paraneoplastic syndrome

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: Case Reports
    一名患有多囊肾病并在55岁时有肾移植史的男子在68岁时发展为血清阴性的类风湿性关节炎(RA)。用生物衍生物治疗导致缓解;然而,患者在2年后复发。改用baricitinib后,患者再次获得缓解。两年后,当病人72岁时,RA复发,右侧天然肾脏因存在大肿瘤而增大。进行了手术肾切除术,肿瘤被归类为肾细胞癌(RCC),未指定。癌组织包括肉瘤样和横纹肌样细胞,有明显的中性粒细胞浸润,肿瘤细胞白细胞介素-6阳性。病人,73岁,在手术干预后经历了关节疼痛的缓解;然而,他们死于全身转移,术后10周。根据临床过程,RA样病变和随后的RCC被认为是副肿瘤综合征.
    A man with polycystic kidney disease and a history of renal transplantation at the age of 55 years developed seronegative rheumatoid arthritis (RA) at the age of 68 years. Treatment with a biological derivative led to remission; however, the patient relapsed 2 years later. After being switched to baricitinib, the patient again achieved remission. After 2 years, when the patient was aged 72 years, RA recurred, and the right native kidney became enlarged due to the presence of a large tumor. Surgical nephrectomy was performed, and the tumor was classified as renal cell carcinoma (RCC), not otherwise specified. The cancer tissue comprised sarcomatoid and rhabdoid cells with marked neutrophil infiltration, and the tumor cells were positive for interleukin-6. The patient, aged 73 years, experienced a resolution of joint pain following surgical intervention; however, they died because of systemic metastases ~10 weeks post-operation. Based on the clinical course, the RA-like lesions and subsequent RCC were considered to represent a paraneoplastic syndrome.
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  • 文章类型: Case Reports
    库欣综合征(CS),由于异位促肾上腺皮质激素(ACTH)的产生,构成各种恶性肿瘤的常见副肿瘤表现,最常见的是小细胞肺癌。在文学中,有文献记载,异位CS与前列腺癌相关的病例不到50例.在本研究中,报道一例76岁男性患有去势抵抗性前列腺腺癌,过去4年接受恩杂鲁胺和促黄体生成素释放激素(LHRH)类似物治疗.患者出现下肢肌无力到急诊科就诊,缓血球和低钾血症。经过全面的诊断评估,确定了高皮质醇血症。低剂量和高剂量地塞米松攻击后无抑制,皮质醇24h排泄增加和正常的垂体磁共振成像导致异位CS的诊断。立即靶向治疗开始与肾上腺类固醇生成抑制剂,包括甲吡酮和酮康唑以及多西他赛和泼尼松龙的化疗。几天之内皮质醇水平显着下降,不再需要住院治疗。患者成功完成了三个周期的化疗;不幸的是,他在诊断为异位CS后三个月内死亡。在本研究中,我们回顾了所有现有的与前列腺癌相关的副肿瘤CS病例。本研究的目的是强调对该实体进行早期诊断和治疗的必要性,因为它可能具有非典型的临床发现,并可能演变成危及生命的状况。
    Cushing\'s syndrome (CS), as a result of ectopic adrenocorticotropic hormone (ACTH) production, constitutes a common paraneoplastic manifestation of various malignancies, with the most common being small cell lung carcinoma. In the literature, fewer than fifty cases associating ectopic CS with prostate cancer have been documented. In the present study, the case of a 76-year old man suffering from castration-resistant prostate adenocarcinoma that had been treated with enzalutamide and luteinizing hormone-releasing hormone (LHRH) analogue for the last four years is presented. The patient presented to the emergency department with lower extremity muscle weakness, bradypsychia and hypokalemia. Following a thorough diagnostic evaluation, hypercortisolemia was identified. No suppression after low- and high-dose dexamethasone challenge, increased cortisol 24 h excretion and normal pituitary magnetic resonance imaging led to the diagnosis of ectopic CS. Immediate targeted therapy was initiated with adrenal steroidogenesis inhibitors, including metyrapone and ketoconazole along with chemotherapy with docetaxel and prednisolone. There was a remarkable decrease in cortisol levels within days and hospitalization was no longer required. The patient managed to complete three cycles of chemotherapy; unfortunately, he succumbed within three months of the diagnosis of ectopic CS. In the present study, all existing cases of paraneoplastic CS related to prostate cancer are reviewed. The aim of the current study was to highlight the need of early diagnosis and treatment of this entity as it may present with atypical clinical findings and potentially evolve to a life-threatening condition.
