原发性小肠子宫内膜间质肉瘤一例并文献复习

doi:10.12280/gjfckx.20220039

摘要/Abstract

摘要：

子宫内膜间质肉瘤（endometrial stromal sarcoma,ESS）是少见的子宫间叶组织来源的恶性肿瘤,主要由子宫内膜间质细胞组成。原发性子宫外ESS是指原发于子宫外、且不合并子宫内病灶的一类特殊ESS,卵巢是最常见的首发部位。原发于肠道的ESS罕见报道,临床病理特征不详。报道1例46岁原发于小肠的ESS患者的手术、化疗、孕激素治疗经过,随访77个月,无病生存。该患者的主要症状为经期上腹胀痛伴腰背部放射痛,既往有子宫内膜异位症病史。ESS的确诊需依靠免疫组织化学检查,根治性手术是一线治疗方案。

关键词: 原发性子宫外子宫内膜间质肉瘤, 肉瘤,子宫内膜间质, 小肠, 病例报告

Abstract:

Endometrial stromal sarcoma (ESS) is a rare mesenchymal neoplasm of the uterus, which is predominantly composed of endometrial stromal cells. Primary extrauterine ESS is a special type originating from extrauterine organ without intrauterine lesions. Ovary is the most common primary site. Primary intestinal ESS is rarely described. Its clinicopathologic features have not been well characterized. We report a case of ESS arising in the small intestine in a 46-year-old woman. She was treated with cytoreductive resection surgery, followed by chemotherapy, megestrol acetate, and has been in remission for more than 77 months with disease-free. The patient′s main symptoms were epigastric pain during menstruation with radiating pain in the lower back, and she had a previous history of endometriosis. The diagnosis of ESS relies on immunohistochemical detection, and radical surgery is the first-line treatment.

Key words: Primary extrauterine endometrial stromal sarcoma, Sarcoma, endometrial stromal, Intestine, small, Case reports

吴珍珍, 王玥元, 王芳, 郑婧, 王惠玲, 刘青. 原发性小肠子宫内膜间质肉瘤一例并文献复习[J]. 国际妇产科学杂志, 2022, 49(5): 560-564.

WU Zhen-zhen, WANG Yue-yuan, WANG Fang, ZHENG Jing, WANG Hui-ling, LIU Qing. Primary Extrauterine Endometrial Stromal Sarcoma of the Small Intestine: A Case Report and Literature Review[J]. Journal of International Obstetrics and Gynecology, 2022, 49(5): 560-564.

图/表 3

图1 小肠肿瘤病理图注：A 肿瘤细胞不规则膨胀性浸润肠壁全层（HE×40）,B 肿瘤细胞不规则、舌状浸润邻近肠壁平滑肌,可见脉管侵犯（HE×100）,C 肿瘤细胞圆形、短梭形,细胞质少,核中度异型,核分裂可见（HE×400）。

