Varan Ali, Sari Neriman, Akalan Nejat, Söylemezoğlu Figen, Akyüz Canan, Kutluk Tezer, Büyükpamukçu Münevver
Department of Pediatric Oncology, Hacettepe University Institute of Oncology, Ankara, Turkey.
J Neurooncol. 2006 Sep;79(2):187-90. doi: 10.1007/s11060-006-9123-3. Epub 2006 Apr 28.
Our aim is to evaluate the clinical features and outcomes of children with primary central nervous system (CNS) tumors who develop extraneural metastasis (ENM). We retrospectively evaluated children diagnosed with primary CNS tumors treated at our institution between 1972 and 2004. Of 1,011 patients these tumors, 10 (0.98%) developed ENM. The histopathologic diagnosis was medulloblastoma in six patients, germ cell tumors in two patients, and ependymoma and atypical teratoid rhabdoid tumor (ATRT) in one patient each. In six patients, the primary tumor was located in the posterior fossa; it had a supratentorial location in the patient with ATRT, was located in the sellar and suprasellar region in the two patients with germ cell tumors, and was found in the distal spinal cord in the patient with an ependymoma. In two patients ENM was detected at the time of diagnosis. In other patients ENM developed between 9 and 25 months after diagnosis. Metastatic sites included bone, bone marrow, lung, cervical lymph nodes, liver, and paranasal sinuses. Of the 10 patients who developed ENM, 8 died of their disease 0.27-16.2 months (median, 2.60 months) after it was detected. One patient with dysgerminoma is alive, without disease, 117.80 months after diagnosis of the ENM. One patient with germ cell tumor is alive with disease 11.3 months after diagnosis of the ENM. Systemic metastasis to other extraneural sites is extremely rare in children with intracranial tumors. In our series the rate of ENM is 0.98%. The liver and lung are the most common site for metastasis, followed by the bone and bone marrow. The outcome is poor in patients with CNS tumors with ENM.
我们的目的是评估发生神经外转移（ENM）的原发性中枢神经系统（CNS）肿瘤患儿的临床特征和预后。我们回顾性评估了1972年至2004年在我院接受治疗的原发性CNS肿瘤患儿。在1011例这些肿瘤患者中，10例（0.98%）发生了ENM。组织病理学诊断为髓母细胞瘤6例，生殖细胞肿瘤2例，室管膜瘤和非典型畸胎样横纹肌样肿瘤（ATRT）各1例。6例患者的原发肿瘤位于后颅窝；ATRT患者的原发肿瘤位于幕上，2例生殖细胞肿瘤患者的原发肿瘤位于鞍区和鞍上区，室管膜瘤患者的原发肿瘤位于脊髓远端。2例患者在诊断时检测到ENM。其他患者在诊断后9至25个月发生ENM。转移部位包括骨、骨髓、肺、颈部淋巴结、肝和鼻窦。在发生ENM的10例患者中，8例在检测到ENM后0.27至16.2个月（中位数，2.60个月）死于疾病。1例生殖细胞瘤患者在ENM诊断后117.80个月存活且无疾病。1例生殖细胞肿瘤患者在ENM诊断后11.3个月带瘤存活。颅内肿瘤患儿发生转移至其他神经外部位极为罕见。在我们的系列研究中，ENM发生率为0.98%。肝和肺是最常见的转移部位，其次是骨和骨髓。发生ENM的CNS肿瘤患者预后较差。