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  • 文章类型: Journal Article
    黑色素瘤相关视网膜病变(MAR)是一种与皮肤转移性黑色素瘤相关的副肿瘤综合征,患者出现视力缺陷,包括夜视功能下降,对比敏感度差,和光视。MAR是由靶向TRPM1的自身抗体引起的,TRPM1是在黑素细胞和视网膜ON双极细胞(ON-BC)中发现的离子通道。当TRPM1自身抗体进入ON-BCs并阻断TRPM1的功能时出现视觉症状,因此在患者血清中检测TRPM1自身抗体是诊断MAR的关键标准。视网膜电图用于测量TRPM1自身抗体对ON-BC功能的影响,并代表MAR的另一个重要诊断工具。迄今为止,MAR病例报告包括一个或两个诊断组件,但只针对患者疾病过程中的单个时间点。这里,我们报告了一例由血清自身抗体检测的纵向分析支持的MAR,视觉功能,眼部炎症,血管完整性,以及对缓释眼内皮质类固醇的反应。将这些数据与患者的肿瘤和眼科记录相结合,揭示了有关MAR发病机制的新见解。programming,和治疗,这可能为新的研究提供信息,扩大我们对这种疾病的集体理解。简而言之,我们发现TRPM1自身抗体即使在westernblot和免疫组织化学几乎检测不到血清水平时也能破坏视力;尽管循环中的TRPM1自身抗体水平很高,但眼内地塞米松治疗可缓解MAR视觉症状,提示抗体进入视网膜是MAR发病机制的关键因素。患者眼睛中炎性细胞因子水平升高可能是观察到的血-视网膜屏障损伤以及随后自身抗体进入视网膜的原因。
    Melanoma-associated retinopathy (MAR) is a paraneoplastic syndrome associated with cutaneous metastatic melanoma in which patients develop vision deficits that include reduced night vision, poor contrast sensitivity, and photopsia. MAR is caused by autoantibodies targeting TRPM1, an ion channel found in melanocytes and retinal ON-bipolar cells (ON-BCs). The visual symptoms arise when TRPM1 autoantibodies enter ON-BCs and block the function of TRPM1, thus detection of TRPM1 autoantibodies in patient serum is a key criterion in diagnosing MAR. Electroretinograms are used to measure the impact of TRPM1 autoantibodies on ON-BC function and represent another important diagnostic tool for MAR. To date, MAR case reports have included one or both diagnostic components, but only for a single time point in the course of a patient\'s disease. Here, we report a case of MAR supported by longitudinal analysis of serum autoantibody detection, visual function, ocular inflammation, vascular integrity, and response to slow-release intraocular corticosteroids. Integrating these data with the patient\'s oncological and ophthalmological records reveals novel insights regarding MAR pathogenesis, progression, and treatment, which may inform new research and expand our collective understanding of the disease. In brief, we find TRPM1 autoantibodies can disrupt vision even when serum levels are barely detectable by western blot and immunohistochemistry; intraocular dexamethasone treatment alleviates MAR visual symptoms despite high levels of circulating TRPM1 autoantibodies, implicating antibody access to the retina as a key factor in MAR pathogenesis. Elevated inflammatory cytokine levels in the patient\'s eyes may be responsible for the observed damage to the blood-retinal barrier and subsequent entry of autoantibodies into the retina.
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  • 文章类型: Case Reports
    掌筋膜炎和多关节炎综合征(PFPAS)是一种极为罕见的风湿病,其特征是掌筋膜纤维化改变并伴有关节疼痛。已知与妇科恶性肿瘤有关,尤其是卵巢腺癌,胃癌,胰腺,前列腺,乳房,还有肺癌.我们介绍了一个独特的病例,该病例是一名75岁的白人女性,在八个月前诊断为卵巢癌。我们的病例强调了将PFPAS视为潜在副肿瘤综合征的重要性。它强调了需要提高认识和进一步研究,以增强对表现出类似非特异性手部症状的患者的潜在恶性肿瘤的早期发现。
    Palmar fasciitis and polyarthritis syndrome (PFPAS) is an exceedingly rare rheumatologic condition characterized by fibrotic changes in the palmar fascia with joint pains. It is known to be associated with gynecological malignancy, especially ovarian adenocarcinoma, gastric cancer, pancreatic, prostate, breast, and lung cancer. We present a unique case of a 75-year-old Caucasian female with PFPAS preceding the diagnosis of ovarian cancer by eight months. Our case highlights the importance of considering PFPAS as a potential paraneoplastic syndrome. It underscores the need for increased awareness and further studies to enhance the early detection of underlying malignancies in patients presenting with similar nonspecific hand symptoms.
