macroadenoma

大腺瘤
  • 文章类型: Journal Article
    背景:催乳素瘤是最常见的垂体腺瘤,目前的治疗方案包括多巴胺激动剂治疗(DA)和手术[17]。由于以前的DA治疗引起的肿瘤纤维化,手术切除可能是具有挑战性的。因此,这项研究调查了术前DA的使用如何影响泌乳素瘤的围手术期治疗和手术结局,旨在确定特定的泌乳素瘤患者亚组是否可以从独家手术干预中获得更大的益处.
    方法:我们回顾性分析了2013-2022年在我们机构中,经手术治疗和组织学证实的n=159例鞍区泌乳素腺瘤。临床,分析了放射学和手术特点。进行单变量和多变量分析。
    结果:在159例催乳素瘤患者中,83.6%曾接受DA治疗,随后接受手术治疗,而只有16.4%的人接受了独家手术。两组的初始肿瘤体积相似(1.9cm3与1.5cm3,p=0.59)和术前催乳素水平(PRL)相等(199.7µg/l与191.0微克/升,p=0.44)。当患者接受先前的DA治疗时,外科手术花费的时间明显更长(79分钟。vs.70分钟,p=0.0479)。手术后六个月,与未接受治疗的患者相比,接受治疗的患者的PRL明显更高(107g/lvs.8.64µg/,p=0.0009)。此外,未经治疗的微泌乳素瘤缓解了100%,而预处理显示88.75%的缓解率。
    结论:当前的研究表明,先前的DA治疗与明显延长的手术有关,较高的复发率和术后PRL水平正常化率较低,特别是在微泌乳素瘤中,并支持垂体学会2023年共识声明的最新建议,该声明主张选择单独手术作为微泌乳素瘤的一线治疗。
    BACKGROUND: Prolactinoma account to the most common pituitary adenomas and current therapy regime constitutes of dopamine agonist therapy (DA) and surgery in selected cases [17]. Due to tumor fibrosis induced by previous DA therapy, surgical removal can be challenging though. Therefore, this study investigates how preoperative DA usage influences perioperative treatment and surgical outcome in prolactinoma and aims to ascertain whether a specific subgroup of prolactinoma patients could derive greater benefit from exclusive surgical intervention.
    METHODS: We retrospectively analyzed n = 159 surgically treated and histologically confirmed prolactinomas in the sella region from 2013-2022 in our institution. Clinical, radiological and surgical features were analyzed. Univariate and multivariate analyses were performed.
    RESULTS: Out of total of 159 prolactinoma patients, 83.6% received previous treatment with DA followed by surgery, while only 16.4% received exclusive surgery. Both groups presented similar initial tumor volumes (1.9cm3 vs. 1.5cm3, p = 0.59) and equal preoperative prolactin levels (PRL) (199.7 µg/l vs. 191.0 µg/l, p = 0.44). Surgical procedures took significantly longer when patients received prior DA treatment (79 min. vs. 70 min., p = 0.0479). Six months after surgery, pretreated patients revealed significantly higher PRL compared to non-treated (107 g/l vs. 8.64 µg/, p = 0.0009). Additionally, untreated microprolactinoma presented a remission of 100%, whereas pretreated exhibited a remission rate of 88.75%.
    CONCLUSIONS: The current study demonstrates that prior DA treatment is associated with significantly longer surgeries, higher recurrence rates and lower rates of normalization of PRL levels after surgery, particularly in microprolactinomas and support the latest recommendations of the Pituitary Society\'s Consensus Statement 2023, which favors the option of surgery alone as first-line therapy for microprolactinomas.
