59 Endoscopic Approaches to the Sella and Suprasellar Region 59 内镜下鞍区和上鞍区手术方法
Sanjeet V. Rangarajan, Marc R. Rosen, and James J. Evans Sanjeet V. Rangarajan, Marc R. Rosen 和 James J. Evans
Abstract 摘要
Transsphenoidal approaches to the sella and suprasellar regions to treat a variety of pathologies have been performed with increasing frequency since their initial introduction and development in the early 20th century. Utilizing the sinonasal cavity and sphenoid sinus as a corridor to access the sellar and suprasellar space is now ubiquitous across the world and has been validated in a plethora of studies measuring outcomes, anatomic variability, economic factors, and more. In this chapter, we discuss the history of the approach, common pathologies encountered, a step-by-step description of the most common endoscopic approaches to the region, and technical nuances an endoscopic skull base surgeon will find useful. Endoscopic reconstruction techniques, identification of potential complications, and perioperative care, all of which are keys to good outcomes in endoscopic sellar and suprasellar surgery, are also discussed in this chapter. 经蝶入路治疗鞍区和鞍上区各种病理情况自 20 世纪初初引入和发展以来,其应用频率逐渐增加。利用鼻窦和蝶窦作为通道进入鞍区和鞍上空间,现在在全球范围内普遍存在,并在大量研究中得到了验证,这些研究测量了结果、解剖变异性、经济因素等。在本章中,我们讨论了该方法的史实、常见的病理情况、该区域最常见内镜方法的逐步描述,以及内镜颅底外科医生会发现有用的技术细节。本章还讨论了内镜重建技术、潜在并发症的识别和围手术期护理,这些都是内镜鞍区和鞍上手术良好结果的关键。
The operating surgeon must enter the sphenoid sinus with preservation of the posterior nasal artery in order to avoid disruption of the nasoseptal flap pedicles. 手术操作者必须保留后鼻动脉,进入蝶窦,以避免鼻中隔瓣蒂的破坏。
The approach to the sella may be tailored to the pathology utilizing unilateral, “1.5,” and “tunnel” techniques without a large posterior septectomy and bilateral wide sphenoidotomies. 该进入蝶鞍的方法可根据病理情况采用单侧、“1.5”和“隧道”技术,无需大范围后隔膜切除术和双侧广泛蝶窦切开术。
Lumbar drains are not necessary for typical patients undergoing sellar/suprasellar surgery, although they may be utilized temporarily in patients with elevated intracranial pressure until cerebrospinal fluid shunt placement can occur. 腰椎引流对于典型接受鞍区/鞍上手术的患者并非必需,尽管在颅内压升高的患者中,在脑脊液分流植入之前,可能暂时使用。
Routine approaches to the sella can often employ tailored, conservative reconstructive techniques. It is unnecessary to routinely use extensive nasal packing or vascularized tissue in every case, 常规鞍区手术常可使用定制、保守的重建技术。在每例手术中常规使用广泛的鼻部填充或血管化组织是不必要的。
59.1 Introduction and History 59.1 简介和历史
Transsphenoidal approaches to the sella for treatment of pituitary lesions originated in the early 20th century when Austrian surgeon Dr. Hermann Schloffer documented the first transnasal pituitary surgery in 1907. ^(1){ }^{1} After further modifications in Europe and subsequent adoption in the United States by Dr. Harvey Cushing at Johns Hopkins, transsphenoidal surgery via the sublabial route became the preferred approach for many surgeons. ^(2){ }^{2} After his first sublabial, transsphenoidal approach in 1909 for a patient with acromegaly, Cushing amassed a series of nearly 20 世纪初,奥地利外科医生赫尔曼·施洛弗博士在 1907 年记录了首次经鼻垂体手术,从而开创了经蝶入路治疗垂体病变的方法。 ^(1){ }^{1} 在欧洲进一步改进并由约翰霍普金斯医院的哈维·库欣博士在美国采纳后,经蝶入路手术通过下唇途径成为许多外科医生的首选方法。 ^(2){ }^{2} 在 1909 年首次为一位患有肢端肥大的患者进行下唇途径经蝶入路手术之后,库欣积累了一系列经验。
