suprasellar mass

鞍上肿块
  • 文章类型: Journal Article
    背景:鞍上肿块通常包括颅咽管瘤和垂体腺瘤。鞍上胶质母细胞瘤极为罕见,文献中只有少数病例报道。鞍上胶质母细胞瘤可在术前模仿颅咽管瘤或其他更常见的鞍上病因。
    方法:一名无明显病史的65岁男性因亚急性精神状态下降而就诊于急诊科。检查发现一个大的鞍上肿块,并延伸到右下内侧额叶和右侧脑室,伴有明显的血管源性水肿。患者接受了半球间的经call体入路,对肿块的室间部分进行了次全切除。病理分析显示胶质母细胞瘤,MGMT部分甲基化,BRAFV600E突变。
    结论:恶性成胶质细胞瘤可以模拟良性鞍上肿块,并且对于具有广泛放射学和临床特征的各种脑肿块应保持差异。对于从脑室进入的复杂病例,垂体复合体无法通过经蝶入路可靠地保留,半球间方法也是一种实用的初始手术选择。除了提供诊断价值外,分子谱分析也可能揭示治疗上显著的基因改变,如BRAF突变.
    BACKGROUND: Suprasellar masses commonly include craniopharyngiomas and pituitary adenomas. Suprasellar glioblastoma is exceedingly rare with only a few prior case reports in the literature. Suprasellar glioblastoma can mimic craniopharyngioma or other more common suprasellar etiologies preoperatively.
    METHODS: A 65-year-old male with no significant history presented to the emergency department with a subacute decline in mental status. Work-up revealed a large suprasellar mass with extension to the right inferior medial frontal lobe and right lateral ventricle, associated with significant vasogenic edema. The patient underwent an interhemispheric transcallosal approach subtotal resection of the interventricular portion of the mass. Pathological analysis revealed glioblastoma, MGMT partially methylated, with a BRAF V600E mutation.
    CONCLUSIONS: Malignant glioblastomas can mimic benign suprasellar masses and should remain on the differential for a diverse set of brain masses with a broad range of radiological and clinical features. For complex cases accessible from the ventricle where the pituitary complex cannot be confidently preserved via a transsphenoidal approach, an interhemispheric approach is also a practical initial surgical option. In addition to providing diagnostic value, molecular profiling may also reveal therapeutically significant gene alterations such as BRAF mutations.
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  • 文章类型: Journal Article
    嗜酸性粒细胞性血管中心纤维化(EAF)是一种罕见的累及鼻腔的进行性纤维化病变,鼻旁窦,和上呼吸道。很少有报道说它很少涉及轨道;然而,没有颅内受累的报告.这里,我们报告并分享了罕见的原发性颅内EAF病例的经验。一位33岁的女性,有鞍上肿块的病史,手术和药物治疗失败。体格检查显示右侧失明和上睑下垂,左侧视力下降,和视野缺陷。脑成像显示轴外硬膜内清晰的大的鞍上肿块,具有鞍旁(右侧更多)和鞍后延伸。通过翼点开颅术和额叶下入路,一个非常坚定的奶油褐色明确定义的纤维化肿块。肿瘤质地太硬,无法完全切除。显微镜离开了手术区,而肿瘤是用骨钳切除的.组织病理学发现有利于EAF。进一步的组织病理学评估未能显示IgG4相关疾病的组织学特征。虽然EAF的术前诊断是不可能的,在MRI(磁共振成像)的T2图像序列上显示低张力的惰性缓慢生长病变的背景下,EAF应被视为鉴别诊断。在这种诊断的设置中,应评估用于诊断IgG4-RD的全身和其他器官受累情况.然而,需要更多的案例来说明这两个实体之间的关系。
    Eosinophilic angiocentric fibrosis (EAF) is a rare progressive fibrosing lesion involving the nasal cavity, paranasal sinuses, and the upper respiratory tract. There are few reports that it rarely involves the orbit; however, there is no report of intracranial involvement. Here, we report and share our experience with a rare case of primary intracranial EAF. A 33-year-old woman with a history of a suprasellar mass and unsuccessful surgical and medical treatment referred to us. Physical examination demonstrated right-sided blindness and ptosis, left-sided decreased visual acuity, and visual field defect. The brain imaging revealed an extra-axial intradural well-defined large suprasellar mass with parasellar (more on the right side) and retrosellar extension. Via pterional craniotomy and subfrontal approach, a very firm creamy-brownish well-defined fibrotic mass was encountered. The tumour texture was too firm to be totally resected. The microscope exited the surgical field off, and the tumour was incompletely resected using a rongeur. The histopathology finding favoured EAF. Further histopathology evaluation failed to show histologic features of IgG4-related disease. Although the preoperative diagnosis of EAF is impossible, in the setting of an indolent slow-growing lesion demonstrating hypointensity on the T2 image sequence of MRI (magnetic resonance imaging), EAF should be considered a differential diagnosis. In the setting of this diagnosis, the systemic and other organ involvement for a diagnosis of IgG4-RD should be evaluated. However, more cases are needed to illustrate the relation between these two entities.
