Therapeutic outcomes of transsphenoidal surgery in pediatric patients with craniopharyngiomas: a single-center study
Therapeutic outcomes of transsphenoidal surgery in pediatric patients with craniopharyngiomas: a single-center study
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DOI：
10.3171/2017.10.peds17254
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发表时间：
2018-06-01
影响因子：
1.9
通讯作者：
Ito, Junko
中科院分区：
文献类型：
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作者：
Yamada, Shozo;Fukuhara, Noriaki;Ito, Junko
目的分析儿童颅咽管瘤经蝶窦手术（TSS）的临床疗效。方法回顾性分析65例儿童颅咽管瘤经蝶窦手术治疗的临床资料（28名女孩和37名男孩，平均年龄9.6岁）在1990年至2015年期间接受TSS治疗（45次初次手术和20次重复手术）。肿瘤被分类为膈下或膈上。对初次手术组和再次手术组的人口统计学和临床特征，包括切除范围、并发症、复发率、术前和术后视力障碍、垂体功能、尿崩症（DI）发生率以及新发肥胖进行分析和比较。26例（58%）位于膈下，19例位于膈上。在再次手术组的20例患者中，9例（45%）患有脑膜下肿瘤，11例患有膈上肿瘤。两个手术组之间唯一具有统计学意义的差异是肿瘤大小;初次手术组的肿瘤（平均最大直径30 mm）大于重复手术组（25 mm）（p = 0.008）。65例病例中有59例（91%）完成了GTR;初次手术组的GTR率高于再次手术组（98% vs 75%，p = 0.009）。在接受GTR的患者中，12%的患者发生了肿瘤复发，中位随访时间为7.8年，初次手术患者的复发频率低于重复手术患者（7% vs 27%，p = 0.06）。在45例初次手术患者中，80%的垂体功能恶化，83%发生DI，而100%的重复手术患者发生这些情况。在术前视力障碍的患者中，62%的患者视力改善，但11%的患者视力恶化。初次手术患者的视力改善率高于再次手术患者（71% vs 47%，p < 0.001），而初次手术后视力恶化率低于再次手术患者（4% vs 24%，p = 0.04）。在57例术前无肥胖的患者中，尽管进行了侵袭性切除术，但在9%的初次手术患者和21%的重复手术患者中发现了新发术后肥胖（p = 0.34），这表明在该系列中，下丘脑功能障碍很少与TSS的GTR相关。然而，由于既往经颅手术，25%的再次手术患者术前存在肥胖。虽然没有围手术期死亡，但12例（18%）出现并发症（6例CSF泄漏，3例脑膜炎，2例短暂性记忆障碍和1例脑积水）。术后脑脊液漏似乎是更常见的重复比在初次手术的患者（20%比4.4%，p = 0.2）。结论TSS的结果，儿童颅咽管瘤在这种情况下，一系列建议，GTR应该是第一次手术尝试的目标。GTR应该是可以实现的，没有严重的并发症，虽然大多数患者需要术后激素替代。当GTR不可能或GTR后发生肿瘤复发时，建议进行放射外科手术以防止肿瘤再生长或进展。
OBJECTIVE The aim of this study was to analyze the outcomes of transsphenoidal surgery (TSS) in a single-center clinical series of pediatric craniopharyngioma patients treated with gross-total resection (GTR).METHODS The authors retrospectively reviewed the surgical outcomes for 65 consecutive patients with childhood craniopharyngiomas (28 girls and 37 boys, mean age 9.6 years) treated with TSS (45 primary and 20 repeat surgeries) between 1990 and 2015. Tumors were classified as subdiaphragmatic or supradiaphragmatic. Demographic and clinical characteristics, including extent of resection, complications, incidence of recurrence, pre- and postoperative visual disturbance, pituitary function, and incidence of diabetes insipidus (DI), as well as new-onset obesity, were analyzed and compared between the primary surgery and repeat surgery groups.RESULTS Of the 45 patients in the primary surgery group, 26 (58%) had subdiaphragmatic tumors and 19 had supradiaphragmatic tumors. Of the 20 patients in the repeat surgery group, 9 (45%) had subdiaphragmatic tumors and 11 had supradiaphragmatic tumors. The only statistically significant difference between the 2 surgical groups was in tumor size; tumors were larger (mean maximum diameter 30 mm) in the primary surgery group than in the repeat surgery group (25 mm) (p = 0.008). GTR was accomplished in 59 (91%) of the 65 cases; the GTR rate was higher in the primary surgery group than in the repeat surgery group (98% vs 75%, p = 0.009). Among the patients who underwent GTR, 12% experienced tumor recurrence, with a median follow-up of 7.8 years, and recurrence tended to occur less frequently in primary than in repeat surgery patients (7% vs 27%, p = 0.06). Of the 45 primary surgery patients, 80% had deteriorated pituitary function and 83% developed DI, whereas 100% of the repeat surgery patients developed these conditions. Among patients with preoperative visual disturbance, vision improved in 62% but worsened in 11%. Visual improvement was more frequent in primary than in repeat surgery patients (71% vs 47%, p < 0.001), whereas visual deterioration was less frequent following primary surgery than repeat surgery (4% vs 24%, p = 0.04). Among the 57 patients without preoperative obesity, new-onset postoperative obesity was found in 9% of primary surgery patients and 21% of repeat surgery patients (p = 0.34) despite aggressive resection, suggesting that hypothalamic dysfunction was rarely associated with GTR by TSS in this series. However, obesity was found in 25% of the repeat surgery patients preoperatively due to prior transcranial surgery. Although there were no perioperative deaths, there were complications in 12 cases (18%) (6 cases of CSF leaks, 3 cases of meningitis, 2 cases of transient memory disturbance, and 1 case of hydrocephalus). Postoperative CSF leakage appeared to be more common in repeat than in primary surgery patients (20% vs 4.4%, p = 0.2).CONCLUSIONS The results of TSS for pediatric craniopharyngioma in this case series suggest that GTR should be the goal for the first surgical attempt. GTR should be achievable without serious complications, although most patients require postoperative hormonal replacement. When GTR is not possible or tumor recurrence occurs after GTR, radiosurgery is recommended to prevent tumor regrowth or progression.