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  • 文章类型: Journal Article
    背景:库欣病(CD)的垂体卒中(PA)很少见,数据仅限于病例报告/系列。
    方法:我们回顾性回顾了1987年至2023年在我们中心管理的CD中PA的病例记录,并进行了系统的文献综述。
    结果:我们确定了58名患者(44名女性),包括我们中心的十二张(12/315CD,CD中PA的患病率为3.8%),系统评价为46。PA诊断时的中位年龄为35岁。最常见的表现是A型(79.3%),症状是头痛(89.6%)。垂体卒中评分(PAS)中位数为2。皮质醇和ACTH水平中位数分别为24.9µg/dl和94.1pg/ml,分别。在55.2%的病例中,中风是基础CD的首发表现,31.1%(14/45)出现低皮质醇血症(血清皮质醇≤5.0µg/dl),强调识别皮质醇增多症的临床体征/症状的重要性。中位最大肿瘤尺寸为1.7cm(53/58为大腺瘤)。在57.8%的病例中,PA通过手术治疗,其余的保守管理。所有5例有微腺瘤的CD患者均通过保守治疗获得缓解,虽然两个后来复发。在患有大腺瘤的初治CD患者中,手术组和保守组之间PA相关的神经缺陷改善相当。然而,更大比例的手术管理患者在缓解时间更长(70%vs.38.5%;p=0.07),平均31vs.10.5个月。
    结论:CD中的PA更常与大腺瘤相关,可能存在低皮质醇血症,和手术治疗倾向于更高和更持久的缓解率。
    BACKGROUND: Pituitary apoplexy (PA) in Cushing\'s disease (CD) is rare with data limited to case reports/series.
    METHODS: We retrospectively reviewed case records of PA in CD managed at our center from 1987 to 2023 and performed a systematic literature review.
    RESULTS: We identified 58 patients (44 females), including twelve from our center (12/315 CD, yielding a PA prevalence in CD of 3.8%) and forty six from systematic review. The median age at PA diagnosis was 35 years. The most common presentation was type A (79.3%) and symptom was headache (89.6%), with a median Pituitary Apoplexy Score (PAS) of 2. Median cortisol and ACTH levels were 24.9 µg/dl and 94.1 pg/ml, respectively. Apoplexy was the first manifestation of underlying CD in 55.2% of cases, with 31.1% (14/45) presenting with hypocortisolemia (serum cortisol ≤ 5.0 µg/dl), underscoring the importance of recognizing clinical signs/symptoms of hypercortisolism. The median largest tumor dimension was 1.7 cm (53/58 were macroadenomas). PA was managed surgically in 57.8% of cases, with the remainder conservatively managed. All five PA cases in CD with microadenoma achieved remission through conservative management, though two later relapsed. Among treatment-naïve CD patients with macroadenoma, PA-related neuro-deficit improvement was comparable between surgical and conservative groups. However, a greater proportion of surgically managed patients remained in remission longer (70% vs. 38.5%; p = 0.07), for an average of 31 vs. 10.5 months.
    CONCLUSIONS: PA in CD is more commonly associated with macroadenomas, may present with hypocortisolemia, and surgical treatment tends towards higher and longer-lasting remission rates.
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  • 文章类型: Case Reports
    巨大泌乳素瘤很少见;其中,淀粉样变异体,有大量球形淀粉样蛋白沉积的泌乳素瘤,是罕见的,仅有30例报告与常规泌乳素腺瘤的医疗管理相反的手术治疗建议。
    我们在此报告一例巨大的淀粉样性泌乳素瘤,患者32岁,在临床上表现非常不典型,放射学,和病理特征,并像正常泌乳素瘤一样对多巴胺激动剂治疗产生反应。
    淀粉样巨大催乳素瘤是罕见的。与通常的信念相反,即使他们仍然有医疗反应;然而,需要更多的文献来决定他们的理想管理。
    UNASSIGNED: Giant prolactinomas are rare; among them, the amyloidogenic variant, prolactinomas with extensive spherical amyloid deposits, are rare, with only 30 cases reported with recommendations of surgical management contrary to the routine prolactinoma\'s medical management.
