Paraneoplastic Syndromes

副肿瘤综合征
  • 文章类型: Case Reports
    背景:Doege-Potter综合征是一种与胸膜孤立性纤维瘤(SFTP)相关的罕见副肿瘤现象。它的特点是存在严重,持续,和治疗难治性低血糖。低血糖,这可能是疾病发作时唯一的症状,是由高分子量胰岛素样生长因子(IGF-2)的分泌介导的。大多数肿瘤表现出良性行为,5年生存率为100%。然而,这些肿瘤中的10%可能表现出侵袭性行为,并伴有局部或转移性复发。我们介绍了一例胸膜良性孤立性纤维瘤患者的临床病例,该患者表现为有症状的低血糖,需要进行肺和胸膜手术切除以控制副肿瘤现象。
    方法:一名46岁的西班牙裔男子因禁食而出现短暂意识改变的15天病史。相关病史包括持续正压(CPAP)治疗的阻塞性睡眠呼吸暂停和先前吸烟。住院研究显示非胰岛素性低血糖和良性SFTP。在患者因低血糖而围手术期接受葡萄糖液和皮质类固醇治疗时,进行了完整的手术切除。随后,低血糖消退,对患者进行随访,无疾病复发。
    结论:Doege-Potter综合征具有挑战性。然而,有效的治疗可以实现高存活率。提高医疗保健专业人员对这种副肿瘤综合征患者的认识将改善诊断怀疑,生化确认,诊断和治疗指南的发展,以及为需要更密切监测的激进演示创建预测指数。
    BACKGROUND: Doege-Potter syndrome is a rare paraneoplastic phenomenon associated with solitary fibrous tumors of the pleura (SFTPs). It is characterized by the presence of severe, sustained, and treatment-refractory hypoglycemia. Hypoglycaemia, which may be the sole symptom at disease onset, is mediated by the secretion of high-molecular-weight insulin-like growth factor (IGF-2). Most tumors exhibit benign behavior, with a 100% survival rate at 5 years. However, 10% of these tumors may display aggressive behavior with local or metastatic recurrence. We present a clinical case of a patient with a benign solitary fibrous tumor of the pleura who presented with symptomatic hypoglycemia and required pulmonary and pleural surgical resection to control the paraneoplastic phenomenon.
    METHODS: A Hispanic 46-year-old man presented with a 15-day history of transient alterations in consciousness worsened by fasting. The relevant medical history included obstructive sleep apnea treated with continuous positive air pressure (CPAP) and previous smoking. In-hospital studies revealed noninsulinemic hypoglycemia and a benign SFTP. Complete surgical resection was performed while the patient received dextrose fluids and corticosteroids perioperatively for hypoglycemia. Subsequently, the hypoglycemia resolved, and the patient was followed-up without disease recurrence.
    CONCLUSIONS: Doege-Potter syndrome is challenging to recognize. However, effective treatment can be achieved with a high survival rate. Raising awareness among healthcare professionals about the recognition of this paraneoplasic syndrome patients will improve diagnostic suspicion, biochemical confirmation, the development of diagnostic and therapeutic guidelines, and the creation of predictive indices for aggressive presentations requiring closer monitoring.
