Pisani-Moreau M N, Merigou D, Gautier C, Bioulac P, Geniaux M, Delaunay M M
Unité de dermatologie-cancérologie, Centre Hospitalier Pellegrin, Bordeaux.
Ann Dermatol Venereol. 1997;124(2):176-81.
Soft tissue melanoma was described in 1965 by Enzinger who used the term clear-cell sarcoma. In 1983, Chung and Enzinger coined the term soft tissue melanoma due to the immunohistochemical similarity with melanoma. We report a case of this rare type of melanoma.
A 59-year-old woman had pain between the first two toes for 3 years. A subcutaneous tumor was found at examination. Histologically, the tumor was composed of weakly eosinophilic cell proliferation. Protein S100 and HMB45 were positive. The cells were organized in theques. Pathology diagnosis was soft tissue melanoma. Complete remission was obtained for 3 years when several local recurrences required surgery and chemotherapy then surgery and radiotherapy. Complete remission has been achieved for 9 months.
This case presented the main characteristics of soft tissue melanoma as described in a review of 209 analyzable cases reported in the literature. This tumor occurs in young subjects with no sex or race predominance. It is an ubiquitous tumor which develops in close relation with tendons and aponevroses, usually in limbs (especially feet). Pain is sometimes the revealing manifestation, but the tumor is often asymptomatic, so the volume is often important at diagnosis. Pathology examination shows rather monomorphic proliferation of cells with a clear or weakly eosinophilic cytoplasm grouped in clusters or theques separated by fibrous septa. Intracytoplasmic melanin is sometimes observed, indicating interest of protein S100 and HMB45 immunohistochemistry which is almost always positive. The principle differential diagnoses are metastasic melanoma and epithelioid sarcoma. Prognosis of soft tissue melanoma is similar to that in sarcomas with a high rate of local recurrence and metastases (lymph nodes, lungs). Mortality reaches 56 p. 100. Treatment is wide surgical exeresis.
Soft tissue melanoma is a rare tumor of the melanocyte. It differs from melanoma by the population involved, its clinical expression and its prognosis which is similar to that in sarcoma.
1965年,恩津格首次描述了软组织黑色素瘤,当时他将其称为透明细胞肉瘤。1983年,钟和恩津格因该肿瘤在免疫组化方面与黑色素瘤相似,而创造了软组织黑色素瘤这一术语。我们报告一例这种罕见类型的黑色素瘤病例。
一名59岁女性,前两趾间疼痛3年。检查时发现一个皮下肿瘤。组织学检查显示,肿瘤由弱嗜酸性细胞增殖构成。蛋白S100和HMB45呈阳性。细胞呈巢状排列。病理诊断为软组织黑色素瘤。3年内实现了完全缓解,期间出现几次局部复发,需要先进行手术及化疗,然后再进行手术及放疗。目前已实现完全缓解9个月。
本病例呈现了软组织黑色素瘤的主要特征,这些特征与文献报道的209例可分析病例综述中所描述的一致。该肿瘤发生于年轻患者,无性别或种族倾向。它是一种普遍存在的肿瘤,通常与肌腱和腱膜密切相关,多发生于四肢(尤其是足部)。疼痛有时是首发表现,但该肿瘤通常无症状,因此诊断时肿瘤体积往往较大。病理检查显示细胞呈相当单一的增殖,细胞质清晰或弱嗜酸性,聚集成团或巢状,被纤维间隔分隔。有时可观察到胞质内黑色素,这表明蛋白S100和HMB45免疫组化具有重要意义,其几乎总是呈阳性。主要鉴别诊断为转移性黑色素瘤和上皮样肉瘤。软组织黑色素瘤的预后与肉瘤相似,局部复发和转移(淋巴结、肺)率较高。死亡率达56%。治疗方法为广泛手术切除。
软组织黑色素瘤是一种罕见的黑素细胞肿瘤。它在受累人群、临床表现及预后方面与黑色素瘤不同,其预后与肉瘤相似。