PDF(Pubmed)
Abstract：
BACKGROUND: Dandy-Walker complex and pleomorphic xanthoastrocytomas are both rare disease entities that typically manifest early in life and are associated with congenital etiological factors. Dandy-Walker complex is a cerebellar malformation associated with a series of anatomical changes. The disease onset is usually at birth or during infancy. Late onset in adulthood is uncommon. Pleomorphic xanthoastrocytoma is a rare WHO grade II astrocytic tumor affecting mainly young adults. Concomitant occurrence of Dandy-Walker complex and pleomorphic xanthoastrocytoma has not been previously reported.
METHODS: A 30-year-old woman with a previous history of unconfirmed resected lateral ventricle meningioma presented with severe headache for 1 day. Imaging examination revealed a mass in the right lateral ventricle with heterogeneous signal patterns, changes in the posterior fossa corresponding to a Dandy-Walker variant, and mild hydrocephalus.
RESULTS: Surgical complete resection of the mass was achieved. postoperative histopathological examination confirmed WHO grade II pleomorphic xanthoastrocytoma. Three years postsurgery, ventriculoperitoneal shunt was performed due to worsening of hydrocephalus. The patient has since remained symptom-free.
CONCLUSIONS: This is the first report of concomitant occurrence of Dandy-Walker complex and pleomorphic xanthoastrocytoma. The association of neurological congenital malformation with intracranial neoplasms may be multifactorial, with underlying role of genetic mutations or chromosome alterations.
METHODS: A 30-year-old woman with a previous history of unconfirmed resected lateral ventricle meningioma presented with severe headache for 1 day. Imaging examination revealed a mass in the right lateral ventricle with heterogeneous signal patterns, changes in the posterior fossa corresponding to a Dandy-Walker variant, and mild hydrocephalus.
RESULTS: Surgical complete resection of the mass was achieved. postoperative histopathological examination confirmed WHO grade II pleomorphic xanthoastrocytoma. Three years postsurgery, ventriculoperitoneal shunt was performed due to worsening of hydrocephalus. The patient has since remained symptom-free.
CONCLUSIONS: This is the first report of concomitant occurrence of Dandy-Walker complex and pleomorphic xanthoastrocytoma. The association of neurological congenital malformation with intracranial neoplasms may be multifactorial, with underlying role of genetic mutations or chromosome alterations.
摘要：
背景：Dandy-Walker复合体和多形性黄色星形细胞瘤都是罕见的疾病实体，通常在生命早期表现，并与先天性病因相关。Dandy-Walker复合体是与一系列解剖学变化相关的小脑畸形。该疾病通常在出生时或婴儿期发病。成年后期发病并不常见。多形性黄色星形细胞瘤是一种罕见的WHOII级星形细胞瘤，主要影响年轻人。先前尚未报道过同时发生Dandy-Walker复合物和多形性黄色星形细胞瘤。
方法：一名30岁女性，既往有未经证实的切除侧脑室脑膜瘤病史，表现为严重头痛1天。影像学检查显示右侧脑室有肿块，信号模式不均匀，对应于Dandy-Walker变体的后窝变化，和轻度脑积水.
结果：手术完全切除了肿块。术后组织病理学检查证实WHOII级多形性黄色星形细胞瘤。手术三年后,由于脑积水恶化，进行了脑室-腹腔分流术。此后，患者一直没有症状。
结论：这是首次报道同时发生Dandy-Walker复合体和多形性黄色星形细胞瘤。神经系统先天性畸形与颅内肿瘤的关系可能是多因素的，具有基因突变或染色体改变的潜在作用。
方法：一名30岁女性，既往有未经证实的切除侧脑室脑膜瘤病史，表现为严重头痛1天。影像学检查显示右侧脑室有肿块，信号模式不均匀，对应于Dandy-Walker变体的后窝变化，和轻度脑积水.
结果：手术完全切除了肿块。术后组织病理学检查证实WHOII级多形性黄色星形细胞瘤。手术三年后,由于脑积水恶化，进行了脑室-腹腔分流术。此后，患者一直没有症状。
结论：这是首次报道同时发生Dandy-Walker复合体和多形性黄色星形细胞瘤。神经系统先天性畸形与颅内肿瘤的关系可能是多因素的，具有基因突变或染色体改变的潜在作用。
