pheochromocytoma

嗜铬细胞瘤
  • 文章类型: Published Erratum
    [这更正了文章DOI:10.3389/fonc.202.965168。].
    [This corrects the article DOI: 10.3389/fonc.2022.965168.].
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  • 文章类型: Journal Article
    <b>简介:</b>肾上腺出血(AH)是一种非常罕见且可能危及生命的疾病,可继发于外伤或非创伤性病因。&lt;b&gt;目的:&lt;/b&gt;该研究的目的是介绍肾上腺出血的特征和治疗方法,并表明肾上腺出血比预期的更常见,并且临床症状不明确。<b>材料与方法:</b>本回顾性研究共纳入199例术后诊断为肾上腺出血的患者。<b>讨论:</b>肾上腺出血的潜在原因是肾上腺皮质癌,嗜铬细胞瘤,和肾上腺腺瘤.研究组共纳入199例术后诊断为AH的患者。结果显示所有术后确诊患者均有嗜铬细胞瘤(n=54),肾上腺腺瘤(n=68),或腺癌(n=17)。如果我们更仔细地观察结果,我们只能发现30%的患者(n=39)术前诊断为AH。这组39名患者准备进行快速手术。在这组患者中,AH的术前诊断为嗜铬细胞瘤28%(n=11),腺癌(n=4),和肾上腺腺瘤(n=9)。<b>结论:</b>肾上腺肿瘤出血由于其不可预测性,仍然是一个未被充分理解的话题,从我们的材料中可以看出,不同的严重程度。在199名患者中,只有30%(n=39)准备手术,术前诊断为AH;其中大多数患有嗜铬细胞瘤。我们建议使用α-肾上腺素受体拮抗剂为术前诊断AH的患者做好手术准备非常重要。延长诊断过程(影像学检查和手术之间的时间)可能导致疾病进展和肾上腺出血。
    <b>Introduction:</b> Adrenal hemorrhage (AH) is a very rare and potentially life-threatening disease which may be secondary to trauma or of non-traumatic etiology.<b>Aim:</b> The aim of the study was to present the characteristics and management of adrenal hemorrhage and show that adrenal hemorrhage is more common than expected and that the clinical symptoms are not specific.<b>Materials and methods:</b> This retrospective study involved 199 patients with postoperative diagnosis of adrenal hemorrhage.<b>Discussion:</b> The factors identified as potential causes of adrenal hemorrhage are adrenocortical carcinoma, pheochromocytoma, and adrenal adenoma. The study group included 199 patients with postoperative diagnosis of AH. It showed that all patients with postoperative diagnosis had pheochromocytoma (n = 54), adrenal adenoma (n = 68), or adenocarcinoma (n = 17). If we look more careful at the results, we can find only 30% of patients (n = 39) with preoperative diagnosis of AH. This group of 39 patients was prepared for expedited surgery. In this group of patients, the preoperative diagnosis of AH was pheochromocytoma 28% (n = 11), adenocarcinoma (n = 4), and adrenal adenoma (n = 9).<b>Conclusions:</b> Bleeding into adrenal tumors is still an insufficiently understood topic due to its unpredictability and, as can be seen in our material, of varying severity. Out of 199 patients, only 30% (n = 39) were prepared for surgery with a preoperative diagnosis of AH; most of them had pheochromocytoma. We suggest that is very important to prepare patients for surgery with a preoperative diagnosis of AH using α-adrenoreceptor antagonists. Prolongation of the diagnostic process (time between the imaging examination and the surgery) may result in the disease progressing and adrenal bleeding.
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  • 文章类型: Journal Article
    背景:3P关联(3PA)是垂体腺瘤和嗜铬细胞瘤/副神经节瘤同时发生的罕见疾病。记录的3PA病例不到100例,可能是零星的或与基因突变有关。本病例报告描述了现有文献中的首例3PA伊朗患者和第90例3PA患者。
    一名36岁的白人男性因头痛和血压突然升高入院。腹部CT扫描显示下腔静脉后方有腹膜后肿块,后来切除并诊断为嗜铬细胞瘤。四年后,他注意到偶尔有轻微的头痛和颈部右侧的无痛肿块。超声检查显示颈动脉体瘤,手术切除了.在他第二次手术一个月后,患者头痛的严重程度加重,他发展出了正确的同音偏盲。脑部MRI显示肿块有利于大腺瘤,颅咽管瘤,或者脑膜瘤,泌乳素水平升高导致大泌乳素瘤的诊断。
    方法:根据提供的历史记录,这个病人被诊断为3PA,一项基因研究发现琥珀酸脱氢酶复合物亚基b突变阳性,可能与他的颈动脉体瘤家族史有关.
