楊宜倩(Yi-Chien Yang) ； 鄭裕文(Yu-Wen Cheng)

《中華皮膚科醫學雜誌》 25卷2期 (2007/06) Pp. 159-164

https://doi.org/10.29784/DS.200706.0014

摘要

隆凸性皮膚纖維肉瘤是一種中度惡性之纖維組織腫瘤，以局部侵犯性之生長和高復發率為特色。轉移則為非常罕見，最常見的轉移部位是肺部和淋巴結。我們報告一例53歲女性罹患位於頭皮隆凸性皮膚纖維肉瘤，並在切除後第四年復發，第六年發現轉移至大腦右額葉。病理切片檢查發現在復發及轉移腫瘤細胞出現退行性分化，從低度惡性之隆凸性皮膚纖維肉瘤轉變為較惡性的纖維肉瘤。在復發位的腫瘤細胞表現CD34陽性反應，但腦部的轉移病灶中，在局部顯現較惡性纖維肉瘤分化的細胞不表現CD34。隆凸性皮膚纖維肉瘤不僅有局部侵犯性並有明確的轉移潛力。尤其對於具有不良預後因子的患者，徹底的切除病灶並密切觀察是十分重要的。

並列摘要

Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor of intermediate malignancy. It is characterized by a locally aggressive growth and a high rate of local recurrence. Metastases are very rare and the most common sites of metastasis are the lungs and lymph nodes. We report a 53-year-old female patient with a DFSP on the scalp. She developed local recurrence of DFSP 4 years after simple excision and right frontal lobe metastasis in the sixth year. The pathological examinations revealed dedifferentiation from low-grade DFSP to higher grade fibrosarcomatous features in the recurrent and metastatic lesions. Immunohistochemical studies showed diffuse CD34 expression in recurrent tumor and focal loss of CD34 expression associated with fibrosarcomatous change in the brain metastatic tumor. DFSP is not only locally invasive but also has a definite metastatic potential. The importance of complete local excision with close surveillance, especially for the patients with poor prognostic factors, should be emphasized.

並列關鍵字

Dermatofibrosarcoma protuberans ； Brain metastasis ； CD34 ； Fibrosarcoma