treatment and prognosis

治疗和预后
  • 文章类型: Case Reports
    大多数卵巢肿瘤表现出纯粹的组织学特征。然而,具有相同组织遗传学起源但不同组织学亚型的肿瘤组合比较常见.此外,具有不同组织遗传学起源的肿瘤的同时发生非常罕见。通常,这些混合肿瘤包括混合上皮肿瘤,混合性上皮间质肿瘤,混合性生殖细胞-性索-间质肿瘤,和混合生殖细胞肿瘤。然而,混合性上皮-性索间质-淋巴造血系统肿瘤是罕见的。目前,临床医生对这类肿瘤的了解有限,和流行病学,诊断,这种疾病的治疗方法尚未建立。
    我们报告一例73岁女性腹胀疼痛3个月。影像学评估显示盆腔肿块较大,超声提示良性卵巢囊肿和平滑肌瘤。此外,计算机断层扫描(CT)和磁共振成像(MRI)显示为恶性肿瘤。血液测试显示CA125和CA199水平显着增加。该患者接受了全腹子宫切除术和双侧附件卵巢切除术。在手术过程中,在右侧卵巢观察到一个大的多结节性囊性实性肿块,最大结节直径为14.2cm。肿块的实心区域呈灰白色和灰褐色,而囊性区域包含透明液体,光滑的壁厚0.2厘米,没有囊内固体区域。左卵巢有单发结节,最大的是直径4厘米。右侧卵巢的显微镜检查显示三种不同的细胞类型。第一种类型的细胞面积是类似的,圆形,梭形,和交错的混合细胞,边界不清,核仁或有丝分裂罕见。第二种类型的细胞区域是囊性扩张区域。囊肿壁被单层扁平上皮覆盖,丰富的嗜酸性细胞浆,均匀核染色质,没有乳头状结构.第三种类型是弥漫性淋巴区域,具有均匀的中等大小的细胞,核染色质粗糙,核仁和有丝分裂明显。左卵巢细胞形态单一,这与右卵巢中第一种类型的细胞区域一致。右卵巢的免疫组织化学显示第一个区域表达波形蛋白,抑制素-α,calretinin,SF-1、WT-1和CD56,Ki-67为5%,没有CKpan表达式。第二个区域表示CKpan,Ki-67为1%。第三区表达CD20、Pax-5、Bcl-6、Bcl-2、MUM1、CD45和C-myc,Ki-67为70%,IGH克隆基因重排阳性。最后,病理诊断为右侧卵巢混合卵巢肿瘤,包括肌瘤-纤维瘤,浆液性囊腺瘤,弥漫性大B细胞淋巴瘤,和左侧卵巢的纤维瘤。15个月后对患者进行的随访检查显示,身体其他部位无肿块或淋巴结肿大,未观察到复发或转移。
    我们介绍了一例绝经后妇女,患有罕见的囊瘤-纤维瘤,卵巢浆液性囊腺瘤和弥漫性大B细胞淋巴瘤。据我们所知,这是此类组合的首例报道。典型的病理形态学和免疫组织化学对该病的诊断至关重要。由于对这种疾病的知识有限,其发病机制和组织起源不明。临床医生应该对此类患者保持谨慎。我们相信此病例报告可能为此类肿瘤患者的诊断和治疗提供一些新的见解。
    UNASSIGNED: Most ovarian tumors exhibit a pure histological characteristic. Nevertheless, a combination of tumors with the same histogenetic origin but different histologic subtypes is relatively common. Additionally, co-occurrence of tumors with different histogenetic origins is very rare. Typically, these mixed tumors include mixed epithelial tumors, mixed epithelial-stromal tumors, mixed germ cell-sex cord-stromal tumors, and mixed germ cell tumors. However, mixed epithelial-sex cord stromal-lymphohematopoietic system tumors are rare. Currently, clinicians have limited knowledge of this type of tumor, and the epidemiology, diagnosis, and treatment of this disease are yet to be established.
