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中华诊断学电子杂志 ›› 2025, Vol. 13 ›› Issue (04) : 275 -279. doi: 10.3877/cma.j.issn.2095-655X.2025.04.011

病例诊断思维

卵巢囊性幼年型粒层细胞瘤的影像及病理特征
陈锦1, 邱君斓2, 陈玲1, 徐新运1,()   
  1. 1210008 南京大学医学院附属鼓楼医院病理科
    2210008 南京大学医学院附属鼓楼医院超声医学科
  • 收稿日期:2025-09-19 出版日期:2025-11-26
  • 通信作者: 徐新运
  • 基金资助:
    南京市卫生科技发展专项资金资助项目(YKK24076)

Imaging and pathological features of cystic juvenile granulosa cell tumor of the ovary

Jin Chen1, Junlan Qiu2, Ling Chen1, Xinyun Xu1,()   

  1. 1Department of Pathology, Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School, Nanjing 210008, China
    2Department of Medical Ultrasound, Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School, Nanjing 210008, China
  • Received:2025-09-19 Published:2025-11-26
  • Corresponding author: Xinyun Xu
引用本文:

陈锦, 邱君斓, 陈玲, 徐新运. 卵巢囊性幼年型粒层细胞瘤的影像及病理特征[J/OL]. 中华诊断学电子杂志, 2025, 13(04): 275-279.

Jin Chen, Junlan Qiu, Ling Chen, Xinyun Xu. Imaging and pathological features of cystic juvenile granulosa cell tumor of the ovary[J/OL]. Chinese Journal of Diagnostics(Electronic Edition), 2025, 13(04): 275-279.

目的

探讨卵巢囊性幼年型粒层细胞瘤(CJGCT)的影像及病理特征。

方法

回顾性分析南京鼓楼医院2025年收治的2例CJGCT患者的症状、体征及影像学和病理等诊断学特征,复习文献总结CJGCT的诊断学要点。

结果

病例1,12岁女性,下腹部隐痛不适伴呕吐数日,超声提示盆腹腔巨大单房囊性占位(约20.6 cm × 20.9 cm × 9.7 cm),右卵巢未显示,较12 d前囊肿体积增长约10%。病例2,16岁女性,发现腹部包块5 d内迅速增大,包块上界达脐上1指,无腹痛或阴道出血。直肠彩超检查示盆腹腔囊性占位,大小为13.8 cm × 7.0 cm × 16.4 cm。两例患者CT平扫显示盆腹腔均匀低密度囊性病变,血清肿瘤标志物、性激素水平正常。两例病理显示肿瘤整体均呈完全囊性结构,外表面光滑,囊壁厚约0.3~0.5 cm,囊腔内含稀薄、水样或黏稠的淡染液体。类固醇生成因子-1(++)、抑制素α(弱+)、钙视网膜蛋白(-);网状纤维染色示肿瘤细胞巢周围阳性,病理诊断CJGCT。两例患者均接受保留生育功能的卵巢病变切除术,随访4~6个月无复发。

结论

CJGCT以完全囊性变为特征,临床罕见且影像学易误诊,确诊依赖术后病理检查证实,需结合临床与影像学特征综合判断。治疗首选保留生育功能的保守性手术,辅助化疗或二次手术的选择应结合临床分期及病理高危因素评估。

Objective

To investigate the imaging and pathological characteristics of cystic juvenile granulosa cell tumor (CJGCT) of the ovary.

Methods

A retrospective analysis of the clinical symptoms, signs, imaging, and pathological characteristics of 2 patients with CJGCT treated at Nanjing Drum Tower Hospital in 2025, and a review of the relevant literature to summarize the key diagnostic points.

Results

Case 1: a 12-year-old girl presented with lower abdominal discomfort and vomiting for several days. Ultrasonography revealed a large unilocular cystic mass in the abdominopelvic cavity (approximately 20.6 cm × 20.9 cm × 9.7 cm), with the right ovary not visualized; the cyst volume had increased by about 10% within 12 days. Case 2: a 16-year-old girl presented with a rapidly enlarging abdominal mass within 5 days, palpated up to 1 cm above the umbilicus, without abdominal pain or vaginal bleeding. Transrectal color Doppler ultrasound showed a cystic lesion in the pelvis (13.8 cm × 7.0 cm × 16.4 cm). On CT, both tumors appeared as homogeneous low density cystic masses. Serum tumor markers and sex hormone levels were within normal limits.Histopathology demonstrated entirely cystic tumors with smooth external surfaces and cyst walls measuring 0.3-0.5 cm in thickness, containing clear or slightly mucoid fluid. Immunohistochemistry showed steroidogenic factor 1 (+ + ), inhibin-α (weak + ), and calretinin (-). Reticulin staining highlighted a positive network surrounding tumor cell nests. The final pathological diagnosis was CJGCT in both cases. Both patients underwent fertility sparing excision of the ovarian lesion and were followed up for 4-6 months without recurrence.

Conclusions

CJGCT is characterized by complete cystic change, is clinically rare, and may be easily misdiagnosed by imaging examination. Definitive diagnosis relies on postoperative histopathological confirmation in conjunction with clinical and imaging findings. Fertility sparing conservative surgery is the preferred treatment, while the necessity of adjuvant chemotherapy or secondary surgery should be determined based on tumor stage and pathological high risk factors.

图1 囊性幼年型粒层细胞瘤患者超声检查图像注:a图为病例1检查图像,盆腹腔内见20.6 cm × 20.9 cm × 9.7 cm液性暗区,包膜完整,厚0.37 cm(红色圈区域);b图为病例2检查图像,盆腹腔内见19.8 cm × 9.6 cm × 12.4 cm液性暗区,包膜完整,厚0.2 cm(红色圈区域)
图2 囊性幼年型粒层细胞瘤患者病理学及分子检测图像注:a图示病变完全呈囊性,囊壁深部可见肿瘤细胞巢(箭头所示)(HE ×20);b图示肿瘤细胞呈滤泡样、巢团状排列,滤泡内含嗜碱性或嗜酸性分泌物(箭头所示)(HE ×100);c图示部分区域肿瘤细胞呈实性片状排列,瘤细胞具有轻-中度的异型性(HE ×400);d图示肿瘤细胞Melan A呈阳性(DAB ×200);e图示肿瘤细胞CK呈阳性(DAB ×200);f图示肿瘤细胞INHA呈阳性(DAB ×200);g图示肿瘤细胞巢周围网状纤维染色呈阳性,而不是单个瘤细胞周围(×200);h图示分子检查,未检出FOXL2基因c.402 C>G突变(标黄字母)。Melan A为黑色素A;CK为细胞角蛋白;INHA为抑制素α
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