高级别子宫内膜间质肉瘤-伴BCOR异位或者片段重复
High grade endometrial stromal sarcoma with BCOR translocation or internal tandem duplication
曹登峰
更新时间：2019-01-19 21:20:00
概述：
发病部位：子宫内膜+/-肌层
诊断要点：
1. 累及子宫内膜和肌层
2. 梭形细胞为主，可伴有少量卵圆形和上皮样细胞，轻度至中度异型，核分裂像范围大 (1-50/10 高倍视野，中位14.5/10 hpfs, 80%的病例超过10/10 hpfs)，核染色质细腻
3. 细胞质量不等，从很少到丰富
4. 间质：大多数可见粘液变，偶尔可见胶原斑块
6. 肿瘤凝固性坏死：可见，但是只有少数肿瘤有
7. 脉管瘤栓：多数肿瘤有
免疫组织化学染色：
分子标记：
鉴别诊断：
1. 低级别子宫内膜间质肉瘤
2. YWHAE-NUTM2 融合高级别子宫内膜间质肉瘤
3. 粘液性平滑肌肉瘤
4. 炎性肌纤维母细胞瘤
5. 粘液性未分化肉瘤
预后：
治疗：
参考文献：
1. Panagopoulos I, Thorsen J, Gorunova L, Haugom L, Bjerkehagen B, Davidson B, Heim S, Micci F. Fusion of the ZC3H7B and BCOR genes in endometrial stromal sarcomas carrying an X;22-translocation. Genes Chromosomes Cancer. 2013 Jul; 52(7):610-8.
2. Kao YC, Sung YS, Zhang L, Jungbluth AA, Huang SC, Argani P, Agaram NP, Zin A, Alaggio R, Antonescu CR. BCOR Overexpression Is a Highly Sensitive Marker in Round Cell Sarcomas With BCOR Genetic Abnormalities. Am J Surg Pathol. 2016 Dec; 40(12):1670-1678.
3. Hoang LN, Aneja A, Conlon N, Delair DF, Middha S, Benayed R, Hensley ML, Park KJ, Hollmann TJ, Hameed MR, Antonescu CR, Soslow RA, Chiang S. Novel High-grade Endometrial Stromal Sarcoma: A Morphologic Mimicker of Myxoid Leiomyosarcoma. Am J Surg Pathol. 2017 Jan;41(1):12-24.
4. Chiang S, Lee CH, Stewart CJR, Oliva E, Hoang LN, Ali RH, Hensley ML, Arias-Stella JA 3rd, Frosina D, Jungbluth AA, et al. BCOR is a robust diagnostic immunohistochemical marker of genetically diverse high-grade endometrial stromal sarcoma, including tumors exhibiting variant morphology. Mod Pathol. 2017 Sep; 30(9):1251-1261.
5. Lewis N, Soslow RA, Delair DF, Park KJ, Murali R, Hollmann TJ, Davidson B, Micci F, Panagopoulos I, Hoang LN, et al. ZC3H7B-BCOR high-grade endometrial stromal sarcomas: a report of 17 cases of a newly defined entity. Mod Pathol. 2018 Apr; 31(4):674-684.
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