    UNASSIGNED: We report a case of a 73-year-old woman with abdominal distension and pain for three months. Imaging evaluation revealed a large pelvic mass, with ultrasound suggesting a benign ovarian cyst along with leiomyoma. Furthermore, computed tomography (CT) and magnetic resonance imaging (MRI) revealed a malignant tumor. Blood tests showed significant increases in CA125 and CA199 levels. The patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. During the surgery, a large multinodular cystic solid mass was observed in the right ovary, and the maximum nodular diameter was 14.2 cm. The solid areas of the mass appeared gray-white and taupe, whereas the cystic areas contained clear liquid with smooth walls 0.2 cm thick and no intracystic solid areas. The left ovary had solitary nodules, the largest being 4 cm in diameter. Microscopic examination of the right ovary revealed three different cell types. The first type of cell area was analogous, round, fusiform, and staggered mixed cells with unclear boundaries and rare nucleolus or mitosis. The second type of cell area was the cystic dilatation area. The cyst wall was covered with a single layer of flat epithelium, rich eosinophilic cytoplasm, uniform nuclear chromatin, and no papillary structures. The third type was a diffuse lymphoid region with uniform medium-sized cells, rough nuclear chromatin and evident nucleoli and mitosis. The morphology of the left ovarian cell was single, which was consistent with the first type of cell area in the right ovary. Immunohistochemistry of the right ovary indicated that the first region expressed vimentin, inhibin-α, calretinin, SF-1, WT-1 and CD56, with Ki-67 at 5 %, and no CKpan expression. The second region expressed CKpan, with Ki-67 at 1 %. The third region expressed CD20, Pax-5, Bcl-6, Bcl-2, MUM1, CD45, and C-myc, with Ki-67 at 70 %, and positive IGH clonal gene rearrangement. Lastly, the pathological diagnosis was a mixed ovarian tumor in the right ovary, comprising thecoma-fibroma, serous cystadenoma, diffuse large B-cell lymphoma, and a thecoma-fibroma in the left ovary. A follow-up examination of the patient after 15 months showed no mass or lymph node enlargement in other parts of the body, and no recurrence or metastasis was observed.
    UNASSIGNED: We present a case of a postmenopausal woman with a rare combination of thecoma-fibroma, serous cystadenoma and diffuse large B-cell lymphoma in the ovary. To the best of our knowledge, this is the first reported case of such a combination. Typical pathological morphology and immunohistochemistry are crucial for the diagnosis of this disease. Owing to the limited knowledge of the disease, its pathogenesis and tissue origin are unknown. Clinicians should be careful about such patients. We believe this case report may provide some novel insights into the diagnosis and therapy of patients with this type of tumor.
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  • 文章类型: Journal Article
    糖尿病视网膜病变(DR)是糖尿病最常见的微血管并发症,也是全球致盲的主要原因之一。应该更加重视诊断,DR的治疗和预后。扫描源光学相干断层扫描血管造影(SS-OCTA)是近年来提出的一种新颖的成像技术。它可以准确地呈现视网膜的各个层面,脉络膜毛细血管,黄斑,和视神经乳头微循环,这对DR的诊断和预后是新的。然而,SS-OCTA受到固定不良或严重介质混浊的限制,并且容易受到运动伪影和分割错误的影响。需要解决未来的局限性,并进行大型前瞻性试验以完善SS-OCTA与DR的相关性。本研究综述了SS-OCTA在临床诊断中的应用进展。DR的治疗和预后。
    Diabetic retinopathy (DR) is the most common microvascular complication of diabetes and one of the leading causes of global blinding. More attention should be paid to the diagnosis, treatment and prognosis of DR. Swept-source optical coherence tomography angiography (SS-OCTA) is a novel imaging technique presented in recent years. It can accurately present the various levels of the retina, choriocapillaris, macula, and the optic papillary microcirculation, which is new to the diagnosis and prognosis of DR. However, SS-OCTA is limited by poor fixation or severe media clouding and is susceptible to motion artefacts and segmentation errors. Future limitations need to be addressed and large prospective trials conducted to refine the relevance of SS-OCTA to DR. The present study reviews the advances in clinical application of SS-OCTA in diagnosis, treatment and prognosis of DR.