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  • 文章类型: Journal Article
    一位50多岁的女性患上了头痛,塌陷,并注意到患有急性非创伤性硬膜下出血(SDH),需要手术撤离和颅内压定向治疗。她的背景包括反复发作的鼻出血,严重的全身性骨痛和多发性骨折功能不全和未分化的自身免疫性结缔组织疾病。慢性低磷酸盐血症,还注意到碱性磷酸酶升高和成纤维细胞生长因子23(FGF23)升高.MRI头和随后的68GaCT/正电子发射断层扫描扫描显示右侧筛窦有强烈的肿瘤,颅内延伸。磷酸盐被积极地取代,并且开始使用阿法骨化醇来规避FGF23对她的肾脏和骨矿物质的影响。对肿瘤进行活检,然后通过鼻内和开颅联合方法明确切除,导致良好的临床改善。FGF23滴度和血清磷酸盐均恢复正常,从而诊断出分泌磷性间充质肿瘤的FGF23,从而导致肿瘤诱导的骨软化症。
    A female in her 50s developed a headache, collapsed and was noted to have an acute atraumatic subdural haemorrhage (SDH) requiring surgical evacuation and intracranial pressure-directed therapy. Her background included recurrent epistaxis, severe generalised bone pain and multiple insufficiency fractures and an undifferentiated autoimmune connective tissue disease. Chronic hypophosphataemia, elevated alkaline phosphatase and raised fibroblast growth factor 23 (FGF23) were also noted. An MRI head and subsequent 68Ga CT/positron emission tomography scan demonstrated an intensely avid tumour in the right ethmoid sinus, extending intracranially. Phosphate was aggressively replaced, and alfacalcidol was initiated to circumvent the effects of FGF23 on her kidneys and bone minerals. The tumour was biopsied and then definitively resected via combined endonasal and craniotomy approaches, resulting in good clinical improvement. FGF23 titre and serum phosphate both normalised leaving the diagnosis of a phosphaturic mesenchymal tumour-secreting FGF23, leading to tumour-induced osteomalacia.
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  • 文章类型: Journal Article
    移植后恶性肿瘤是实体器官移植的重要并发症。肾移植受者患癌症的风险特别高。癌变最相关的危险因素是使用免疫抑制剂和致癌病毒感染。此外,由这些因素引起的免疫失调可能导致各种类型的器官损伤。副肿瘤性肾小球疾病是最有趣且研究不足的癌症表现之一。在肾移植受者中,对副肿瘤肾小球损伤的适当诊断可能具有挑战性。由于伴随用药和常见合并症的固有因素。分子和临床肾脏病学领域的最新进展使我们对肾小球疾病及其更有针对性的治疗的认识有了显着提高。另一方面,新型抗癌药物的引入极大地提高了患者的生存率,以肾脏相关副作用为代价。我们的综述旨在为副肿瘤肾小球疾病的诊断和治疗提供见解。特别关注肾移植受者。
    Posttransplant malignancies are an important complication of solid organ transplantation. Kidney transplant recipients are at particularly high risk of cancer development. The most relevant risk factors of carcinogenesis are the use of immunosuppressive agents and oncogenic viral infections. Additionally, immune dysregulation caused by these factors may predispose to various types of organ damage. Paraneoplastic glomerular diseases are one of the most interesting and understudied cancer manifestations. The appropriate diagnosis of paraneoplastic glomerular damage can be challenging in kidney transplant recipients, due to factors inherent to concomitant medication and common comorbidities. Recent advances in the field of molecular and clinical nephrology led to a significant improvement in our understanding of glomerular diseases and their more targeted treatment. On the other hand, introduction of novel anticancer drugs tremendously increased patients\' survival, at the cost of kidney-related side effects. Our review aims to provide insights into diagnosis and treatment of paraneoplastic glomerular diseases, with a special attention to kidney transplant recipients.
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  • 文章类型: Case Reports
    僵硬人综合征(SPS)是一种罕见的神经系统疾病,其特征是慢性和进行性轴向肌肉僵硬和阵发性疼痛性肌肉痉挛。本病例研究描述了一名SPS患者(血清和脑脊液中抗GAD65抗体增加)合并桥本甲状腺炎,C3补体水平降低。临床表现,诊断方法,对这一独特病例的治疗方法进行了全面详细的描述。在这种情况下,我们全面介绍了一例SPS合并桥本甲状腺炎和相关的血清C3补体降低的病例,以及关于这一主题的当前数据的讨论。
    Stiff-person syndrome (SPS) is a rare neurological disorder characterized by chronic and progressive axial muscle rigidity and paroxysmal painful muscle spasms. The present case study described an SPS patient (increased anti-GAD65 antibody in serum and cerebrospinal fluid) with co-occurring Hashimoto\'s thyroiditis and decreased C3 complement levels. The clinical presentation, diagnostic approach, and treatment employed for this unique case were comprehensively described in detail. In this case, we comprehensively presented a case of SPS with co-occurring Hashimoto\'s thyroiditis and an associated decrease in serum C3 complement, as well as a discussion on the current data on this topic.
