paraneoplastic syndrome

副肿瘤综合征
  • 文章类型: Journal Article
    恶性腹膜间皮瘤(MPM)是一种罕见的侵袭性肿瘤,一些患者在病程中会出现副肿瘤综合征(PS)。这篇综述总结了与MPM相关的PS,关注血液学的临床特点和治疗进展,内分泌,风湿病,神经学,泌尿,和其他系统,以减少漏诊和误诊,有助于早期诊断和及时治疗,为该类患者的临床决策提供指导。
    Malignant peritoneal mesothelioma (MPM) is a rare and invasive tumor, and some patients will develop paraneoplastic syndrome (PS) during the course of the disease. This review summarizes PS associated with MPM, focusing on the clinical characteristics and treatment progress in hematological, endocrine, rheumatic, neurological, urinary, and other systems to decrease missed diagnosis and misdiagnosis, help early diagnosis and prompt treatment, and provide guidance for the clinical decision-making of this kind of patients.
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  • 文章类型: Systematic Review
    背景:肾病综合征(NS)可以作为与各种类型癌症相关的副肿瘤疾病发生。然而,副肿瘤肾病综合征(PNS)常被误诊为特发性肾病综合征或肿瘤治疗的不良反应,导致诊断延迟和治疗欠佳。与实体恶性肿瘤相关的NS的特征尚未阐明。我们系统总结了128例NS合并实体恶性肿瘤的临床资料,旨在告知PNS的临床处理。
    方法:我们在PubMed数据库中搜索了从开始之日到2023年10月发表的文章,使用以下关键词:“癌症”或“恶性肿瘤”或“瘤形成”或“肿瘤”和“肾病综合征”,“肾病”或“综合征”,肾病\“。所有数据均来自病例报告和病例系列,并且提取包括用于识别个体水平的患者数据的方法。
    结果:通过文献检索,发现了105例PNS和23例因癌症治疗引起的NS。诊断时的中位年龄为60岁,男女比例为1.8:1。在PNS患者中,以前发生过NS的表现,同时,或在诊断出肿瘤后(36%,30%,34%的病例,分别)。膜性肾病(49%)是最常见的肾脏病理,特别是在肺部患者中发现。结直肠,或者乳腺癌.不管是单独治疗还是与NS联合治疗,缓解的可能性很高.
    结论:NS的病理类型可能与PNS患者的特定恶性肿瘤有关。PNS的迅速识别以及适当的治疗干预对患者的预后有重大影响。
    BACKGROUND: Nephrotic syndrome (NS) can occur as a paraneoplastic disorder in association with various types of carcinoma. However, paraneoplastic nephrotic syndrome (PNS) is often misdiagnosed as idiopathic nephrotic syndrome or as an adverse effect of oncology treatment, leading to delayed diagnosis and suboptimal treatment. The characteristics of NS associated with solid malignancies are not yet elucidated. We systematically summarized the clinical data for 128 cases of NS combined with solid malignancies with the aim of informing the clinical management of PNS.
    METHODS: We searched the PubMed database for articles published from the date of inception through to October 2023 using the following keywords: \"cancer\" or \"malignant neoplasms\" or \"neoplasia\" or \"tumors\" and \"nephrotic syndrome\", \"nephrotic\" or \"syndrome, nephrotic\". All data were extracted from case reports and case series, and the extraction included a method for identifying individual-level patient data.
    RESULTS: A literature search yielded 105 cases of PNS and 23 of NS induced by cancer therapy. The median age at diagnosis was 60 years, with a male to female ratio of 1.8:1. In patients with PNS, manifestations of NS occurred before, concomitantly with, or after diagnosis of the tumor (in 36%, 30%, and 34% of cases, respectively). Membranous nephropathy (49%) was the most prevalent renal pathology and found particularly in patients with lung, colorectal, or breast carcinoma. Regardless of whether treatment was for cancer alone or in combination with NS, the likelihood of remission was high.
    CONCLUSIONS: The pathological type of NS may be associated with specific malignancies in patients with PNS. Prompt identification of PNS coupled with suitable therapeutic intervention has a significant impact on the outcome for patients.
