Department of Radiotherapy and Oncology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Department of Radiotherapy and Oncology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Clin Neurol Neurosurg. 2021 Mar;202:106508. doi: 10.1016/j.clineuro.2021.106508. Epub 2021 Jan 27.
Embryonal tumors with multilayered rosettes (ETMR) is an extremely rare and highly aggressive tumor. It includes three distinct entities i.e, embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL) and medulloepithelioma (MEPL). Here, we present our institutional experience of seven ETMR cases treated over a period of five years.
Patients' records from 2015 to 2019 were reviewed manually and electronically to retrieve the data. Clinicopathological and outcome details of ETMR cases were entered in a predesigned proforma.
A total of seven cases of ETMR were registered from 2015 to 2019 with a median age at presentation of four years (range 3-7 years). All patients underwent surgery. However, only three patients completed the planned adjuvant treatment, comprising of focal radiotherapy (RT) alone, craniospinal irradiation (CSI) alone and CSI followed by six cycles of chemotherapy in one patient each respectively. Two patients commenced CSI but deteriorated during RT and thereafter needed best supportive care. Two patients could not be started on any adjuvant treatment. Unfortunately, six patients succumbed to their disease within one year of their diagnosis. Only one patient who received both CSI and adjuvant chemotherapy is alive at 15 months of diagnosis.
ETMR is a rare and aggressive entity. Majority of the patients die within one year of the diagnosis despite multimodality treatment.
具有多层玫瑰花结的胚胎性肿瘤（ETMR）是一种极其罕见且高度侵袭性的肿瘤。它包括三个不同的实体，即富含神经胶质的胚胎性肿瘤伴真性玫瑰花结（ETANTR）、室管膜母细胞瘤（EBL）和髓上皮瘤（MEPL）。在此，我们介绍了我们机构在五年期间治疗的七例 ETMR 病例的经验。
手动和电子检索 2015 年至 2019 年患者的记录，以获取数据。将 ETMR 病例的临床病理和结局详细信息输入到预先设计的表格中。
2015 年至 2019 年共登记了 7 例 ETMR，中位发病年龄为 4 岁（范围 3-7 岁）。所有患者均接受了手术。然而，只有 3 名患者完成了计划的辅助治疗，包括单独局部放疗（RT）、单纯颅脊髓照射（CSI）和 CSI 后各有 1 名患者接受 6 个周期化疗。2 名患者开始接受 CSI，但在 RT 期间恶化，此后需要最佳支持治疗。2 名患者无法接受任何辅助治疗。不幸的是，6 名患者在诊断后一年内死于该病。仅 1 名接受 CSI 和辅助化疗的患者在诊断后 15 个月仍存活。
ETMR 是一种罕见且侵袭性的实体瘤。尽管采用了多模式治疗，但大多数患者在诊断后一年内死亡。
