Division of Pediatric Hematology-Oncology at Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, California 90027, USA.
Curr Opin Neurol. 2010 Dec;23(6):576-82. doi: 10.1097/WCO.0b013e3283404ef1.
Pineal tumors are rare in children, with pineoblastoma and germ cell tumors (GCTs) being the most common. Here we discuss recent advances in treatment and controversies in the management of these tumors.
There is significant heterogeneity in the clinical behavior of pineoblastoma in children. We will discuss differences in outcome of children with pineoblastoma who are less than and greater than 3 years of age, and between pineoblastoma and nonpineal supratentorial primitive neuro-ectodermal tumors when treated with multiple different strategies. Significant controversies exist in the treatment of GCTs as well, including the levels of tumor markers in the blood and cerebrospinal fluid that are required to establish without biopsy the diagnosis of a GCT, the role of surgery in GCTs and the optimal treatment for germinomas as well as mixed malignant GCTs.
Although pineoblastoma in infants and very young children still remains a therapeutic challenge, significant progress has been made in the treatment of pineal GCTs with treatment strategies using a combination of chemotherapy and reduced dose and volume irradiation, resulting in increased survival rates and reduced long-term morbidity.
松果体肿瘤在儿童中较为罕见，其中以松果体母细胞瘤和生殖细胞肿瘤（GCT）最为常见。本文讨论了这些肿瘤治疗方面的最新进展和争议。
儿童松果体母细胞瘤的临床行为存在显著异质性。我们将讨论接受多种不同治疗策略的 3 岁以下和 3 岁以上儿童的松果体母细胞瘤、以及松果体母细胞瘤和非松果体幕上原始神经外胚层肿瘤之间的预后差异。GCT 的治疗也存在显著争议，包括在不进行活检的情况下，通过血液和脑脊液中的肿瘤标志物水平来诊断 GCT 的标准、手术在 GCT 中的作用以及生殖细胞瘤和混合恶性 GCT 的最佳治疗方法。
尽管婴儿和非常年幼儿童的松果体母细胞瘤仍然是一个治疗挑战，但通过化疗联合低剂量和小体积照射的治疗策略，松果体生殖细胞肿瘤的治疗已取得显著进展，从而提高了生存率，降低了长期发病率。
