Supratentorial leptomeningeal hemangioblastoma

A familial case of von Hippel-Lindau syndrome with a supratentorial leptomeningeal hemangioblastoma is reported. This is the first case in which the meningeal origin of a supratentorial hemangioblastoma has been histologically verified in von Hippel-Lindau syndrome.     

Supratentorial cystic hemangioblastoma with infratentorial extension—a unique location and a rare infant case

Introduction Supratentorial occurrence of hemangioblastoma is an exceedingly rare event. Even rarer is the occurrence of a supratentorial hemangioblastoma in infancy.Case report We hereby report the case of an 18-month-old girl who presented with irritability, increasing head size, and an open fontanelle. MR scans demonstrated triventricular hydrocephalus and a cystic mass within the left lateral ventricle. There was an 18×15×13 mm enhancing nodule along the medial aspect of the cyst. The cystic mass was mainly supratentorial but there was a caudal extension through the tentorial incisura that compressed the cerebellum. At surgery, the content of the cyst was xanthochromic. Enhancing medial nodule was extremely vascular and was extirpated totally. The pathological diagnosis was reticular variant of hemangioblastoma. Conclusion The child is well at 4 years of age. An extensive review of the English literature revealed only three such cases. All three cases survived the operation.     

Hemangioblastoma originating from the right cerebellopontine angle

The morbidity of hemangioblastoma in the supratentorial region is very low, and is seldom found in the area of the cerebellopontine angle, so it is easily misdiagnosis before surgery. We report and discuss a case of hemangioblastoma originating at the right cerebellopontine angle in a 42-year-old female patient.     

Neonatal tumors of the CNS: a report of 9 cases and a review

Neonatal central nervous system (CNS) tumors are an uncommon and histologically heterogeneous group of neoplasms with different clinical and biological features from those arising in childhood. We report 9 cases in which a diagnosis of CNS tumor was confirmed by biopsy or autopsy during the years 1982-1997 in the Vall d'Hebrón Children's Hospital, Barcelona. Two cases were fetal tumors detected by fetal sonography, 3 patients were symptomatic in the first days after birth and 4 patients presented initial clinical signs in the first weeks or months of life. Eight lesions were supratentorial and 1 was located in the spinal cord. According to histologic types, there were 2 glioneuronal tumors, 1 anaplastic astrocytoma, 1 choroid plexus carcinoma, 1 immature teratoma, 1 craniopharyngioma, 1 hemangioblastoma, 1 astroblastoma and 1 hemangioendothelioma. Extensive review of the literature indicates that our cases of hemangioblastoma, astroblastoma and hemangioendothelioma are exceptional and one more of the very rare and isolated previously published cases.     

Meningeal Supratentorial Hemangioblastoma in a Patient with Von Hippel-Lindau Disease Mimicking Angioblastic Menigioma

Hemangioblastomas are sporadic tumors found in the cerebellum or spinal cord. Supratentorial hemangioblastomas are rare, and those with meningeal involvement are extremely rare and have been reported in only approximately 130 patients. Here, we report the case of a 51-year-old female patient with supratentorial meningeal hemangioblastoma detected 5 years after surgical resection of an infratentorial hemangioblastoma associated with von Hippel-Lindau disease. Patients with von Hippel-Lindau syndrome are at risk for developing multiple hemangioblastomas, with new tumor formation and growth and possible meningeal infiltration. Regular lifelong follow-up in at-risk patients is recommended and should include the differential diagnosis of dural-based tumors such as angioblastic meningioma and metastatic renal cell carcinoma.     

Supratentorial hemangioblastomas in von Hippel–Lindau wild-type patients – case series and literature review

Background: Supratentorial hemangioblastoma is an uncommon lesion with few data available. Resemblance to other tumours represents a struggle concerning pre-operative diagnosis and management.

Objectives: The aim of this study was to review the current literature, integrating two new cases with uncommon features.

Methods: A search of English language peer-reviewed articles in PubMed®, Cochrane Library®, Google Scholar® and summary of the current knowledge.