300 cases. He and others modified the procedure to an inferior transseptal approach utilizing a nasal speculum. Later, Cushing abruptly abandoned the method in 1929 in favor of the transcranial route. This change was a result of refinements and improved outcomes of craniotomies, as well as dissatisfaction with decompression of suprasellar structures and poor illumination with the transnasal approach. ^(3){ }^{3} Due to the worldwide influence of Cushing and other reputable transcranial surgeons, the transsphenoidal approach to the sella fell into relative obscurity for almost four decades. 300 例。他和其他人将手术方法修改为使用鼻镜的次优经蝶窦入路。后来,库欣在 1929 年突然放弃了这种方法,转而采用经颅入路。这种变化是由于颅骨切开术的改进和效果的提高,以及对鞍上结构减压的不满和经鼻入路照明不足。由于库欣和其他知名经颅外科医生对全球的影响,经蝶窦入路对鞍区的处理几乎在接下来的四十年里变得相对隐秘。
Norman Dott in Scotland, who trained under Cushing, was one of the few surgeons worldwide to continue the inferior transnasal approach. Dott improved the visualization by adding lights to the speculum and his student, Guiot, added the use of fluoroscopy for intraoperative imaging. After learning from Guiot in France, Jules Hardy reintroduced the approach to North America in the 1960s. Hardy subsequently improved the visualization by adding the operating microscope and refined the nasal speculum among other surgical instruments. ^(2){ }^{2} These advances, along with the availability of intraoperative fluoroscopy for navigation of instrumentation, expanded the indications for the transsphenoidal approach beyond pituitary tumors to include craniopharyngiomas, meningiomas, and other sellar/parasellar pathology. ^(4){ }^{4} Thus began a period of rapid technological development that eventually saw the emergence of endoscopic, endonasal techniques, real-time image-guided navigation, and the development of new surgical instruments. The publication of the first major series of patients treated for sellar lesions using the endoscopic, endonasal approach by Jho and Carrau in 1997 signified the beginning of the “modern era” of purely endoscopic sellar and suprasellar surgery. ^(5){ }^{5} Their initial success gave way to our understanding of the advantages, limitations, and surgical pearls, which will be discussed in this chapter. 诺曼·道特在苏格兰,师从库欣,是全球少数继续采用下鼻道入路的外科医生之一。道特通过在窥镜中添加灯光来改善可视化,他的学生纪尧姆则增加了术中影像学检查中使用荧光镜。在法国从纪尧姆学习后,朱尔斯·哈迪在 20 世纪 60 年代将这种方法重新引入北美。哈迪随后通过添加手术显微镜和改进鼻窥镜等手术器械来改善可视化。这些进步,加上术中荧光镜导航仪器的可用性,将经蝶入路的适应症从垂体瘤扩展到颅咽管瘤、脑膜瘤和其他蝶鞍/蝶鞍旁病理。因此,开始了快速技术发展的时期,最终出现了内镜、鼻内技术、实时图像引导导航和新手术器械的发展。 1997 年,Jho 和 Carrau 首次发表使用内镜、鼻内入路治疗垂体瘤的主要病例系列,标志着“现代时代”纯内镜垂体和鞍上手术的开始。他们的初步成功使我们理解了其优势、局限性和手术要点,这些将在本章中讨论。
59.2 Patient Selection/Indications 59.2 患者选择/适应症
While pituitary adenomas are the most common sellar lesion, endoscopic endonasal approaches enable the treatment of a variety of pathologies including craniopharyngiomas, Rathke’s cleft cysts (RCCs), meningiomas, and other benign and malignant tumors. We will summarize the salient features of each in the subsequent pages. 垂体腺瘤是最常见的鞍区病变,内镜经鼻入路可以治疗包括颅咽管瘤、Rathke 裂囊肿(RCCs)、脑膜瘤以及其他良性及恶性肿瘤等多种疾病。我们将在后续页面中总结每种疾病的显著特征。
