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颅内生殖细胞肿瘤:来自印度三个三级医疗中心的多机构经验。

Intracranial germ cell tumors: a multi-institutional experience from three tertiary care centers in India.

作者信息

Kakkar Aanchal, Biswas Ahitgani, Kalyani Nikhil, Chatterjee Uttara, Suri Vaishali, Sharma Mehar C, Goyal Nishant, Sharma Bhawani S, Mallick Supriya, Julka Pramod K, Chinnaswamy Girish, Arora Brijesh, Sridhar Epari, Chatterjee Sandip, Jalali Rakesh, Sarkar Chitra

机构信息

Department of Pathology, All India Institute of Medical Sciences, New Delhi, 110029, India.

Department Radiation Oncology, All India Institute of Medical Sciences, New Delhi, 110029, India.

出版信息

Childs Nerv Syst. 2016 Nov;32(11):2173-2180. doi: 10.1007/s00381-016-3167-2. Epub 2016 Jul 30.

Abstract

OBJECTIVE

Central nervous system germ cell tumors (CNS GCTs) are relatively rare neoplasms. Incidence of CNS GCTs in Western literature is low (0.3-0.6 %) as compared to East Asia (3-4 %). No large study is available on CNS GCTs from India.

METHODS

Intracranial GCT cases were retrieved from databases of three tertiary care institutes in India; clinicopathological data was reviewed.

RESULTS

Ninety-five intracranial GCT cases were identified, accounting for 0.43 % of CNS tumors. Median age was 12 years (range, birth to 48 years); male preponderance was noted (66 %). Most patients (86.3 %) were aged <18 years. Pineal location was most common (45 %) and was associated with male gender and age >14 years. Germinoma was the commonest histopathological type (63.2 %), followed by teratoma (20 %). Suprasellar location was associated with germinoma histology. Follow-up was available for 71 patients (median, 15 months). Of these, 48 received adjuvant chemotherapy and/or radiotherapy. At the last follow-up, 44 patients showed no evidence of disease. Age >10 years, male gender, pineal location, and germinoma histology were associated with favorable outcome.

CONCLUSION

This is the first multicentric study from India establishing that incidence of CNS GCT in India is similar to that in the West and differs from that in East Asian countries. However, similar to both, germinoma is the commonest histological type, and pineal location is most frequent. Studies on molecular alterations based on ethnicity and geographical location are necessary to provide clarity on differences in incidence. Attention needs to be focused on decreasing treatment heterogeneity and minimizing treatment-related morbidity and mortality, improving the cure rate of these highly treatable tumors.

摘要

目的

中枢神经系统生殖细胞肿瘤(CNS GCTs)是相对罕见的肿瘤。与东亚地区(3%-4%)相比,西方文献中CNS GCTs的发病率较低(0.3%-0.6%)。目前尚无来自印度的关于CNS GCTs的大型研究。

方法

从印度三家三级医疗机构的数据库中检索颅内GCT病例;对临床病理数据进行回顾。

结果

共识别出95例颅内GCT病例,占中枢神经系统肿瘤的0.43%。中位年龄为12岁(范围,出生至48岁);男性占优势(66%)。大多数患者(86.3%)年龄<18岁。松果体区是最常见的部位(45%),且与男性性别和年龄>14岁相关。生殖细胞瘤是最常见的组织病理学类型(63.2%),其次是畸胎瘤(20%)。鞍上部位与生殖细胞瘤组织学相关。71例患者获得随访(中位随访时间为15个月)。其中,48例接受了辅助化疗和/或放疗。在最后一次随访时,44例患者无疾病证据。年龄>10岁、男性性别、松果体区部位和生殖细胞瘤组织学与良好预后相关。

结论

这是来自印度的第一项多中心研究,证实印度CNS GCT的发病率与西方相似,与东亚国家不同。然而,与两者相似的是,生殖细胞瘤是最常见的组织学类型,松果体区部位最常见。有必要开展基于种族和地理位置的分子改变研究,以明确发病率差异。需要关注减少治疗异质性,将治疗相关的发病率和死亡率降至最低,提高这些高度可治性肿瘤的治愈率。

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