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  • 文章类型: Letter
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  • 文章类型: Case Reports
    皮肌炎是一种自身免疫性疾病,其特征是明显的皮肤和肌肉表现。皮肌炎的年发病率约为(5〜10)/100万人。值得注意的是,与普通人群相比,恶性肿瘤患者发生皮肌炎的风险较高.然而,在皮肌炎与恶性肿瘤同时发生的情况下,单纯激素治疗的疗效往往不理想.此外,关于肿瘤治疗和皮肌炎治疗之间相关性的报道很少.一名60岁的男性患者出现皮肌炎,最初表现为皮疹等症状,肌肉无力,和吞咽困难.尽管接受了标准激素治疗,皮肌炎症状无明显改善.考虑到病人伴随的麻烦咳嗽,进行了进一步调查,包括CT,PET-CT,和病理活检。这些评估证实了局限期小细胞肺癌(T1cN3M0IIIB)的诊断。值得注意的是,在这个病人身上,皮肌炎被怀疑是与小细胞肺癌相关的副肿瘤综合征。采用标准化疗和放疗治疗小细胞肺癌,导致两个治疗周期后部分缓解。随着恶性肿瘤的消退,观察到皮肌炎症状显着改善,随后导致处方激素剂量逐渐减少。总之,在整个治疗过程中,我们对皮肌炎作为副肿瘤综合征进行了全面的案例研究。对肿瘤治疗的反应与皮肌炎症状的改善相吻合。因此,对于诊断为皮肌炎的患者,必须进行勤奋的恶性肿瘤筛查。
    Dermatomyositis represents an autoimmune disorder characterized by notable skin and muscular manifestations. The annual incidence of dermatomyositis stands at approximately (5~10)/1 million individuals. Notably, patients with malignant tumors exhibit an elevated risk of developing dermatomyositis compared to the general population. However, in cases where dermatomyositis co-occurs with malignancy, the efficacy of hormone therapy alone tends to be suboptimal. Moreover, reports addressing the correlation between tumor treatment and the management of dermatomyositis are scarce. A 60-year-old male patient presented with dermatomyositis, initially manifesting through symptoms such as rash, muscle weakness, and dysphagia. Despite undergoing standard hormone therapy, there was no discernible improvement in the dermatomyositis symptoms. Considering the patient\'s concomitant troublesome cough, further investigations were conducted, including CT, PET-CT, and pathological biopsy. These assessments confirmed the diagnosis of limited-stage small cell lung cancer (T1cN3M0 IIIB). Notably, in this patient, dermatomyositis was suspected to be a paraneoplastic syndrome associated with small cell lung cancer. Standard chemotherapy and radiotherapy were employed to treat the small cell lung cancer, resulting in partial remission after two treatment cycles. As the malignancy regressed, a notable improvement in dermatomyositis symptoms was observed, subsequently leading to a gradual reduction in the prescribed hormone dosage. In conclusion, we present a comprehensive case study of dermatomyositis as a paraneoplastic syndrome throughout the treatment process. The response to tumor therapy coincided with the amelioration of dermatomyositis symptoms. Therefore, diligent malignancy screening is imperative for patients diagnosed with dermatomyositis.
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  • 文章类型: Case Reports
    洛拉替尼是新一代ALK激酶抑制剂。我们描述了一名52岁的ALK阳性晚期肺腺癌患者,该患者在多线治疗联合Lorlatinib治疗的副肿瘤类白血病反应后获得缓解。
    一名52岁男性患者被诊断为IV期右肺腺癌,ALK:(+),以前曾口服克唑替尼和阿莱替尼。血常规显示疾病进展后白细胞异常升高,最大白细胞计数为179.14×10^9/L该患者参加了名为“第二阶段”的研究,多中心,开放标签,双队列研究,以评估LORLATINIB单药治疗在中国ALK抑制剂治疗的局部晚期或转移性ALK阳性非小细胞肺癌患者中的疗效和安全性。口服洛拉替尼,给药两周后,白细胞计数从179.14×10^9/L下降至正常。PFS为4.5个月。当随访影像学显示病变进展时,白细胞计数再次增加,诊断副肿瘤性白血病反应。OS为5.2个月。
    在这种情况下,四线Lorlatinib治疗ALK阳性的晚期副肿瘤类白血病反应患者是有效的.ClinicalTrials.gov标识符:NCT03909971。
    UNASSIGNED: Lorlatinib is a new generation ALK kinase inhibitor. We describe a 52-year-old patient with ALK-positive advanced lung adenocarcinoma who achieved remission after multi-line therapy combined with paraneoplastic leukemoid reaction treated with Lorlatinib.