59.2.1 Pituitary Adenoma 垂体腺瘤
Pituitary adenomas are classified as either functional or nonfunctional based on their secretory profile as determined by blood and urine tests. They can be further classified based on their size as microadenomas ( < 10mm<10 \mathrm{~mm} ), macroadenomas ( > 10mm>10 \mathrm{~mm} ), or giant adenomas ( >= 40mm\geq 40 \mathrm{~mm} ). Roughly one-half of pituitary tumors presenting with clinical symptoms are classified as macroadenomas. ^(6){ }^{6} As a group, pituitary adenomas are 垂体腺瘤根据其分泌谱(由血液和尿液检测确定)被分为功能性或非功能性。它们还可以根据大小进一步分类为微腺瘤( < 10mm<10 \mathrm{~mm} )、大腺瘤( > 10mm>10 \mathrm{~mm} )或巨大腺瘤( >= 40mm\geq 40 \mathrm{~mm} )。大约一半出现临床症状的垂体肿瘤被归类为大腺瘤。作为一个群体,垂体腺瘤被
invariably common, comprising almost 90%90 \% of all sellar lesions. ^(7){ }^{7} Postmortem and radiographic data reveal the presence of pituitary adenomas in at least 10%10 \% of individuals, while clinical epidemiological studies estimate the prevalence to range from 1 in 865 persons to 1 in 2,688 persons. ^(6){ }^{6} 普遍常见,约占所有垂体病变的 90%90 \% 。 ^(7){ }^{7} 尸检和放射学数据表明,至少 10%10 \% 的人存在垂体腺瘤,而临床流行病学研究表明,患病率范围从每 865 人中有 1 人至每 2,688 人中有 1 人。 ^(6){ }^{6}
Nearly two-thirds of pituitary adenomas are functional, with prolactinomas comprising the majority. ^(6){ }^{6} Prolactinomas are generally treated with a dopamine agonist and are considered for surgery if they remain refractory to medical treatment. Functioning pituitary adenomas may also secrete adrenocorticotrophic hormone (ACTH) causing Cushing’s disease or growth hormone (GH) causing acromegaly. ^(8){ }^{8} These functional tumors are preferentially treated with surgery, although medical therapy and stereotactic radiotherapy may also be necessary. 近三分之二垂体腺瘤为功能性,其中催乳素瘤占多数。催乳素瘤通常使用多巴胺激动剂治疗,如果对药物治疗无反应,则考虑手术治疗。功能性垂体腺瘤也可能分泌肾上腺皮质激素(ACTH)导致库欣病或生长激素(GH)导致肢端肥大症。这些功能性肿瘤优先考虑手术治疗,尽管可能还需要药物治疗和立体定向放射治疗。
Functional pituitary adenomas are typically diagnosed at a smaller size due to the physical or physiological effects of hormone secretion, while nonfunctional tumors are usually diagnosed due to the symptoms from local mass effect as they increase in size. The mass effect caused by a larger pituitary macroadenoma may cause visual disturbance from compression of the optic chiasm (e.g., bitemporal hemianopsia), headache, hormonal insufficiency, and cranial nerve deficits such as diplopia due to extension into the cavernous sinus. These symptoms are generally suitable indications for surgery. 功能性垂体腺瘤通常因激素分泌的物理或生理效应而在较小尺寸时被诊断,而非功能性肿瘤则通常因局部肿块效应增大时的症状而被诊断。较大的垂体巨腺瘤引起的肿块效应可能导致由于视交叉受压(例如,双颞侧偏盲)、头痛、激素不足以及由于扩展到海绵窦而引起的颅神经缺陷,如复视。这些症状通常适合作为手术的适应症。
