颅咽管瘤的手术治疗和复发因素分析

目的探讨颅咽管瘤的手术策略和治疗效果以及影响肿瘤复发的因素。方法回顾性分析我经手术治疗的375例颅咽管瘤病人的临床资料,分析和评价不同类型的颅咽管瘤的手术方式、治疗效果和复发情况。结果 375例颅咽管瘤病人中,鞍上型218例,鞍内型43例,三脑室内114例。手术入路选择:经典开颅翼点或额下入路288例,经鼻蝶入路46例,经纵裂胼胝体前入路23例,经皮层脑室入路17例,联合入路1例。经术中观察和术后影像学证实全切272例,次全切103例。术后病人症状改善356例,效果不佳19例,其中死亡5例。结论颅咽管瘤应根据生长方式和特点选择个体化的手术治疗方式。在保护垂体-下丘脑功能基础上的肿瘤的全切仍然是降低复发获得良好预后的关键。     

110例颅咽管瘤手术经验

目的 探讨手术全切除颅咽管瘤的有效方法。方法 110例颅咽管瘤患者,男性62例,女性58例。年龄15．0－67．0岁,平均34．4岁。其中102例患者取翼点入路,5例经额下入 路,3例经纵裂从胼胝体至穹窿间隙和第三脑室前部切除肿瘤。术中根据肿瘤位置,从视交叉前间隙、视神经外侧间隙和颈内动脉外侧间隙切除肿瘤。术中注意保护下丘脑神经结构和防止穿通动脉的 损伤。结果 101例患者达到肿瘤全切除,6例次全切除,3例大部切除。术中垂体柄保留57例,断裂29例,未见垂体柄24例。术后54例患者有多饮多尿,52例3个月内恢复,2例持续1年以上;52例患者有血钠紊乱,均在3个月内恢复;3例术后视力减退加重;3例动眼神经麻痹,其中1例1年以上仍无恢复,2例暂短性肢体瘫;4例死亡。术后随访1个月－5．0年,平均1．1年。101例肿瘤全切除患者,有4例复发。本组患者术后99例能参加日常工作,5例生活自理,2例需要生活照顾。结论 选择合适的手术入路,保护下丘脑结构和功能,是颅咽管瘤全切除并获得良好效果的关键。     

经眶-额-颞入路显微手术切除颅咽管瘤

目的 总结经眶-额-颞入路显微手术切除颅咽管瘤的经验,探索肿瘤全切除的手术技巧。方法 采用经眶-额-颞入路显微手术切除颅咽管瘤18例。肿瘤位于鞍上7例、鞍上-第三脑室6例、鞍上-鞍内4例、第三脑室1例。12例肿瘤有钙化、13例囊性变、5例完全实质性。术中根据肿瘤的生长方向,分别从不同的间隙切除肿瘤。结果 肿瘤全切除14例,次全切除2例,部分切除2例。术后17例患者获随访,随访时间8～41个月。肿瘤全切除的14例未见肿瘤复发;次全切除和部分切除的3例于术后1个月内行辅助放疗,在随访期间肿瘤未见明显增大。Karnofsky预后评分(KPS)80～90分12例,60～70分5例,50分1例,无一例死亡。结论 经眶-额-颞入路能清楚地显露鞍区颅咽管瘤及其周围结构,减少对脑组织的牵拉和无效脑暴露。显微手术技巧是安全切除肿瘤、避免穿通动脉损伤和保护下丘脑功能的重要保障,也是手术成败的关键。     

经蝶窦入路显微手术切除小儿颅咽管瘤

目的 探讨经蝶窦入路切除小儿颅咽管瘤的手术技巧及适应证。方法 10例接受了11次经蝶窦手术,10次采用唇下－鼻中隔－蝶窦入路,1次采用鼻外侧－筛窦－蝶窦入路,在X线透视监测及显微放大10－15倍下切除肿瘤,对未能全切除病例术后加后放疗1个疗程。结果 无死亡、 无严重并发症,全切除4例,次全切除4例,部分切除2例,其中1例残瘤病例再次经蝶窦手术获得全切除。随访3个月－9年,肿瘤消失5例,残瘤静止4例,1例复发。结论 小儿颅咽管瘤局限于鞍内或伴蝶窦扩大的鞍内 －鞍上型可经蝶窦手术,但钙化明显的肿瘤经该入路难以获得令人满意的切除。     