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  • 文章类型: Journal Article
    复发性多软骨炎(RP)是一种罕见的自身免疫性疾病,可引起软骨和富含蛋白聚糖的结构的炎症,包括耳朵,鼻子,和气道。副肿瘤RP是在某些类型的癌症的检测和治疗之后发生在一些个体中的RP的子集。FDGPET/CT有助于RP的早期诊断,即使没有症状,也能识别炎症区域,并指导选择合适的活检部位。这里,我们介绍了一例以RP副肿瘤症状为首发表现的肺腺癌,化疗3个周期后症状缓解。
    UNASSIGNED: Relapsing polychondritis (RP) is an uncommon autoimmune disease that causes inflammation of the cartilage and proteoglycan-rich structures, including the ear, nose, and airway. Paraneoplastic RP is a subset of RP that occurs in some individuals following the detection and treatment of certain types of cancers. FDG PET/CT helps with early diagnosis of RP, identifying inflammatory areas even in the absence of symptoms, and guiding the selection of appropriate biopsy sites. Here, we present a case of adenocarcinoma of the lung presenting with paraneoplastic symptoms of RP as initial presentation, and symptoms were resolved after 3 cycles of chemotherapy.
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  • 文章类型: Journal Article
    副肿瘤肾小球疾病(PGD)由肿瘤细胞产物发展而来,导致肾功能不全.与直接的肿瘤效应不同,PGD说明了癌症与不同临床表现和结果之间的复杂关联。最初在霍奇金病患者中发现,目前的研究已经根据PGD症状和癌症进展确定了诊断标准.PGD,虽然罕见(发现在<1%的成人癌症患者明显的肾脏表现),是至关重要的,因为它们可以预示癌症的发作,并且经常抵抗标准的肾小球肾炎治疗。新兴的眼科研究肾脏疾病和癌症之间的这种关系。许多PGD病例的确切原因仍然未知。这篇综述研究了PGD,他们的临床病理特征,相关癌症,和机制,强调肾脏疾病和相关癌症的早期诊断和定制治疗的必要性。
    Paraneoplastic glomerular disease (PGD) develops from tumor cell products, leading to renal dysfunction. Unlike direct tumor effects, PGD illustrates the complex association between cancer and diverse clinical presentations and outcomes. Initially detected in a Hodgkin\'s disease patient, current research has defined diagnostic criteria based on PGD symptoms and cancer progression. PGDs, although rare (found in <1% of adult cancer patients with overt renal manifestations), are crucial, as they can signal cancer onset and frequently resist standard glomerulonephritis treatments. The emerging field of onconephrology studies this relationship between kidney disorders and cancers. The exact cause of many PGD cases remains unknown. This review examines PGDs, their clinicopathological features, related cancers, and mechanisms, emphasizing the need for early diagnosis and tailored treatment for kidney disease and linked cancer.
