摘要目的 分析卵巢幼年型粒层细胞瘤的临床病理特点,探讨其诊断标准、特殊形态及预后因素等.方法 对7例幼年型粒层细胞瘤的临床资料行回顾性分析总结、组织切片进行形态观察,对其中6例行免疫组织化学EliVision法染色.6例进行了随访.结果 7例幼年型粒层细胞瘤的平均年龄为24岁(6～53岁).5例有性激素紊乱的临床表现,2例以腹胀及腹痛为主要症状.除1例(例2)行全子宫和双附件切除,其余6例均为一侧附件切除.5例Ⅰ A期,2例Ⅰ C期.6例随访病例中除1例(例1)术后1年余死于肿瘤转移,其余5例健在,随访时间平均4.3年(1～10年).肿瘤最大径7～20 cm(平均13.4 cm),4例囊实性,2例单房囊性,1例实性.镜下全部为弥漫生长和不典型滤泡样结构,未见Call-Exner小体.肿瘤细胞核小、较圆、深染,极少见核沟,3例重度异形,1例中度,3例轻度.1例(例2)小灶有成年型粒层细胞瘤特征.5例核分裂象＞5/10 HPF,2例核分裂象不易见.6例CK(AE1/AE3)阴性、波形蛋白强阳性;1例α-抑制素阳性;4例CD99阳性、Calretinin阴性.结论 幼年型粒层细胞瘤以不典型滤泡样结构而非Call-Exner小体为结构特点,以核小、圆形、深染而无核沟为主要细胞学特点,以大多数CK阴性和波形蛋白阳性为特殊免疫表型,兼以有少量成年型粒层细胞肿瘤结构及单房囊性肿瘤为其特殊形态.肿瘤破裂可能是影响预后的一个重要因素,核异形、核分裂象多见及肿瘤体积大并不能提示肿瘤具有恶性潜能.

abstractsObjective To study the clinicopathologic features, diagnostic criteria and prognostic parameters of juvenile granulosa cell tumor of ovary. Methods The clinical and pathologic findings of 7 cases of juvenile granulosa cell tumor were retrospectively reviewed. Immunohistochemical study was carried out in 6 of these cases. The follow-up data were also analyzed. Results The mean age of the patients was 24 years ( range = 6 to 53 years). Four patients presented with hormonal disturbance, while 3 patients presented with abdominal pain or swelling. Six patients underwent unilateral salpingo-oophorectomy. Six cases were in stage Ⅰ A and the remaining case in stage Ⅰ C. Follow-up information was available in 6 patients and the duration of follow up ranged from 1 to 10 years (mean =4.3 years). Five patients remained healthy, with no evidence of tumor recurrence. One patient died of tumor metastasis one year after the diagnosis. Gross examination showed that the tumor masses ranged from 7 to 20 cm in the greatest dimension (average = 13.4 cm). Four of the 7 tumors were mixed solid-cystic in appearance and 2 cases were unilocular cystic in nature. Microscopic examination showed diffuse atypical follicular structures formed by granulosa cells. The granulosa cells contained round hyperchromatic nuclei, without nuclear grooves or Call-Exner body formation (6/7). In one of the cases studied, minor foci resembling adult granulosa cell tumor were also demonstrated. The degree of cellular atypia varied (3 cases with severe atypia, 1 case with moderate atypia and 3 cases with mild atypia). The mitotic count ranged from 1 to more than 5 per 10 highpower fields. Immunohistochemical study showed diffuse positivity for vimentin (6/6). The staining for cytokeratin ( AE1/AE3) and calretinin was negative. Four cases expressed CD99 and 1 case was positive for inhibin. Conclusions Juvenile granulosa cell tumor is characterized by the presence of diffuse atypical follicular structures formed by small round cells, without nuclear grooves or Call-Exner bodies. Rare cases contain minor foci of adult granulosa cell tumor. They can be unilocular cystic in nature. The degree of nuclear atypia, mitotic activity and size of the tumor vary and do not correlate with the risk of recurrence and aggressive biologic behavior.