• Title/Summary/Keyword: von Hippel Lindau Disease

Search Result 15, Processing Time 0.021 seconds

Prognostic Factors of Spinal Intramedullary Hemangioblastoma : Analysis of Surgical Outcomes and Tumor Characteristics

  • Hyun-Jun Jang;Bong-Ju Moon;Kyung-Hyun Kim;Jeong-Yoon Park;Dong-Kyu Chin;Yong-Eun Cho;Keun-Su Kim
    • Journal of Korean Neurosurgical Society
    • /
    • v.67 no.6
    • /
    • pp.637-645
    • /
    • 2024
  • Objective : Spinal intramedullary hemangioblastoma is a rare and highly vascularized benign tumor. The characteristics of the tumor, its corresponding location, and surgical outcomes remain unknown. The purpose of this study was to identify risk factors and strategies for neurologic deterioration following hemangioblastoma surgery. Methods : A comprehensive retrospective analysis was undertaken to evaluate patients who underwent surgical intervention for intramedullary hemangioblastoma at our institution from 1993 to 2022. Patients with at least 1 year of follow-up data were included. The analysis covered patient demographics, pre- and post-operative modified McCormick scale (MMCS), tumor location, and tumor size. Results : This study included 25 cases. One-year after surgery, neurological deterioration was observed in five cases (20.0%), and neurological improvement was found in nine cases (36.0%). Five cases were ventrally located, and twelve cases were dorsally located. Ventrally located cases were larger in tumor axial size (p=0.029) than dorsal location tumors, resulting in poorer follow-up MMCS and a higher prevalence of von Hippel-Lindau syndrome (VHL) (p=0.042). Three of them were confirmed to be supplied by the anterior spinal artery. In the case of dorsally located cases, there was no neurologic deterioration. Conclusion : In intramedullary spinal cord hemangioblastomas, cases located ventrally had a higher incidence of neurological deterioration following surgery than those located dorsally or in intramedullary extramedullary cases. Ventrally located hemangioblastomas were larger than those in other locations. They were mainly supplied by the anterior spinal artery in VHL patients.

Sporadic Hemangioblastoma in the Pituitary Stalk : A Case Report and Review of the Literature

  • Lee, Gun-Ill;Kim, Jae-Min;Choi, Kyu-Sun;Kim, Choong-Hyun
    • Journal of Korean Neurosurgical Society
    • /
    • v.57 no.6
    • /
    • pp.465-468
    • /
    • 2015
  • Hemangioblastomas in the pituitary stalk are especially rare. Most pituitary stalk hemangioblastomas reported in the literature were associated with von Hippel-Lindau (VHL) disease. Here, we report only the 3rd case of sporadic pituitary stalk hemangioblastoma diagnosed in a 60-year-old woman. Despite the danger of potential complications due to excessive vascularity or proximity to important neural structures, the tumor in our case was successfully removed while preserving pituitary function. In this case, complete surgical excision was shown to be an effective treatment option for symptomatic pituitary stalk hemangioblastoma, and we suggest careful evaluation of any highly enhancing mass with a signal void in the pituitary stalk preoperatively, even if no VHL disease is evident.

Multiple Supra- and Infratentorial Hemangioblastomas : Case Report and Review of the Literature (천막상부 및 하부에 다발성으로 발생한 혈관모세포종 - 증례보고 -)

  • Kim, Cheol-Soo;Jung, Shin;Kang, Sam-Suk;Lee, Jung-Kil;Kim, Tae-Sun;Kim, Jae-Hyoo;Kim, Soo-Han;Lee, Je-Hyuk
    • Journal of Korean Neurosurgical Society
    • /
    • v.30 no.2
    • /
    • pp.217-220
    • /
    • 2001
  • A rare case of multiple supratentorial and infratentorial hemangioblastomas in a 50-year old man is presented. There were neither manifestations of visceral tumors nor familial history. The two tumors were totally removed in two sessions and the diagnosis in both tumors were hemangioblastomas.

