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Background: Pediatric hemangioblastomas are rare, and the clinical features, timing of surgical intervention, optimal treatment, and clinical outcomes are still unclear. Methods: We performed a retrospective study of all patients with CNS hemangioblastomas who were treated at West China Hospital from January 2003 to March 2015. Patients under the age of 16 years were included in the study. The medical records of these patients were reviewed and statistically analyzed. Results: Twenty-five children (15 females and ten males, [mean age 12.6 ± 4.7 years, range 1-16 years]) presented with hemangioblastomas. Tumors were detected in the cerebellum, brainstem, and spinal cord in 40, 28, and 32% of patients, respectively. Sixteen children (64%) had VHL syndrome. The most frequent symptoms were those related to increased intracranial pressure. The mean duration of symptoms was 1.5 ± 2.1 months. Preoperative hydrocephalus was noted in 11 children (44%). Gross total resection was achieved in all children. Clinical symptoms improved in 19 children (76%), unchanged in four children (16%), and aggravated in two children (8%), respectively. The mean follow-up was 44.5 ± 32.3 months. Five patients (20%) experienced disease progression. Using univariate analysis, both tumor-associated cysts ( P = 0.027) and VHL disease ( P = 0.032) were significantly related to postoperative outcomes. Conclusions: Pediatric hemangioblastomas have many different clinical features compared with adult cases. A high degree of suspicion for VHL disease should be raised in pediatric hemangioblastomas. Despite many challenges involved, surgical outcomes for pediatric hemangioblastomas are favorable. Lifelong follow-up is mandatory to detect the disease progression. [ABSTRACT FROM AUTHOR]

Objective Hemangioblastomas with enhanced cyst walls represent a rare radiologic presentation of hemangioblastomas with poor understandings. We aimed to summarize the clinical and radiologic features, important differential diagnosis, surgical strategy, and clinical outcome of this rare entity. Methods From June 2008 to March 2017, 12 patients with cystic hemangioblastomas presenting with enhanced wall thickness on MRI were treated in our department. The clinical presentations, radiologic investigations, surgical treatment, neurologic outcome, and recurrence rate were evaluated. Important preoperative differential diagnosis and surgical strategy of this entity were discussed. Results Twelve patients with cystic hemangioblastomas presenting with an enhanced cyst wall on magnetic resonance imaging were analyzed retrospectively. There were 5 male and 7 female subjects, with a mean age of 41.4 years (range, 13–78 years) and an average duration of symptoms before diagnosis of 2.23 months (range, 0.5–8.0 months). Radiologically, enhancement of both tumoral nodule and cyst were observed in 8 patients, while pure ring-enhanced cyst without typical tumoral nodule was found in 4 patients. Histopathologic examination confirmed the diagnosis of hemangioblastomas, and the enhanced cyst wall and mural nodule shared the same histopathologic pattern. Postoperative complications occurred in only 1 patient with postoperative cerebellar hemorrhage. During follow-up, 8 patients achieved favorable neurologic outcomes (Karnofsky score: 100) without recurrence; however, 4 patients experienced local tumor recurrence after the initial surgery. Conclusions Hemangioblastomas with enhanced cyst wall possess distinctive radiologic features, and they are frequently misdiagnosed preoperatively. Favorable tumor control can be achieved only when gross total resection of both the tumor nodule and cyst wall are performed. Close follow-up is necessary because of the high recurrence rate in this subset of hemangioblastomas. [ABSTRACT FROM AUTHOR]

Objectives Hemangioblastomas in the cerebellopontine angle (CPA) are uncommon and have rarely been reported. They may be easily misdiagnosed because of the atypical location and clinical and imaging features. The present study aimed to characterize clinical and radiologic features, treatment strategies, and outcomes in these rare lesions and to investigate various factors that may affect postoperative outcomes. Methods The medical records of patients with CPA hemangioblastomas who underwent surgery from 2003–2016 at the West China Hospital were reviewed retrospectively and statistically analyzed. Results Twenty-three patients (14 males and 9 females) presented with CPA hemangioblastomas. Eight patients (34.8%) had von Hippel-Lindau (VHL) syndrome. Gross total resection was achieved in 22 patients (95.6%). The mean follow-up was 45.1 ± 36.2 months (range 3–144 months). After surgery, the symptoms improved in 18 cases (78.3%), remained unchanged in 3 cases (13%), and were aggravated in 2 cases (8.7%). Four patients showed local recurrence during follow-up (17.4%). Patients with cystic hemangioblastomas had a better neurologic improvement ( P = 0.041) compared with patients with solid tumors. Furthermore, patients with maximal diameter of tumors >3 cm ( P = 0.035) or solid tumors ( P = 0.018) showed a higher incidence of postoperative complications. The local recurrence was correlated with VHL disease ( P = 0.027). Conclusions Although hemangioblastomas of the CPA are challenging lesions to treat surgically, they can be removed safely when these lesions are appropriately diagnosed and treated. Patients with VHL disease are more likely to have a local recurrence. A regular follow-up is recommended to detect the local and distant recurrence, even if the clinical course is benign and the tumor is totally resected. [ABSTRACT FROM AUTHOR]

Purpose This study aimed to determine the expression of angiopoietin-1 (Ang-1), angiopoietin-2 (Ang-2), and endothelial tyrosine kinase receptor (Tie-2) in central nervous system (CNS) hemangioblastomas (HBs) and explore the correlation with peritumoral cyst formation. Method A total of 22 paraffin-embedded specimens derived from 11 cyst-associated and 11 solid solitary CNS HBs were collected for streptavidin-peroxidase immunohistochemical staining. The expression levels of Ang-1, Ang-2 and Tie-2 in cystic and solid HBs were estimated and compared according to the integrated optical density (IOD) of staining in the sections. To further confirm the differential expression of Ang-1 and Ang-2 between cystic and solid HBs, an additional 9 frozen specimens from 5 cyst-associated and 4 solid solitary HBs were collected for Western blot analysis. Clinical histories and radiological records of the patients were reviewed retrospectively. Results Compared to normal cerebellum tissue, Ang-1, Ang-2 and Tie-2 were prominently overexpressed in both the stromal cells and endothelial cells of CNS HBs. The expression of Ang-2 and the ratio of Ang-2/Ang-1 in the cystic group were significantly higher than those in the solid group. The intensity of Ang-2 expression in the cystic group was negatively correlated with age. There was no correlation between the expression level of the proteins and sex, tumor location, tumor volume or cyst volume. Conclusions Ang/Tie signaling is upregulated in HBs and is likely associated with the process of intense vascularization and cyst formation. Higher Ang-2 levels and a higher Ang-2/Ang-1 ratio may contribute to cyst formation by increasing the vascular permeability. The decrease in Ang-2 expression with advanced age in cystic-associated HBs may be associated with the reduced growth rate of peritumoral cysts among elderly patients. Ang-2 shows great potential as an effective therapeutic target of symptomatic CNS HBs associated with cysts. [ABSTRACT FROM AUTHOR]