    UNASSIGNED: A 52-year-old male patient was diagnosed with stage IV right lung adenocarcinoma, ALK: (+), previously received oral Crizotinib and Alectinib. Blood routine showed white blood cells abnormally elevated after disease progression, and maximum white blood cell count was 179.14×10^9/L. The patient was enrolled in study entitled \"a phase II, multicenter, open-label, dual-cohort study to evaluate the efficacy and safety of LORLATINIB monotherapy in ALK inhibitor-treated locally advanced or metastatic ALK-positive non-small cell lung cancer patients in China\". With oral Lorlatinib, the white blood cell count decreased from 179.14×10^9/L to normal after two weeks of administration. PFS was 4.5 months. When follow up imaging showed lesions progression, the white blood cell count increased again, diagnosing a paraneoplastic leukemic reaction. OS was 5.2 months.
    UNASSIGNED: In this case, fourth-line Lorlatinib treatment is effiective in ALK-positive advanced patient with paraneoplastic leukemoid reaction. ClinicalTrials.gov Identifier: NCT03909971.
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  • 文章类型: Journal Article
    背景:恶性腹膜间皮瘤(MPM)是一种罕见且高度侵袭性的肿瘤。其临床表现多样,症状并不具体。一些患者在病程中会出现副肿瘤综合征(PS)。本研究旨在分析MPM患者发生PS的危险因素及其对预后的影响。
    方法:回顾性分析2015年6月至2023年5月在我中心行细胞减灭术联合腹腔热灌注化疗(CRS+HIPEC)的MPM患者的临床资料。根据诊断标准将MPM患者分为PS组和非PS组。单因素和多因素分析探讨MPM患者发生PS的危险因素,并分析PS对预后的影响。
    结果:本研究共有146例MPM患者,其中PS患者60例(41.1%),无PS患者86例(58.9%)。PS发病率最高的是血小板增多症(33.6%),其次是肿瘤热(9.6%)。单因素分析显示8个因素(P<0.05),两组间差异有统计学意义:手术前评分,靶向治疗史,Karnofsky表演状态得分,术前碳水化合物抗原(CA)125水平,血管肿瘤栓子,腹膜癌指数,细胞减灭术(CC)评分和术中腹水的完整性。多因素分析确定了与PS相关的3个独立因素:术前CA125水平,血管肿瘤栓子,CC得分。生存分析显示MPM合并PS患者预后较差,虽然PS不是独立的预后因素。
    结论:PS在MPM患者中并不罕见,并且与术前CA125水平独立相关,血管肿瘤栓子和CC评分。PS常提示疾病进展和预后不良。
    BACKGROUND: Malignant peritoneal mesothelioma (MPM) is a rare and highly aggressive tumor. Its clinical manifestations are diverse, and the symptoms are not specific. Some patients will develop paraneoplastic syndrome (PS) during the disease course. This study aims to analyze the risk factors of PS in patients with MPM and their impacts on prognosis.
    METHODS: The clinical data of MPM patients who underwent cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy (CRS + HIPEC) at our center from June 2015 to May 2023 were retrospectively analyzed. MPM patients were divided into PS group and non-PS group according to the diagnostic criteria. Univariate and multivariate analyses were performed to explore the risk factors of PS in MPM patients, and to analyze the impact of PS on prognosis.