图2 肿瘤细胞免疫组织化学图（DAB法×100）注：A 肿瘤细胞ER呈强阳性表达;B 肿瘤细胞PR呈强阳性表达;C 肿瘤细胞CD10呈强阳性表达。

图3 血清肿瘤标志物变化趋势

参考文献 29

[1]	Asada Y, Isomoto H, Akama F, et al. Metastatic low-grade endometrial stromal sarcoma of the sigmoid colon three years after hysterectomy[J]. World J Gastroenterol, 2005, 11(15):2367-2369. doi: 10.3748/wjg.v11.i15.2367. doi: 10.3748/wjg.v11.i15.2367
[2]	Tropé CG, Abeler VM, Kristensen GB. Diagnosis and treatment of sarcoma of the uterus. A review[J]. Acta Oncol, 2012, 51(6):694-705. doi: 10.3109/0284186X.2012.689111. doi: 10.3109/0284186X.2012.689111 pmid: 22793037
[3]	Böcker W. WHO classification of breast tumors and tumors of the female genital organs: pathology and genetics[J]. Verh Dtsch Ges Pathol, 2002, 86:116-119. pmid: 12647359
[4]	Chang KL, Crabtree GS, Lim-Tan SK, et al. Primary extrauterine endometrial stromal neoplasms: a clinicopathologic study of 20 cases and a review of the literature[J]. Int J Gynecol Pathol, 1993, 12(4):282-296. pmid: 8253545
[5]	Kim L, Choi SJ, Park IS, et al. Endometrial stromal sarcoma of the small bowel[J]. Ann Diagn Pathol, 2008, 12(2):128-133. doi: 10.1016/j.anndiagpath.2006.03.020. doi: 10.1016/j.anndiagpath.2006.03.020 pmid: 18325474
[6]	Masand RP, Euscher ED, Deavers MT, et al. Endometrioid stromal sarcoma: a clinicopathologic study of 63 cases[J]. Am J Surg Pathol, 2013, 37(11):1635-1647. doi: 10.1097/PAS.0000000000000083. doi: 10.1097/PAS.0000000000000083 pmid: 24121169
[7]	Heaps JM, Nieberg RK, Berek JS. Malignant neoplasms arising in endometriosis[J]. Obstet Gynecol, 1990, 75(6):1023-1028. pmid: 2188180
[8]	Irvin W, Pelkey T, Rice L, et al. Endometrial stromal sarcoma of the vulva arising in extraovarian endometriosis: a case report and literature review[J]. Gynecol Oncol, 1998, 71(2):313-316. doi: 10.1006/gyno.1998.5142. doi: 10.1006/gyno.1998.5142 pmid: 9826478
[9]	McCluggage WG, Singh N, Gilks CB. Key changes to the World Health Organization (WHO) classification of female genital tumours introduced in the 5th edition (2020)[J]. Histopathology, 2022, 80(5):762-778. doi: 10.1111/his.14609. doi: 10.1111/his.14609 pmid: 34996131
[10]	Micci F, Heim S, Panagopoulos I. Molecular pathogenesis and prognostication of "low-grade" and "high-grade" endometrial stromal sarcoma[J]. Genes Chromosomes Cancer, 2021, 60(3):160-167. doi: 10.1002/gcc.22907. doi: 10.1002/gcc.22907
[11]	Amador-Ortiz C, Roma AA, Huettner PC, et al. JAZF1 and JJAZ1 gene fusion in primary extrauterine endometrial stromal sarcoma[J]. Hum Pathol, 2011, 42(7):939-946. doi: 10.1016/j.humpath.2010.11.001. doi: 10.1016/j.humpath.2010.11.001 pmid: 21316079
[12]	Sun L, Zhao W, Zhao Z, et al. JAZF1, YWHAE and BCOR gene translocation in primary extrauterine low-grade and high-grade endometrial stromal sarcomas[J]. Histopathology, 2022, 80(5):809-819. doi: 10.1111/his.14608. doi: 10.1111/his.14608
[13]	Kusaka M, Mikuni M, Nishiya M. A case of high-grade endometrial stromal sarcoma arising from endometriosis in the cul-de-sac[J]. Int J Gynecol Cancer, 2006, 16(2):895-899. doi: 10.1111/j.1525-1438.2006.00236.x. doi: 10.1111/j.1525-1438.2006.00236.x pmid: 16681780
[14]	Lauchlan SC. The secondary Müllerian system[J]. Obstet Gynecol Surv, 1972, 27(3):133-146. doi: 10.1097/00006254-197203000-00001. doi: 10.1097/00006254-197203000-00001 pmid: 4614139
[15]	Lauchlan SC. The secondary müllerian system revisited[J]. Int J Gynecol Pathol, 1994, 13(1):73-79. doi: 10.1097/00004347-199401000-00009. doi: 10.1097/00004347-199401000-00009 pmid: 8112958