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  • 文章类型: Journal Article
    塞勒,鞍上,而星际空间包含了大量的病状,包括肿瘤,先天性,血管,炎症,和传染性病因。症状,如果存在,包括头痛的组合,眼睛疼痛,眼肌麻痹,视野缺损,颅神经病,和内分泌表现。特别关注CT和MRI的关键特征,这些特征可以帮助区分不同的病理。虽然大多数病变最终需要进行组织病理学评估,颅底解剖的专业知识与关键影像学特征的认识相结合,可用于限制鉴别诊断和指导管理。外科技术,包括鼻内镜和经颅神经外科手术入路的详细描述。
    The sellar, suprasellar, and parasellar space contain a vast array of pathologies, including neoplastic, congenital, vascular, inflammatory, and infectious etiologies. Symptoms, if present, include a combination of headache, eye pain, ophthalmoplegia, visual field deficits, cranial neuropathy, and endocrine manifestations. A special focus is paid to key features on CT and MRI that can help in differentiating different pathologies. While most lesions ultimately require histopathologic evaluation, expert knowledge of skull base anatomy in combination with awareness of key imaging features can be useful in limiting the differential diagnosis and guiding management. Surgical techniques, including endoscopic endonasal and transcranial neurosurgical approaches are described in detail.
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  • 文章类型: Case Reports
    The pedicled nasoseptal flap (NSF) is the mainstay for endoscopic skull base reconstruction. We present a novel technique using a semirigid chondromucosal NSF that improves the reinforcement and protection of intracranial structures.
    Composite NSFs were performed to repair intraoperative high-flow cerebrospinal fluid leaks in 2 patients who had undergone endoscopic endonasal resection of a suprasellar mass. The surgical technique and postoperative outcomes are described.
    The flaps were sufficient for defect coverage, and the patients did not experience any cerebrospinal fluid leak in the immediate and delayed postoperative periods. No complications related to the composite flap had developed.
    The composite chondromucosal NSF is a reliable reconstruction option for select ventral cranial base reconstruction cases with the potential to improve the protection of intracranial structures. Additional surgical cases and longer follow-up are required for a better assessment of long-term outcomes.
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  • 文章类型: Journal Article
    UNASSIGNED: To illustrate the structure-function relationship of compressive optic neuropathy (CON) at the time of diagnosis.
    UNASSIGNED: Thirty-two eyes of newly diagnosed suprasellar CON and 60 healthy eyes were included in the study. The peripapillary retinal nerve fiber layer (RNFL) thickness and macular ganglion cell-inner plexiform layer (GCIPL) thickness were obtained using Cirrus spectral domain optical coherence tomography (SD-OCT). CON eyes were stratified based on the similar degree and pattern of both RNFL and GCIPL.
