Pediatric Spinal Hemangioblastomas: Clinical Features and Surgical Outcomes of 39 Cases
Objective Spinal hemangioblastomas (HBs) are a rare pathology, especially in the pediatric population. The natural history and long-term outcomes of pediatric patients with spinal HBs remain unclear due to their scarcity. Methods A retrospective review of the clinical data and treatment outcomes of...
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| Format: | Article |
| Language: | English |
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Korean Spinal Neurosurgery Society
2023-03-01
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| Series: | Neurospine |
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| Online Access: | http://e-neurospine.org/upload/pdf/ns-2244970-485.pdf |
| _version_ | 1827361552428171264 |
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| author | Bo Han Liang Zhang Wenqing Jia |
| author_facet | Bo Han Liang Zhang Wenqing Jia |
| author_sort | Bo Han |
| collection | DOAJ |
| description | Objective Spinal hemangioblastomas (HBs) are a rare pathology, especially in the pediatric population. The natural history and long-term outcomes of pediatric patients with spinal HBs remain unclear due to their scarcity. Methods A retrospective review of the clinical data and treatment outcomes of children with spinal HBs in our institution from 2012 to 2021 was conducted. Results Thirty-nine pediatric patients were included, with an average age of 15.9 ± 2.9 years (range, 8–18 years), and 51.3% were female. Children were more likely to have von Hippel-Lindau (VHL) disease (p < 0.001), a family history of VHL (p < 0.001), multiple symptoms (p = 0.006), a shorter duration of symptoms (p < 0.001), and a larger lesion size (p = 0.004) and volume (p = 0.008) than their adult counterparts. The VHL-associated group of patients was more likely to present with multiple symptoms (p = 0.026), have a family history of VHL (p < 0.001), have multiple HBs (p < 0.001) and have synchronous intracranial lesions (p < 0.001) than the sporadic group. After surgery, 15 patients (38.5%) showed improved clinical outcomes, 17 patients (43.6%) remained unchanged, 4 patients (10.2%) worsened, and 3 patients (7.7%) died of tumor progression. During follow-up, there was a high rate of recurrence and repeated surgery, especially for children in the VHL-associated group. Conclusion Pediatric patients with spinal HBs appear to have a higher relapse risk than their adult counterparts. Therefore, life-long follow-up of these patients is necessary, especially for VHL-associated cases. Surgery can benefit children with HBs and should be considered early to avoid irreversible neurological deterioration. |
| first_indexed | 2024-03-08T07:07:00Z |
| format | Article |
| id | doaj.art-af1a71418c3b4c0b915214114e90720d |
| institution | Directory Open Access Journal |
| issn | 2586-6583 2586-6591 |
| language | English |
| last_indexed | 2024-03-08T07:07:00Z |
| publishDate | 2023-03-01 |
| publisher | Korean Spinal Neurosurgery Society |
| record_format | Article |
| series | Neurospine |
| spelling | doaj.art-af1a71418c3b4c0b915214114e90720d2024-02-03T04:29:37ZengKorean Spinal Neurosurgery SocietyNeurospine2586-65832586-65912023-03-0120134335210.14245/ns.2244970.4851377Pediatric Spinal Hemangioblastomas: Clinical Features and Surgical Outcomes of 39 CasesBo Han0Liang Zhang1Wenqing Jia2 Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China National Cancer Center/National Clinical Research Center for Cancer/Hebei Cancer Hospital, Chinese Academy of Medical Sciences, Langfang, China Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, ChinaObjective Spinal hemangioblastomas (HBs) are a rare pathology, especially in the pediatric population. The natural history and long-term outcomes of pediatric patients with spinal HBs remain unclear due to their scarcity. Methods A retrospective review of the clinical data and treatment outcomes of children with spinal HBs in our institution from 2012 to 2021 was conducted. Results Thirty-nine pediatric patients were included, with an average age of 15.9 ± 2.9 years (range, 8–18 years), and 51.3% were female. Children were more likely to have von Hippel-Lindau (VHL) disease (p < 0.001), a family history of VHL (p < 0.001), multiple symptoms (p = 0.006), a shorter duration of symptoms (p < 0.001), and a larger lesion size (p = 0.004) and volume (p = 0.008) than their adult counterparts. The VHL-associated group of patients was more likely to present with multiple symptoms (p = 0.026), have a family history of VHL (p < 0.001), have multiple HBs (p < 0.001) and have synchronous intracranial lesions (p < 0.001) than the sporadic group. After surgery, 15 patients (38.5%) showed improved clinical outcomes, 17 patients (43.6%) remained unchanged, 4 patients (10.2%) worsened, and 3 patients (7.7%) died of tumor progression. During follow-up, there was a high rate of recurrence and repeated surgery, especially for children in the VHL-associated group. Conclusion Pediatric patients with spinal HBs appear to have a higher relapse risk than their adult counterparts. Therefore, life-long follow-up of these patients is necessary, especially for VHL-associated cases. Surgery can benefit children with HBs and should be considered early to avoid irreversible neurological deterioration.http://e-neurospine.org/upload/pdf/ns-2244970-485.pdfhemangioblastomapediatricsvon hippel-lindau diseasespinal cordnatural history |
| spellingShingle | Bo Han Liang Zhang Wenqing Jia Pediatric Spinal Hemangioblastomas: Clinical Features and Surgical Outcomes of 39 Cases Neurospine hemangioblastoma pediatrics von hippel-lindau disease spinal cord natural history |
| title | Pediatric Spinal Hemangioblastomas: Clinical Features and Surgical Outcomes of 39 Cases |
| title_full | Pediatric Spinal Hemangioblastomas: Clinical Features and Surgical Outcomes of 39 Cases |
| title_fullStr | Pediatric Spinal Hemangioblastomas: Clinical Features and Surgical Outcomes of 39 Cases |
| title_full_unstemmed | Pediatric Spinal Hemangioblastomas: Clinical Features and Surgical Outcomes of 39 Cases |
| title_short | Pediatric Spinal Hemangioblastomas: Clinical Features and Surgical Outcomes of 39 Cases |
| title_sort | pediatric spinal hemangioblastomas clinical features and surgical outcomes of 39 cases |
| topic | hemangioblastoma pediatrics von hippel-lindau disease spinal cord natural history |
| url | http://e-neurospine.org/upload/pdf/ns-2244970-485.pdf |
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