  • PDF

Treatment Strategy of Multiple Hemangioblastomas

  • Kim, Eui-Hyun;Park, Yong-Sook;Chang, Jong-Hee;Chang, Jin-Woo;Park, Yong-Gou
    • Journal of Korean Neurosurgical Society
    • /
    • v.38 no.3
    • /
    • pp.184-189
    • /
    • 2005
  • Objective : Hemangioblastomas are highly vascular and benign neoplasm of the central nervous system[CNS]. They can often be found as multiple lesions, as is commonly observed in von Hippel-Lindau[VHL] disease. The aim of this study is to determine the proper management for multiple hemangioblastomas. Methods : Since 1990, 78cases of hemangioblastoma have been encountered. Among these, 9cases were multiple hemangioblastomas that were treated with surgical resection with or without radiosurgery. The medical, radiological, surgical and histological records were reviewed retrospectively and analyzed statistically. Results : Nine patients presented with multiple hemangioblastomas and were diagnosed as VHL disease. The mean follow-up duration was 75.7months [$6.6{\sim}159.2months$] after the first surgical treatment. Three patients were treated with surgical resection alone and six patients were treated by both surgical resection and radiosurgery. Twenty-one surgical procedures [13 surgical resections and 8 radiosurgery] were performed. One patient required ventriculoperitoneal shunt and a posterior fossa decompressive craniectomy because of post-radiation brain swelling. Another patient refused additional treatment for the newly developed lesions after the successful treatment of initial lesions. The other patient who presented with numerous lesions in the whole brain and spine underwent cranio-spinal irradiation. Remaining patients showed good results. Conclusion : The surgical outcomes for the patients with a single lesion of the CNS hemangioblastoma are favorable. However. the treatment of multiple hemangioblastoma is more difficult, and should be treated by surgical resection and radiosurgery with careful consideration.

Clinical Analysis of Spinal Cord Hemangioblastoma (척수 혈관모세포종의 임상 분석)

  • Lee, Dae Kyu;Choe, Woo Jin;Kim, Dong Yoon;Lee, Chul Hee;Chung, Chun Kee;Kim, Hyun Jib
    • Journal of Korean Neurosurgical Society
    • /
    • v.30 no.11
    • /
    • pp.1291-1299
    • /
    • 2001
  • Objectives : The authors present a retrospective analysis of 14 patients treated for spinal cord hemangioblastoma (SCH) between Dec. 1986 and Mar. 2000. This study was conducted to evaluate and compare the difference of the functional outcomes associated with the extent of surgical removal of SCH. Methods : Eleven patients were male and three patients were female. Their mean age was 37.2 years that ranged from 19 to 62 years. Preoperative magnetic resonance(MR) imaging of the spine was performed in all cases, and preoperative angiography in eleven cases. They were followed from 15 months to 161 months(median follow-up period, 47 months), and we investigated the change of neurological symptoms and functional outcomes with radiological features, especially on MR imaging. Results : Six patients were accompanied by von Hippel-Lindau disease, and three of them had multiple CNS tumors. Thirteen patients had intramedullary tumor, and the remaining one had extradural. Syringomyelia around the tumor was observed in ten cases. All patients underwent surgical removal, and gross total removal(GTR) was achieved in ten cases. Preoperative embolization was performed in four cases. In four patients who were treated with preoperative embolization, intraoperative loss of blood was minimal and GTR was possible. One patient developed a transient swallowing difficulty postoperatively without permanent postoperative neurological deficits. In three of four patients in whom GTR was not possible, their functional outcomes were worsened postoperatively. The functional status at discharge was improved in seven patients, stationary in four patients, and worse in three. At the last follow-up(15-161 months), one of four patients who had been stationary at discharge showed improvement but, the rest did not show any change. All patients who showed neurological improvement were patients with GTR, and the patients with GTR had significant better outcome than those without GTR(p=0.015). Conclusion : Surgical treatment, and especially, GTR is considered as treatment of choice for spinal cord hemangioblastoma. Preoperative embolization may prevent intraoperative bleeding and improve surgical outcome.

  • PDF