    UNASSIGNED: From 32 eyes of newly diagnosed suprasellar CON eyes, 27 eyes had a predominantly nasal hemiretina thinning of macular GCIPL, 4 eyes showed a generalized macular thinning, and 1 eye showed a predominantly superior macular thinning. The corresponding temporal peripapillary RNFL thinning with nasal hemiretina GCIPL thinning were inconsistently manifested. Structure-function analysis of stratified CON eyes with similar thinning profiles showed that a range rather than a fixed value of visual field loss based on mean deviation (MD) index was associated to each thinning profile. The maximal limit of visual field loss range was ubiquitously nonrestricted to any structural thinning profile. While the minimal limit of the associated MD range was gradually reduced from 0 to about -16.0 dB, the nasal hemiretina macular GCIPL thinning was the only manifestation and decreased from 75 to 45 µm. However, the different degrees of temporal hemiretina macular GCIPL and superior-inferior peripapillary RNFL thinning were only seen in 10 of 32 eyes of which their nasal hemiretina GCIPL and temporal RNFL thinning had reached significant thinning. Interestingly when present, the minimal limit of associated MD range continued to decrease from -16.0 to -32.0 dB.
    UNASSIGNED: CON eyes can present with variable structure and function relationship at the time of diagnosis. Using structural parameters at the time of diagnosis to predict the prognosis should be used with caution.
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  • 文章类型: Case Reports
    海绵状畸形是可在整个中枢神经系统中出现的良性血管病变。视神经或交叉内海绵状畸形的发生,然而,是非常罕见的。该视频中描述的病例涉及一名36岁的妇女,她在接受左额颞骨开颅手术切除视神经海绵状畸形后3个月出现。她最初因视力丧失而去了外面的医院,确认并切除左侧视神经病变。尽管据报道她的视力在术后略有改善,3个月后,她因双侧主观模糊和新的视野缺陷而醒来。磁共振成像显示左光学装置出血性病变扩大,对应于残余海绵状畸形。考虑到出血复发和相关的视觉症状,患者接受了重做左额颞部开颅手术切除视神经和交叉损伤。组织病理学评估显示厚壁血管具有局灶性神经胶质组织,肿瘤细胞的缺失,出血,与海绵状畸形一致。患者对手术耐受良好。术后,她的视觉症状立即得到改善。她在术后第3天出院回家,通过手术后14个月的最后一次眼科预约,她的双时视野缺损继续逐步改善。术后和随后的监测神经影像学显示海绵状畸形完全切除,没有复发的证据。这种情况证明了用于确保在这个非常雄辩的区域中完全切除畸形的技术。患者同意发表。
    Cavernous malformations are benign vascular lesions that can arise throughout the central nervous system. The occurrence of a cavernous malformation within the optic nerve or chiasm, however, is extremely uncommon. The case described in this video involved a 36-yr-old woman who presented 3 mo after undergoing a left frontotemporal craniotomy for resection of an optic nerve cavernous malformation. She had initially presented to an outside hospital with vision loss, and the left optic nerve lesion was identified and resected. Although her vision had reportedly improved slightly postoperatively, she awoke 3 mo later with bilateral subjective blurriness and new visual field deficits. Magnetic resonance imaging revealed enlargement of the left optic apparatus hemorrhagic lesion, corresponding to residual cavernous malformation. Given the recurrence of hemorrhage and the associated visual symptoms, the patient underwent a redo left frontotemporal craniotomy for resection of the optic nerve and chiasmal lesion. Histopathologic evaluation revealed thick-walled vessels with focal intervening glial tissue, an absence of neoplastic cells, and hemorrhage, consistent with a cavernous malformation. The patient tolerated the procedure well. Postoperatively, she experienced immediate amelioration in her visual symptoms. She was discharged home on postoperative day 3, and her bitemporal visual field deficit continued to progressively improve through her last ophthalmologic appointment 14 mo after surgery. Postoperative and subsequent surveillance neuroimaging demonstrated complete resection of the cavernous malformation without evidence of recurrence. This case demonstrates the techniques utilized to ensure complete resection of the malformation in this very eloquent region. The patient provided consent for publication.