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  • 文章类型: Journal Article
    背景与其他子宫癌相比,子宫平滑肌肉瘤(LMS)具有更多的复发和更高的死亡率。考虑到文献中案例系列的数量有限,标准治疗方法的有效性有限,以及分子生物标记的不足,我们计划研究这些患者的治疗方法和生存结局的效果.方法本研究是回顾性设计的,以及在安卡拉大学医学院接受随访和治疗的患者的记录,肿瘤内科诊所,在2011年1月1日至2021年12月31日之间进行了审查。包括病理诊断为子宫LMS的年龄超过18岁的患者。人口统计,临床,使用医院数据库记录病理数据。根据AJCC癌症分期手册,对每位患者重新评估了国际妇产科联合会(FIGO)分期,第八版(2017年)。肿瘤大小,location,和等级也进行了评估。治疗的类型,协议,并记录不良反应。复发的病人,复发定位,记录并比较复发时的治疗方法。结果28例患者纳入。患者的平均年龄为53.7岁。中位随访时间为39.3个月。在22例(78.57%)患者中可以检测到LMS的定位,其中20例(90.9%)患者有壁间,1例(4.5%)有粘膜下,1例(4.5%)有浆膜下LMS。所有患者(26,92.8%)均接受了初次手术,除了2例(7.14%)在诊断时转移的患者。在多学科肿瘤理事会中,对7例(25%)复发风险较高的患者提出了辅助治疗建议。在2例(7.1%)转移性患者中,有1例(3.5%)观察到部分缓解,在另一个观察到稳定的疾病。22例(84.6%)患者出现复发。15例(53.6%)患者在随访期间死亡。绝经前患者的生存率较好(99.2个月比51.6个月,P=0.056)。当比较接受和未接受辅助治疗的患者的生存率时,没有发现显着差异。在复发患者中,接受和未接受手术治疗的患者之间的生存率无显著差异.结论子宫LMS是一种罕见且侵袭性的恶性肿瘤,诊断方法有限。频繁复发,高死亡率,有限使用非手术治疗。辅助治疗对生存的积极影响尚未得到证实。需要进一步的研究来研究激素受体状态对预后和新的生物标志物的影响。
    Background Uterine leiomyosarcomas (LMS) are associated with more recurrence and higher mortality compared to other uterine cancers. Considering the limited number of case series in the literature, the limited effectiveness of standard treatment methods, and the inadequacy of molecular biomarkers, we planned to investigate the effects of treatment methods and survival outcomes in these patients. Methodology The study was designed retrospectively, and the records of patients who were followed up and treated at Ankara University Faculty of Medicine, Medical Oncology Clinic, between January 1, 2011, and December 31, 2021, were reviewed. Patients over 18 years of age with a pathological diagnosis of uterine LMS were included. Demographic, clinical, and pathological data were recorded using the hospital database. The International Federation of Gynecology and Obstetrics (FIGO) staging was reassessed for each patient in accordance with the AJCC Cancer Staging Manual, Eighth Edition (2017). Tumor size, location, and grade were also evaluated. Types of treatments, protocols, and adverse effects were recorded. Relapsed patients, relapse localization, and treatments given at relapse were recorded and compared.  Results Twenty-eight patients were included. The mean age of the patients was 53.7 years. The median follow-up time was 39.3 months. The localization of LMS could be detected in 22 (78.57%) patients, among them 20 (90.9%) patients had intramural, 1 (4.5%) had submucosal, and 1 (4.5%) had subserosal LMS. All patients (26, 92.8%) underwent primary surgery, except for 2 (7.14%) patients who were metastatic at the time of diagnosis. Adjuvant treatment suggestion was made for 7 (25%) patients with a high risk of recurrence in the multidisciplinary tumor council. Partial response was observed in 1 (3.5%) of the 2 (7.1%) metastatic patients, and stable disease was observed in the other. Recurrence was detected in 22 (84.6%) patients . Fifteen (53.6%) patients died during the follow-up period. Survival was better in premenopausal patients (99.2 versus 51.6 months, P = 0.056). No significant difference was found when the survival of patients who received and did not receive adjuvant treatment were compared. In relapsed patients, there was no significant difference in survival between patients who underwent and did not undergo surgical treatment. Conclusions Uterine LMS is a rare and aggressive malignancy with limited diagnostic methods, frequent recurrences, high mortality, and limited use of nonsurgical treatments. The positive effect of adjuvant treatment on survival has not been demonstrated. Further studies are needed to investigate the effect of hormone receptor status on prognosis and new biomarkers.