A small percentage ( ∼2%\sim 2 \% ) of pituitary tumors may present with an acute onset of symptoms called apoplexy. ^(9){ }^{9} Pituitary apoplexy is caused by intratumor hemorrhage or infarction and causes rapid development of symptoms such as headache, visual decline, cranial nerve palsy, hypocortisolemia, and altered mental status. ^(10){ }^{10} Patients presenting with symptoms suspicious for pituitary apoplexy should immediately receive intravenous fluids, stress-dose steroids, imaging with computed tomography (CT) and/or magnetic resonance imaging (MRI), and be evaluated for prompt surgical intervention. In a recent review of 44 patients, Zaidi et al found that headaches, visual acuity, and pituitary dysfunction were more likely to improve quickly following surgical decompression compared with ophthalmoparesis (from cranial nerve dysfunction), which tended to persist for longer periods of time. ^(10){ }^{10} Endocrinopathies may also be more persistent. Bills et al reported that 80%80 \% of patients required long-term steroids or thyroid hormone supplementation, 11%11 \% required desmopressin, and 64% of male patients required testosterone replacement. ^(11){ }^{11} The appropriate time course for surgery is debated and ranges from within 24 hours to 1 week of symptom onset, but these authors recommend intervention as soon as the patient is medically stable. ^(12){ }^{12} 一小部分( ∼2%\sim 2 \% )垂体肿瘤可能表现为急性发作的症状,称为卒中。 ^(9){ }^{9} 垂体卒中由肿瘤内出血或梗死引起,导致头痛、视力下降、颅神经麻痹、低皮质醇血症和意识状态改变等症状迅速发展。 ^(10){ }^{10} 对于出现疑似垂体卒中的症状的患者,应立即接受静脉输液、应激剂量皮质类固醇、计算机断层扫描(CT)和/或磁共振成像(MRI)检查,并评估是否需要及时进行手术治疗。在一项对 44 名患者的近期回顾性研究中,Zaidi 等人发现,与眼肌麻痹(由颅神经功能障碍引起)相比,头痛、视力敏锐度和垂体功能障碍在手术减压后更有可能迅速改善,而眼肌麻痹往往持续更长时间。 ^(10){ }^{10} 内分泌疾病也可能更持久。Bills 等人报告称, 80%80 \% 的患者需要长期使用皮质类固醇或甲状腺激素补充剂, 11%11 \% 的患者需要去氨加压素,64%的男性患者需要睾酮替代治疗。 手术的适宜时间尚有争议,从症状出现后 24 小时内到 1 周不等,但这些作者建议在患者病情稳定后尽快干预。
59.2.2 Craniopharyngioma 颅咽管瘤
Craniopharyngiomas constitute nearly 3%3 \% of all intracranial tumors and occur in the sellar and parasellar regions. ^(13){ }^{13} They are thought to arise from the epithelial remnants of the craniopharyngeal duct, which extends from Rathke’s cleft to the floor of the third ventricle, or from buccal mucosa rests. Craniopharyngiomas are divided into two categories that have different clinical features: the adamantinomatous type, which occurs primarily in a bimodal distribution between children and older 颅咽管瘤占所有颅内肿瘤的近 0#,发生在鞍区和邻近区域。1#人们认为它们起源于颅咽管残留的鳞状上皮,该管从 Rathke 裂延伸到第三脑室底部,或起源于颊黏膜残留。颅咽管瘤分为两种具有不同临床特征的类型:硬质瘤型,主要发生在儿童和老年人之间的双峰分布。
adults (50-60 years), and the papillary type, which mainly occurs in adults. ^(13){ }^{13} Adamantinomatous craniopharyngiomas are much more common and present as solid/cystic lesions, compared to their papillary counterparts, which have a more solid constitution. ^(14){ }^{14} The cystic portion of craniopharyngiomas often contains a brown fluid likened to “motor oil,” which contains lipids, cholesterol crystals, and epithelial cells. 成人(50-60 岁),以及乳头状型,主要发生在成人。 ^(13){ }^{13} 与其乳头状对应物相比,腺样颅咽管瘤更为常见,表现为实性/囊性病变,其构成更为坚实。 ^(14){ }^{14} 颅咽管瘤的囊性部分通常含有类似“机油”的棕色液体,其中含有脂质、胆固醇晶体和上皮细胞。