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  • 文章类型: Case Reports
    膀胱小细胞癌是一种极其罕见且侵袭性的疾病,总体生存率低,因为它通常在后期被诊断出来。同样,副肿瘤性血小板减少症也是文献中很少描述的罕见现象。鉴于其罕见但对化疗的反应性,对非典型表现的认识有助于促进适当的治疗。一名76岁的绅士因5个月的厌食症而从一家偏远的小型医院住进了澳大利亚地区医院,嗜睡,减肥,新发胸膜炎性胸痛,既往有前列腺肿大病史,有局限性精原细胞瘤的遥远病史,手术切除和单纯放疗治疗。体格检查显示新的快速心房颤动和轻度缺氧,同时右上腹压痛和饱胀。病人做了胸膜引流,细胞学,和计算机断层扫描,随后被诊断为膀胱小细胞癌,并迅速发展为孤立性血小板减少症,通过卡铂/依托泊苷住院化疗改善。经过长时间的入院后,他最终出院了。关于后续行动,在患者的偏远小医院接受姑息治疗之前,他作为门诊接受了第2周期的治疗。这凸显了迅速识别和治疗快速生长的小细胞癌的重要性,因为它们首先非典型地出现无特征的副肿瘤综合征,以降低发病率和死亡率。
    Small cell carcinoma of the bladder is an extremely rare and aggressive disease with poor overall survival, as it is often diagnosed in later stages. Similarly, paraneoplastic thrombocytopenia is also a rare phenomenon infrequently described in the literature. Given its rarity but responsiveness to chemotherapy, awareness of atypical presentations helps facilitate appropriate treatment. A 76-year-old gentleman was admitted to an Australian regional hospital from a small remote hospital with complaints of five months of anorexia, lethargy, weight loss, and new-onset pleuritic chest pain with a past medical history of prostatomegaly and a distant history of localised seminoma treated with surgical resection and radiotherapy alone. Physical examination revealed new rapid atrial fibrillation and mild hypoxia alongside right upper quadrant tenderness and fullness. The patient underwent pleural drainage, cytology, and computed tomography, was subsequently diagnosed with small cell carcinoma of the bladder, and rapidly developed isolated thrombocytopenia that improved with inpatient chemotherapy with carboplatin/etoposide. He was eventually discharged home after a lengthy admission. On follow-up, he had cycle 2 of treatment as an outpatient before undergoing palliative treatment at the patient\'s small remote hospital. This highlights the importance of both prompt recognition and treatment of rapidly growing small cell carcinomas when they first present atypically with uncharacteristic paraneoplastic syndromes to reduce morbidity and mortality.
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  • 文章类型: Case Reports
    前纵隔肿块,包括胸腺瘤,可以出现胸部症状或副肿瘤综合征,尤其是40岁以上的成年人。诊断涉及成像和活检,治疗包括手术切除和化疗,取决于舞台。一个31岁的男性,有酗酒史和以前的吸烟者,伴随着越来越多的胃灼热,呼吸急促,左肩疼痛,和胸痛。影像学显示前纵隔肿块,胸膜增厚和少量积液。活检证实为B2型胸腺瘤。初始治疗包括环磷酰胺,阿霉素,和顺铂,导致显著的肿瘤缩小和胸腔积液消退。患者在新辅助化疗后接受了计划的手术切除。该病例强调了晚期胸腺瘤治疗的复杂性和新辅助化疗在降低肿瘤负担方面的有效性。相关的积液,改善结果。持续的随访和进一步的研究对于优化晚期胸腺瘤的治疗方案至关重要。
    Anterior mediastinal masses, including thymomas, can present with thoracic symptoms or paraneoplastic syndromes, especially in adults over 40. Diagnosis involves imaging and biopsy, and treatment includes surgical resection and chemotherapy, depending on the stage. A 31-year-old male, with a history of alcohol use disorder and a former smoker, presented with increasing heartburn, shortness of breath, left shoulder pain, and chest pain. Imaging revealed an anterior mediastinal mass with pleural thickening and a small effusion. A biopsy confirmed a B2-type thymoma. Initial treatment included cyclophosphamide, doxorubicin, and cisplatin, resulting in significant tumor reduction and pleural effusion resolution. The patient underwent planned surgical resection following neoadjuvant chemotherapy. This case highlights the complexity of advanced thymoma treatment and the effectiveness of neoadjuvant chemotherapy in reducing tumor burden, the associated effusions, and improving outcomes. Continuous follow-up and further studies are essential to optimize treatment protocols for advanced thymoma.