    结果:患者在每3个月就诊期间保持无症状。
    结论:全球诊断为3PA的病例数正在增加。使用临床和遗传评估,我们可以及时诊断和充分监测患有3PA或处于3PA风险的个体。
    BACKGROUND: 3P association (3PA) is a rare condition with co-occurrence of pituitary adenoma and pheochromocytoma/paraganglioma. There have been less than a hundred documented cases of 3PA, which can be sporadic or related to genetic mutations. The present case report describes the first Iranian patient with 3PA and a 90th case of 3PA in the available literature.
    UNASSIGNED: A 36-year-old Caucasian male was admitted with headache and sudden increase in blood pressure. An abdominal CT scan revealed a retroperitoneal mass posterior to the inferior vena cava, later removed and diagnosed as a pheochromocytoma. Four years later, he noticed occasional mild headaches and a painless mass on the right side of his neck. The ultrasonography evaluations suggested a carotid body tumor, which was surgically removed. About a month after his second surgery, the severity of the patient\'s headaches worsened, and he developed right homonymous hemianopia. A brain MRI showed a mass in favor of macroadenoma, craniopharyngioma, or meningioma, and elevated prolactin level led to the diagnosis of macroprolactinoma.
    METHODS: Based on the provided history, this patient was diagnosed with 3PA, and a genetic study identified a positive succinate-dehydrogenase-complex subunit b mutation, possibly linked to his family history of carotid body tumor.
    RESULTS: He has remained symptom-free during his visits every 3 months.
    CONCLUSIONS: The number of cases diagnosed with 3PA worldwide is increasing. Using clinical and genetic assessments, we can timely diagnose and adequately monitor individuals with or at risk of 3PA.
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  • 文章类型: English Abstract
    The understanding of the nature of catecholamine-secreting tumors has changed significantly in recent years, affecting terminology and classification. Phaeochromocytoma/paraganglioma (PCC/PG) is a rare neuroendocrine tumor from chromaffin tissue that produces and secretes catecholamines. The incidence of PCC/PG is relatively low, with 2-8 cases per 1 million population per year; among patients with arterial hypertension, their prevalence is 0.2-0.6%. However, delayed diagnosis of PCC/PG is associated with a high risk of cardiovascular complications and a high mortality rate. The consensus presents the clinical manifestations of the disease with an emphasis on the course of arterial hypertension as the most common symptom in PCC/PG; modern ideas about the features of diagnosis, aspects of preoperative preparation, treatment, and follow-up of patients with PCC/PG are considered.
    Понимание природы катехоламин-секретирующих опухолей существенно изменилось в последние годы, что отразилось и на терминологии, и на классификации. Феохромоцитома/параганглиома (ФХЦ/ПГ) является редкой нейроэндокринной опухолью из хромаффинной ткани, продуцирующей и секретирующей катехоламины. Частота развития ФХЦ/ПГ относительно невелика и составляет 2–8 случаев на 1 млн населения в год, среди пациентов с артериальной гипертонией их распространенность – 0,2–0,6%. Однако несвоевременно диагностированные случаи ФХЦ/ПГ сопряжены с высоким риском развития сердечно-сосудистых осложнений и высоким уровнем смертности. В консенсусе представлены клинические проявления течения заболевания с акцентом на течение артериальной гипертонии как наиболее часто встречающегося симптома при ФХЦ/ПГ; рассматриваются современные представления об особенностях диагностики, аспектах предоперационной подготовки, лечении и последующем наблюдении пациентов с ФХЦ/ПГ.