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  • 文章类型: Journal Article
    The orbitopterional approach provides an excellent combination of basal access and suprasellar access. This approach also allows for less brain retraction when resecting larger suprasellar tumors that are more superiorly projecting due to a more frontal and inferior trajectory. In this operative video, the authors thoroughly detail an orbitopterional craniotomy utilizing a one-piece modified orbitozygomatic technique. This technique involves opening the craniotomy through a standard pterional incision. The craniotomy is performed using the standard three burr holes of a pterional approach; however, the osteotomy is extended anteriorly through the frontal process of the zygomatic bone as well as through the supraorbital rim. In this operative video atlas, the authors illustrate the operative anatomy, as well as surgical strategy and techniques to resect a large suprasellar craniopharyngioma in a 4-year-old male. Other reasonable approach options for a lesion of this size would include a standard pterional approach, a supraorbital approach, or expanded endoscopic transsphenoidal approach. The lesion was quite high and thus, the supraorbital approach may confine access to the superior portion of the tumor. While recognizing that some groups may have chosen the endoscopic expanded transsphenoidal approach for this lesion, the authors describe more confidence in achieving the goal of a safe and maximal resection with the orbitopterional approach. The link to the video can be found at: https://youtu.be/eznsK16BzR8 .
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  • 文章类型: Case Reports
    Hemangioblastomas are rare vascular tumors most often found in the posterior fossa and cervical spinal cord and commonly associated with von Hippel-Lindau Disease. We report a case of sporadic hemangioblastoma in a patient without von Hippel-Lindau Disease. Imaging characteristics included a solid, suprasellar mass that was homogeneously enhancing. These findings most resembled a pituicytoma or choroid glioma because of the close association with the infundibulum and the homogeneous avid enhancement. Microscopically, the neoplasm was seen to be composed of vascular channels associated with foamy stromal cells, containing clear cytoplasmic vacuoles. Microscopic and immunohistochemical findings were consistent with hemangioblastoma. Hemangioblastomas are a rare form of vascular tumor most commonly associated with von-Hippel Lindau disease. Our finding of non-cystic hemangioblastoma arising from the infundibulum demonstrates that, while rare, hemangioblastomas should be considered on the differential diagnosis for an avidly enhancing suprasellar mass.
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  • 文章类型: Journal Article
    Masses in the sella and parasellar region comprise about 10% of all pediatric brain tumors but type and frequency differs from those in adults. Imaging is critical for diagnosis and characterization of these lesions. By assessing the site of origin, signal and contrast enhancement characteristics, and the presence or absence of characteristic patterns, differential diagnosis can narrow the possibilities. The clinical presentation is often characteristic for lesion type and should be considered. This article summarizes the characteristic imaging features of the most frequent pediatric tumors and tumor-mimicking lesions in children in this region.
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  • 文章类型: Case Reports
    Desmoplastic small round cell tumors (DSRCTs) are rare, aggressive neoplasms that typically arise from abdominal and pelvic peritoneum in young adults. Other primary sites are uncommon, and an intracranial origin is exceptionally rare. Here the authors report the first case of a DSRCT presenting as a primary suprasellar tumor causing panhypopituitarism and severe bitemporal hemianopia in a young man. Macroscopic debulking of the tumor was undertaken, and histology revealed features of DSRCT. Reverse transcription polymerase chain reaction confirmed the presence of Ewing\'s sarcoma-Wilms tumor 1 (EWS-WT1) gene rearrangement specific to DSRCT. Postoperative whole-body imaging showed no primary malignancy elsewhere. The tumor recurred 4 months after surgery, and this was followed by cervical and mediastinal lymph node metastases. The patient died 20 months after initial presentation of rapidly progressive disease. DSRCTs should be included in the differential diagnosis of an unusual suprasellar mass in young adults. Early diagnosis is essential, and once the tumor is identified histologically, gross-total resection and radical postoperative treatment involving radiotherapy, chemotherapy, and close surveillance are required because of the lesion\'s potential for rapidly progressive malignancy.
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