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  • 文章类型: Case Reports
    与原发性肺癌相关的双侧肾上腺增生可能起因于促肾上腺皮质激素(ACTH)依赖性副肿瘤综合征或转移性增生。异位库欣综合征表现为以恶性细胞分泌促肾上腺皮质激素为特征的副肿瘤综合征。这种分泌反过来会过度刺激肾上腺皮质,导致肾上腺皮质增生,然后是皮质醇增多症。虽然罕见,肺癌可以转移到双侧肾上腺,偶尔导致出血,导致大小迅速增加。这份尸体报告旨在描绘一个不寻常的转移性肺癌病例,表现为双侧肾上腺增生和两个腹主动脉瘤(AAA)。在例行课程中解剖了一名84岁的白人男性尸体。右肺解剖显示浅黄色肿块,大小为10.0x7.4x7.0cm。一个不规则的定义,坏死,测量5.5x4.5x3.3cm的溃疡病变在左肩的侧面是明显的,与转移性肺癌病史一致。在腹部解剖时,观察到双侧肾上腺增大和两个AAAs。这些AAAs在其各自最宽的横向直径处测得6.0cm和11.0cm。左右肾上腺测量10.0x6.5x4.5cm和7.3x4.7x3.5cm,分别。我们旨在讨论该尸体中这些异常发现的可能病理生理相关性。
    Bilateral adrenal hyperplasia associated with primary lung cancer may arise from either an adrenocorticotropic hormone (ACTH)-dependent paraneoplastic syndrome or metastatic hyperplasia. Ectopic Cushing\'s syndrome manifests as a paraneoplastic syndrome characterized by the secretion of ACTH from malignant cells. This secretion can in turn overstimulate the adrenal cortex, resulting in adrenal cortical hyperplasia followed by hypercortisolism. Though rare, lung cancer can metastasize to the adrenal glands bilaterally, occasionally resulting in hemorrhage causing a rapid increase in size. This cadaveric report aims to delineate an unusual case of metastatic lung cancer, presenting with bilateral adrenal hyperplasia and two abdominal aortic aneurysms (AAA). An 84-year-old white male cadaver was dissected during routine coursework. Dissection of the right lung revealed a pale-yellow mass measuring 10.0 x 7.4 x 7.0cm. An irregularly defined, necrotic, ulcerated lesion measuring 5.5 x 4.5 x 3.3cm was evident on the lateral surface of the left shoulder, consistent with a history of metastatic lung carcinoma. Upon abdominal dissection, bilaterally enlarged adrenal glands and two AAAs were observed. These AAAs measured 6.0cm and 11.0cm at their respective widest transverse diameters. The right and left adrenal glands measured 10.0 x 6.5 x 4.5cm and 7.3 x 4.7 x 3.5cm, respectively. We aim to discuss the possible pathophysiological correlation of these unusual findings in this cadaver.
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  • 文章类型: Case Reports
    畸胎瘤占所有颅内生殖细胞肿瘤的18-20%,主要发生在松果体区域,文献中描述的小儿鞍和鞍上畸胎瘤只有少数病例。这里,我们介绍了一例患有颅内成熟畸胎瘤的儿童,其胰腺特征导致血管痉挛和随后的中风,发现CDKN2A阳性-与恶性肿瘤和小血管疾病相关的独立变异导致卒中。
    Teratomas account for 18-20% of all intracranial germ cell tumors and mostly occur in the pineal region with only a few cases of pediatric sellar and suprasellar teratomas described in the literature. Here, we present a case of a child with an intracranial mature teratoma with pancreatic features causing vasospasm and subsequent stroke, found to be positive for CDKN2A-an independent variant associated with malignancy and small vessel disease leading to stroke.
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  • 文章类型: Case Reports
    鳞状细胞癌(SCC)是第二常见的皮肤癌。由于紫外线照射是一个重要的危险因素,SCC通常发生在面部,嘴唇,头皮,手,和高跟鞋。脚是表现SCC的不寻常位置。在这份报告中,我们介绍了一例44岁女性,其右足跟严重局部复发,最初切除小学四年后,小病变。由于各种原因,在此期间,患者没有到诊所进行随访评估.考虑到病变的程度和感染风险,受影响的腿在小腿的三分之一处被截肢。此病例报告强调了对患者进行SCC风险教育并协助他们进行随访的重要性。此外,应充分注意外观可疑的足部病变。及早发现可以最大限度地减少系统性风险,包括转移和感染,并在手术干预后最大限度地保留功能。
    Squamous cell carcinoma (SCC) is the second most common type of skin cancer. As ultraviolet exposure represents an important risk factor, SCC commonly occurs on the face, lips, scalp, hands, and heels. The foot is an unusual location to manifest SCC. In this report, we present a case of a 44-year-old woman with severe local recurrence of SCC in the right heel, four years after an initial excision of a primary, small lesion. For various reasons, the patient did not visit the clinic for follow-up assessment during this period. Considering the extent of the lesion and infection risk, the affected leg was amputated at one-third of the lower leg. This case report underlines the importance of educating patients about the risk of SCC and assisting them in attending follow-up visits. In addition, adequate attention should be given to foot lesions with suspicious appearance. Early detection would minimize systemic risks, including metastasis and infection, and maximize preserved function after surgical intervention.
