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儿童及青少年卵巢幼年型颗粒细胞瘤:一项前瞻性合作研究中登记的33例患者的结果

Juvenile granulosa cell tumors of the ovary in children and adolescents: results from 33 patients registered in a prospective cooperative study.

作者信息

Calaminus G, Wessalowski R, Harms D, Göbel U

机构信息

Department of Pediatrics, Heinrich-Heine University, Düsseldorf, Germany.

出版信息

Gynecol Oncol. 1997 Jun;65(3):447-52. doi: 10.1006/gyno.1997.4695.

DOI:10.1006/gyno.1997.4695
PMID:9190974
Abstract

Clinical and pathological data from 33 prospective registered patients who suffered from juvenile granulosa cell tumors (JGCT) were evaluated according to treatment and outcome. The median age at the time of diagnosis was 7.6 years (range, 6 months to 17.5 years). Fourteen patients showed signs of a pseudo-precocious puberty. In 1 patient premenarcheal bleeding was the only clinical symptom of the disease. A pelvic tumor or an abdominal distention was found in 6 children, revealing signs of an acute abdomen in 3 children. Tumor staging was performed according to the FIGO (International Federation of Gynecology and Obstetrics) classification for ovarian tumors. Twenty children and adolescents were classified as FIGO stage Ia; 8 children had stage Ic tumors. In 4 patients stage IIc and in 1 patient stage IIIc tumors were observed. For local tumor control all 33 patients underwent tumor resection and oophorectomy, which was complete in 28 patients. Adjuvant combination chemotherapy was used in 1 girl who presented with high mitotic pathological index features in FIGO stage Ia. In 8 other children between FIGO stage Ic and IIIc, treatment was also intensified by multidrug chemotherapy. After a follow-up period of 168 months, an event-free survival of 0.75 +/- 0.07 was observed. From our data we conclude that multidrug chemotherapy including cisplatin-based regimens may be useful to enhance treatment results of JGCT, especially in advanced FIGO stages.

摘要

根据治疗情况和结果,对33例登记在册的青少年颗粒细胞瘤(JGCT)患者的临床和病理数据进行了评估。诊断时的中位年龄为7.6岁(范围为6个月至17.5岁)。14例患者出现假性性早熟迹象。1例患者经前出血是该疾病的唯一临床症状。6名儿童发现盆腔肿瘤或腹部膨隆,3名儿童出现急腹症迹象。根据国际妇产科联盟(FIGO)的卵巢肿瘤分类法进行肿瘤分期。20名儿童和青少年被分类为FIGO Ia期;8名儿童患有Ic期肿瘤。4例患者为IIc期肿瘤,1例患者为IIIc期肿瘤。为了进行局部肿瘤控制,所有33例患者均接受了肿瘤切除和卵巢切除术,其中28例患者手术完整。1名FIGO Ia期有高有丝分裂病理指数特征的女孩接受了辅助联合化疗。在其他8名FIGO Ic期至IIIc期的儿童中,也通过多药化疗加强了治疗。经过168个月的随访,观察到无事件生存率为0.75±0.07。根据我们的数据,我们得出结论,包括基于顺铂的方案在内的多药化疗可能有助于提高JGCT的治疗效果,尤其是在FIGO晚期。

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