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  • 文章类型: Journal Article
    原发性小肠淋巴瘤(PSIL)的全球发病率正在增加。然而,对这种疾病的临床和内镜特征知之甚少。这项研究的目的是调查PSIL患者的临床和内镜资料,为了增强我们对这种疾病的认识,提高诊断准确性,并促进更准确的预后估计。
    对2012-2021年山东大学齐鲁医院诊断为PSIL的94例患者进行回顾性研究。临床数据,肠镜检查结果,治疗方式,和生存时间被收集和分析。
    94例PSIL患者(52例男性)纳入本研究。发病年龄中位数为58.5岁(范围:19-80岁)。弥漫大B细胞淋巴瘤(n=37)是最常见的病理类型。腹痛(n=59)是最常见的临床表现。回盲区(n=32)是最常见的受影响的部位,11.7%的患者有多个病灶。在诊断的时候,大多数患者(n=68)处于I-II期.开发了一种新的PSIL内镜分类,包括肥厚型,外生型,卵泡/息肉样类型,溃疡性类型,和扩散类型。手术未显示总生存期的显着增加;化疗是最常用的治疗方法。T细胞淋巴瘤,阶段III-IV,“B”症状,溃疡型与不良预后相关。
    本研究对94例患者的PSIL的临床和内窥镜特征进行了全面分析。这突出了在小肠镜检查中考虑临床和内镜特征对于准确诊断和预后评估的重要性。PSIL的早期检测和治疗与良好的预后相关。我们的发现还表明,某些风险因素,如病理类型,“B”症状,和内窥镜类型,可能影响PSIL患者的生存。这些结果强调了在PSIL的诊断和治疗中需要仔细考虑这些因素。
    UNASSIGNED: The worldwide incidence of primary small intestinal lymphoma (PSIL) is increasing. However, little is known about the clinical and endoscopic characteristics of this disease. The aim of this study was to investigate the clinical and endoscopic data of patients with PSIL, with the goal of enhancing our understanding of the disease, improving diagnostic accuracy, and facilitating more accurate prognosis estimation.
    UNASSIGNED: Ninety-four patients diagnosed with PSIL were retrospectively studied at Qilu Hospital of Shandong University between 2012 and 2021. The clinical data, enteroscopy findings, treatment modalities, and survival times were collected and analyzed.
    UNASSIGNED: Ninety-four patients (52 males) with PSIL were included in this study. The median age of onset was 58.5 years (range: 19-80 years). Diffuse large B-cell lymphoma (n=37) was the most common pathological type. Abdominal pain (n=59) was the most frequent clinical presentation. The ileocecal region (n=32) was the most commonly affected site, and 11.7% of patients had multiple lesions. At the time of diagnosis, the majority of patients (n=68) were in stages I-II. A new endoscopic classification of PSIL was developed, including hypertrophic type, exophytic type, follicular/polypoid type, ulcerative type, and diffusion type. Surgery did not show a significant increase in overall survival; chemotherapy was the most commonly administered treatment. T-cell lymphoma, stages III-IV, \"B\" symptoms, and ulcerative type were associated with poor prognosis.
    UNASSIGNED: This study provides a comprehensive analysis of the clinical and endoscopic features of PSIL in 94 patients. This highlights the importance of considering clinical and endoscopic characteristics for accurate diagnosis and prognosis estimation during small bowel enteroscopy. Early detection and treatment of PSIL is associated with a favorable prognosis. Our findings also suggest that certain risk factors, such as pathological type, \"B\" symptoms, and endoscopic type, may affect the survival of PSIL patients. These results underscore the need for careful consideration of these factors in the diagnosis and treatment of PSIL.
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  • 文章类型: Journal Article
    OBJECTIVE: Diffuse panbronchiolitis (DPB) is a chronic airway inflammation with low specificity and its diagnosis is often missed or delayed. This study aims to summarize the clinical characteristics and treatment of DPB in order to improve the understanding and diagnosis of the disease.
    METHODS: The clinical data of 32 DPB patients were collected, analyzed and summarized from March 1, 2013 to March 1, 2022 in the Second Xiangya Hospital of Central South University. The basic information, clinical manifestations, laboratory tests, pulmonary function, imaging tests, treatment, and regression of patients were analyzed.