    UNASSIGNED: We report here a case of giant amyloidogenic prolactinoma in a 32-year-old male patient who had a very atypical presentation in terms of clinical, radiological, and pathological features and responded to dopamine agonist therapy like a normal prolactinoma.
    UNASSIGNED: Amyloidogenic giant prolactinomas are rare. Contrary to usual belief, even they remain medically responsive; however, more literature is required to decide their ideal management.
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  • 文章类型: Case Reports
    垂体腺瘤可以出现或没有最小的视觉障碍。我们介绍了一例40岁的男性垂体腺瘤,尽管在初始视野测试中检测到最小的不对称非特异性变化,但在光学相干断层扫描(OCT)上突出显示双颞部乳头周围视网膜神经纤维层(NFL)变薄是交叉损伤的主要标志。双时NFL变薄促使MRI进一步评估,这证实了垂体大腺瘤的存在。尽管有巨大的腺瘤,卡麦角林治疗导致消退,和病人的视野改善。该病例强调了OCT在检测与垂体瘤相关的细微结构变化方面的重要性,因为它可以促进早期诊断和及时干预以获得最佳的视觉结果。
    Hypophyseal adenomas can present with or without minimal visual disturbances. We present a case of a 40-year-old male with a hypophyseal adenoma, highlighting bitemporal peripapillary retinal nerve fiber layer (NFL) thinning on optical coherence tomography (OCT) as a major sign of chiasmal damage despite minimal asymmetrical nonspecific changes detected on initial visual field testing. The bitemporal NFL thinning prompted further evaluation with MRI, which confirmed the presence of a macroadenoma of the hypophysis. Despite the large adenoma, treatment with cabergoline led to regression, and the patient\'s visual field improved. This case underscores the importance of OCT in detecting subtle structural changes associated with pituitary tumors, as it can facilitate early diagnosis and prompt intervention for optimal visual outcomes.
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  • 文章类型: Journal Article
    无功能垂体神经内分泌肿瘤患者的术后肿瘤进展是可变的。这项研究的目的是使用机器学习(ML)模型来改善NFPitNET患者术后结局的预测。我们研究了383例接受或不接受放疗的患者的数据,随访期在6个月至15年之间。ML模型,包括k-最近邻(KNN),支持向量机(SVM),决策树,与使用逻辑回归的参数统计模型相比,在预测肿瘤进展方面表现出优异的性能,与SVM实现最高性能。肿瘤进展的最强预测指标是手术切除的程度,患者年龄,肿瘤体积,放疗的使用也显示出影响。完全切除后,没有特征显示与肿瘤复发有关。总之,这项研究证明了ML模型在预测NFPitNET患者术后结局方面的潜力.未来的工作应该包括额外的,更颗粒状,多中心数据,包括合并成像和手术视频数据。
    Post-operative tumour progression in patients with non-functioning pituitary neuroendocrine tumours is variable. The aim of this study was to use machine learning (ML) models to improve the prediction of post-operative outcomes in patients with NF PitNET. We studied data from 383 patients who underwent surgery with or without radiotherapy, with a follow-up period between 6 months and 15 years. ML models, including k-nearest neighbour (KNN), support vector machine (SVM), and decision tree, showed superior performance in predicting tumour progression when compared with parametric statistical modelling using logistic regression, with SVM achieving the highest performance. The strongest predictor of tumour progression was the extent of surgical resection, with patient age, tumour volume, and the use of radiotherapy also showing influence. No features showed an association with tumour recurrence following a complete resection. In conclusion, this study demonstrates the potential of ML models in predicting post-operative outcomes for patients with NF PitNET. Future work should look to include additional, more granular, multicentre data, including incorporating imaging and operative video data.