Patients with craniopharyngiomas present with endocrinopathies up to 80 to 90%90 \% of the time, and visual disturbance and headache are also common due to mass effect from the lesion. ^(14){ }^{14} Imaging with CT and/or MRI generally reveals a cystic sellar/suprasellar lesion with intralesional calcifications. Given their propensity for locally aggressive growth, resection is the definitive treatment for these lesions. 颅咽管瘤患者 80%至 90%90 \% 的时间出现内分泌疾病,由于病变的占位效应,视力障碍和头痛也较为常见。 ^(14){ }^{14} 通常通过 CT 和/或 MRI 成像显示为含有病变内钙化的囊性垂体/鞍上病变。鉴于其局部侵袭性生长的倾向,切除是这些病变的根治性治疗方法。
Craniopharyngiomas have been historically difficult to treat, once earning Dr. Cushing’s designation as the “most forbidding of the intracranial tumors.” ^(15){ }^{15} Efforts to classify craniopharyngiomas based on their relationship to the infundibulum and optic chiasm have been well described and can help predict ease of resection and risk of recurrence. ^(16){ }^{16} While rates of gross total resection (GTR) have improved in the endoscopic era due to increasing surgeon experience, many patients require postoperative radiation for residual tumor. ^(15){ }^{15} The learning curve for successful endoscopic treatment of craniopharyngiomas is significant, but improvements in the degree of resection while minimizing morbidity can be achieved with experience. Degree of success can be defined as GTR, near total resection (NTR; > 95%>95 \% resection), and subtotal resection (STR). Those undergoing NTR or STR followed by adjuvant radiation may have similar recurrence rates compared with those who underwent GTR. ^(17){ }^{17} Striving for GTR should not take place at the expense of potentially increased morbidity. Overall recurrence rates vary between centers, but have been reported to be nearly 15%15 \% in recent reports. ^(17){ }^{17} 颅咽管瘤历史上治疗困难,一度被库欣博士称为“颅内肿瘤中最令人畏惧的。”根据颅咽管瘤与漏斗和视交叉的关系进行分类的努力已被充分描述,并有助于预测切除的容易程度和复发的风险。在内窥镜时代,由于外科医生经验的增加,全切除率(GTR)有所提高,但许多患者需要术后放疗以消除残留肿瘤。成功进行颅咽管瘤内窥镜治疗的学习曲线非常显著,但通过经验可以实现切除程度的提高同时最小化并发症。成功程度可以定义为 GTR、近全切除(NTR;切除)和次全切除(STR)。接受 NTR 或 STR 并随后进行辅助放疗的患者,其复发率可能与接受 GTR 的患者相似。不应以增加潜在并发症为代价追求 GTR。总体复发率在不同中心之间有所差异,但据最近报道,几乎为@5%。
59.2.3 Rathke's Cleft Cyst 59.2.3 拉特克裂囊肿
RCCs are benign, epithelium-lined remnants of the craniopharyngeal duct and are commonly discovered incidentally in the sellar and suprasellar regions. The lesions commonly present in adulthood, between ages 40 and 60 years. Females are slightly more likely to be affected. These lesions are commonly asymptomatic despite their high prevalence of up to 22%22 \%, and only 2 to 9%9 \% of patients undergoing endoscopic, endonasal approaches to the sella are being treated for an RCC. ^(18){ }^{18} When the cysts begin to exert mass effects from growth, they can cause headache, hormonal dysfunction, and visual deficits. ^(19){ }^{19} Rarely, they can present with chemical meningitis or intracystic hemorrhage, an analog to pituitary apoplexy. RCCs 是颅咽管残留物,由上皮细胞衬里,通常在鞍区和鞍上区偶然发现。这些病变通常在成年期出现,在 40 至 60 岁之间。女性受影响的几率略高。尽管这些病变的患病率高达 22%22 \% ,但通常无症状,只有 2 到 9%9 \% 接受经内镜、经鼻入路鞍区手术的患者被诊断为 RCC。 ^(18){ }^{18} 当囊肿开始因生长而产生质量效应时,它们可以引起头痛、激素功能障碍和视力障碍。 ^(19){ }^{19} 罕见情况下,它们可能表现为化学性脑膜炎或囊内出血,类似于垂体卒中。