Results: A total of 162 cases, with no predominance between von Hippel–Lindau disease and wild-type sporadic lesions. The frontal lobe is the most common topography, followed by the pituitary stalk, with image resemblance to a glioma or a metastasis. From these, 20 cases revealed dural attachment, mimicking a meningioma. Symptoms are due to mass effect or epilepsy, with low haemorrhagic risk. Clinical outcome on supratentorial hemangioblastoma depends on resection extension, with no recognised complementary treatment. Post-operative follow-up is essential, even in wild-type, sporadic cases, concerning recent reports of tumour dissemination.

Conclusions: This review compiles the main characteristics of supratentorial hemangioblastoma, that despite its rarity, should be a concern on differential diagnosis, treatment planning and expected prognosis.     

Hemangioblastoma of the cauda equina

A very rare hemangioblastoma of the filum terminale with sciatic pain and partial cauda syndrome in a 36-year-old man is presented. The difference between solitary hemangioblastoma, hemangioblastomatosis and von Hippel-Lindau disease is stressed. The literature is reviewed and the diagnosis of cauda equina neoplasms is discussed. Even nowadays often the final diagnosis is made late and only after operation and microscopical tissue examination.     

家族性同一部位血管母细胞瘤的诊断治疗(附两个家系报告)

目的 探讨家族性同一部位血管母细胞瘤(HB)的诊断和治疗.方法 回顾分析两个家系2例小脑HB、3例脑干HB的临床资料和手术疗效,结合文献进行讨论.结果 两个家系均确诊为希佩尔-林道病(VHL).5例肿瘤全部切除,随访6-48个月,5例KPS评分均≥90分.结论 HB诊断主要依靠MRI,基因检测对VHL病的诊断有重要价值.显微手术技巧可显著提高本病的疗效;术后控制正常灌注压突破综合征、及时发现和处理相关并发症对本病的预后有重要意义.

Abstract：

Objective To study the diagnosis and treatment of familical hemangioblastoma with the same parts of central nervous system.Methods The clinical presentation, surgical treatment and outcomes in two families of two cerebellar hemangioblastoma and three hemangioblastoma of brainstem were analyzed retrospectively and literature review was performed.Results Family A was suspected to be VHL, family B was confirmed to be VHL.Five tumors were removed completely.The KPS of all patients were more than 90 during follow - up period ( from 6 months to 48 months ).Conclusion MRI and DSA may be the most important methods for the diagnosis of HBs.Genetic testing bears great value in the diagnosis of VHL.Microsurgical technique play a key role in the curative effect of hemangioblastoma.NPPB could be controlled in the perioperative period and management of complications is important for prognosis.     

A report of supratentorial leptomeningeal hemangioblastoma and a literature review

Hemangioblastomas of the CNS are solid or cystic vascular‐rich tumors, most common in the cerebellum, less frequent in the brainstem or spinal cord, and rare in supratentorial locations with meningeal involvement. We document a case in a 58‐year‐old man who presented with about 2 months history of motor weakness and speech dysfunction. The tumor was a heterogeneously enhanced dural‐based tumor with high vascularity and perifocal edema in the left frontal lobe. The tumor was completely removed followed by embolization and preoperative radiotherapy. Histologic examination revealed a hemangioblastoma with features resembling angiomatous meningioma. Immunohistochemistry for epithelial membrane antigen (EMA) and S100 may be helpful to make differential diagnosis. Electron microscopic investigation is essential to differentiate between meningiomas and other leptomeningeal tumors.     

侧脑室实性血管母细胞瘤1例并文献复习

目的 探讨侧脑室实性血管母细胞瘤(HBS)的临床特点与治疗方法 .方法 回顾分析1例目前国内外年龄最小的侧脑室实性血管母细胞瘤患者的临床资料;并复习相关文献.结果 患者为女性,15岁;表现间断性头痛3个月,头痛加重伴发热1周;查体无异常神经系统体征.头颅MRI检查示,右侧脑室体部不规则形实性混杂信号影,增强扫描病灶呈明...     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.