经胼胝体-穹窿间入路显微手术切除第三脑室肿瘤

目的探讨经胼胝体-穹窿间入路显微手术切除第三脑室肿瘤的方法，并对相应的显微解剖学基础加以讨论。方法经胼胝体-穹窿间入路显微手术切除第三脑室肿瘤12例，其中突入第三脑室颅咽管瘤8例，丘脑内侧型胶质瘤2型，室管膜瘤1例，畸胎瘤1例。结论10例在手术显微镜下肿瘤全切，2例次全切除，全切除率83．3％，其中8例颅咽管瘤中7例全切，术后复查头颅MR未见肿瘤残留及复发。无死亡病例，近期并发症主要为多饮、多尿、电解质紊乱。经处理后1-2周消失。远期并发症为2例脑积水，其中1例半年后行脑室腹腔分流，2例硬膜下积液，3月后自行吸收。全组病例随访最短2个月，最长2年半，1例颅咽管瘤大部切除8月后复发，1例丘脑胶质瘤术后1年复发，其余10例中9例生活正常，1例生活自理。结论经胼胝体-穹窿间入路显微手术切除第三脑室肿瘤系通过胚胎组织残留的组织间隙进入第三脑室，几乎不损伤正常脑组织，手术视野大，直视下操作，可切除第三脑室前、中、后各部位肿瘤，肿瘤全切除率高，并发症少。     

内镜辅助眶上锁孔入路切除颅咽管瘤手术方法及术后护理对策

目的:总结内镜辅助眶上锁孔入路显微手术切除颅咽管瘤方法及术后护理对策.方法:采用眶上锁孔入路,内镜辅助显微技术切除颅咽管瘤11例.其中鞍内-鞍上型2例,鞍上-视定叉周围-脑室外型8例,脑室内-外型1例.结果:术后复查MRI,肿瘤全切除8例,次全切除3例,残余瘤体给予放疗,无手术死亡.随访3个月-2.3年,肿瘤复发1例.颅咽管瘤术前原有症状均有明显不同程度的好转.结论:内镜辅助眶上锁孔入路能清楚显露颅咽管瘤与周围结构的关系,提高了显微手术的精细度与安全性,及时有效的术后护理,提高了疾病的治愈率,防止并发症的发生.     

颅咽管瘤显微外科手术62例分析

目的 探讨颅咽管瘤显微外科手术的临床效果. 方法 应用显微外科手术切除颅咽管瘤62例,其中翼点入路19例、额下入路25例、经胼胝体入路11例、经蝶入路7例,根据临床、影像学术后随访结果分析临床效果.结果 手术全切除37例,垂体柄完全保留19例,肿瘤复发1例,死亡1例;手术部分切除25例,垂体柄完全保留24例,肿瘤复发5例,死亡1例. 结论 应用显微外科手术,选择合理的手术入路,可以改善肿瘤全切除率、垂体柄的保留率,减少复发率以及并发症的发生.     

经蝶入路显微外科切除颅咽管瘤适应证选择与操作要点

有关颅咽管瘤的手术一直是神经外科医师最感困惑的问题。本文报道经蝶显微手术切除颅咽管瘤18例,均经CT或MRI扫描确诊。本入路适用起源于鞍底或向鞍上扩展的肿物。手术采取经唇下-鼻中隔-蝶窦入路或经鼻前庭-鼻中隔-蝶窦入路两种方式行肿瘤切除术。9例肿瘤获得全切除,4例次全切除,其余5例为部分切除,术后无死亡。15例获长期(平均3.1年)随访,有12例(80.0％)恢复良好,3例影像学检查提示肿瘤复发,需行再次手术,放疗或放射外科治疗。文中对颅咽管瘤手术适应证选择及操作要点进行了讨论。     

