PDF(Pubmed)
Abstract：
BACKGROUND: Pineal region tumors have historically been challenging to treat. Advances in surgical techniques have led to significant changes in care and outcomes for these patients, and this is well demonstrated by our single institution\'s experience over a 17-year-period in which the evolution of diagnosis, treatment, and outcomes of pineal tumors in pediatric patients will be outlined.
METHODS: We retrospectively collected data on all pediatric patients with pineal region lesions treated with surgery at Children\'s National Hospital (CNH) from 2005 to 2021. Variables analyzed included presenting symptoms, presence of hydrocephalus, diagnostic and surgical approach, pathology, and adverse events, among others. IRB approval was obtained (IRB: STUDY00000009), and consent was waived due to minimal risk to patients included.
RESULTS: A total of 43 pediatric patients with pineal region tumors were treated during a 17-year period. Most tumors in our series were germinomas (n = 13, 29.5%) followed by pineoblastomas (n = 10, 22.7%). Twenty seven of the 43 patients (62.8%) in our series received a biopsy to establish diagnosis, and 44.4% went on to have surgery for resection. The most common open approach was posterior interhemispheric (PIH, transcallosal) - used for 59.3% of the patients. Gross total resection was achieved in 50%; recurrence occurred in 20.9% and mortality in 11% over a median follow-up of 47 months. Endoscopic third ventriculostomy (ETV) was employed to treat hydrocephalus in 26 of the 38 patients (68.4%) and was significantly more likely to be performed from 2011 to 2021. Most (73%) of the patients who received an ETV also underwent a concurrent endoscopic biopsy. No difference was found in recurrence rate or mortality in patients who underwent resection compared to those who did not, but complications were more frequent with resection. There was disagreement between frozen and final pathology in 18.4% of biopsies.
CONCLUSIONS: This series describes the evolution of surgical approaches and outcomes over a 17-year-period at a single institution. Complication rates were higher with open resection, reinforcing the safety of pursuing endoscopic biopsy as an initial approach. The most significant changes occurred in the preferential use of ETVs over ventriculoperitoneal shunts. Though there has been a significant evolution in our understanding of and treatment for these tumors, in our series, the outcomes for these patients have not significantly changed over that time.
METHODS: We retrospectively collected data on all pediatric patients with pineal region lesions treated with surgery at Children\'s National Hospital (CNH) from 2005 to 2021. Variables analyzed included presenting symptoms, presence of hydrocephalus, diagnostic and surgical approach, pathology, and adverse events, among others. IRB approval was obtained (IRB: STUDY00000009), and consent was waived due to minimal risk to patients included.
RESULTS: A total of 43 pediatric patients with pineal region tumors were treated during a 17-year period. Most tumors in our series were germinomas (n = 13, 29.5%) followed by pineoblastomas (n = 10, 22.7%). Twenty seven of the 43 patients (62.8%) in our series received a biopsy to establish diagnosis, and 44.4% went on to have surgery for resection. The most common open approach was posterior interhemispheric (PIH, transcallosal) - used for 59.3% of the patients. Gross total resection was achieved in 50%; recurrence occurred in 20.9% and mortality in 11% over a median follow-up of 47 months. Endoscopic third ventriculostomy (ETV) was employed to treat hydrocephalus in 26 of the 38 patients (68.4%) and was significantly more likely to be performed from 2011 to 2021. Most (73%) of the patients who received an ETV also underwent a concurrent endoscopic biopsy. No difference was found in recurrence rate or mortality in patients who underwent resection compared to those who did not, but complications were more frequent with resection. There was disagreement between frozen and final pathology in 18.4% of biopsies.
CONCLUSIONS: This series describes the evolution of surgical approaches and outcomes over a 17-year-period at a single institution. Complication rates were higher with open resection, reinforcing the safety of pursuing endoscopic biopsy as an initial approach. The most significant changes occurred in the preferential use of ETVs over ventriculoperitoneal shunts. Though there has been a significant evolution in our understanding of and treatment for these tumors, in our series, the outcomes for these patients have not significantly changed over that time.
摘要：
背景：松果体区肿瘤历来具有治疗挑战性。外科技术的进步导致这些患者的护理和结果发生了重大变化，我们的单一机构在17年的诊断演变期间的经验充分证明了这一点。治疗,和小儿患者松果体肿瘤的结果将被概述。
方法：我们回顾性收集了2005-2021年在儿童国家医院（CNH）接受手术治疗的所有松果体区病变患儿的数据。分析的变量包括出现症状，脑积水的存在，诊断和手术方法，病理学,和不良事件，在其他人中。获得IRB批准(IRB:STUDY00000009),由于纳入患者的风险最小,因此放弃了同意书.
结果：在17年期间共治疗了43例松果体区肿瘤患儿。我们系列中的大多数肿瘤是生殖细胞瘤（n=13，29.5％），其次是松果体母细胞瘤（n=10，22.7％）。我们系列的43例患者中有27例（62.8%）接受了活检以确定诊断，44.4%继续接受手术切除。最常见的开放入路是后半球（PIH，经call骨）-用于59.3％的患者。在47个月的中位随访时间内，总切除率为50％；复发率为20.9％，死亡率为11％。在38例患者中，有26例（68.4％）采用内窥镜第三脑室造口术（ETV）治疗脑积水，并且从2011年至2021年进行的可能性更大。接受ETV的大多数患者（73％）也接受了并发内窥镜活检。与未切除的患者相比，接受切除的患者的复发率或死亡率没有差异，但手术切除后并发症更常见。在18.4％的活检中，冷冻病理和最终病理之间存在分歧。
结论：本系列描述了单一机构在17年期间手术方法和结果的演变。开腹手术并发症发生率较高,加强内镜活检作为初始方法的安全性。最显着的变化发生在优先使用ETV而不是脑室腹膜分流器。尽管我们对这些肿瘤的理解和治疗有了重大的进展，在我们的系列中,这些患者的结局在这段时间内没有显著变化.
方法：我们回顾性收集了2005-2021年在儿童国家医院（CNH）接受手术治疗的所有松果体区病变患儿的数据。分析的变量包括出现症状，脑积水的存在，诊断和手术方法，病理学,和不良事件，在其他人中。获得IRB批准(IRB:STUDY00000009),由于纳入患者的风险最小,因此放弃了同意书.
结果：在17年期间共治疗了43例松果体区肿瘤患儿。我们系列中的大多数肿瘤是生殖细胞瘤（n=13，29.5％），其次是松果体母细胞瘤（n=10，22.7％）。我们系列的43例患者中有27例（62.8%）接受了活检以确定诊断，44.4%继续接受手术切除。最常见的开放入路是后半球（PIH，经call骨）-用于59.3％的患者。在47个月的中位随访时间内，总切除率为50％；复发率为20.9％，死亡率为11％。在38例患者中，有26例（68.4％）采用内窥镜第三脑室造口术（ETV）治疗脑积水，并且从2011年至2021年进行的可能性更大。接受ETV的大多数患者（73％）也接受了并发内窥镜活检。与未切除的患者相比，接受切除的患者的复发率或死亡率没有差异，但手术切除后并发症更常见。在18.4％的活检中，冷冻病理和最终病理之间存在分歧。
结论：本系列描述了单一机构在17年期间手术方法和结果的演变。开腹手术并发症发生率较高,加强内镜活检作为初始方法的安全性。最显着的变化发生在优先使用ETV而不是脑室腹膜分流器。尽管我们对这些肿瘤的理解和治疗有了重大的进展，在我们的系列中,这些患者的结局在这段时间内没有显著变化.
