Paraneoplastic Syndromes

副肿瘤综合征
  • 文章类型: Case Reports
    卵巢癌,虽然不是最常见的癌症之一,仍然是女性癌症相关死亡的重要原因。几种副肿瘤综合征已经被联系起来,这个案例研究代表了卵巢癌的一种罕见表现,表现为非胰岛细胞瘤低血糖(NICTH),以肿瘤细胞过度产生胰岛素样生长因子-II(IGF-II)为特征。我们报告了一名55岁的妇女,她因腹胀和严重的难治性低血糖而来到我们医院。实验室数据显示血清胰岛素和C肽水平受到抑制。胰岛素样生长因子II(IGF-II)/胰岛素样生长因子1(IGF1)比值>32。因此,低血糖归因于非胰岛细胞肿瘤类型,它可能是由不完全加工的IGF-II的肿瘤分泌驱动的。实验室发现表明NICTH的存在。腹部计算机断层扫描显示存在左卵巢肿块和腹膜癌。CT引导下腹膜病变活检显示恶性分化低,与卵巢癌肉瘤(OCS)一致。患者接受连续输注葡萄糖治疗。她甚至接受了口服泼尼松和胰高血糖素输注。开始使用卡铂和紫杉醇进行化疗,但不幸的是,她死于多器官衰竭的并发症。据我们所知,这是第一例NICTH转移性OCS的新病例,强调了卵巢癌表现的复杂性,以及在管理罕见的副肿瘤综合征中采取综合方法的必要性,如NICTH。
    Ovarian cancer, although not among the most commonly diagnosed cancers, remains a significant cause of cancer-related mortality in females. Several paraneoplastic syndromes have been associated, and this case study represents a rare manifestation of ovarian cancer, presenting as non-islet cell tumor hypoglycemia (NICTH), characterized by the excessive production of insulin-like growth factor-II (IGF-II) by tumor cells. We report a 55-year-old woman who presented to our hospital with abdominal distension and severe refractory hypoglycemia. The laboratory data revealed the suppression of serum insulin and C-peptide levels. The insulin-like growth factor II (IGF-II)/insulin-like growth factor 1 (IGF1) ratio was >32. The hypoglycemia was hence attributed to the non-islet cell tumor type, and it is likely driven by tumoral secretion of incompletely processed IGF-II. The lab findings suggested the existence of NICTH. Abdominal computed tomography demonstrated the presence of a left ovarian mass and peritoneal carcinomatosis. CT-guided biopsy of the peritoneal lesions showed poorly differentiated malignancy consistent with ovarian carcinosarcoma (OCS). The patient was treated with a continuous infusion of glucose. She even received oral prednisone and glucagon infusion. Chemotherapy with carboplatin and paclitaxel was initiated, but unfortunately, she died from complications of multiorgan failure. To our knowledge, this is the first novel case of an initial presentation of metastatic OCS with NICTH, underscoring the complexity of ovarian cancer presentations and the necessity of a comprehensive approach in managing rare paraneoplastic syndromes, such as NICTH.
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  • 文章类型: Journal Article
    胆囊癌(GBC)是一种罕见的胃肠道肿瘤,据报道在美国的发病率为100,000分之一。GBC可能会出现细微的体征和症状,在常规检查和/或与其他疾病混淆。不幸的是,它的微妙表现经常导致晚期诊断,因此,预后不良。几种副肿瘤综合征与GBC有关。尽管它们与肿瘤疾病有很强的联系,这些综合征发展的确切病理生理机制仍然知之甚少.鉴于他们最初的体征和症状的模糊性,这些综合征通常被诊断为独立实体,并且仅在后来与可能已经转移到其他器官的隐匿性恶性肿瘤相关.医生需要了解这些副肿瘤综合征的体征和症状,并将潜在的恶性肿瘤作为鉴别诊断的一部分。这篇综述提供了与GBC相关的副肿瘤综合征的详细讨论。
    Gallbladder carcinoma (GBC) is a rare gastrointestinal tumor with a reported incidence of 1 in 100,000 in the United States. GBC may present with subtle signs and symptoms that can be missed on routine examination and/or confused with other conditions. Unfortunately, its subtle presentation frequently leads to late diagnosis and, thus, a poor prognosis. Several paraneoplastic syndromes have been associated with GBC. Despite their strong associations with neoplastic disease, the precise pathophysiologic mechanisms underlying the development of these syndromes remain poorly understood. Given the vague nature of their initial signs and symptoms, these syndromes are frequently diagnosed as independent entities and only later associated with occult malignancies that may have already metastasized to other organs. Physicians need to be aware of the signs and symptoms of these paraneoplastic syndromes and include an underlying malignancy as part of the differential diagnosis. This review provides a detailed discussion of the paraneoplastic syndromes associated with GBC.
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  • 文章类型: Case Reports
    Trousseau综合征,也称为血栓性静脉炎偏头痛或转移性浅表血栓性静脉炎,是与各种癌症相关的罕见但显著的副肿瘤表现。该综合征的特征是恶性肿瘤患者反复发生深静脉或浅静脉血栓形成。癌症患者静脉血栓形成的风险大大增加,特别是在诊断后的最初几个月和存在远处转移的情况下。本文介绍一例72岁女性患者右下肢深静脉血栓形成,这导致了继发于非霍奇金淋巴瘤的Trousseau综合征。
    Trousseau syndrome, also known as thrombophlebitis migrans or migratory superficial thrombophlebitis, is a rare but significant paraneoplastic manifestation associated with various cancers. This syndrome is characterized by the occurrence of recurrent deep or superficial venous thrombosis in patients with malignancies. Patients with cancer have a greatly increased risk of venous thrombosis, especially in the first few months after diagnosis and in the presence of distant metastases. This article describes the case of a 72-year-old female patient who suffered a deep vein thrombosis in the right lower limb, which led to Trousseau syndrome secondary to non-Hodgkin\'s lymphoma.
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  • 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
    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: 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: Case Reports
    副肿瘤神经系统疾病是多发性肿瘤的罕见并发症,比如肺,睾丸,和乳房,并且可以与抗Hu阳性抗体(抗神经元核抗体1型或ANNA-1)相关,反Ta,反马,和未鉴定的抗体,或者是抗体阴性.潜在肿瘤的早期治疗是最可能导致副肿瘤神经症状消退的方式。这里,我们介绍了一例73岁女性患者,该患者因ANNA-1脑炎出现新发作的癫痫发作,该患者被发现患有未确诊的小细胞肺癌,以强调需要进一步检查恶性肿瘤.
    Paraneoplastic neurological disorders are a rare complication of multiple neoplasms, such as lung, testis, and breast, and can be associated with positive antibody anti-Hu (anti-neuronal nuclear antibody type 1 or ANNA-1), anti-Ta, anti-Ma, and uncharacterized antibody, or be antibody-negative. Early treatment of the underlying tumor is the most likely modality that will lead to regression of the paraneoplastic neurological symptoms. Here, we present a case of a 73-year-old female with new-onset seizure activity from ANNA-1 encephalitis found to have undiagnosed small cell lung cancer to highlight the need for further workup for malignancy.
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