小儿多形性黄细胞瘤伴肿瘤性脑膜炎:附细胞病理学证据的病例报告及文献综述

IF 0.3 Q4 SURGERY Indian Journal of Neurosurgery Pub Date : 2023-11-20 DOI:10.1055/s-0043-1774814
K. S. V. Karanth, Suchanda Bhattacharjee, Ramanadha Reddy, Megha Uppin, Indian J Neurosurg, MS Mch Vishwakumar Karanth
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引用次数: 0

摘要

摘要 Pleomorphic xanthoastrocytoma(PXA)被认为是二级肿瘤,预后良好。世界卫生组织中枢神经系统5级分类(WHO CNS5)将PXA归入环形星形胶质瘤,并根据组织学将其分为2级或3级。此类肿瘤很少出现脑脊液(CSF)和脑膜扩散。本病例报告描述了一名患有肿瘤性脑膜炎的年轻男性的 PXA 3 级肿瘤。这名 17 岁的男童有癫痫发作、颅内压升高和脑膜刺激症状。右额叶可见界限清楚、T2异质高强化病灶(5.5*4.8 厘米),伴轻度异质对比度增强和邻近脑膜增厚。患者接受了右额叶开颅手术和近全切术。术后采用脑脊液引流术治疗脑积水。组织病理学显示上皮样和横纹样形态,伴有明显的细胞多形性和不典型有丝分裂,符合PXA 3级。脑脊液细胞学检查显示,大量肿瘤细胞具有明显的核和细胞质多形性。PXA是一种罕见的儿童和青少年恶性肿瘤,常见于颞叶。BRAF 点突变 V600E 型在 2 级 PXA 中最为常见。脑膜播散在 PXA 中并不常见,出现脑膜播散则意味着预后不佳。PXA2级肿瘤可在大体全切除后进行连续成像随访。3级PXA肿瘤可通过最大限度的安全切除、放疗和/或化疗来治疗。伴有脑脊液扩散的 3 级 PXA 在临床过程中往往会迅速衰退,建议进行常规的基线和随访颅骨筛查,并需要积极的治疗。
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Pediatric Pleomorphic Xanthoastrocytoma with Neoplastic Meningitis: A Case Report with Cytopathological Evidence with Literature Review
Abstract Pleomorphic xanthoastrocytoma (PXA) was regarded as grade II tumor and considered to be associated with favorable outcome. The World Health Organization Central Nervous System 5 (WHO CNS5) has classified PXA under circumscribed astrocytic gliomas and graded 2 or 3 depending on histology. Cerebrospinal fluid (CSF) and leptomeningeal spread are observed rarely in these tumors. The present case report describes a PXA, grade 3 tumor in a young male with neoplastic meningitis. This 17-year-old male child presented with history of seizure, signs of raised intracranial pressure, and meningeal irritation. Well-defined, T2 heterogeneously hyperintense lesion (5.5*4.8 cm) was seen in right frontal lobe with mild heterogenous contrast enhancement and adjacent pachy-meningeal enhancement. Right frontal craniotomy and near total excision were done. Postoperative hydrocephalus was treated with CSF diversion. Histopathology showed epithelioid and rhabdoid morphology with significant cellular pleomorphism and atypical mitosis consistent with the PXA, grade 3. The CSF cytology showed numerous tumor cells with marked nuclear and cytoplasmic pleomorphism. PXA is a rare malignancy of children and young adults, commonly seen in the temporal lobes. BRAF point mutations of V600E type are most common in PXA, grade 2. Meningeal dissemination is uncommon in PXA and its presence marks poor outcome. PXA, grade 2 tumors could be followed with serial imaging following gross total resection. PXA, grade 3 tumors are managed with maximal-safe resection, radiotherapy, and/ or chemotherapy. PXA, grade 3 with CSF spread tends to have rapid decline in the clinical course and it is advisable to get routine baseline and follow-up craniospinal screening and needs aggressive management.
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CiteScore
0.40
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发文量
52
审稿时长
12 weeks
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