子宫外子宫内膜间质肉瘤的临床病理特征分析

Clinicopathologic features,diagnosis and treatment with extrauterine stromal sarcoma

目的：探讨子宫外子宫内膜间质肉瘤（ESS）的临床病理特点、免疫组织化学特征、生物学行为及诊治经验。方法：回顾性分析自1998年4月至2012年9月间温州市人民医院及温州医科大学附属第一医院收治的10例ESS患者的临床及病理资料,并进行免疫组织化学检测及预后随访。结果：9例病理诊断为低级别ESS,1例为子宫外未分化子宫内膜肉瘤（EUES）;6例发生于卵巢,其余4例分别累及双侧卵巢＋子宫直肠窝、右卵巢＋生殖静脉＋下腔静脉、左卵巢＋腹膜后、输卵管;临床表现以腹痛、腹部包块及痛经为主,其中5例伴有明确的子宫内膜异位症。免疫组织化学显示：CD10（8/10）、ER（5/10）、PR（9/10）、SMA（5/10）、Desmin（2/10）、Caldesmon（1/10）。所有患者均接受手术彻底切除,其中9例术后辅以放化疗或内分泌治疗;10例均获随访,随访时间为19~127个月,有5例出现复发,首次复发时间均在2年以内,复发的5例中有2例（包括1例EUES）为多次复发并最终死亡,其余患者在随访结束时均为存活状态。结论：ESS是非常少见的疾病,ESS以卵巢发生最为常见,大部分与子宫内膜异位症有关,治疗首选彻底手术切除,术后可辅以放化疗和内分泌治疗。低级别ESS容易短期内一次或多次复发,但复发后仍可较长时间存活;免疫组织化学标记CD10较为特异。

Objective： To study the clinicopathologic and immunohistochemical features, biological behavior, diagnosis and treatment of extrauterine stromal sarcoma （ESS）. Methods： A retrospective clinicopathologic analysis was made on 10 cases of ESS admitted to the Third Clinical Institute Affiliated to Wenzhou Medical University and the First Affiliated Hospital of Wenzhou Medical University from April 1998 to September 2012. Results： Among 10 patients, 9 had low-grade ESS and 1 had extrauterine undifferentiated endometrial sarcoma （EUES）. 6 tumors located in the ovary, while the other 4 cases involved bilateral ovaries/uterine rectum nest, the right ovary/genital vein/inferior vena cava, the left ovary/retroperitoneal and fallopian tube respectively. The clinical manifestations were mainly abdominalgia, abdominal mass and algomenorrhea, 5 patients had endome- triosis. The others were alive until the end of follow-up time. Immunohistolochemically, CD10 （8/10）, Estrogen receptor （5/10）, Progesterone receptor （9/10）, Smooth muscle actin （5/10）, Desmin （2/10）, Caldesmon （1/10）, All 10 patients were treated with complete surgical resection and received postoperative adjuvant therapy. The follow-up time for all patients ranged from 19-127 months. 5 patients had one or more recurrences, the time of first recurrences was within 2 years. 2 of 5 patients died of multiple recurrences including 1 EUES. Conclusion： ESS which is the most common in ovary is an extremely rare neoplasm. Most of them are associated with endo-metriosis. The preferred therapy is complete excision and postoperative adjuvant therapy. ESS with low-grade had one or more replases easily in short time, but the patients should be alive for a long time. CD10 as immuno-histochemical marker is specific.