Auroy S, Contesso G, Spatz A, Genin J, Margulis A, Lecesne A, Avril M F
Service de Dermatologie, Institut Gustave-Roussy, Villejuif.
Ann Dermatol Venereol. 1999 Mar;126(3):235-42.
Superficial leiomyosarcomas are rare tumors, which may be confined to the dermis or extend to subcutaneous tissues.
We report the results of a retrospective study of 32 patients treated for leiomyosarcomas through a twenty-two year period (from 1975 to 1997).
Mean age was 45 years, with 50 p. 100 of patients less than 35 years of age. Forty seven percent of the tumors were located on the lower limbs and mean diameter was 2.8 cm. Three clinical types have been isolated: nodule beneath normal epidermis (50 p. 100), purple nodule ulcerated or not (28 p. 100), swelling tumor (22 p. 100). Sixteen percent were intradermal, whereas sixty nine percent involved subcutaneous tissues. With regard to tumor grade, 37 p. 100 of tumors were grade I, 44 p. 100 of tumors were grade II, and 19 p. 100 were grade III. Immunohistochemical staining showed positive reactions for all tumors with anti-vimentin and anti-alpha smooth muscle actin. Main treatment was complete surgical excision. Follow-up informations were available for all patients and 75 p. 100 of them had a follow up period longer than a year. Five patients with leiomyosarcomas involving the subcutis developed local recurrences, and two of them died of the disease.
Leiomyosarcomas can occur at any age without predominant sex-ratio. Main prognostic factors are tumor size, distal location, depth of tumor invasion and pathological grade. Immunohistological staining with anti-alpha smooth muscle actin is more sensitive and specific than with anti-desmin or anti-HHF 35. Main treatment is surgical excision with wide margins.
浅表性平滑肌肉瘤是罕见肿瘤,可局限于真皮或延伸至皮下组织。
我们报告了一项对32例平滑肌肉瘤患者进行的回顾性研究结果,该研究历时22年(从1975年至1997年)。
平均年龄为45岁,50%的患者年龄小于35岁。47%的肿瘤位于下肢,平均直径为2.8厘米。已分离出三种临床类型:正常表皮下结节(50%)、紫色结节(有无溃疡,28%)、肿胀性肿瘤(22%)。16%为真皮内肿瘤,而69%累及皮下组织。关于肿瘤分级,37%的肿瘤为I级,44%为II级,19%为III级。免疫组织化学染色显示所有肿瘤对抗波形蛋白和抗α平滑肌肌动蛋白均呈阳性反应。主要治疗方法是完整手术切除。所有患者均有随访信息,其中75%的患者随访期超过一年。5例累及皮下组织的平滑肌肉瘤患者出现局部复发,其中2例死于该病。
平滑肌肉瘤可发生于任何年龄,无明显性别差异。主要预后因素为肿瘤大小、远端位置、肿瘤浸润深度和病理分级。抗α平滑肌肌动蛋白免疫组织化学染色比抗结蛋白或抗HHF 35染色更敏感和特异。主要治疗方法是广泛切缘的手术切除。