[16]	Baiocchi G, Kavanagh JJ, Wharton JT. Endometrioid stromal sarcomas arising from ovarian and extraovarian endometriosis: report of two cases and review of the literature[J]. Gynecol Oncol, 1990, 36(1):147-151. doi: 10.1016/0090-8258(90)90126-6. doi: 10.1016/0090-8258(90)90126-6 pmid: 2403959
[17]	Bosincu L, Massarelli G, Cossu Rocca P, et al. Rectal endometrial stromal sarcoma arising in endometriosis: report of a case[J]. Dis Colon Rectum, 2001, 44(6):890-892. doi: 10.1007/BF02234715. doi: 10.1007/BF02234715 pmid: 11391154
[18]	Cho HY, Kim MK, Cho SJ, et al. Endometrial stromal sarcoma of the sigmoid colon arising in endometriosis: a case report with a review of literatures[J]. J Korean Med Sci, 2002, 17(3):412-414. doi: 10.3346/jkms.2002.17.3.412. doi: 10.3346/jkms.2002.17.3.412
[19]	Roşca E, Venter A, Muţiu G, et al. Endometrial stromal sarcoma developed on outer endometriosis foci[J]. Rom J Morphol Embryol, 2011, 52(Suppl 1):489-492.
[20]	Ayuso A, Fadare O, Khabele D. A case of extrauterine endometrial stromal sarcoma in the colon diagnosed three decades after hysterectomy for benign disease[J]. Case Rep Obstet Gynecol, 2013, 2013:202458. doi: 10.1155/2013/202458. doi: 10.1155/2013/202458
[21]	Son HJ, Kim JH, Kang DW, et al. Primary extrauterine endometrial stromal sarcoma in the sigmoid colon[J]. Ann Coloproctol, 2015, 31(2):68-73. doi: 10.3393/ac.2015.31.2.68. doi: 10.3393/ac.2015.31.2.68
[22]	Mourra N, Tiret E, Parc Y, et al. Endometrial stromal sarcoma of the rectosigmoid colon arising in extragonadal endometriosis and revealed by portal vein thrombosis[J]. Arch Pathol Lab Med, 2001, 125(8):1088-1090. doi: 10.5858/2001-125-1088-ESSOTR. doi: 10.5858/2001-125-1088-ESSOTR pmid: 11473465
[23]	Kovac D, Gasparović I, Jasic M, et al. Endometrial stromal sarcoma arising in extrauterine endometriosis: a case report[J]. Eur J Gynaecol Oncol, 2005, 26(1):113-116. pmid: 15755017
[24]	Chen CW, Ou JJ, Wu CC, et al. High-grade endometrial stromal sarcoma arising from colon endometriosis[J]. Int J Colorectal Dis, 2007, 22(12):1551-1553. doi: 10.1007/s00384-006-0267-6. doi: 10.1007/s00384-006-0267-6
[25]	Wang Q, Zhao X, Han P. Endometrial stromal sarcoma arising in colorectal endometriosis: a case report and review of the literature[J]. Case Rep Obstet Gynecol, 2015, 2015:534273. doi: 10.1155/2015/534273. doi: 10.1155/2015/534273
[26]	Yantiss RK, Clement PB, Young RH. Neoplastic and pre-neoplastic changes in gastrointestinal endometriosis: a study of 17 cases[J]. Am J Surg Pathol, 2000, 24(4):513-524. doi: 10.1097/00000478-200004000-00005. doi: 10.1097/00000478-200004000-00005 pmid: 10757398
[27]	Masand RP. Unusual Presentations of Gynecologic Tumors: Primary, Extrauterine, Low-Grade Endometrioid Stromal Sarcoma[J]. Arch Pathol Lab Med, 2018, 142(4):536-541. doi: 10.5858/arpa.2017-0241-RA. doi: 10.5858/arpa.2017-0241-RA pmid: 29293021
[28]	Zaza KJ, Arafah MA, Al-Badawi IA. Vulvar extrauterine endometrial stromal sarcoma: A case report and literature review[J]. Hematol Oncol Stem Cell Ther, 2015, 8(3):125-129. doi: 10.1016/j.hemonc.2014.12.003. doi: 10.1016/j.hemonc.2014.12.003 pmid: 25585306
[29]	Ramia JM, De la Plaza R, Garcia I, et al. Liver metastasis of endometrial stromal sarcoma[J]. World J Hepatol, 2012, 4(12):415-418. doi: 10.4254/wjh.v4.i12.415. doi: 10.4254/wjh.v4.i12.415 pmid: 23355923