原发部位在子宫肌层的卵泡膜细胞瘤一例
Primary Thecoma Arising in the Uterine Myometrium: A Case Report
DOI: 10.12677/acm.2025.1572026, PDF, HTML, XML,   
作者: 莫天瑶, 邓馨仪, 刘金香, 罗惠娟*:暨南大学附属第一医院妇产科,广东 广州
关键词: 卵泡膜细胞瘤子宫平滑肌瘤手术疾病特点Thecoma Uterine Leiomyoma Operation Disease Characteristic
摘要: 卵泡膜细胞瘤(thecomas)通常为卵巢性索间质肿瘤,罕见于卵巢外部位。本文报告一例原发性颗粒细胞瘤发生于49岁绝经后女性子宫肌层的病例。患者因偶然发现的腹部肿块就诊,无特异性症状。超声检查显示子宫前壁肌层见两个实性低回声团,最大者尺寸为110 mm × 73 mm × 90 mm。最初诊断为子宫平滑肌瘤。手术过程中,发现子宫内肿块呈黄色,质地松软。手术标本送冰冻病理检查,回报:间叶源性肿瘤,最终病理检查回报:泡膜细胞瘤。此病例提醒我们,绝经后出现的子宫大肿块可能是其他组织来源的罕见肿瘤。
Abstract: Thecomas are typically ovarian sex cord-stromal tumors that rarely occur in extraovarian locations. We report a case of primary thecoma arising in the uterine myometrium of a 49-year-old postmenopausal woman. The patient presented with an incidentally discovered abdominal mass without specific symptoms. Ultrasound examination revealed multiple hypoechoic masses in the myometrium, with the largest measuring 110 mm × 73 mm × 90 mm. Initially diagnosed as a uterine leiomyoma. During surgery, an intramural uterine mass was identified, which appeared yellow in color and friable in consistency. The surgical specimen was submitted for pathological examination, which confirmed the diagnosis of thecoma. This case reminds us that the large mass of the uterus that appears after menopause may be a rare tumor of other tissue origins.
文章引用:莫天瑶, 邓馨仪, 刘金香, 罗惠娟. 原发部位在子宫肌层的卵泡膜细胞瘤一例[J]. 临床医学进展, 2025, 15(7): 575-579. https://doi.org/10.12677/acm.2025.1572026

1. 引言

卵泡膜细胞瘤是一种罕见的性索间质肿瘤,通常发生在卵巢中,约占所有卵巢肿瘤的0.5%~1% [1]。卵巢外卵泡膜细胞瘤极为罕见。迄今为止,仅报告了三例卵巢外卵泡膜细胞瘤[2]-[4]。我们报告了第四例卵巢外卵泡膜细胞瘤,该病例发现于一名误诊为子宫平滑肌瘤的患者。该肿瘤起源于子宫肌层,没有卵巢病变。

2. 病例简介

一名49岁的绝经后女性因偶然发现下腹部肿块而到妇科就诊。否认异常阴道出血、腹痛或其他不适,否认高血压、糖尿病病史。孕3产1流产2,顺产史1次。没有绝经后阴道出血。否认有任何遗传性疾病或肿瘤的家族史。

Figure 1. (A) Preoperative transvaginal ultrasound scan revealed a large uterine mass of heterogeneous echogenicity. (B) Cut section of the uterus shows a yellow, friable tumor within the myometrium

1. (A) 术前经阴道超声扫描显示子宫肌壁间肿块,回声不均匀。(B) 子宫切面显示肌层内有一个黄色、脆弱的肿瘤

体格检查显示生命体征稳定(体温36.8℃,脉搏78次/分,呼吸频率16次/分,血压105/54 mmHg,身高155 cm,体重55 kg,BMI 22.9 kg/m2)。腹部叩诊未发现移动性浊音。妇科检查:宫颈光滑且增大,无宫颈举痛,无接触性出血。双合诊检查显示子宫增大,表面光滑,活动度正常。在子宫底部可触及一个约10 cm × 10 cm的坚实肿块,无压痛。双侧附件未清晰触及。经阴道超声检查显示子宫增大,大小为134 mm × 92 mm × 104 mm。在前壁肌层内发现一个明显的低回声肿块,大小为110 mm × 73 mm × 90 mm。肿块呈规则的椭圆形,略向外突出,内部回声均匀且低水平,边界清晰。彩色多普勒血流成像(CDFI)显示无明显血流信号。双侧卵巢外观正常,无占位性病变。超声检查提示子宫肌瘤(图1(A))。

根据临床发现和影像学结果,患者被诊断为子宫平滑肌瘤。治疗计划包括术前实验室检查(如血常规、血型、心电图、胸部X光片等),因患者拒绝切除卵巢,故拟行腹腔镜全子宫切除术及双侧输卵管切除术。手术将根据术中实际情况进行调整。

手术中,腹腔镜探查显示子宫增大,双侧附件萎缩。子宫和双侧输卵管通过腹腔镜成功完整切除。由于子宫体积较大,无法通过阴道完整取出。因此,子宫被放入标本袋中,并在阴道取出时进行切分。剖视切除的子宫发现一个壁间肿块,呈黄色,质地脆弱(图1(B))。标本送冰冻切片分析,报告为间叶性肿瘤。在与患者家属充分讨论并获得知情同意后,进行了双侧卵巢切除术。