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  • 文章类型: Case Reports
    一名60岁的妇女,有先前诊断的鞍区肿块和最近发作的严重头痛的病史,视力丧失,和头晕。患者在影像学上发现有较大的肿块并伴有曲线钙化。组织病理学证实存在异常腺泡的垂体腺瘤,与腺瘤一致,和中等数量的颗粒状嗜酸性细胞浆。对钙化模式和放射学形态的详细分析对于区分垂体腺瘤和颅咽管瘤至关重要。识别这些模式可以帮助区分这些条件,提供更准确的诊断和有效的治疗方案。
    A 60-year-old woman presented with a history of a previously diagnosed sellar mass and a recent onset of severe headache, vision loss, and dizziness. The patient was found to have a large mass with curvilinear calcification on imaging. Histopathology confirmed the presence of a pituitary adenoma with abnormal acini, consistent with adenoma, and moderate amounts of granular eosinophilic cytoplasm. A detailed analysis of the patterns of calcification and the radiological morphology is crucial to distinguishing between pituitary adenoma and craniopharyngioma. Recognition of these patterns can aid in distinguishing between these conditions, providing a more accurate diagnosis and an effective treatment plan.
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  • 文章类型: Observational Study
    视觉功能障碍是无功能垂体大腺瘤(NFPM)患者的普遍症状;OCT在此类患者中的作用尚未确定。这是一项为期6年的前瞻性纵向观察研究,在20例视交叉的放射学压缩而没有视敏度(VA)和视野(VF)障碍的患者中。主要终点是评估NFPA对神经轴突丢失的影响,方法是使用包含时(T0)的OCT测量RNFL厚度,12个月(T1),24个月(T2),36个月(T3),分别。次要终点是监测OCT随时间的演变,并评估OCT改变程度与放射学和临床视交叉压迫综合征程度之间的任何关系。在包括的20名患者中,八个(40%)在诊断时显示RNFL-OCT改变,而其余12人(60%)显示正常模式。在平均60个月的眼科随访中,4例患者(20%)表现出无症状的RNFL-OCT厚度减少,尽管所有20例患者的VA/VF稳定。据我们所知,这项研究是首次尝试纵向评估放射学无症状交叉压迫综合征患者RNFL-OCT的自然史和演变。结果并未清楚地表明OCT作为视觉功能障碍的早期预后因素的作用。
    Visual dysfunction is a prevalent symptom in patients with non-functioning pituitary macroadenoma (NFPM); the role of OCT in such patients has not been yet determined. This is a prospective longitudinal observational study over a period of 6 years, on 20 patients presenting a radiological compression of the optic chiasma without visual acuity (VA) and visual field (VF) disturbances. The primary endpoint was to evaluate the impact of NFPA on neuro-axonal loss by measuring RNFL thickness using OCT at inclusion (T0), 12 months (T1), 24 months (T2), and 36 months (T3), respectively. The secondary endpoint was to monitor the evolution of OCT over time and assess any relationship between the degree of OCT alteration and the degree of radiological and clinical optic chiasm compression syndrome. Among the 20 patients included, eight (40%) showed an altered RNFL-OCT at diagnosis, while the remaining 12 (60%) showed a normal pattern. During a mean ophthalmologic follow-up of 60 months, 4 patients (20%) presented an asymptomatic reduction of RNFL-OCT thickness although all 20 had a VA/VF stable. To our knowledge, this study represents the first attempt to longitudinally evaluate the natural history and evolution of RNFL-OCT in patients with radiologically asymptomatic chiasmatic compression syndrome. The results do not clearly demonstrate the role of the OCT as an early prognostic factor for visual dysfunction.
