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: Case Reports
    在具有适当流行病学危险因素的患者中,应将神经囊虫病作为鞍上或鞍旁肿块病变的鉴别诊断的一部分。由于神经影像学检查结果可能是非特异性的,血清学可能会有所帮助,但是当仍然有疑问时,脑活检,和组织病理学可能需要做出正确的诊断。
    神经囊虫病(NCC)是一种有据可查的中枢神经系统蠕虫感染,经常在发展中国家观察到。NCC感染的已知部位包括高血管灰白质交界处,基底水箱,脑实质,蛛网膜下腔,心室系统,和脊髓。此病例突出显示了鞍上区域内NCC感染的罕见但有趣的部位,表现为与鞍上肿块或病变相似的临床和影像学特征。这位44岁的女性最初抱怨头痛和恶心,持续了5年,并发展为视力问题和短期记忆丧失。最初怀疑是颅咽管瘤,基于部分钙化的鞍上肿瘤的影像学表现。然而,通过组织病理学和血清学检测囊尾蚴IgG抗体阳性证实囊尾蚴病。患者成功接受了阿苯达唑和逐渐减少剂量的类固醇治疗,这改善了她的表现症状并解决了先前的影像学发现。此病例提醒在鞍区和鞍区上肿块或病变的鉴别诊断中考虑NCC,特别是当存在流行病学危险因素时。
    UNASSIGNED: In patients with appropriate epidemiological risk factors, neurocysticecosis should be considered as part of the differential diagnosis of suprasellar or parasellar mass lesions. As neuroimaging findings can be nonspecific, serology may be helpful, but when still in doubt, brain biopsy, and histopathology may be necessary to make the correct diagnosis.
    UNASSIGNED: Neurocysticercosis (NCC) is a well-documented central nervous system helminth infection that is, frequently observed in developing countries. Known sites of NCC infection include the highly vascular gray-white matter junction, basal cistern, brain parenchyma, subarachnoid space, ventricular system, and spinal cord. This case highlights an uncommon yet intriguing site of NCC infection within the suprasellar area, which presented with similar clinical and imaging characteristics as suprasellar masses or lesions. The 44-year-old female initially complained of headaches and nausea that persisted for 5 years and progressed to vision problems and short-term memory loss. A craniopharyngioma was initially suspected, based on imaging findings of a partially calcified suprasellar tumor. However, cysticercosis was confirmed by histopathology and serological testing positive for Cysticercus IgG antibodies. The patient was successfully treated with albendazole and tapering doses of steroids, which improved her presenting symptoms and resolved prior imaging findings. This case serves as a reminder to consider NCC in the differential diagnosis of sellar and suprasellar masses or lesions, particularly when an epidemiologic risk factor is present.
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  • 文章类型: Case Reports
    浆细胞瘤是作为孤立性病变或与多发性骨髓瘤结合出现的浆细胞的集合。颅内位置并不常见,但应考虑为不同的管理。鞍上区域的浆细胞瘤很少见,但在鉴别诊断鞍上肿块时应予以考虑。临床表现和影像学表现在垂体腺瘤和浆细胞瘤之间有相似性和重叠,所以诊断取决于活检和病理评估。由于与腺瘤的结构相似性,免疫组织学染色通常是必要的。孤立的病例可以单独使用放射治疗,并且由于肿块效应而保留手术治疗症状。如果有多发性骨髓瘤的证据,则给予全身治疗。在这个案例报告中,我们介绍了一名52岁的男性,他表现出与头痛和鼻出血相关的视力模糊恶化,持续4个月。头部CT显示大肿块,累及蝶鞍和颅底。实验室显示钙正常,肌酐,和完整的垂体功能。肿块的活检最初被诊断为垂体腺瘤,但重复病理显示浆细胞瘤。身体影像学显示弥漫性溶解性病变。骨髓活检和血清电泳与多发性骨髓瘤的诊断一致。患者接受鞍上肿块放射治疗,然后用硼替佐米全身治疗多发性骨髓瘤,来那度胺,还有地塞米松.患者获得了非常好的部分反应。
    Plasmacytomas are a collection of plasma cells that occur as a solitary lesion or in conjunction with multiple myeloma. Intracranial location is uncommon but should be considered as management differs. Plasmacytomas in the suprasellar region are rare but should be considered in the differential diagnosis of suprasellar masses. Clinical presentation and imaging findings have similarities and overlap between pituitary adenomas and plasmacytomas, so the diagnosis depends on biopsy and pathological evaluation. Immunohistological staining is often necessary due to structural similarities to adenomas. Isolated cases may be treated with radiation alone and surgery is reserved for symptoms due to mass effect. Systemic therapy is given if there is evidence of multiple myeloma. In this case report, we present a 52-year-old male who presented with worsening blurry vision associated with headaches and epistaxis of four months duration. CT of the head showed a large mass involving the sella and skull base. Labs showed normal calcium, creatinine, and intact pituitary function. Biopsy of the mass was initially diagnosed as a pituitary adenoma but repeat pathology revealed plasmacytoma. Body imaging revealed diffuse lytic lesions. Bone marrow biopsy and serum electrophoresis were consistent with a diagnosis of multiple myeloma. The patient underwent radiation therapy to the suprasellar mass followed by systemic therapy for multiple myeloma with bortezomib, lenalidomide, and dexamethasone. The patient achieved a very good partial response.
