Pleomorphic xanthoastrocytoma: Favorable outcome after complete surgical resection
Pleomorphic xanthoastrocytoma: Favorable outcome after complete surgical resection
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DOI：
10.1093/neuonc/3.3.184
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发表时间：
2001-07-01
期刊：
影响因子：
15.9
通讯作者：
Gajjar, A
中科院分区：
文献类型：
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作者：
Fouladi, M;Jenkins, J;Gajjar, A
为了描述多形性黄色星形细胞瘤（PXA）患者的临床特征、组织学特征和治疗，我们回顾了13例经组织学证实的PXA患儿的资料，这些患儿在1985年至1999年间被转诊至神经肿瘤科。CT和/或MRI的神经影像学记录了解剖位置、肿瘤范围和切除程度。有3名男性和10名女性;中位年龄为12.9岁（范围，8.2-17.2岁）。最常见的表现包括癫痫发作（n = 8）和头痛（n = 5）。肿瘤部位包括颞叶（n = 5）、顶叶（n = 3）、额叶（n = 1）、额顶叶（n = 1）、顶枕叶（n = 1）和颞顶叶（n = 1）以及脊髓（n = 1）。CT/MRI显示6例囊性成分，3例囊壁强化。实性成分均匀强化11例。9例患者出现血管源性水肿，4例患者出现钙化。组织学表现包括脑膜侵犯12例，钙化4例，坏死2例。在8例患者中观察到核分裂像（每个高倍视野1-12个）。大体全切除8例，近全切除1例，次全切除4例。10名患者存活，中位随访时间为41个月。2例患者死于疾病进展，1例死于无关的结论，多形性黄色星形细胞瘤是一种罕见的肿瘤在儿童，通常表现为癫痫发作。在我们的系列研究中，7例患者中有6例（85%）在没有辅助治疗的情况下进行了大体全切除，从而延长了疾病控制时间。
To describe the clinical features, histologic characteristics, and management of patients with pleomorphic xanthoastrocytoma (PXA), we reviewed data on 13 children who had histologically confirmed PXA and were referred to the neuro-oncology service between 1985 and 1999. Neuro-imaging with CT and/or MRI documented the anatomic location, tumor extent, and degree of resection. There were 3 males and 10 females; median age was 12.9 years (range, 8.2-17.2 years). The most frequent presentations included seizures (n = 8) and headache (n = 5). Tumor sites included temporal (n = 5), parietal (n = 3), frontal (n = 1), frontoparietal (n = 1), parietooccipital (n = 1), and temporoparietal (n = 1) lobes and the spinal cord (n = 1). CT/MRI revealed a cystic component in 6 patients, with cyst wall enhancement in 3 patients. The solid component was uniformly enhancing in 11 patients. Vasogenic edema was present in 9 patients, and calcification was noted in 4 patients. Histopathologic findings included meningeal invasion in 12 patients, calcifications in 4, and necrosis in 2. Mitotic figures (1-12 per high-power field) were seen in 8 patients. Gross total resection was achieved in 8 patients, near total resection in 1, and subtotal resection in 4. Ten patients were alive with a median follow-up of 41 months at this writing. Two patients died of progressive disease, and 1 died of an unrelated In conclusion, pleomorphic xanthoastrocytoma is a rare neoplasm in childhood, commonly presenting with seizures. Gross total resection without adjuvant therapy provides prolonged disease control, as seen in 6 of 7 patients (85%) in our series.