Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT): A review of 47 cases.
Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT): A review of 47 cases.
复制标题
DOI：
10.1016/j.ygyno.2015.11.004
复制
发表时间：
2016-01
影响因子：
4.7
通讯作者：
Schmeler KM
中科院分区：
文献类型：
--
作者：
Callegaro-Filho D;Gershenson DM;Nick AM;Munsell MF;Ramirez PT;Eifel PJ;Euscher ED;Marques RM;Nicolau SM;Schmeler KM
卵巢高钙型小细胞癌是一种罕见的疾病，预后不良。SCCOHT最近被证明与SMARCA 4基因突变以及与恶性横纹肌样瘤（MRT）的分子和遗传相似性相关。我们研究的目的是描述47例SCCOHT患者的临床特征、治疗方式和结局。我们对1990年至2014年期间在MD安德森癌症中心接受评估的47例SCCOHT患者进行了回顾性分析。回顾病历中的人口统计学信息、病理学发现、治疗方案和结局。诊断时的中位年龄为30岁（范围5-46岁）。所有患者均接受手术，其中26例（55%）患者行单侧输卵管卵巢切除术（USO），21例（45%）患者行子宫切除术和双侧输卵管卵巢切除术（BSO）。16例患者（34.0%）为I期疾病，6例（12.8%）为II期疾病，23例（48.9%）为III期疾病，2例（4.3%）为IV期疾病。43例患者的辅助治疗信息可用：83.3%仅接受化疗，9.5%化疗后放疗，2.4%放化疗，4.8%未接受任何辅助治疗。中位随访时间为13.2个月（范围：0.1 - 210.7），中位总生存期为14.9个月。多药化疗和放疗预后较好。我们的研究结果表明，积极的治疗，包括多药化疗和可能的放疗可能会延长生存期。需要进一步的研究来改善这些患者的预后，包括采用MRT中使用的全身治疗以及开发针对特定突变的新型药物。
Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT) is a rare disease with a poor prognosis. SCCOHT has recently been shown to be associated with SMARCA4 gene mutations as well as molecular and genetic similarities to malignant rhabdoid tumors (MRT). The objective of our study is to describe the clinical characteristics, treatment modalities and outcomes of 47 patients with SCCOHT. We performed a retrospective analysis of 47 patients with SCCOHT evaluated at MD Anderson Cancer Center between 1990 and 2014. Medical records were reviewed for demographic information, pathologic findings, treatment regimens and outcomes. Median age at diagnosis was 30 years (range 5-46). All patients underwent surgery with unilateral salpingo-oophorectomy (USO) performed in 26 patients (55%), and hysterectomy with bilateral salpingooophorectomy (BSO) in 21 patients (45%). Sixteen patients (34.0%) had stage I disease, six (12.8%) stage II, 23 (48.9%) stage III, and two patients (4.3%) had stage IV disease. Information on adjuvant treatment was available for 43 patients: 83.3% received chemotherapy alone, 9.5% chemotherapy followed by radiotherapy, 2.4% chemoradiation, and 4.8% did not receive any adjuvant therapy. Median follow-up was 13.2 months (range, 0.1 to 210.7) with a median overall survival of 14.9 months. Multi-agent chemotherapy and radiotherapy were associated with a better prognosis. Our findings suggest that aggressive therapy including multi-agent chemotherapy and possibly radiotherapy may extend survival. Further study is needed to improve outcomes in these patients including the adoption of systemic therapies used in MRT as well as the development of novel agents targeting specific mutations.
登录
查看更多内容
DOI：
10.1111/igc.0b013e3181a1a116
发表时间：
2009-04-01
影响因子：
4.8
作者：
Dykgraaf, Ramon H. M.;de Jong, Diederick;van der Burg, Maria E. L.
通讯作者：
van der Burg, Maria E. L.
影响因子：
7.3
作者：
Foulkes, William D.;Clarke, Blaise A.;McCluggage, W. Glenn
通讯作者：
McCluggage, W. Glenn
影响因子：
2.6
作者：
Estel, Rebecca;Hackethal, Andreas;Muenstedt, Karsten
通讯作者：
Muenstedt, Karsten
影响因子：
6.4
作者：
Vujanic, GM;Sandstedt, B;Delemarre, JFM
通讯作者：
Delemarre, JFM
影响因子：
5.6
作者：
YOUNG, RH;OLIVA, E;SCULLY, RE
通讯作者：
SCULLY, RE