    RESULTS: A total of 32 patients were enrolled in the final analysis, with a male-to-female ratio at 1.67. The median age at symptom onset was 26.5 (11.0-69.0) years, and the median age of diagnosis was 47.5 (16.0-77.0) years. All patients presented with chronic cough and copious sputum production. A total of 26 patients had post activity shortness of breath and 14 patients had a positive result (blood cold agglutination test titer≥1꞉64). Pulmonary function examination was performed in 31 patients, 18 patients showed mixed pulmonary ventilation dysfunction, 12 patients showed obstructive pulmonary ventilation, and 1 patient had normal pulmonary ventilation function. A total of 31 patients had a bilateral, diffuse, small nodule pattern on chest CT. All patients were treated with macrolides. A total of 31 patients showed improvement, and 20 patients showed improvement in partial pressure of oxygen and blood oxygen saturation compared with before at discharge. A total of 12 patients were re-examined by chest CT after completing macrolides treatment, 6 cases showed less diffuse nodules, 5 cases showed no significant changes, and 1 case showed more diffuse nodules, which indicated the disease progression. Seven patients received pulmonary function tests after completing macrolides treatment, forced expiratory volume in one second (FEV1) and FEV1/forced vital capacitywere improved, but forced expiratory flow at 25% of vital capacity did not change significantly.
    CONCLUSIONS: The clinical manifestations of DPB are nonspecific. Early diagnosis and treatment are very important for the prognosis of patients.
    目的: 弥漫性泛细支气管炎(diffuse panbronchiolitis,DPB)是一种慢性气道炎症,临床表现缺乏特异性,漏诊及误诊率高。本研究旨在通过总结DPB的临床特征及治疗情况,提高临床医师对该病的认识及诊断水平。方法: 收集2013年3月1日至2022年3月1日中南大学湘雅二医院收治的32例DPB患者的临床资料,分析患者的基础信息、临床表现、实验室检查、肺功能、影像学检查、治疗及转归情况。结果: 32例DPB患者的男女比例为1.67꞉1,中位发病年龄为26.5(11.0~69.0)岁,中位确诊年龄为47.5(16.0~77.0)岁。32例患者入院时均有咳嗽、咳痰,26例存在活动后气促。14例患者血冷凝集试验效价≥1꞉64。31例患者行肺功能检查,18例表现为混合型肺通气功能障碍,12例表现为阻塞型肺通气功能,1例肺通气功能正常。31例肺部CT表现为弥漫性小结节影。32例患者均接受大环内酯类药物治疗,出院时31例患者临床症状好转,20例患者复查血气分析提示氧分压、血氧饱和度较前好转。12例患者治疗后复查肺部CT,6例提示弥漫性小结节影较前减少,5例与治疗前相比无明显变化,1例提示弥漫性小结节影较前增多,提示疾病进展。7例患者治疗后行肺功能检查,治疗前后第1秒用力呼气量(forced expiratory volume in one second,FEV1)及FEV1/用力肺活量有改善,用力呼出25%肺活量时最大用力呼气流量无明显变化。结论: DPB临床表现无特异性,早诊断、早治疗对患者的预后至关重要。.
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  • 文章类型: Case Reports
    妊娠急性脂肪肝(AFLP)是一种罕见的,在妊娠晚期或产后期影响妇女的灾难性疾病。我们报告了一例24岁的G2A1,GA为35周,患有闭经,恶心,发烧,呕吐,头痛和眼睛发黄。患者被诊断为重度先兆子痫伴宫内死亡(IUD),溶血升高肝酶低血小板(HELLP)。调查显示低血糖,血小板计数低,并提高了肝脏酶的凝血谱。病人被关在重症监护室,用米索前列醇进行诱导,她接生了一个宫内节育器婴儿.病人的状况恶化了,她出现了肺水肿.因此,她被插管了.肝脏超声检查(USG)显示回声结构改变。然后患者的状况得到改善。AFLP的早期诊断需要高度怀疑。没有明显/妊娠期糖尿病的孕妇的低血糖以及肝脏紊乱和血小板减少症为诊断AFLP提供了线索。及时诊断和干预可以降低孕产妇和胎儿的发病率和死亡率。
    Acute fatty liver of pregnancy (AFLP) is a rare, catastrophic disease affecting women in the third trimester of pregnancy or the postpartum period. We report a case of 24-year-old G2A1 with GA of 35 weeks who presented with amenorrhea, nausea, fever, vomiting, headache and yellowness of eyes. The patient was diagnosed with severe preeclampsia with Intrauterine death (IUD) with hemolysis elevated liver enzymes low platelets (HELLP). Investigations showed hypoglycemia, low platelet count, and raised liver enzymes with an altered coagulation profile. The patient was kept in the Medicine Intensive care unit, induction was done with misoprostol, and she delivered an IUD baby. The condition of the patient deteriorated, and she developed pulmonary edema. Thus, she was intubated. Ultrasonography (USG) of the liver showed altered echotexture. The condition of the patient was then improved. AFLP requires a high index of suspicion for early diagnosis. Hypoglycemia in a pregnant woman without overt /gestational diabetes mellitus along with deranged liver panels and thrombocytopenia gives a clue in diagnosing AFLP. Timely diagnosis and intervention can reduce both maternal and fetal morbidity and mortality.