眶上经眉弓锁孔入路显微手术治疗鞍区肿瘤

目的探讨经眉弓锁孔入路显微手术切除鞍区肿瘤的技术. 方法 2001年7月～2004年6月我院采用经眉弓2.0 cm×3.0 cm游离小骨窗锁孔入路,开放鞍区脑池,显露深部结构,切除鞍区肿瘤33例. 结果 26例垂体瘤全切除19例,次全切除7例;4例颅咽管瘤全切除3例,1例次全切除;2例脑膜瘤全切除;1例视交叉胶质瘤大部分切除.术后5例出现一过性尿崩症,1例发生癫痫大发作,无出血、感染等术后并发症.33例随访4～36个月,平均27个月,全切24例肿瘤无复发,7例垂体瘤、1例颅咽管瘤、1例视交叉胶质瘤术后放疗肿瘤未见增大. 结论经眉弓锁孔入路对脑组织无效暴露少,创伤小,术后并发症少,提高了手术安全性.     

经蝶入路显微外科切除颅咽管瘤适应证选择与操作要点

有关颅咽管瘤的手术一直是神经外科医师最感困惑的问题。本报道经蝶显微手术切除颅咽管瘤18例，均经CT或MRI扫描确认。本入路适用起源于鞍底或向鞍上扩展的肿物。手术采取经唇下-鼻中隔-蝶窦入路或经鼻前庭-鼻中隔-蝶窦入路两种方式行肿瘤切除术。9例肿瘤获得全切除，4例次全切除，其余5例为部分切除，术后无死亡。15例获长期（平均3.1年）随访，有12例（80.0%）恢复良好，3例影像学检查提示肿瘤复发，需行再行手术，放疗或放射外科治疗。中对颅咽管瘤手术适应证选择及操作要点进行了讨论。     

Extended transsphenoidal surgery for suprasellar craniopharyngiomas: infrachiasmatic radical resection combined with or without a suprachiasmatic trans-lamina terminalis approach

BACKGROUND: An extended transsphenoidal approach allowed for direct midline exposure of the parasellar structures such as the hypothalamic-pituitary axis and the third ventricle. To evaluate the capability of this approach for removal of suprasellar craniopharyngiomas, surgical outcomes were retrospectively analyzed. METHODS: During a 9-year period, 20 consecutive patients with suprasellar craniopharyngioma underwent transsphenoidal tumor resection. The average follow-up period was 55 months. No patient had a purely intrasellar tumor, 9 had prechiasmatic tumors, 9 had retrochiasmatic tumors, and 2 had purely intraventricular tumors. RESULTS: Total resection was achieved in 86% of operations. Even in mostly intraventricular cases, a transsphenoidal trans-lamina terminalis approach afforded complete resection. Visual improvement and preservation of the pituitary stalk were achieved in 84% and 95% of cases, respectively. New postoperative deterioration of pituitary function occurred in about 65% of cases, and no patient resolved their preoperative hormonal disturbance after surgery. The overall percentage of patients with diabetes insipidus increased to 61% postoperatively from 11% preoperatively. Nonendocrinologic surgical complications were observed: worsening of vision in 3 patients, hyperphagia in 3 patients, short-term memory loss in 2 patients, and cerebrospinal fluid leakage in 3 patients. Recurrence after total resection occurred in 2 (11%) patients with retrochiasmatic tumors. CONCLUSIONS: Reasonable surgical results in this study suggest that the extended transsphenoidal approach is safe and effective for removal of craniopharyngiomas. Although preservation of the pituitary stalk can be achieved in a high percentage of patients, postoperative endocrinopathy still remains as a significant problem after radical removal of the craniopharyngioma.     

