Yang Michael M H, Singhal Ash, Rassekh Shahrad Rod, Yip Stephen, Eydoux Patrice, Dunham Christopher
Department of Surgery, Division of Neurosurgery, Children's and Women's Health Centre of British Columbia and University of British Columbia, Vancouver, British Columbia.
J Neurosurg Pediatr. 2012 May;9(5):517-23. doi: 10.3171/2012.1.PEDS11326.
The authors describe an infant girl who, at 10 months of age, presented with a large right parietooccipital tumor causing increased intracranial pressure, mass effect, and midline shift. The tumor was completely resected, and the entirety of the histology was consistent with glioblastoma. She was subsequently placed on adjuvant high-dose chemotherapy consisting of carboplatin, vincristine, and temozolomide, according to Head Start III, Regimen C. Three months after the complete resection, tumor recurrence was noted on MR imaging, during the third cycle of chemotherapy, and biopsy revealed malignant astrocytoma. Given the recurrence and the patient's intolerance to chemotherapy, a palliative course was pursued. Unexpectedly, the patient was alive and had made significant developmental improvements 18 months into palliation. Subsequently, however, signs of increased intracranial pressure developed and imaging demonstrated a very large new tumor growth at the site of prior resection. The recurrence was again fully resected, but microscopy surprisingly revealed pleomorphic xanthoastrocytoma throughout. The clinicopathological and genetic features of this girl's unusual neoplasm are detailed and potential pathogenic hypotheses are explored in this report.
作者描述了一名10个月大的女婴，她因右侧顶枕叶巨大肿瘤导致颅内压升高、占位效应和中线移位。肿瘤被完全切除，组织学检查结果完全符合胶质母细胞瘤。随后，根据“Head Start III，方案C”，她接受了由卡铂、长春新碱和替莫唑胺组成的辅助大剂量化疗。在完全切除肿瘤三个月后，即化疗第三个周期期间，磁共振成像显示肿瘤复发，活检显示为恶性星形细胞瘤。鉴于肿瘤复发且患者对化疗不耐受，于是采取了姑息治疗方案。出乎意料的是，在姑息治疗18个月后，患者仍然存活且有显著的发育进步。然而，随后出现了颅内压升高的迹象，影像学检查显示在先前切除部位有一个非常大的新肿瘤生长。复发肿瘤再次被完全切除，但显微镜检查令人惊讶地发现整个肿瘤均为多形性黄色星形细胞瘤。本报告详细阐述了这名女童罕见肿瘤的临床病理和基因特征，并探讨了潜在的致病假说。
