Clinicopathological features with outcomes of a series of conventional and proximal-type epithelioid sarcomas, diagnosed over a period of 10 years at a tertiary cancer hospital in India
Clinicopathological features with outcomes of a series of conventional and proximal-type epithelioid sarcomas, diagnosed over a period of 10 years at a tertiary cancer hospital in India
复制标题
DOI：
10.1007/s00428-008-0639-0
复制
发表时间：
2008-08-01
期刊：
影响因子：
3.5
通讯作者：
Chinoy, R. F.
中科院分区：
文献类型：
--
作者：
Rekhi, Bharat;Gorad, Biru D.;Chinoy, R. F.
上皮样肉瘤（ES）是一种罕见的肉瘤。最近，其变体，包括近端型 ES，已被识别。本研究重点介绍了 26 例 (65%) 传统 ES 病例和 14 例 (35%) 近端型 ES 病例的临床病理特征。 38% 的病例发生在 21-30 岁年龄组，其中 77.5% 的病例为男性。四肢是这两种亚型中最常见的部位。组织学上，传统型ES显示结节状肿瘤聚集并坏死，而近端型则显示大的“横纹肌样”细胞的固体排列。更多近端型病例（64.2%）为 3 级。考虑了一系列鉴别诊断。最重要的免疫组织化学标记物是波形蛋白、上皮膜抗原、细胞角蛋白、CD34 和结蛋白。最多（72.5%）病例接受手术治疗。近端型复发和转移较多。传统亚型的 7 年无病生存率为 19.4%，近端亚型则为零 (p = 0.06)。近端总生存率 (31.3%) 也低于传统类型 (90.2%；p
Epithelioid sarcoma (ES) is an uncommon sarcoma. Lately, its variants, including proximal-type ES, have been recognized. The present study highlights clinicopathological features of 26 (65%) conventional and 14 (35%) cases of proximal-type ES. Thirty-eight percent of cases were seen in 21-30-year age group, including 77.5% cases in men. Extremities were the commonest sites in both the subtypes. Histologically, conventional-type ES displayed nodular tumor aggregates with necrosis, while proximal-type showed solid arrangement of large, "rhabdoid-like" cells. More cases (64.2%) of the proximal type were of grade 3. A range of differential diagnoses was considered. Most important immunohistochemical markers were vimentin, epithelial membrane antigen, cytokeratin, CD34, and desmin. Maximum (72.5%) cases were treated Surgically. Recurrences and metastasis were observed more in the proximal type. The 7-year disease-free survival was 19.4% in the conventional and nil in the proximal subtype (p = 0.06). The overall survival rate was also lower in the proximal (31.3%) than conventional type (90.2%; p