RCCs are identified on MRI as well-circumscribed intra-/ suprasellar lesions with an enhancing rim in lesions whose cyst wall has undergone squamous metaplasia. The cysts will often separate the anterior and posterior glands of the pituitary gland. Signal intensity of the cyst on T1 and T2 MRI sequences depends on the protein content of the fluid. RCCs 在 MRI 上被识别为界限清晰的鞍内/上病变,病变的囊肿壁发生鳞状化生时,病变周围有增强的边缘。囊肿通常将垂体前叶和后叶分开。囊肿在 T1 和 T2 MRI 序列上的信号强度取决于液体的蛋白质含量。
The asymptomatic patient with an incidentally detected RCC can generally be followed with annual imaging and ophthalmologic evaluation. Patients who exhibit growth of the cyst on follow-up imaging or experience compressive symptoms from 无症状的偶然发现肾细胞癌(RCC)患者通常可以通过年度影像学和眼科评估进行随访。在随访影像学检查中表现出囊肿生长或经历压迫症状的患者,
an RCC benefit from endoscopic endonasal drainage with a wide dural opening and cyst fenestration to prevent recurrence. 一例通过内镜经鼻引流术,结合广泛硬脑膜开口和囊肿窗孔术,以预防复发的 RCC 受益者。
59.2.4 Meningioma 59.2.4 脑膜瘤
Meningiomas are predominantly benign, slow-growing tumors that originate from the meningothelial cells of the arachnoid. An estimated 2 to 3%3 \% of the population have an incidental asymptomatic meningioma. ^(20){ }^{20} While they account for almost 25%25 \% of all intracranial masses, they only comprise roughly 1%1 \% of sellar masses. ^(21){ }^{21} Their incidence is double in women and they tend to peak in incidence during the fifth and sixth decades of life. ^(21){ }^{21} Previous exposure to radiation is a recognized risk factor. The clinical presentation of meningiomas is highly variable and can range from incidental discovery to severe visual field deficits, decreased visual acuity, focal and general neurological deficits, hypopituitarism, and headache. Histological grading of meningiomas is based on the World Health Organization (WHO) classification system, and includes grade I (benign), grade II (atypical), and grade III (anaplastic). ^(20){ }^{20} 脑膜瘤主要是由蛛网膜的脑膜细胞起源的良性、生长缓慢的肿瘤。估计有 2%至 3%3 \% 的人口偶然发现无症状的脑膜瘤。 ^(20){ }^{20} 虽然它们占所有颅内肿瘤的近 25%25 \% ,但它们只占鞍区肿瘤的大约 1%1 \% 。 ^(21){ }^{21} 其发病率在女性中是男性的两倍,并且它们在人生的第五和第六十年达到发病率高峰。 ^(21){ }^{21} 既往接触辐射是一个已知的危险因素。脑膜瘤的临床表现高度多样,可以从偶然发现到严重的视野缺陷、视力下降、局灶性和一般性神经功能障碍、垂体功能减退和头痛。脑膜瘤的病理分级基于世界卫生组织(WHO)的分类系统,包括 I 级(良性)、II 级(不典型)和 III 级(间变)。 ^(20){ }^{20}
Meningiomas are extra-axial tumors and are represented on imaging as fairly well-defined masses that displace the surrounding brain. ^(20){ }^{20} They enhance with contrast and appear hyperdense compared to normal brain tissue on CT. MRI is the preferred imaging study for meningiomas and will appear isointense or mildly hypointense to normal brain on T 1 sequences, and hyperintense to brain on T 2 sequences. ^(20){ }^{20} They enhance vividly with gadolinium contrast. The “dural tail,” which represents the characteristic marginal dural thickening that tapers peripherally, is a pathognomonic imaging finding for meningiomas. 脑膜瘤是外周肿瘤,在影像学上表现为较为明确的肿块,可推移周围脑组织。增强扫描后,与正常脑组织相比,在 CT 上呈现高密度。MRI 是脑膜瘤的首选影像学检查,T1 序列上表现为等信号或轻度低信号,T2 序列上对脑组织呈高信号。它们在钆增强扫描中明显增强。所谓的“硬脑膜尾”,代表特征性的边缘硬脑膜增厚,向周围逐渐变细,是脑膜瘤的特征性影像学发现。