病理回报:镜下见肿瘤细胞呈梭形,胞核呈圆形或短柱状,泡状核,有小核仁,核分裂象少见,肿瘤边界清晰,向宫肌层结节状推挤性生长;免疫组化:ER (80%),PR (100%+),CR (+),inhibinα (+),Desmin (−),SMA (弱+),CD34 (部分+),H-caldesmon (−),S100 (−),SOX10 (−),CD10 (−),STAT6 (胞浆+),BCOR (−),ALK (−),P16 (−),HMB45 (−),Ki67 (2%+)。结合镜下形态及免疫组化,考虑卵泡膜细胞瘤,双侧卵巢白体形成,输卵管无明显异常。

3. 讨论及文献复习

根据世界卫生组织(WHO)分类,性索间质肿瘤(Sex Cord-Stromal Tumors, SCSTs)是卵巢肿瘤的主要类别之一,与上皮性肿瘤、生殖细胞肿瘤及其他类型肿瘤不同[5]。SCSTs约占所有卵巢肿瘤的5%~8% [6] [7],其中卵泡膜细胞瘤是一种特定亚型,通常起源于卵巢组织。

卵巢外的卵泡膜细胞瘤极为罕见。迄今为止,仅报道了三例卵巢外卵泡膜细胞瘤病例[2]-[4]。我们报告了第四例卵巢外卵泡膜细胞瘤病例,该病例最初被诊断为子宫平滑肌瘤。该肿瘤起源于子宫肌层,且未发现卵巢病变。从组织发生学角度来看,卵巢外部位出现SCST可归因于性索细胞及其衍生物起源于体腔上皮,而非胚胎发育过程中原始生殖细胞迁移的卵巢间质[8]。异位性腺间质组织和多余卵巢也被认为是性腺外部位发生SCST的另一种解释[9]

卵巢外SCST通常发生于腹膜后、输卵管、卵巢系膜、肾上腺、肠系膜、阔韧带和脐疝囊等部位[10]。我们报告的这例子宫肌层起源的卵泡膜细胞瘤此前从未被报道过。该病例的术前影像学结果具有误导性,因为根据超声特征,病变最初被解释为子宫平滑肌瘤。

卵泡膜细胞瘤具有独特的组织学特征,表现为空泡化的梭形细胞,胞质淡染且边界不清,形成合体样生长模式,呈弥漫性或结节性排列。肿瘤细胞核无异型性,核分裂活性低,有时可见核沟。间质中含有胶原沉积和透明斑块,可能包含性索成分。免疫组化特征通常为多种标志物阳性,包括calretinin、inhibin、WT1、CD56、ER/PR、CD10和FOXL2,并具有特征性的网状纤维染色模式。要确认病变为原发于子宫肌层的卵泡膜细胞瘤,双侧卵巢必须显示正常且皮质完整,卵巢与肿瘤之间存在正常组织[11]。在本病例中,病理检查结果与这些典型特征基本一致。肿瘤完全位于子宫肌层内,未发现卵巢或输卵管病变。显微镜下,肿瘤显示为梭形卵巢细胞,核呈圆形或杆状,核呈空泡状。免疫组化分析显示关键标志物ER (80%阳性)、PR (阳性)和inhibin (阳性)呈阳性,与典型特征一致。Ki67指数低(2%)证实了其低增殖活性(图2)。

虽然SCST通常被认为是良性肿瘤且预后良好,但其临床行为和预后尚未明确。治疗方案根据肿瘤类型和分期而异:对于良性SCST,保守手术(卵巢切除术或卵巢囊肿切除术)即可;对于卵泡膜细胞瘤,围绝经期和绝经后患者需行全子宫切除术及双侧输卵管卵巢切除术,但对于年轻患者可考虑保留生育功能的手术;对于恶性SCST,需通过开腹或腹腔镜进行完整的手术分期(不推荐常规淋巴结清扫),晚期疾病需辅助化疗,对于可切除的复发性疾病,手术切除是首选[12]。鉴于卵巢外SCST的罕见性,目前尚无标准化治疗方案,但现有卵巢性索间质肿瘤指南建议进行全面的手术分期,对于早期或低风险且希望保留生育功能的病例可选择保留生育功能的手术,而美国国家综合癌症网络(NCCN)建议在完成生育后行全子宫切除术及双侧输卵管卵巢切除术[13]

Figure 2. Histological and immunohistochemical images of mass. Histopathology showed characteristic spindle-shaped tumor cells arranged in fascicles and storiform pattern (A, B, H&E, ×40). The tumor cells exhibited uniform nuclei with no significant atypia at higher magnification (C, H&E, ×100). Immunohistochemistry demonstrated strong positive staining for CR (D) and inhibin α (E) (×40)

2. 肿块的组织学和免疫组化图像。组织病理学显示特征性的梭形肿瘤细胞呈束状和席纹状排列(A、B,H&E染色,×40)。高倍镜下,肿瘤细胞核均匀,无明显异型性(C,H&E染色,×100)。免疫组化显示CR (D)和inhibin α (E)呈强阳性染色(×40)

4. 结论

卵巢外SCST,尤其是起源于子宫肌层的卵泡膜细胞瘤,非常罕见,术前诊断极具挑战性。免疫组化染色对于诊断至关重要。手术是首选的治疗方案,需根据肿物的位置、患者的年龄、生育要求等决定术式。

NOTES

*通讯作者。

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