Clear cell sarcoma (malignant melanoma) of soft parts: a clinicopathologic study of 52 cases.
Clear cell sarcoma (malignant melanoma) of soft parts: a clinicopathologic study of 52 cases.
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DOI：
10.1155/2012/984096
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发表时间：
2012
影响因子：
1.5
通讯作者：
Robert C
中科院分区：
文献类型：
--
作者：
Hocar O;Le Cesne A;Berissi S;Terrier P;Bonvalot S;Vanel D;Auperin A;Le Pechoux C;Bui B;Coindre JM;Robert C
透明细胞肉瘤是侵袭性的，罕见的软组织肿瘤，由于其在两种类型的肿瘤中均发现的临床，病理和分子特性，其在黑色素瘤或肉瘤中的分类仍未确定。这是一项回顾性研究，对1979年4月至2005年4月间在两个机构就诊的52例CCS患者进行了研究。在31例患者中研究了EWS-ATF-1融合转录物，在22例患者中研究了BRAF或NRAS基因的激活突变。研究对象为30名男性和22名女性，平均年龄为33岁。43例（82.69%）肿瘤位于四肢，尤以足部（19例）居多。初始肿瘤中位大小为4.8 cm （1 ~ 15 cm）。坏死累及50%以上肿瘤细胞14例（26.92%）。有丝分裂率高25例（48.07%）。31例患者中有28例（53.84%）发现EWS/ATF-1易位，22例患者中仅有2例（3.84%）发现BRAF或NRAS突变。在肿瘤相关参数中，只有肿瘤大小（bbb4cm）是一个重要的预后因素。49例（94.23%）患者在诊断时有局限性疾病，并立即手术切除（90%）或在新辅助化疗（CT）后（10%）。37例（71.15%）患者采用不同的CT治疗方案，均无明显疗效。5年和10年的OS率分别为59%和41%。肿瘤大小是我们研究中唯一出现的预后因素。完全手术切除仍然是这种侵袭性化疗耐药肿瘤的最佳治疗方法。
Clear cell sarcomas are aggressive, rare soft tissue tumors and their classification among melanoma or sarcoma is still undetermined due to their clinical, pathologic, and molecular properties found in both types of tumors. This is a retrospective study of 52 patients with CCS seen between April 1979 and April 2005 in two institutions. The EWS-ATF-1 fusion transcript was studied in 31 patients and an activating mutation of the BRAF or NRAS gene was researched in 22 patients. 30 men and 22 women, with a mean age of 33 were studied. Forty-three tumors (82.69%) were located in the extremities, specially the foot (19 tumors). Median initial tumor size was 4.8 cm (1 to 15 cm). Necrosis involving more than 50% of the tumor cells was found in 14 cases (26.92%). High mitotic rate (>10) was found in 25 cases (48.07%). The EWS/ATF-1 translocation was found in 28 (53.84%) of 31 patients studied, and mutation of BRAF or NRAS was found in only 2 of 22 patients analyzed cases (3.84%). Among the tumor-associated parameters, only tumor size (>4 cm) emerged as a significant prognostic factor. Forty-nine patients had a localized disease at diagnosis (94.23%) and underwent surgical resection immediately (90%) or after neoadjuvant chemotherapy (CT) (10%). Various CT regimens were used in 37 patients (71.15%) with no significant efficacy. The 5- and 10-year OS rates were 59% and 41%, respectively. Tumor size was the only emerging prognosis factor in our series. Complete surgical resection remains the optimal treatment for this aggressive chemoresistant tumor.