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  • 文章类型: Journal Article
    暂无摘要。
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  • 文章类型: Case Reports
    由于常染色体显性多囊肾病(ADPKD)和晚期慢性肾病患者的高血压患病率很高,诊断继发性高血压带来挑战.我们介绍了ADPKD患者中罕见的嗜铬细胞瘤病例,以强调在终末期肾病(ESRD)患者中识别嗜铬细胞瘤/副神经节瘤(PPGL)引起的继发性高血压的诊断困难。
    一名患有ADPKD和ESRD的48岁女性经历了复发性高血压危象(高达220/135mmHg),并伴有心悸和震颤,在过去2年中复发。在抗高血压治疗中引入β受体阻滞剂加重了她的症状。尿中肾上腺素升高的最初文献被解释为由于肾衰竭而导致的假阳性发现。随后对游离血浆中的肾上腺素的测量显示出明显的升高,引起了对PPGL的怀疑。磁共振成像确定了29毫米的右肾上腺肿块。患者接受了右肾上腺切除术,从而解决了高血压危机。
    由于非特异性临床症状和诊断缺陷,PPGL的诊断可能存在重大挑战,并且在ESRD中进一步复杂化。据报道,ESRD患者的PPGL病例少于20例。β受体阻滞剂治疗的不耐受,以及使用PPGL可能性的评分系统应该引起怀疑。
    在所有未控制的高血压和β受体阻滞剂不耐受的患者中应考虑PPGL,即使存在其他病因机制,如ESRD。在肾功能受损的情况下,测量游离血浆中的肾上腺素可提供最可靠的生化筛查。
    UNASSIGNED: Due to the high prevalence of hypertension in patients with autosomal dominant polycystic kidney disease (ADPKD) and advanced chronic kidney disease, diagnosing secondary hypertension poses challenges. We present a rare case of pheochromocytoma in an ADPKD patient to highlight the diagnostic difficulties in identifying secondary hypertension due to pheochromocytoma/paraganglioma (PPGL) in end-stage renal disease (ESRD) patients.
    UNASSIGNED: A 48-year-old female with ADPKD and ESRD experienced recurrent hypertensive crises (up to 220/135 mmHg) accompanied by palpitations and tremors that recurred over the past 2 years. Introduction of a betablocker to the antihypertensive therapy aggravated her symptoms. The initial documentation of elevated urinary metanephrines was interpreted as false positive finding due to renal failure. Subsequent measurements of free plasma metanephrines revealed significant elevations raising suspicion of PPGL. Magnetic resonance imaging identified a 29 mm right adrenal mass. The patient underwent right adrenalectomy resulting in resolution of the hypertensive crises.
    UNASSIGNED: The diagnosis of PPGLs can present significant challenges and is further complicated in ESRD due to nonspecific clinical symptoms and diagnostic pitfalls. Less than 20 PPGL cases have been reported in patients with ESRD. The intolerance of beta-blocker therapy, as well as the use of a scoring system for the likelihood of PPGL should have raised suspicion.
    UNASSIGNED: PPGL should be considered in all patients with uncontrolled hypertension and beta-blockers intolerance, even in the presence of other etiologic mechanisms such as ESRD. Measuring free plasma metanephrines provides the most reliable biochemical screening in the context of impaired renal function.