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  • 文章类型: Case Reports
    在并发肺肉瘤样反应和肺腺癌的背景下,将眼部肉瘤样反应描述为副肿瘤综合征的独特表现。
    单例报告和叙述性回顾。
    一名59岁的男性患者,有一年的视力下降和体重减轻的病史。临床检查显示全葡萄膜炎和多发性脉络膜视网膜病变。胸部CT扫描显示纵隔和肺门淋巴结肿大以及恶性的右下肺结节。随后的支气管镜检查和活检证实前哨淋巴结中有肺腺癌和非干酪性肉芽肿。
    尽管肺肉芽肿反应可以在肺部恶性肿瘤中看到,眼部类肉瘤样反应可能表现为系统性恶性肿瘤的副肿瘤表现,伴随的肺和眼部类肉瘤样反应的存在区分了这种情况。这些发现强调了对有体质症状的患者进行系统性检查的重要性,因为副肿瘤综合征和转移性疾病可能模拟葡萄膜炎。识别副肿瘤结节病作为潜在的临床表现是至关重要的,尤其是有慢性病指标的患者,需要对恶性肿瘤进行全面评估。
    UNASSIGNED: To describe ocular sarcoid-like reaction as a unique manifestation of paraneoplastic syndrome in the context of concurrent pulmonary sarcoid-like reaction and lung adenocarcinoma.
    UNASSIGNED: Single case report and narrative review.
    UNASSIGNED: A 59-year-old male patient presented with a year-long history of diminished vision and weight loss. Clinical examination revealed panuveitis and multiple chorioretinal lesions. A CT scan of the chest revealed mediastinal and hilar lymphadenopathy as well as a spiculated right lower lung nodule concerning for malignancy. Subsequent bronchoscopy and biopsy confirmed lung adenocarcinoma and non-caseating granulomas in sentinel lymph nodes.
    UNASSIGNED: Although pulmonary granulomatous reaction can be seen in the setting of lung malignancy, and ocular sarcoid-like reaction may present as a paraneoplastic manifestation of systemic malignancy, the presence of concomitant pulmonary and ocular sarcoid-like reactions distinguishes this case. The findings underscore the importance of a systemic workup for patients with concerning constitutional symptoms, as paraneoplastic syndromes and metastatic diseases may mimic uveitis. Recognition of paraneoplastic sarcoidosis as a potential clinical manifestation is essential, especially in patients with chronic illness indicators, necessitating a comprehensive evaluation for malignancy.
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  • 文章类型: Journal Article
    报告1例双侧弥漫性葡萄膜黑素细胞增生(BDUMP),随后出现大量单侧葡萄膜增生。
    回顾性病例报告。
    一名47岁女性,有转移性卵巢癌病史,最初表现为双侧视力丧失和后极多灶性红斑,与BDUMP一致。五年后,她表现为双侧新生血管性青光眼和单侧虹膜和睫状体肿块,考虑为恶性肿瘤。摘除术显示弥漫性葡萄膜生长,几乎涉及整个葡萄膜。
    BDUMP很少与葡萄膜增生相关。建议进行常规检查以监测有关恶性肿瘤的任何变化。
    UNASSIGNED: To report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP) followed by massive unilateral uveal proliferation.
    UNASSIGNED: Retrospective case report.
    UNASSIGNED: A 47-year-old female with history of metastatic ovarian carcinoma initially presented with bilateral vision loss and multifocal red patches on posterior poles consistent with BDUMP. Five years later, she presented with bilateral neovascular glaucoma and unilateral iris and ciliary body mass concerning for malignancy. Enucleation revealed diffuse uveal growth involving almost the entirety of the uveal tract.
    UNASSIGNED: BDUMP can rarely be associated with uveal proliferation. Routine examinations are recommended to monitor for any changes concerning malignancy.
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