    RESULTS: There were 146 MPM patients in this study, including 60 patients (41.1%) with PS and 86 patients (58.9%) without PS. The highest incidence of PS was thrombocytosis (33.6%), followed by neoplastic fever (9.6%). Univariate analysis revealed 8 factors (P < 0.05) with statistically significant differences between the two groups: prior surgical scores, targeted therapy history, Karnofsky performance status score, preoperative carbohydrate antigen (CA) 125 level, vascular tumor embolus, peritoneal cancer index, completeness of cytoreduction (CC) score and intraoperative ascites. Multivariate analysis identified 3 independent factors associated with PS: preoperative CA 125 level, vascular tumor embolus, and CC score. Survival analysis demonstrated that MPM patients with PS had worse prognosis, although PS was not an independent prognostic factor.
    CONCLUSIONS: PS is not rare in patients with MPM, and is independently associated with preoperative CA 125 level, vascular tumor embolus and CC score. PS often indicates advanced disease and poor prognosis.
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  • 文章类型: Review
    背景:关于抗代谢谷氨酸受体5(mGluR5)脑炎的报道非常有限,尤其是缺乏儿科研究。这种疾病主要伴有肿瘤,主要是霍奇金淋巴瘤.没有其他肿瘤的报告,如神经节细胞瘤已被报道与抗mGluR5脑炎有关。
    我们报告一例12岁男孩抗mGluR5脑炎合并神经节细胞瘤。患者患有精神障碍,包括幻听,和睡眠障碍。他的头颅磁共振成像(MRI)显示右岛叶异常。脑脊液和血清中自身免疫性脑炎抗体检测mGluR5IgG抗体呈阳性(分别为1:3.2,1:100)。腹部CT示:左侧腹膜后肿块,病理证实为神经节细胞瘤。患者接受神经节细胞瘤切除术。一线免疫疗法后(糖皮质激素,丙种球蛋白),他的病情得到了改善。此外,我们提供了6例儿科抗mGluR5脑炎病例的总结。他们大多数患有霍奇金淋巴瘤,除了目前报告的病例与神经节细胞瘤共病。疗效满意。
    结论:我们报告了首例抗mGlur5脑炎合并神经节细胞瘤的患者。提示除了关注与抗mGlur5脑炎相关的常见淋巴瘤,我们还应该筛查其他肿瘤的可能性,以便早期发现原因,积极治疗和预防复发。
    BACKGROUND: There are very limited reports on anti-metabolic glutamate receptor5 (mGluR5) encephalitis, especially lacking of pediatric research. The disease was mostly accompanied by tumors, mainly Hodgkin\'s lymphoma. No reports of other tumors, such as gangliocytoma have been reported to associate with anti-mGluR5 encephalitis so far.
    UNASSIGNED: We reported a case of a 12-year-old boy with anti-mGluR5 encephalitis complicated with gangliocytoma. The patient suffered from mental disorders including auditory hallucination, and sleep disorders. His cranial magnetic resonance imaging (MRI) showed an abnormality in the right insular lobe. Autoimmune encephalitis antibodies testing was positive for mGluR5 IgG antibody both in cerebrospinal fluid and serum (1:3.2, 1:100 respectively). Abdominal CT indicated a mass in left retroperitoneal confirmed with gangliocytoma via pathology. The patient underwent resection of gangliocytoma. After first-line immunotherapy (glucocorticoid, gamma globulin), his condition was improved. Furthermore, we provide a summary of 6 pediatric cases of Anti-mGluR5 encephalitis. Most of them complicated with Hodgkin\'s lymphoma, except the case currently reported comorbid with gangliocytoma. The curative effect is satisfactory.