经眶上翼点入路显微手术切除巨大鞍区肿瘤

目的 总结经眶上翼点入路显微手术切巨大鞍区肿瘤的经验。方法 经上翼点入路显微手术切除巨大鞍区肿瘤18例,其中垂体腺瘤7例,颅咽管瘤8例,生殖细胞瘤、脑膜瘤、成熟性畸胎瘤各1例。结果 肿瘤全切除12例,次全切除6例。术后随访4－23个月,恢复良好12例,生活自理3例,生活需人照顾2例,死亡1例。结论 眶上翼点入路能很好地显露鞍区 肿瘤及其周围结构,显微手术是安全切除肿瘤、保护下丘脑功能的关键。     

Intrinsic third ventricular craniopharyngiomas: report on six cases and a review of the literature

BACKGROUND: Craniopharyngiomas constitute 2 to 4% of intracranial neoplasms. However, the purely intraventricular variety are rare. Their magnetic resonance imaging (MRI) characteristically shows an intact third ventricular floor, a patent suprasellar cistern, an intact pituitary stalk, and the absence of sellar abnormalities. METHODS: Between 1994 and June 2002, 6 patients with purely intraventricular craniopharyngioma were surgically managed. There were 4 cystic and 2 solid lesions. The surgical approaches utilized included a frontal, parasagittal, transcallosal approach with the third ventricle being accessed using either the transforaminal or subchoroidal approach (n = 3); pterional, transsylvian (n = 1), and bifrontal interhemispheric (n = 2) approaches in which the third ventricle was accessed via the lamina terminalis. A ventriculoperitoneal shunt was required for one of the 3 patients with hydrocephalus. RESULTS: Total excision was performed in 3 patients with cystic craniopharyngioma, while a small residual lesion was left adherent to the third ventricular floor in the others. There was one perioperative mortality because of septicemia. Two patients required thyroxine supplementation. Two patients developed transient and one other patient a sustained diabetes insipidus. The 2 patients with solid tumors received radiotherapy for the residual lesions. At follow up ranging from 8 to 36 months, neither tumor recurrence nor regrowth was observed in any of the patients. The symptoms of raised intracranial pressure, hypothalamic dysfunction or visual field defects had resolved. CONCLUSIONS: Intraventricular craniopharyngiomas occur in an older population and present mainly with raised intracranial pressure. Visual and endocrinologic imbalances are much less in these lesions compared to the suprasellar craniopharyngiomas. They mainly attach to the third ventricular floor. The surgical approaches to the third ventricle, along with radiotherapy and hormone supplementation, were successful in the management of these rare tumors.     

Surgical outcomes in 31 patients with craniopharyngiomas extending outside the suprasellar cistern: an evaluation of the frontobasal interhemispheric approach

OBJECT: Craniopharyngiomas frequently grow from remnants of the Rathke pouch, which is located on the cisternal surface of the hypothalamic region. These lesions can also extend elsewhere in the infundibulohypophyseal axis. The aim of this study was to establish the usefulness of the frontobasal approach made through a relatively small craniotomy window for the removal of tumors protruding from the sellar-suprasellar region into the third and basal cistern. METHODS: Thirty-one patients who were surgically treated for craniopharyngiomas extending outside the sellar-suprasellar region were evaluated. The diagnoses were established in all cases by using magnetic resonance and computerized tomography imaging. The initial symptoms and signs were increased intracranial pressure in eight, vision impairment or visual field defect in 16, hypopituitarism in 17, and psychological disturbances in three cases. All patients underwent surgery via the frontobasal interhemispheric approach, and the average follow-up period was 30 months. Total removal of the lesion was achieved in 22 cases, six patients underwent subtotal resection, and three underwent partial removal due to tumor recurrence after previous surgeries performed with or without adjunctive radiotherapy. Major complications, including impairment of the cranial nerves, were not observed in the immediate postoperative period. One patient exhibited transient memory disturbance due to infarction of the perforating vessels; after 3 months this symptom was ameliorated. None of the patients died during long-term follow up; however, four of the 22 who underwent total removal and six of the nine patients who underwent subtotal or partial removal suffered recurrence. Of the 10 patients with recurrence, six experienced a small recurrence of the lesion (average 3 months postsurgery); after gamma knife surgery (GKS), the size of two of the lesions was unchanged and in four reoperation was performed due to tumor enlargement during the follow-up period. Ultimately, a total of eight patients (four with recurrence and four who had been treated with GKS) underwent reoperation, with gross-total removal via the same approach or combined with the orbitozygomatic approach in patients with very short optic nerves. In no patient was deterioration of visual acuity and visual field observed after surgery. Although all patients except four children and one adult were receiving some form of hormone replacement therapy, their endocrine status was stably controllable. CONCLUSIONS: In the authors' experience, the frontobasal interhemispheric approach, even made through a small craniotomy window, is a valid choice for the removal of craniopharyngiomas extending outside the sellar-suprasellar region. Via this approach, tumors can be removed without significant sequelae related to the surgical method, due to ease of preservation of the pituitary stalk, hypothalamic structures, and perforating vessels. This approach offers a safe and minimally invasive means of treating craniopharyngiomas.     