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  • 文章类型: Case Reports
    弥漫性肺泡出血(DAH)可由各种条件引起,分为自身免疫和非自身免疫。免疫因子介导的血管炎,比如韦格纳肉芽肿病,显微镜下多血管炎,Goodpasture综合征,结缔组织疾病,和抗磷脂抗体综合征,是常见的自身免疫原因。非自身免疫因素包括感染性或毒性暴露和肿瘤状况。DAH的诊断,由于肾上腺嗜铬细胞瘤或肾上腺外副神经节瘤释放过多的儿茶酚胺,可以提出诊断挑战,需要及时治疗。在这份报告中,我们介绍一例嗜铬细胞瘤,表现为肾上腺偶发瘤(在胆囊切除术后突发性DAH治疗期间诊断).病例报告:一名39岁女性肾上腺偶发瘤患者在胆囊切除术后发展为DAH,表现为突发性咯血和呼吸困难。糖皮质激素的给药,已知会导致嗜铬细胞瘤危机(PCC),在确定原因之前需要。由于持续的低氧性呼吸衰竭和急性呼吸窘迫综合征(ARDS),必须进行插管和机械通气。在这种情况下,患者在进行机械通气时经历了两次PCC的水肿。随后的检查显示有26×25mm的左肾上腺腺瘤,激素证实儿茶酚胺分泌过多。八个月后进行了腹腔镜肾上腺切除术,以切除左肾上腺。随后的组织检查显示嗜铬细胞瘤,从而验证初步诊断。结论:肾上腺偶发瘤可能是嗜铬细胞瘤(肾上腺偶发瘤可表现为嗜铬细胞瘤),即使没有肾上腺素能症状.建议在进行侵入性手术或接受皮质类固醇治疗之前,对肾上腺偶发瘤进行嗜铬细胞瘤的评估。在不进一步阐明的情况下考虑DAH的潜在原因时,包括嗜铬细胞瘤或副神经节瘤(PPGL)在鉴别诊断中具有重要意义。
    Diffuse alveolar hemorrhage (DAH) can be caused by various conditions, categorized as autoimmune and non-autoimmune. Immunofactor-mediated vasculitis, such as Wegener granulomatosis, microscopic polyangiitis, Goodpasture syndrome, connective tissue disorders, and antiphospholipid antibody syndrome, are common autoimmune causes. Non-autoimmune factors include infectious or toxic exposures and neoplastic conditions. The diagnosis of DAH, resulting from excessive catecholamine release from an adrenal pheochromocytoma or extra-adrenal paraganglioma, can present diagnostic challenges and necessitate prompt treatment. In this report, we present a case of pheochromocytoma that manifested as an adrenal incidentaloma (diagnosed during the management of sudden-onset DAH after cholecystectomy). Case report: A 39-year-old female patient with adrenal incidentaloma developed DAH following a cholecystectomy procedure, presenting with sudden-onset hemoptysis and dyspnea. Administration of glucocorticoids, known to precipitate pheochromocytoma crisis (PCC), was required before the cause was determined. Intubation and mechanical ventilation were necessary due to persistent hypoxemic respiratory failure and acute respiratory distress syndrome (ARDS). The patient in this case experienced two epidoses of PCC while she was on mechanical ventilation. Subsequent work-up revealed a 26 × 25 mm left adrenal adenoma with hormonal confirmation of catecholamine hypersecretion. A laparoscopic adrenalectomy was done eight months later to excise the left adrenal gland. Subsequent examination of the tissue revealed pheochromocytoma, thereby validating the initial diagnosis. Conclusion: Adrenal incidentalomas may be pheochromocytomas (adrenal incidentalomas can manifest as pheochromocytomas), even without adrenergic symptoms. It is recommended that adrenal incidentalomas undergo evaluation for pheochromocytoma before undergoing invasive surgery or receiving corticosteroid treatment. When considering potential causes of DAH without further elucidation, including a pheochromocytoma or paraganglioma (PPGLs) in the differential diagnosis is important.
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    文章类型: Case Reports
    A 16-year-old castrated male shih tzu dog was brought to the emergency service because of an acute (24 h) history of gagging, coughing, and lethargy. Physical examination revealed dyspnea concurrent with diffuse, bilateral, pulmonary crackles and wheezes. Oxygen saturation, measured with a pulse oximeter, was < 95%. Thoracic radiographs revealed a multifocal alveolar pattern superimposed over a diffuse interstitial pattern with normal heart size. The dog continued to be dyspneic despite oxygen therapy. In accordance with the radiographic findings, further supportive care was recommended. Due to the unknown prognosis and financial constraints, the owner elected humane euthanasia. Necropsy confirmed a pheochromocytoma and lung changes without evidence of congestive heart failure. Findings were consistent with diffuse alveolar damage. These findings correlated with the dog\'s clinical diagnosis of acute respiratory distress syndrome (ARDS). No other disease processes associated with ARDS were identified. The purpose of this case report is to describe an unusual presentation of ARDS likely associated with a pheochromocytoma and confirmed by necropsy. Acute respiratory distress syndrome associated with a pheochromocytoma has been described in medical literature but has never been reported in veterinary medicine. Key clinical message: Pheochromocytomas should be added to the list of risk factors associated with ARDS in dogs. Dogs with a suspected diagnosis of pheochromocytoma whose owners elect against surgical removal should be closely monitored for an acute onset of respiratory distress, which could suggest the development of ARDS.