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  • 文章类型: Journal Article
    目的:确定临床特征,病理类型,肿瘤标志物,治疗,中国原发性泪囊淋巴瘤患者的预后。
    方法:本病例回顾性研究分析了15例原发性泪囊淋巴瘤患者。收集的临床数据包括性别,诊断时的年龄,症状,影像学检查结果,病理诊断,病原体鉴定,肿瘤标志物,治疗,后续行动,和预后。使用描述性统计来表征患者。无进展生存期(PFS)定义为从手术到最后一次随访的时间,首次记录肿瘤复发,或死亡。
    结果:左眼(n=6)或右眼(n=9)有7名男性和8名女性患有单侧原发性泪囊淋巴瘤。13例患者的最初症状是泪溢,2例患者出现泪囊区红肿。所有的病人最终都出现了泪溢,12个泪囊区有肿块。术前血浆肿瘤标志物分析提示14例同型半胱氨酸升高,9有升高的β2-微球蛋白,2名患者的乳酸脱氢酶(LDH)升高;2名患者的所有三种标志物均升高,1例患者无任何标记物升高。所有患者均接受手术切除,12例患者接受术后化疗。病理类型为DLBCL(n=8),MALT淋巴瘤(n=5),和NK/T细胞淋巴瘤,鼻型(n=2)。平均随访时间为25.8mo(范围:4-41),2例患者死亡。7例接受肿块切除联合泪囊鼻腔吻合术(DCR)的患者术后无泪溢。8例仅接受肿块切除术的患者术后出现不同程度的溢唇。术前LDH升高与NK/T细胞淋巴瘤,鼻型与不良预后相关。
    结论:对于大多数原发性泪囊淋巴瘤患者,早期诊断和治疗可以导致良好的预后。肿块切除联合DCR可减少术后泪溢的发生。病理类型和肿瘤标志物状态与预后相关。
    OBJECTIVE: To determine the clinical characteristics, pathological types, tumor markers, treatments, and outcomes of Chinese patients with primary lacrimal sac lymphoma.
    METHODS: This case-based retrospective study analyzed 15 Chinese patients with primary lacrimal sac lymphoma. The clinical data collected included gender, age at diagnosis, symptoms, imaging examination results, pathologic diagnosis, pathogen identification, tumor markers, treatments, follow-up, and prognosis. Descriptive statistics were used to characterize the patients. Progression-free survival (PFS) was defined as the time from surgery to the last follow-up, first record of tumor recurrence, or death.
    RESULTS: There were 7 males and 8 females with unilateral primary lacrimal sac lymphoma in the left eye (n=6) or right eye (n=9). The initial symptom in 13 patients was epiphora, and 2 patients had redness and swelling in the lacrimal sac area. All patients ultimately developed epiphora, and 12 had masses in the lacrimal sac area. Analysis of preoperative plasma tumor markers indicated 14 patients had elevated homocysteine, 9 had elevated β2-microglobulin, and 2 had elevated lactate dehydrogenase (LDH); 2 patients had elevations of all three markers, and 1 patient had no elevation of any marker. All patients underwent surgical resection and 12 patients received postoperative chemotherapy. The pathological types were DLBCL (n=8), MALT lymphoma (n=5), and NK/T-cell lymphoma, nasal type (n=2). The mean follow-up time was 25.8mo (range: 4-41) and 2 patients died. Seven patients who underwent mass excision combined with dacryocystorhinostomy (DCR) had no postoperative epiphora. Eight patients who only underwent mass excision had varying degrees of postoperative epiphora. Preoperative LDH elevation and NK/T-cell lymphoma, nasal type were associated with poor prognoses.
    CONCLUSIONS: Early diagnosis and treatment can lead to a good prognosis for most patients with primary lacrimal sac lymphoma. Mass resection combined with DCR can reduce the occurrence of post-surgical epiphora. The pathology type and tumor marker status are associated with prognosis.