    CONCLUSIONS: We report the first patient with anti-mGlur5 encephalitis complicated with gangliocytoma. It suggests that in addition to paying attention to the common lymphoma associated with anti-mGlur5 encephalitis, we should also screen the possibility of other tumors for early detection of the cause, active treatment and prevention of recurrence.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    低血糖有多种原因,但最常见的是胰岛素治疗的并发症。除了胰岛素治疗,还应考虑引起副肿瘤综合征的胰腺起源的胰岛素瘤和胰腺外肿瘤。胸膜孤立性纤维瘤(SFTP)是罕见的肿瘤,当与低血糖相关时,会导致Doege-Potter综合征。本文报道了一例69岁的Doege-Potter综合征患者,并接受了SFTP的首次手术切除。然而,9年后肿瘤复发,伴有低血糖症状和植入物转移。这种复发性肿瘤起源于内脏胸膜,更具侵略性,并侵入隔膜和顶叶胸膜。第二次手术切除肿瘤后,低血糖症状消失。
    Hypoglycemia has multiple causes, but the most common is a complication of insulin treatment. In addition to insulin therapy, tumors such as insulinomas of pancreatic origin and extrapancreatic tumors causing paraneoplastic syndromes should also be considered. Solitary fibrous tumors of the pleura (SFTP) is rare tumor, which when associated with hypoglycemia causes Doege-Potter syndrome. This article reports a case of a 69-year-old man with Doege-Potter syndrome and underwent the first surgical resection for SFTP. However, the tumor recurred 9 years later with hypoglycemic symptoms and implant metastasis. This recurrent tumor originated from the visceral pleura, was more aggressive and invaded the diaphragm and parietal pleura. After the second surgical removal of the tumor, the hypoglycemic symptoms disappeared.
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  • 文章类型: Review
    背景:抗α-氨基-3-羟基-5-甲基-4-异恶唑丙酸受体(AMPAR)脑炎,一种罕见的自身免疫性脑炎(AE)亚型,经常发现与胸腺瘤等肿瘤有关,肺癌,卵巢肿瘤,和乳腺癌,肿瘤通常是在脑炎开始后的筛查过程中检测到的。肿瘤被认为是AE的触发因素,但机制尚不清楚。
    方法:一名53岁女性在高级别浆液性卵巢癌原发性细胞减灭术后两天出现短期记忆丧失(HGSOC,图IC3阶段)。基于细胞的测定发现AMPARCluA2IgG在血清(1:3.2)和脑脊液(1:32)中均为阳性。此外,在卵巢肿瘤组织石蜡切片中也发现轻度AMPARGluA1和强GluA2阳性表达,表明卵巢细胞减灭术可能刺激受体抗原释放到循环系统中。病人的病情在两周内恶化,发展意识和自主神经功能障碍,导致ICU入院。口服类固醇,静脉注射免疫球蛋白,血浆置换,利妥昔单抗治疗,3个月后患者的意识明显改善。
    结论:我们介绍了第一例抗AMPAR脑炎发生在原发性细胞减灭术后的HGSOC患者,并检索了副肿瘤抗AMPAR脑炎病例(n=66)。妇科医生应注意卵巢肿瘤切除后不久出现认知功能障碍或精神症状的患者,并始终将AE纳入鉴别诊断。
    Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis, a rare subtype of autoimmune encephalitis (AE), is often found associated with tumors such as thymoma, lung cancer, ovarian tumors, and breast cancer, and the tumors were generally detected during the screening process after the encephalitis initiated. The tumor is considered a trigger of AE, but the mechanism remains unclear.
    A 53-year-old woman presented short-term memory loss two days after the primary cytoreduction for high-grade serous ovarian cancer (HGSOC, FIGO stage IC3). Cell-based assay found AMPAR CluA2 IgG positive in both serum (1:3.2) and cerebrospinal fluid (1:32). Moreover, mild AMPAR GluA1 and strong GluA2 expressions were also found positive in the paraffin sections of ovarian tumor tissue, indicating the ovarian cytoreduction surgery might stimulate the release of receptor antigens into the circulation system. The patient\'s condition deteriorated within two weeks, developing consciousness and autonomic dysfunction, leading to ICU admission. With oral steroids, intravenous immunoglobulin, plasmapheresis, and rituximab treatment, the patient\'s consciousness markedly improved after three months.
    We presented the first case of anti-AMPAR encephalitis developed right after the primary cytoreduction of a patient with HGSOC and retrieved paraneoplastic anti-AMPAR encephalitis cases (n = 66). Gynecologists should pay attention to patients who develop cognitive dysfunction or psychiatric symptoms shortly after the ovarian tumor resection and always include AE in the differentiation diagnosis.
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