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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: Case Reports
    Erdheim-Chester病(ECD)是一种罕见的全身性疾病,其特征是非朗格汉斯组织细胞增生症。垂体受累,经常表现为尿崩症,是最常见的中枢神经系统(CNS)病变。然而,压缩光学设备的大量质量形成很少报道。我们介绍了一例ECD相关的鞍上肿块,经鼻内窥镜入路治疗,重点是手术策略和术中发现。质量是纤维状的,非常困难,并牢固地粘附在视神经上,导致视力障碍。在保留肿块和视神经之间的粘连的情况下进行了次全切除术,手术后她的视觉症状明显改善.我们强调ECD相关鞍上肿块的外科手术,从内窥镜的角度来看。由于质量与周围光学设备和穿孔器的牢固粘附,完全切除可能是有害的;明智的肿块减少并保留这种粘连将有助于更好的视觉效果。
    Erdheim-Chester disease (ECD) is a rare systemic disease characterized by non-Langerhans histiocytosis. Pituitary involvement, often manifesting as diabetes insipidus, is the most common central nervous system (CNS) lesion. However, significant mass formation compressing the optic apparatus is rarely reported. We present a case of ECD-related suprasellar mass treated with an endoscopic transnasal approach, with emphasis on the surgical strategy and the intraoperative findings. The mass was fibrous, significantly hard, and strongly adhered to the optic nerves, causing visual impairment. A subtotal resection was performed with preserving the adhesion between the mass and the optic nerves, and her visual symptoms improved remarkably after surgery. We highlight the surgical procedure of ECD-related suprasellar mass, from an endoscopic point of view. Due to strong adhesion of the mass to the surrounding optic apparatus and perforators, complete resection may be harmful; judicious mass reduction with preserving such adhesion would contribute to better visual outcomes.
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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
    Myxedema coma and pituitary apoplexy are well-known life-threatening endocrine emergencies. The coincidence of these entities is exceedingly rare. Myxedema coma occurring as a result of pituitary lesion is a much less seen entity. A high index of suspicion is often required for early diagnosis as it is of particular importance in improving survival outcomes. We present a rare case of a patient with myxedema coma presenting as bradycardia and hypotension secondary to pituitary apoplexy, which was confirmed on magnetic resonance imaging (MRI). The patient was managed conservatively with levothyroxine and stress doses of steroid, with the resolution of hemodynamic changes and a decrease in the size of the suprasellar mass.
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  • 文章类型: Case Reports
    原发性垂体炎是指与其他次要原因无关的垂体的孤立炎症。在其组织病理学亚型中,黄瘤是最罕见的。
    我们描述了一位22岁的女性,患有黄瘤性垂体炎(XH),其8年的临床进展以及泼尼松龙和硫唑嘌呤的治疗效果。我们的患者在14岁时因严重身材矮小和青春期延迟而首次转诊。
    调查显示多种垂体缺陷。磁共振成像显示垂体肿块,因此进行了部分切除。由于肿块的位置,完全切除是不可行的。组织病理学分析与XH一致。肿瘤二次锻炼的结果,感染,自身免疫,炎症性疾病呈阴性。手术后,观察到肿块逐渐增大。给予两个疗程的泼尼松龙,质量显着减少。添加硫唑嘌呤是由于泼尼松龙逐渐减少时的不持续作用以及继续使用类固醇毒性的担忧。使用硫唑嘌呤6个月后,没有发现质量进一步增加。
    糖皮质激素和免疫疗法是XH的治疗选择;然而,需要更多的病例来更好地了解其发病机制和临床进展。
    UNASSIGNED: Primary hypophysitis refers to the isolated inflammation of the pituitary gland not associated with other secondary causes. Among its histopathologic subtypes, xanthomatous is the rarest.
    UNASSIGNED: We describe a 22-year-old woman with xanthomatous hypophysitis (XH), its clinical progression over 8 years as well as the treatment effects of prednisolone and azathioprine. Our patient was first referred for severe short stature and delayed puberty at the age of 14 years.
    UNASSIGNED: Investigations revealed multiple pituitary deficiencies. Magnetic resonance imaging showed a pituitary mass whereby a partial resection was performed. A full resection was not feasible due to the location of the mass. The histopathologic analysis of the tissue was consistent with XH. The results of secondary workout for neoplasm, infection, autoimmune, and inflammatory disorders were negative. After surgery, a progressive enlargement of the mass was observed. Two courses of prednisolone were administered with a significant reduction in the mass size. Azathioprine was added due to the unsustained effects of prednisolone when tapered off and the concern of steroid toxicity with continued use. No further increase in the mass size was noted after 6 months on azathioprine.
    UNASSIGNED: Glucocorticoid and immunotherapy are treatment options for XH; however, more cases are needed to better understand its pathogenesis and clinical progression.
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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: Case Reports
    BACKGROUND: Hamartoma of the hypothalamus represents a well-known but rare cause of central precocious puberty and gelastic epilepsy. Due to the delicate site in which a tumor is located, surgery is often difficult and associated with considerable risks.
    METHODS: 10-Year old girl presented with early and regular menstruation at the age of 1 year each cycle lasted for 3 days. She had developed breast, axillary and pubic hair at the age of five, and seven years respectively, with history of difficulty in speech especially articulation and epilepsy since childhood for which she is on medications. She had attacks of an inappropriate laugh. The laboratory tests were consisted with central precocious puberty, MRI shows suprasellar mass. She received leuprolide and antiepileptic medicines until surgery planned. Surgery was done with complete resection, with histopathology showing hypothalamic hamartoma. After surgery, there was complete remission of seizure. She developed recurrence 4 years later and she is currently on anticonvulsant medications with few attacks of convulsions per week, and she has intellectual disabilities and low school performance.
    CONCLUSIONS: The treatment of hypothalamic hamartoma associated with generalized epilepsy has been found to improve seizures and behavioral disturbances with an acceptable morbidity rate by using a variety of surgical approaches. Partial resection of a tumor may be sufficient to reduce seizure frequency and to improve behavior and quality of life with few side effects. Best outcomes are achieved when the patients are managed by an experienced multidisciplinary team and lifelong follow up is recommended.
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