Department of Pathology, University of Kentucky, Lexington, Kentucky 40536, USA.
Neuropathology. 2011 Jun;31(3):286-91. doi: 10.1111/j.1440-1789.2010.01156.x. Epub 2010 Sep 29.
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.
小儿脑胶质瘤和胚胎性肿瘤的生物学行为可能变化多样。有少数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，推测是由辅助化疗和/或放疗引起的。在此，我们描述了一例先天性幕上高级别肿瘤，具有星形细胞特征，在近全切除术后未接受辅助治疗。13 年后，患者在原手术部位出现复发性肿瘤。与原发性肿瘤相比，复发性肿瘤的形态完全不同，有神经节细胞分化的证据，并且变化更类似于低度多形性黄色星形细胞瘤。据作者所知，这是首例未经治疗的高级别小儿肿瘤自发分化为低级别肿瘤的病例。简要讨论了这一现象的临床和生物学意义。