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  • 文章类型: Case Reports
    文献中已充分证明了血管炎的总称下的各种情况。这些被分类为小的,中等,大血管血管炎.此外,血管炎已被归类为辐射诱导,系统性,和副肿瘤。其中,副肿瘤性血管炎占所有血管炎病例的2-5%,文献记载较少。我们介绍了一个有乳腺癌病史的女性患者,表现为上消化道(GI)出血,随后揭示了系统性血管炎的潜在诊断,可能是副肿瘤.此病例强调了成像对于揭示潜在血管炎作为胃肠道出血病因的重要性。
    Various conditions under the umbrella term of vasculitis have been well documented in the literature. These have been classified into small, medium, and large vessel vasculitis. In addition, vasculitis has been categorized into radiation-induced, systemic, and paraneoplastic. Of these, paraneoplastic vasculitis accounts for 2-5% of all cases of vasculitides and is less well documented. We present a case of a female patient with a history of breast cancer presenting with an upper gastrointestinal tract (GI) bleed, which subsequently revealed an underlying diagnosis of systemic vasculitis, possibly paraneoplastic. This case highlights the importance of imaging for revealing underlying vasculitis as an etiology of GI bleed.
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  • 文章类型: Journal Article
    背景:很少有研究分析住院肿瘤患者高钙血症的特征。我们的目的是描述副肿瘤性高钙血症住院患者的临床特征,并确定死亡率的预后变量。
    方法:这是一个观察性的,纵向,回顾性,和双中心研究。其中包括马拉加两家医院收治的成年患者,西班牙(2014-2018)。最短随访期为2年或直至死亡。
    结果:共纳入154例患者;大多数(71.4%)进入内科。中位随访时间为3.5周(四分位距[IQR]1.1-11.5)。平均(标准差)年龄为67.6(12.3)岁,以男性为主(58.4%)。入院时血清钙中位数(IQR)为13.2(11.8-14.6)mg/dl。最常见的肿瘤是肺(27.3%),血液学(23.4%),泌尿外科(13%),和乳房(12.3%)。此外,56.5%的病例在诊断时具有已知的肿瘤病史。甲状旁腺激素(PTH)水平测定为24%;其中,10.8%的水平升高。总之,95.5%的患者在随访期间死亡。中位生存期为3.4周(95%置信区间2.6-4.3)。与较高死亡率相关的因素是年龄,入院时血清钙,以前的肿瘤病史,多发性骨髓瘤以外的病因,和未纠正高钙血症。
    结论:在住院患者中,副肿瘤性高钙血症与高短期死亡率相关.在这些患者中发现了与预后较差相关的几个因素。
    BACKGROUND: There are few studies that have analyzed the characteristics of hypercalcemia in hospitalized oncological patients. Our objectives were to describe the clinical characteristics of hospitalized patients with paraneoplastic hypercalcemia and to identify prognostic variables for mortality.
    METHODS: This was an observational, longitudinal, retrospective, and bicentric study. It included adult patients admitted to two hospitals in Málaga, Spain (2014-2018). The minimum follow-up period was 2 years or until death.
    RESULTS: A total of 154 patients were included; the majority (71.4%) were admitted to the internal medicine department. The median follow-up was 3.5 weeks (interquartile range [IQR] 1.1-11.5). The mean (standard deviation) age was 67.6 (12.3) years, with a predominance of males (58.4%). The median (IQR) serum calcium at admission was 13.2 (11.8-14.6) mg/dl. The most common neoplasms were pulmonary (27.3%), hematologic (23.4%), urological (13%), and breast (12.3%). Furthermore, 56.5% of cases had a known history of neoplasia at the time of diagnosis. The parathyroid hormone (PTH) level was determined in 24%; of these, 10.8% had elevated levels. In all, 95.5% of patients died during follow-up. The median survival was 3.4 weeks (95% confidence interval 2.6-4.3). Factors associated with higher mortality were age, serum calcium at admission, previous history of neoplasia, etiology other than multiple myeloma, and noncorrection of hypercalcemia.
    CONCLUSIONS: In hospitalized patients, paraneoplastic hypercalcemia was associated with high short-term mortality. Several factors associated with a worse prognosis were identified in these patients.
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