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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Low-grade recurrence of a congenital high-grade supratentorial tumor with astrocytic features in the absence of adjuvant therapy.
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Low-grade recurrence of a congenital high-grade supratentorial tumor with astrocytic features in the absence of adjuvant therapy.

机译：在没有辅助治疗的情况下，具有星形细胞特征的先天性高级幕上肿瘤的低度复发。

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摘要

The biological behavior of pediatric gliomas and embryonal tumors can be highly variable. A few case reports have described differentiation of primitive neuroectodermal tumors (PNETs) and medulloblastomas, presumably induced by adjuvant chemotherapy and/or radiation. Herein we describe a case of a congenital supratentorial high-grade tumor with astrocytic features that, after near-total surgical resection, was not treated with adjuvant therapies. Thirteen years later the patient presented with recurrent tumor at the original surgical site. The recurrent tumor had completely different morphology compared to the original, with evidence of ganglion cell differentiation and changes more reminiscent of a low-grade pleomorphic xanthoastrocytoma. To the authors' knowledge, this is the first documented case of an untreated high-grade pediatric tumor that spontaneously differentiated into a low grade tumor. The clinical and biological implications of this are briefly discussed.
机译：小儿神经胶质瘤和胚胎肿瘤的生物学行为可能高度可变。一些病例报告描述了原始神经外胚层肿瘤（PNET）和髓母细胞瘤的分化，大概是辅助化疗和/或放射诱导的。在这里，我们描述了一种具有星形细胞特征的先天性幕上高度恶性肿瘤，在几乎全部手术切除后，并未接受辅助治疗。十三年后，患者在原始手术部位出现了复发性肿瘤。复发的肿瘤与原始肿瘤的形态完全不同，有神经节细胞分化和变化的证据，更让人联想到低度的多形性黄体星形细胞瘤。据作者所知，这是未经治疗的高等级小儿肿瘤自发分化为低等级肿瘤的首例文献报道。对此进行了临床和生物学意义的简要讨论。

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