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首页> 外文期刊>Neurosurgery >Composite pleomorphic xanthoastrocytoma-ganglioglioma presenting as a suprasellar mass: case report.
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Composite pleomorphic xanthoastrocytoma-ganglioglioma presenting as a suprasellar mass: case report.

机译：复合性多形性黄体星形细胞瘤-神经胶质瘤表现为鞍上肿块：病例报告。

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摘要

OBJECTIVE AND IMPORTANCE: Composite pleomorphic xanthoastrocytoma (PXA)-ganglioglioma (GG) is a recently recognized, rare type of neoplasm that most commonly presents as a temporal seizure focus among male patients less than 30 years of age. This case represents the only reported suprasellar presentation, with the youngest reported age at diagnosis. CLINICAL PRESENTATION: We present the case of a 12-year-old boy with new-onset diplopia who was diagnosed as having a large suprasellar mass, which was presumptively designated a craniopharyngioma on the basis of its clinical and radiological appearance. INTERVENTION: Gross total resection of a well-encapsulated tumor with adjacent cysts was achieved, without postoperative neurological deficits. Frozen-section studies suggested pilocytic astrocytoma; however, the final histological diagnosis was composite PXA-GG. CONCLUSION: Composite PXA-GG, although extremely rare, may present as a pediatric suprasellar mass. The histopathological diagnosis is contingent on the demonstration of distinct coincident PXA and GG components. The prognosis after gross subtotal resection is likely favorable; however, long-term follow-up monitoring is indicated for these rare neoplasms.
机译：目的和重要性：复合型多形性黄体星形细胞瘤（PXA）-神经胶质瘤（GG）是一种最近公认的罕见肿瘤，在30岁以下的男性患者中最常表现为短暂性癫痫发作。该病例是唯一报告的鞍上表现，诊断时报告的年龄最小。临床表现：我们介绍了一个病例，该病例为一名12岁男孩，患有新发病的复视，被诊断为具有巨大的鞍上肿块，根据其临床和影像学表现，推测其被称为颅咽管瘤。干预：完全包涵完整的肿瘤并伴有相邻囊肿的全切除术，无术后神经功能缺损。冷冻切片研究提示毛细细胞星形细胞瘤。然而，最终的组织学诊断是复合PXA-GG。结论：复合物PXA-GG尽管极少见，但可能以小儿鞍上存在。组织病理学诊断取决于不同的PXA和GG成分的重合。大体次全切除术后的预后可能良好;然而，对于这些罕见肿瘤，需要进行长期随访监测。

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