核磁共振多平面重建在鞍区肿瘤显微手术入路选择的意义

目的 报道核磁共振多平面和曲面重建技术在鞍区占位病变对鞍区重要结构的显示效果和探讨其在显微外科手术入路选择的作用。方法 通过应用核磁共振和核磁共振多平面重建技术对鞍区常见肿瘤进行术前检查，重建鞍区病变和周围重要结构整体解剖三维切面，显示视神经、视交叉、垂体柄和视丘部等的三维结构，指导手术计划。结果 13例鞍区肿瘤通过核磁共振多平面重建技术能清楚显示受肿瘤压迫移位的视神经、视交叉、动眼神经、视丘部和垂体柄等结构。其中垂体瘤8例、颅咽管瘤3例、鞍结节脑膜瘤2例均获全切除。结论 核磁共振多平面重建技术在显示鞍区整体结构的效果良好，术前应用核磁共振多平面重建技术检查鞍区病变有助于其显微外科手术入路的选择。     

Craniopharyngioma: a pathologic, clinical, and surgical review

Craniopharyngioma is a rare and mostly benign epithelial tumor of the sellar and suprasellar region. Two principal patterns of craniopharyngioma are recognized: papillary and adamantinomatous. Papillary craniopharyngiomas are encountered in adults and may lack the cystic spaces filled with "motor oil" as well as the palisading peripheral rows of epithelial cells, keratinization, or calcification typical of pediatric adamantinomatous craniopharyngioma. Secondary to their anatomic location, craniopharyngiomas may present with endocrinologic dysfunction and visual disturbances. Differential diagnosis includes Rathke's cleft cyst, pituitary adenoma, dermoid/epidermoid cysts, and other rare sellar/suprasellar lesions as pituicytomas. Many controversies exist concerning the preferred surgical approach for these tumors. Endoscopic endonasal surgery is no longer reserved only for sellar or small cystic suprasellar lesions. Prechiasmatic/preinfundibular lesions are effectively removed using an endonasal transtuberculum/transplanum approach; subchiasmatic/transinfundibular tumors require the addition of a transellar approach with inferior pituitary transposition; and retrochiasmatic/retroinfundibular lesions are better accessed performing an endonasal superior pituitary transposition. Compared with well-established trancranial approaches (pterional, subfrontal, presigmoid), endoscopic endonasal surgery combines the virtues of the caudocranial and midline approaches, allowing for appropriate infrachiasmatic exposure without the need for manipulation of surrounding neurovascular structures to access the tumor. This anatomic advantage, combined with high-definition wide-angle visualization, exquisite endonasal microsurgical techniques, and devoted instrumentation facilitates a high rate of endocrine function preservation and visual improvement, while concurrently achieving comparable resections. Endoscopic skull base reconstruction with the vascularized nasoseptal flap has dramatically reduced the incidence of cerebrospinal fluid leak, consolidating endoscopic endonasal surgery as an effective and safe alternative for the treatment of these challenging tumors.     