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  • 文章类型: Journal Article
    未经授权:分泌性乳腺癌(SBC)是一种罕见的乳腺恶性肿瘤。关于SBC的大多数现有研究是病例报告或小病例系列,由于数据库的限制,少数可用的大样本研究缺乏关键信息。提高对SBC的认识,为临床实践提供参考,我们系统地回顾了人口统计学,临床,病理性,和SBC的遗传特征,以及它的治疗和预后。
    UNASSIGNED:我们进行了PubMed搜索,关键词为“分泌性乳腺癌”或“青少年乳腺癌”。1966年1月至2022年2月出版的相关英文出版物以3个级别进行了手动筛选-标题,abstract,和全文-为了确定呈现人口统计的文章,临床,病理性,和SBC的遗传特征,以及它的治疗和预后。
    UNASSIGNED:SBC缺乏特异性临床表现,具有典型的病理和分子特征,包括细胞内和细胞外嗜酸性粒细胞分泌,免疫谱类似于激素受体阳性肿瘤,和ETV6-NTRK3融合基因。手术仍然是SBC的主要治疗方法。大多数研究人员建议成人SBC术后放疗,但不适用于儿科患者。化疗和内分泌治疗的证据不足,ETV6-NTRK3融合基因的靶向治疗显示出良好的反应。除少数经历远处转移的患者外,大多数SBC患者预后良好。未来的研究将集中在预后不良的SBC患者的分子特征上。
    UNASSIGNED:组织病理学和分子遗传学的发展促进了SBC临床诊断的进展。这篇综述的目的是为更好的SBC的临床治疗提供指导。特别是在疾病识别和患者预后分类方面。
    UNASSIGNED: Secretory breast carcinoma (SBC) is a rare breast malignancy. Most available studies on SBC are case reports or small case series, and the few large-sample studies available lack critical information due to database limitations. To improve the understanding of SBC and provide a reference for clinical practice, we systematically reviewed the demographic, clinical, pathologic, and genetic characteristics of SBC, as well as its treatment and prognosis.
    UNASSIGNED: We conducted a PubMed search with the keywords \"secretory breast carcinoma\" or \"juvenile breast carcinoma\". Relevant English-language publications published from January 1966 to February 2022 were screened manually at 3 levels-title, abstract, and full text-to identify the articles that presented the demographic, clinical, pathologic, and genetic characteristics of SBC, as well as its treatment and prognosis.
    UNASSIGNED: SBC lacks specific clinical manifestations and has typical pathological and molecular characteristics, including intracellular and extracellular eosinophilic secretions, immune spectrum similar to hormone receptor-positive tumors, and the ETV6-NTRK3 fusion gene. Surgery remains the primary treatment for SBC. Postoperative radiotherapy is recommended by most researchers for adult SBC but not for pediatric patients. The evidence of chemotherapy and endocrine therapy is insufficient, and targeted therapy of the ETV6-NTRK3 fusion gene shows a good response. Most patients with SBC have a good prognosis except for a few patients who experience distant metastases. Future studies will be focused on the molecular characteristics of those patients with SBC who have a poor prognosis.
    UNASSIGNED: The development of histopathology and molecular genetics has promoted the progress of the clinical diagnosis of SBC. The purpose of this review is to serve as a guide for the better clinical treatment of SBC, particularly in the areas of disease identification and prognosis classification for patients.