    Syndrome de détresse respiratoire aiguë associé à un phéochromocytome chez un chien adulteUn chien shih tzu mâle castré de 16 ans a été amené aux services d’urgence en raison d’antécédents aigus (24 heures) de haut-le-coeur, de toux et de léthargie. L’examen physique a révélé une dyspnée concomitante à des crépitements et des respirations sifflantes diffuses, bilatérales et pulmonaires. La saturation en oxygène, mesurée avec un oxymètre de pouls, était < 95 %. Les radiographies thoraciques ont révélé un motif alvéolaire multifocal superposé à un motif interstitiel diffus avec une taille cardiaque normale. Le chien est resté dyspnéique malgré l’oxygénothérapie. Conformément aux résultats radiographiques, des soins de soutien supplémentaires ont été recommandés. En raison du pronostic inconnu et des contraintes financières, le propriétaire a opté pour l’euthanasie sans cruauté. La nécropsie a confirmé un phéochromocytome et des modifications pulmonaires sans signe d’insuffisance cardiaque congestive. Les résultats concordaient avec des lésions alvéolaires diffuses. Ces résultats étaient en corrélation avec le diagnostic clinique du syndrome de détresse respiratoire aiguë (ARDS) du chien. Aucun autre processus pathologique associé à l’ARDS n’a été identifié. Le but de ce rapport de cas est de décrire une présentation inhabituelle de l’ARDS probablement associée à un phéochromocytome et confirmée par la nécropsie. Le syndrome de détresse respiratoire aiguë associé à un phéochromocytome a été décrit dans la littérature médicale mais n’a jamais été rapporté en médecine vétérinaire.Message clinique clé:Les phéochromocytomes doivent être ajoutés à la liste des facteurs de risque associés à l’ARDS chez le chien. Les chiens avec un diagnostic suspecté de phéochromocytome dont les propriétaires choisissent de ne pas procéder à l’ablation chirurgicale doivent être étroitement surveillés pour détecter l’apparition aiguë d’une détresse respiratoire, ce qui pourrait suggérer le développement d’un ARDS.(Traduit par Dr Serge Messier).
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  • 文章类型: Journal Article
    单侧和双侧肾上腺病变的病因可能不同,具有不同的临床意义和管理指南,后者有增生等病因,感染,浸润性病变和瘤形成。双侧肿瘤更有可能具有遗传性/综合征性关联。关于双侧肾上腺病变的临床和病理特征的数据有限。
    这是一项回顾性研究,从2016年1月至2022年8月到我们研究所就诊的266例肾上腺病变患者中选择双侧肾上腺病变患者。人口统计,从医院信息系统和患者病例档案中检索实验室和影像学数据.
    该研究包括51名患者;就诊时的平均年龄为51.15岁(范围为14至82岁)。48例患者(94.1%)出现症状,症状平均持续时间为10.68个月(范围为10天至1年)。最常见的表现是肾上腺功能不全18例(38%),其次是发热17例(36%)。最常见的病因,正如在组织病理学上所揭示的,是组织胞浆菌病(n=22,43%),其次是嗜铬细胞瘤(n=11,21.5%),转移(n=6,11.7%),肾上腺增生(n=5,9.8%),肾上腺皮质腺瘤(n=1,1.9%),淋巴瘤(n=3,5.8%),神经母细胞瘤(n=1,1.9%),骨髓脂肪瘤(n=1,1.9%)和结核(n=1,1.9%)。组织胞浆菌病和转移性病变常见于老年人,嗜铬细胞瘤与年轻有关。6/11诊断为双侧嗜铬细胞瘤的患者与家族史有关,基因突变和肾上腺外受累。
    双侧肾上腺病变的方法与单侧病变的方法不同,原因是病因不同,遗传学在某些双侧肿瘤中的作用更重要。演讲的年龄,出现症状,病变大小和生化特征有助于描绘各种潜在的病因。
    UNASSIGNED: The aetiologies in unilateral and bilateral adrenal lesions can be different with different clinical implications and management guidelines, the latter having aetiologies like hyperplasia, infections, infiltrative lesions and neoplasia. Bilateral tumours are more likely to have hereditary/syndromic associations. There is limited data on the clinical and pathological profile of bilateral adrenal lesions.