The endoscopic endonasal approach is typically used for meningiomas that arise from the tuberculum sella, planum sphenoidale, diaphragma sella, and olfactory groove. The ideal meningioma for this approach is centered in the posterior aspect of the frontal fossa skull base and possessing a higher craniocaudal-to-anteroposterior ratio, without significant lateral growth beyond the carotid arteries and/or cranial nerves. ^(19){ }^{19} Close observation following resection with scheduled postoperative imaging to monitor for recurrence is recommended. 内镜经鼻入路通常用于起源于鞍结节、蝶骨平台、鞍膈和嗅沟的脑膜瘤。理想的脑膜瘤适用于此入路应位于颅底额窦后部中央,并具有更高的颅尾向-前后向比率,且在颈动脉和/或颅神经外侧无显著生长。建议术后定期进行影像学检查,以监测复发。
59.2.5 Chordoma 59.2.5 软骨肉瘤
Chordomas are rare, slow-growing neoplasms that originate typically in the midline from the notochord remnants along the skull base and spinal column. ^(7,19){ }^{7,19} It is sometimes difficult to distinguish between chordoma, chondromas, and chondrosarcoma on preoperative evaluation. Chordomas have an incidence of less than 0.1 patients per 100,000 per year and account for 1 to 4%4 \% of all primary malignant bone tumors. ^(22){ }^{22} Chordomas often arise in the spheno-occipital region of the clivus, making the endoscopic, endonasal approach an ideal technique for surgical treatment of these lesions. 脊索瘤是罕见的、生长缓慢的肿瘤,通常起源于颅底和脊柱沿线的脊索残留物。有时在术前评估中难以区分脊索瘤、软骨瘤和软骨肉瘤。脊索瘤的发病率低于每年每 10 万人中 0.1 例,占所有原发性恶性骨肿瘤的 1 至 4%4 \% 。脊索瘤通常起源于斜坡的蝶枕区域,使得内镜、鼻内入路成为这些病变手术治疗的理想技术。
Pain secondary to bony destruction is a common symptom, and patients will often also present with cranial nerve deficits (oculomotor or abducens nerve most commonly) due to extension into the cavernous sinus and Dorello’s canal. 7,23 Chordomas tend to demonstrate a proclivity for the clivus, which was 骨破坏引起的疼痛是常见症状,患者通常还会出现颅神经功能障碍(最常见的是动眼神经或滑车神经),这是由于病变扩展到海绵窦和 Dorello 管。7,23 脊索瘤倾向于倾向于侵犯斜坡。
involved in 90%90 \% of patients in a study of 109 patients with chordoma and chondrosarcoma. ^(23){ }^{23} On MRI examination, chordomas demonstrate isointensity or hypointensity on T 1 sequences, and hyperintensity on T2. More than 70%70 \% of chordomas will enhance with administration of gadolinium contrast. ^(24){ }^{24} CT imaging can be helpful to determine the extent of bony destruction and to assist with surgical planning. Metastasis of chordoma is rare and additional imaging studies, such as CT of the neck and/or chest, are generally unnecessary unless there is clinical suspicion. 参与 109 名患者中 90%90 \% 的研究,其中患有脊索瘤和软骨肉瘤。 ^(23){ }^{23} 在 MRI 检查中,脊索瘤在 T1 序列上表现为等信号或低信号,在 T2 上表现为高信号。超过 70%70 \% 的脊索瘤在给予钆对比剂后将会增强。 ^(24){ }^{24} CT 成像有助于确定骨破坏的范围并协助手术规划。脊索瘤的转移很少见,除非有临床怀疑,否则通常无需进行颈部和/或胸部的额外影像学检查。
Chordomas are a surgical challenge due to their midline position and tendency to erode and envelop critical skull base neural and vascular structures. Thus, they have a significant tendency to recur. GTR of skull base chondroma can be difficult, and patients often receive adjuvant radiotherapy to increase disease-free survival. In cases of recurrence, experienced surgeons may utilize the endoscopic, endonasal approach perform re-resection without additional increased morbidity. ^(25){ }^{25} 脊索瘤由于其中线位置和侵蚀并包裹关键颅底神经和血管结构的倾向,构成了手术挑战。因此,它们有很高的复发倾向。颅底软骨瘤的 GTR 可能很困难,患者通常接受辅助放疗以提高无病生存率。在复发的情况下,经验丰富的外科医生可能会利用内镜、鼻内途径进行再次切除,而不会增加额外的发病率。 ^(25){ }^{25}