Stereotactic management of craniopharyngiomas

Eleven patients with craniopharyngiomas underwent 13 stereotactic procedures (intracavitary irradiation, n = 7; radiosurgery, n = 6). Nine patients (82%) had recurrent tumors after prior surgery (median, 2 operations). The median patient age was 50 years (range, 6-63 years). At a median follow-up of 38 months (range, 24-102 months), no patient had progression of the treated tumor component. Two patients developed new cysts, so the actuarial 2- and 4-year progression-free survival rates were 91 and 73%, respectively. Visual function improved in 3 patients, remained stable in 6 patients, and worsened in 1 patient. Two patients (both without prior surgery) not having diabetes insipidus pre-operatively continued to have normal posterior pituitary function. Stereotactic techniques should be considered for patients with craniopharyngiomas, especially those who have failed prior surgical resection.     

Transsphenoidal approaches for the extracapsular resection of midline suprasellar and anterior cranial base lesions.

OBJECTIVE: The transsphenoidal approach is an effective method for treating tumors contained within the sella or extending into the suprasellar cistern. The technique of tumor dissection is predicated on preservation of the integrity of the diaphragma, i.e., intracapsular removal. Gross total extracapsular dissection may, however, be accomplished either by using a standard approach to the pituitary fossa or by extending the exposure to include removal of a portion of the planum sphenoidale and division of the superior intercavernous sinus. METHODS: Included in this series were 14 patients with parasellar or sellar tumors with extension into the anterior fossa and/or suprasellar cistern. For 4 of 14 patients (29%), extracapsular access was gained by broaching the tumor capsule from within the pituitary fossa. For the remaining 10 of 14 patients (71%), the dura of the floor of the sella and the planum sphenoidale was exposed, using neuronavigation to verify the limits of bony dissection; extracapsular tumor resection was performed using the operating microscope and endoscopy as indicated. The dural defect was repaired with abdominal fat, the sellar floor and planum sphenoidale were reconstructed, and in selected cases a lumbar drain was placed. RESULTS: Seven of 14 tumors (50%) were craniopharyngiomas, 3 of 14 (21%) were pituitary adenomas, and 2 of 14 (14%) were meningiomas. There was one case of lymphocytic hypophysitis and one yolk sac tumor. Gross total resection was possible in 11 of 14 cases (79%). Immediate postoperative visual function worsened in 2 of 14 cases (14%), improved in 3 of 14 cases (21%), and was stable in the remainder of cases. Postoperatively, 2 of 14 patients (14%) developed bacterial meningitis. Overt postoperative cerebrospinal fluid rhinorrhea was not observed. CONCLUSION: Gross total extracapsular resection of midline suprasellar tumors via a transsphenoidal approach is possible but is associated with a higher risk of complications than is standard transsphenoidal surgery.     

巨大垂体腺瘤的治疗策略

目的通过回顾性分析32例巨大垂体腺瘤病例的临床资料，探讨其治疗策略。方法男性17例，女性15例，平均年龄46．7岁，平均病程4．7年。其中垂体生长激素(GH)腺瘤1例，泌乳素(PRL)腺瘤6例，无功能腺瘤25例。采用经口鼻蝶窦入路手术24例，经额下人路手术1例，经蝶窦和经颅分期手术6例，药物治疗1例。结果手术显微镜下肿瘤全部切除20例，7欠全切除9例，部分切除2例；手术后放疗10例，药物治疗5例。术后死亡1例。随访6个月～4年，影像学检查显示肿瘤全部切除15例，次全切除14例，部分切除2例；肿瘤无复发。结论对巨大垂体腺瘤，应根据患者的临床表现、内分泌学检查和肿瘤的生长方式决定治疗方案；大多数巨大垂体腺瘤可以首选经蝶窦入路手术，并发症较少，死亡率低；对侵袭性生长的肿瘤，应采用手术、放疗和药物综合治疗。