    UNASSIGNED: This was a retrospective study where patients with bilateral adrenal lesions were selected from a total of 266 patients with adrenal lesions who presented to our institute between January 2016 and August 2022. The demographic, laboratory and imaging data were retrieved from the Hospital Information System and patient case files.
    UNASSIGNED: The study included 51 patients; the mean age at presentation was 51.15 years (range 14 to 82 years). Forty-eight patients (94.1%) were symptomatic at presentation with an average duration of symptoms being 10.68 months (range 10 days to 1 year). The most common presentation was adrenal insufficiency in 18 cases (38%), followed by fever in 17 cases (36%). The commonest aetiology, as revealed on histopathology, was histoplasmosis (n = 22, 43%), followed by pheochromocytoma (n = 11, 21.5%), metastases (n = 6, 11.7%), adrenal hyperplasia (n = 5, 9.8%), adrenocortical adenoma (n = 1, 1.9%), lymphoma (n = 3, 5.8%), neuroblastoma (n = 1, 1.9%), myelolipoma (n = 1, 1.9%) and tuberculosis (n = 1, 1.9%). Histoplasmosis and metastatic lesions were commonly seen in older people, and pheochromocytoma was associated with young age. 6/11 patients with a diagnosis of bilateral pheochromocytoma were associated with family history, genetic mutation and extra-adrenal involvement.
    UNASSIGNED: The approach to bilateral adrenal lesions differs from that of unilateral lesions due to differences in aetiologies and the more significant role of genetics in some bilateral tumours. The age at presentation, presenting symptoms, lesion size and biochemical features help delineate varied underlying aetiologies.
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  • 文章类型: Case Reports
    目的:本病例报告采用吲哚菁绿(ICG)荧光成像技术成功手术切除犬嗜铬细胞瘤。
    方法:10岁,6.87千克,被撒下的雌性贵宾犬因腹部肿块和八次呕吐而被转诊。计算机断层扫描显示脾脏和左肾上腺有肿块。根据血浆去甲肾上腺素水平,患者被诊断为嗜铬细胞瘤。随后,进行了全脾切除术和左肾上腺切除术.在手术前24小时静脉内施用5mg/kgICG的剂量。使用ICG允许的肿瘤边缘可视化,帮助完全切除并最大限度地减少与麻醉相关的风险。
    结论:在12个月内没有观察到复发或转移的证据。据我们所知,这是在兽医学中使用术中ICG成像手术切除嗜铬细胞瘤的第一份报告.此案例强调了ICG荧光成像作为兽医学中肾上腺肿瘤外科治疗的有价值工具的功效。
    OBJECTIVE: This case report presents the successful surgical removal of a pheochromocytoma in a dog using indocyanine green (ICG) fluorescence imaging.
    METHODS: A 10-year-old, 6.87 kg, spayed female poodle was referred for an abdominal mass and eight episodes of vomiting. Computed tomography revealed masses in the spleen and left adrenal gland. Based on the plasma normetanephrine levels, the patient was diagnosed with pheochromocytoma. Subsequently, a total splenectomy and left adrenalectomy were performed. A dose of 5 mg/kg ICG was administered intravenously 24 h prior to surgery. Using ICG allowed visualization of tumor margins, aiding in complete resection and minimizing anesthesia-related risks.
    CONCLUSIONS: No evidence of recurrence or metastasis has been observed for 12 months. To our knowledge, this is the first report of the surgical removal of a pheochromocytoma using intraoperative ICG imaging in veterinary medicine. This case highlights the efficacy of ICG fluorescence imaging as a valuable tool for the surgical management of adrenal tumors in veterinary medicine.
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