59.2.6 Metastases 59.2.6 转移灶
Sellar metastasis are rare and comprise less than 1%1 \% of pituitary and sellar tumors. The most common metastases presenting in this area are from breast and lung cancer, followed by prostate and renal carcinomas. ^(7){ }^{7} When these metastases are found in the sellar area, they are often part of a generalized metastatic spread of the primary neoplasm and can be associated with several more additional sites in the body, especially bone. ^(26){ }^{26} Metastases have a propensity for the posterior lobe of the pituitary gland and the infundibulum, thus causing significant incidence of diabetes insipidus (DI), as high as 100%100 \% in some series. ^(26){ }^{26} The anterior pituitary gland does not have a direct arterial blood supply, perhaps explaining the predilection for metastases to arise in the posterior pituitary gland via hematogenous spread. Cranial nerve deficits and anterior pituitary dysfunction are less common as a result. Other nonspecific symptoms such as headache and visual disturbance can also occur due to local mass effect. 垂体转移罕见,占垂体和鞍区肿瘤不到 1%1 \% 。在此区域最常见的转移是来自乳腺癌和肺癌,其次是前列腺和肾癌。 ^(7){ }^{7} 当这些转移在鞍区发现时,它们通常是原发性肿瘤广泛转移的一部分,并可伴随身体其他几个部位,尤其是骨骼。 ^(26){ }^{26} 转移倾向于垂体后叶和漏斗,因此导致尿崩症(DI)的发病率显著,在某些系列中高达 100%100 \% 。 ^(26){ }^{26} 前垂体没有直接的动脉血供,这或许可以解释为什么转移倾向于通过血源性传播在垂体后叶发生。颅神经缺陷和前垂体功能障碍较少见,因此。其他非特异性症状,如头痛和视力障碍,也可能由于局部肿块效应而发生。
Imaging characteristics of metastases are highly variable and they can be difficult to distinguish from benign pituitary tumors. CT and MRI are useful and enhancement is variable with each. Bony erosion of the sella or surrounding structures can be present but is not pathognomonic. On MRI, metastasis may be hypointense or isointense with normal brain tissue on T 1 sequences, or hyperintense on T 2 sequences. PET/CT may help identify systemic metastasis. 转移瘤的影像学特征高度可变,且难以与良性垂体瘤区分。CT 和 MRI 很有用,增强程度各有不同。鞍区或周围结构的骨侵蚀可能存在,但并非特异性病理特征。在 MRI 上,转移瘤在 T1 序列上可能呈低信号或与正常脑组织等信号,或在 T2 序列上呈高信号。PET/CT 有助于识别全身转移。
Because sellar metastases often present with other systemic metastases concurrently, a careful examination and metastatic workup must be completed. Surgical intervention may be limited to biopsy to establish a tissue diagnosis, but therapeutic excision and/or debulking may serve some purposes to reduce symptoms in some clinical situations. The patient’s overall prognosis must be kept in mind prior to recommending any surgery beyond biopsy for these patients. Discussion in a multidisciplinary tumor board setting can be helpful to identify options, which may include palliative radiation and/or chemotherapy. Medical management of DI (e.g., vasopressin) is often necessary. 因为垂体转移瘤通常与其他系统性转移瘤同时出现,必须进行仔细检查和转移性检查。手术干预可能仅限于活检以确定组织诊断,但在某些临床情况下,治疗性切除和/或减瘤可能有助于减轻症状。在推荐这些患者进行活检以外的任何手术之前,必须考虑患者的整体预后。在多学科肿瘤委员会的讨论中可以有所帮助,以确定可能包括姑息性放疗和/或化疗的选项。DI(例如,加压素)的医学管理通常是必要的。