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  • 文章类型: Case Reports
    垂体炎是一种极其罕见的炎症性疾病,可以模仿垂体腺瘤的临床和放射学特征。在这个案例报告中,我们描述了一名45岁女性,患有继发性黄色肉芽肿性垂体炎(XGH),表现为垂体大腺瘤.病人抱怨头痛,视力障碍,闭经-溢乳综合征.体检正常。实验室调查显示促肾上腺皮质激素,促甲状腺激素,和促性腺激素缺乏。她的右眼视力也很低,视野也改变了。垂体磁共振成像显示鞍内和鞍上肿块13×11×16mm,伴有出血性坏死,这对患者的视交叉和垂体柄有离散的质量影响。患者接受了氢化可的松和左甲状腺素治疗,然后转到神经外科进行全蝶窦切除。肿瘤的组织学检查允许对重塑的Rathke囊囊肿进行XGH诊断。系统性疾病,如结核病,结节病,排除其他肉芽肿性疾病。XGH的病因仍然不明确,但它可能是淋巴细胞性垂体炎的进行性形式或重塑的Rathke囊囊肿。筛查自身免疫性病理学和全身性疾病对于指导适当的管理至关重要。
    Hypophysitis is an extremely rare inflammatory disease that can mimic the clinical and radiological features of a pituitary adenoma. In this case report, we describe a 45-year-old woman with secondary xanthogranulomatous hypophysitis (XGH) who presented with signs of a pituitary macroadenoma. The patient complained of headaches, visual impairment, and amenorrhea-galactorrhea syndrome. Her physical examination was normal. Laboratory investigation revealed corticotropin, thyrotropin, and gonadotropin deficiencies. She also had low visual acuity in her right eye and an altered visual field. Pituitary magnetic resonance imaging revealed an intra and suprasellar mass measuring 13 × 11 × 16 mm, with hemorrhagic necrosis, that was having a discrete mass effect on the patient\'s optic chiasm and pituitary stalk. The patient was treated with hydrocortisone and levothyroxine, and then transferred to the Neurosurgery department for total transsphenoidal resection of the mass. Histological examination of the tumor permitted a diagnosis of XGH of a remodeled Rathke\'s pouch cyst to be made. Systemic conditions such as tuberculosis, sarcoidosis, and other granulomatous diseases were excluded. The etiopathogenesis of XGH remains poorly characterized, but it may be a progressive form of lymphocytic hypophysitis or a remodeled Rathke\'s pouch cyst. Screening for autoimmune pathology and systemic diseases is essential to guide appropriate management.
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  • 文章类型: Journal Article
    背景:垂体腺瘤(PPAs)在儿童和青少年时期并不常见,占所有颅内肿瘤的2-6%。青春期延迟,生长迟缓,溢乳和体重增加是儿科患者的常见特征。功能性肿瘤构成PPA的绝大多数(90%),最常见的是催乳素瘤。
    方法:对7例小儿垂体大腺瘤患者的临床特征和结局进行回顾性分析。通过磁共振(MRI),我们将PPAs纳入了18岁以下直径大于10mm的诊断患者。6名患者为男性(85%),诊断年龄为8至15岁(中位数14±2.8SDS)。导致就医的主要症状是生长迟缓,巨人症和继发性闭经。3例(42%)视野缩小。3名受试者存在鞍上延伸,其中一人患有巨大的腺瘤。6例(85%)的腺瘤具有临床功能(3例泌乳素腺瘤,两个生长激素瘤,一个分泌FSH和一个无生产者)。催乳素瘤对卡麦角林治疗有反应。其余的,一个需要经蝶窦手术,另外三个需要手术和放射治疗。所有接受放疗的患者均有继发性全垂体功能减退。关于基因研究,两名患者出现AIP基因和MEN1的致病性突变。
    小儿垂体大腺瘤是一个独特的实体,主要在男性中发现,并且主要是功能性肿瘤,除了神经眼科表现外,还会对生长和青春期产生不利影响。在18岁以下出现的大腺瘤患者中进行遗传研究很重要,因为该年龄段的遗传和综合征关联更为频繁。
    BACKGROUND: Pituitary adenomas (PPAs) are uncommon in childhood and adolescence, accounting for 2-6% of all intracranial neoplasms. Delayed puberty, growth retardation, galactorrhea and weight gain are common features at presentation in pediatric patients. Functional tumors constitute a vast majority (90%) of PPAs, with the most frequent being prolactinomas.
