高级别肉瘤，以子宫主要神经外胚层和少量胚胎性横纹肌肉瘤为主：一例报告。
High grade sarcoma, with predominant neuroectodermal and minor embryonal rhabdomyosarcomatous tumor of the uterus: A case report.
作者信息
Chang Leona, Enriquez Miriam, Lerman Nati, Wilson-Smith Robin
机构信息
Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, MD Anderson Cancer Center at Cooper, Cooper University Health System, Camden, NJ, USA.
Department of Pathology, Cooper University Health System, Camden, NJ, USA.
出版信息
Gynecol Oncol Rep. 2019 Apr 13;28:128-132. doi: 10.1016/j.gore.2019.04.001. eCollection 2019 May.
BACKGROUND
There have been few documented cases of combined primitive neuroectodermal and embryonal rhabdomyosarcomas (ERMS) in the uterus. Due to their rarity, there is no consensus on the optimal treatment for patients with primitive neuroectodermal tumor (PNET) and ERMS of the uterus. Studies on treatment and outcome are limited.
CASE PRESENTATION
A 32 year-old female presented with heavy vaginal bleeding. Ultrasound revealed an 18 cm uterus with thickened endometrium. Histopathology revealed embryonal rhabdomyosarcoma. She underwent a total abdominal hysterectomy, bilateral salpingectomy, lymph node dissection, and omentectomy. Pathologic review confirmed a tumor with mainly central-type PNET and focally ERMS within the uterus and cervix. She was treated with adjuvant chemoradiation.
CONCLUSION
Treatment of the predominant tumor, PNET, should be the primary goal of therapy. Vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide with tumor directed radiation may be efficacious for the treatment of this specific high grade uterine sarcoma.
背景
子宫内同时存在原始神经外胚层肿瘤和胚胎性横纹肌肉瘤（ERMS）的病例报道较少。由于其罕见性，对于子宫原始神经外胚层肿瘤（PNET）和ERMS患者的最佳治疗方案尚无共识。关于治疗和预后的研究有限。
病例介绍
一名32岁女性因大量阴道出血就诊。超声检查显示子宫大小为18厘米，子宫内膜增厚。组织病理学检查显示为胚胎性横纹肌肉瘤。她接受了全腹子宫切除术、双侧输卵管切除术、淋巴结清扫术和大网膜切除术。病理检查证实子宫和宫颈内的肿瘤主要为中心型PNET，局部为ERMS。她接受了辅助放化疗。
结论
治疗主要肿瘤PNET应是治疗的首要目标。长春新碱、多柔比星、环磷酰胺、异环磷酰胺和依托泊苷联合针对肿瘤的放疗可能对治疗这种特定的高级别子宫肉瘤有效。
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