Jennings M T, Slatkin N, D'Angelo M, Ketonen L, Johnson M D, Rosenblum M, Creasy J, Tulipan N, Walker R
Department of Neurology, Vanderbilt University Medical Center, Nashville, TN 37212-3375.
J Child Neurol. 1993 Oct;8(4):306-12. doi: 10.1177/088307389300800403.
Seven children and young adults initially presented with subacute meningitis and/or increased intracranial pressure. The diagnosis of neoplastic meningitis secondary to a primitive neuroectodermal neoplasm was delayed by the absence of an obvious primary tumor. The neuroradiologic appearance was that of a basimeningeal infiltrative process, complicated by communicating hydrocephalus or "pseudotumor cerebri." Myelography was important in the diagnosis of disseminated meningeal malignancy in four cases. Cerebrospinal fluid cytologic diagnosis was insensitive but ultimately confirmed in five cases. All seven patients experienced progressive disease despite neuraxis radiotherapy and intensive chemotherapy; six have died. Systemic dissemination to bone and/or peritoneum occurred in three patients while on therapy. In two, a primary parenchymal brain or spinal cord tumor could not be identified at postmortem examination. The presentation of a primitive neuroectodermal tumor as subacute meningitis without an evident primary tumor heralds an aggressive and refractory neoplasm.
7名儿童和青年最初表现为亚急性脑膜炎和/或颅内压升高。由于缺乏明显的原发性肿瘤，继发于原始神经外胚层肿瘤的肿瘤性脑膜炎的诊断被延迟。神经放射学表现为基底脑膜浸润性病变，并伴有交通性脑积水或“假性脑瘤”。脊髓造影对4例播散性脑膜恶性肿瘤的诊断很重要。脑脊液细胞学诊断不敏感，但最终在5例中得到证实。尽管进行了全脑脊髓放疗和强化化疗，所有7例患者病情仍进展；6例死亡。3例患者在治疗期间出现骨和/或腹膜的全身播散。2例患者在尸检时未发现原发性脑实质或脊髓肿瘤。原始神经外胚层肿瘤表现为亚急性脑膜炎且无明显原发性肿瘤，预示着肿瘤具有侵袭性且难治。