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  • 文章类型: Case Reports
    青少年双侧卵巢上皮性肿瘤很少见。此外,交界性粘液性肿瘤与局部上皮内癌和间变性癌的发生率更低。在这里,我们介绍了1例(1名17岁女性)月经周期规律,进食时胃痛,被诊断患有伴有壁结节的左卵巢肿瘤.右卵巢囊肿,左卵巢,手术切除了输卵管.术中诊断提示双侧卵巢肿瘤伴壁结节,其中包括三种不同的病理类型:肉瘤样转化,间变性癌,和肉瘤.术后给予紫杉醇联合卡铂6个周期,在化疗开始时给予促性腺激素释放激素激动剂(GnRHa)3个周期的卵巢功能保护。定期随访(末次随访为术后48个月)妇科超声及肿瘤指标均未提示复发。在临床实践中,有必要注意青春期女性的腹痛等症状。建议在手术前进行常规无创盆腔超声检查,以充分评估肿瘤的性质,并决定操作模式。此外,术中应尽快对组织进行冰冻病理检查。
    Bilateral ovarian epithelial neoplasms in adolescents are rare. Moreover, borderline mucinous neoplasms with local intraepithelial carcinoma with anaplastic carcinoma are even more infrequent. Herein, we presented a single case (a 17-year-old female) with regular menstrual cycles and stomach pain when eating who was diagnosed with a left ovarian tumor accompanied by mural nodules. The right ovarian cyst, the left ovary, and the fallopian tube were removed by surgery. Intraoperative diagnosis suggested a bilateral ovarian tumor with mural nodules, which include three different pathological types: sarcomatoid transformation, anaplastic carcinoma, and sarcoma. Paclitaxel combined with carboplatin was given for 6 cycles after an operation, and gonadotropin-releasing hormone agonist (GnRHa) was given at the beginning of chemotherapy for 3 cycles for ovarian function protection. Regular follow-up (the last follow-up was performed 48 months after the operation) of gynecological ultrasound and tumor indicators did not indicate recurrence. In clinical practice, it is necessary to pay attention to the symptoms such as abdominal pain in adolescent females. Routine non-invasive pelvic ultrasound is recommended to fully evaluate the nature of the tumor before surgery, and decide the operation mode. Also, intraoperative frozen pathology of the tissue should be performed as soon as possible.
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  • 文章类型: Journal Article
    本研究旨在确定中国重症自身免疫性脑炎(AE)的临床特征,评估免疫治疗的疗效和长期预后。
    临床特征,实验室或放射学发现,收集2014年1月1日至2020年12月31日期间60例重症AE患者的治疗结果.连续变量使用t检验和非参数Mann-WhitneyU检验进行比较,视情况而定。进行了单变量和多变量逻辑回归分析,以评估因素之间的相关性,治疗反应,和严重AE的预后。
    症状发作的中位年龄为35岁。在23.3%的患者中发现了肿瘤,36/60(60%)患者对一线免疫疗法有反应.26/60(43.3%)患者实施了二线免疫疗法。在接受较低剂量利妥昔单抗治疗的19/26(73.1%)患者中观察到了显着的临床益处;7例患者仍然难治性,并接受了硼替佐米作为附加治疗。在最后一次随访中,48/60(80%)患者取得了良好的预后(mRS,0-2),10人死亡。17名患者经历了复发。高CD19+B细胞计数(或,1.197;95%CI[1.043-1.496];p=0.041)和较低的中性粒细胞与淋巴细胞比率(NLR;OR,0.686;95%CI[0.472-0.884];p=0.015)预测对一线治疗的反应和良好预后,分别。
    患有严重AE的患者在基线时处于危急状态,但在有效的抢救免疫疗法后可以挽救。较低剂量的利妥昔单抗可能是严重AE的最佳选择。CD19+B细胞计数和NLR可以为预测严重AE的治疗反应和结果提供预后信息。
    This study aimed to determine the clinical characteristics and evaluate the efficacy of immunotherapy and the long-term prognosis of severe autoimmune encephalitis (AE) in China.
    Clinical features, laboratory or radiological findings, and treatment outcomes of 60 severe patients with AE from January 1, 2014, to December 31, 2020, were collected. Continuous variables were compared using the t-test and the nonparametric Mann-Whitney U test, as appropriate. Univariate and multivariable logistic regression analyses were performed to assess the correlations between factors, treatment responses, and prognosis of severe AE.
    The median age of symptom onset was 35 years. Tumors were identified in 23.3% of patients, and 36/60 (60%) patients responded to first-line immunotherapy. Second-line immunotherapy was implemented in 26/60 (43.3%) patients. A significant clinical benefit was observed in 19/26 (73.1%) patients treated with lower dosage rituximab; seven patients were still refractory and received bortezomib as an add-on therapy. During the last follow-up, 48/60 (80%) patients achieved good outcomes (mRS, 0-2), and 10 died. Seventeen patients experienced relapses. A high CD19+ B-cell count (OR, 1.197; 95% CI [1.043-1.496]; p = 0.041) and a lower neutrophil-to-lymphocyte ratio (NLR; OR, 0.686; 95% CI [0.472-0.884]; p = 0.015) predict the response to first-line treatment and good prognosis, respectively.
    Patients with severe AE were in critical condition at baseline but could be salvaged after effective rescue immunotherapy. A lower dosage of rituximab could be an optimal option for severe AE. CD19+ B-cell count and NLR may provide prognostic information for predicting treatment response and outcome of severe AE.
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