皮膚原發性平滑肌肉瘤是一罕見惡性腫瘤。在任何年紀都可能發生,常見於50到70 歲之間。由於疾病本身罕見,已往並無確定臨床治療原則。本文回溯分析12位皮膚原發性平滑肌肉肉瘤在台北榮民總醫院為期30年之臨床資料,並同時回顧文獻資料及討論該腫瘤在臨床上之表徵,病理組織診斷及治療方式。12位病患有7位女性5位男性,平均年齡54.4歲。腫瘤均為單一病灶,平均大小爲2.5公分。腫瘤發生部位分別是上,下肢體(7病例), 軀幹(3病例)及頭頸部(2病例)。臨床上主要治療方式是外科切除。共有4位病患在接受外科切除後,病灶局部再發。再發病灶分別接受後續再切除手術。所有病患均無遠處轉移。依據病患臨床資料及腫瘤病理表徵,我們認為皮膚原發性平滑肌肉瘤雖在組織學上是惡性分化腫瘤,但病程表現近似良性。在臨床治療方面,我們建議外科切除至於其他輔助性治療,如放射治療或化學治療等並無一定論,而且大多數病患並無療效與文獻報告相同。
Primary cutaneous leiomyosarcomas are rare malignant tumors. They can occur at any age, but most commonly between the fifth and seventh decade of life. Because of the small number of patients treated, treatment recommendations are still evolving. We report the results of a retrospective study of 12 patients treated for leiomyosarcomas through a thirty year period (from 1973 to 2002). Five males and seven females (mean age, 54.4 years; age range, 22-81 years), more than 58% of the patients were at least 50 years old. The tumors presented mainly as solitary lesions and were located on the upper and lower extremities (seven lesions), trunk (three lesions), and the head and neck (two lesions). The main treatment of choice was surgical wide excision. Clinical follow-up revealed local recurrences in four patients after a period ranging from 3 to 72 months after surgical excision. No distant metastases have been observed in our series. We reviewed published articles. The clinical findings, pathologic examinations and treatments were analyzed. We believe primary cutaneous leiomyosarcoma should be considered biologically benign with regard to distant metastases, despite the malignant histologic appearance. Wide local excision is recommended, and it would seem that narrow margins with a tumor-free plane should be sufficient.