    METHODS: A retrospective review of the clinical features and outcomes of 7 pediatric patients with pituitary macroadenomas was conducted. We included PPAs in patients under 18 years at diagnosis with diameters larger than 10 mm by magnetic resonance (MRI). Six patients were males (85%), with age at diagnosis ranging from 8 to 15 (median 14 ± 2.8SDS). The primary symptoms that led to medical attention were growth retardation, gigantism and secondary amenorrhea. The visual field was reduced in three cases (42%). Suprasellar extension was present in 3 subjects, and one had a giant adenoma. Adenomas were clinically functioning in 6 patients (85%) (three prolactinomas, two somatropinomas, one secreting FSH and one no-producer). The prolactinomas responded to treatment with cabergoline. For the rest, one required transsphenoidal surgery and the other three both surgery and radiotherapy. All patients undergoing radiotherapy had secondary panhypopituitarism. In relation to the genetic studies, two patients presented a pathogenic mutation of the AIP gene and one of the MEN1.
    UNASSIGNED: Pediatric pituitary macroadenomas are a distinct entity, mostly found in males and with a predominance of functional tumors leading to detrimental effects on growth and puberty in addition to neuro-ophthalmological manifestations. It is important to perform genetic studies in patients with macroadenomas appearing under the age of 18 years as genetic and syndromic associations are more frequent in this age group.
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  • 文章类型: Case Reports
    目的:介绍一个新的大GH患者病例,这可能会干扰不同的GH测定,导致血清样品中的假阳性结果。
    方法:一名61岁女性因垂体大腺瘤和生长激素水平升高而转诊。实验室检查显示空腹GH水平升高,通过夹心化学发光免疫测定(LIAISON®XL)测量,口服葡萄糖耐量试验和正常IGF-1无抑制。患者没有肢端肥大症的典型体征和症状。患者接受了垂体肿瘤的经蝶窦切除术,仅显示α亚基免疫染色。术后GH水平仍然升高。怀疑对GH水平的测定有干扰。GH通过三种不同的免疫测定分析,UniCelDxI600,Cobase411和hGH-IRMA。血清样本中未检测到嗜异抗体和类风湿因子。用25%聚乙二醇(PEG)沉淀后的GH回收率为12%。尺寸排阻色谱法证实血清样品中存在宏GH。
    结论:如果实验室检查结果与临床检查结果不一致,免疫化学测定中可能存在干扰。为了识别由宏GH引起的干扰,应使用PEG方法和尺寸排阻色谱法。
    OBJECTIVE: Presentation of a new case of a patient with macro-GH, that may interfere with different GH assays leading to false-positive results in serum samples.
    METHODS: A 61-year-old female was referred with a pituitary macroadenoma and elevated growth hormone levels. The laboratory tests showed increased fasting GH level, measured by a sandwich chemiluminescence immunoassay (LIAISON® XL) without suppression on oral glucose tolerance test and normal IGF-1. The patient did not have the typical signs and symptoms of acromegaly. The patient underwent a transsphenoidal resection of a pituitary tumor, showing only α-subunit immunostaining. Postoperative GH levels remained elevated. An interference in the determination of GH level was suspected. GH was analyzed by three different immunoassays, UniCel DxI 600, Cobas e411 and hGH-IRMA. Heterophilic antibodies and rheumatoid factor were not detected in serum sample. GH recovery after precipitation with 25 % polyethylene glycol (PEG) was 12 %. Size-exclusion chromatography confirmed the presence of macro-GH in serum sample.
    CONCLUSIONS: If results of laboratory tests are not consistent with the clinical findings, the presence of an interference within immunochemical assays could be suspected. To identify interference caused by the macro-GH, the PEG method and size-exclusion chromatography should be used.
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