vanishing

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  • 文章类型: Case Reports
    先天性肢体减少缺陷影响了在美国出生的1900名婴儿中的1名。肢体减少是指肢体或肢体的特定节段的缩短或完全不存在。上肢的先天性缺陷是最常见的位置,占新生儿肢体减少缺陷的58.5%。纵向缺陷会影响肢体的长轴,并可能主要影响一个骨骼,也可能是多个骨骼的发育不全或发育不全。据我们所知,这个案例是一个独特的讨论,婴儿出生时没有任何其他常见的相关综合征的纵向尺骨缺损。
    Congenital limb reduction defects affect 1 in 1,900 babies born in the US. Limb reduction refers to the shortening or total absence of a limb or a specific segment of a limb. Congenital deficiencies of the upper limb are the most common location and comprise 58.5% of newborn limb reduction deficiencies. Longitudinal deficiencies affect the long axis of the limb and can affect one bone predominantly or can be hypoplasia or aplasia of several bones. To our knowledge, this case is a unique discussion of a baby born with longitudinal ulnar deficiency without any other commonly seen associated syndrome.
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  • 文章类型: Journal Article
    目的:我们询问为什么子宫内膜癌有时会消失。
    方法:在过去5年中,共纳入454例诊断为子宫内膜样型子宫内膜癌(EC)(通过子宫内膜取样)并在我们的诊所接受治疗的患者。患者分为两组:消失和残留,取决于术后子宫切除术标本中是否检测到肿瘤。患者年龄,怀孕和分娩的数量,更年期状态,全身性疾病状态,血象参数,国际妇产科联合会(FIGO)年级,和侵袭状态(在磁共振成像[MRI]上很明显)进行了组间比较。
    结果:42例(9.25%)患者的ECs消失。FIGO1级患者的消失率为19.7%(37/187),2级患者为2.1%(5/238),3级患者为0%(0/29)。消失组的平均年龄低于残留组,但绝经前状态和1级肿瘤发生率较高(均p<0.001)。在消失组(p<0.001)中更常见的是没有侵袭(如MRI显示的)。消失组没有复发,但在残留组中,有3.3%(14/412)出现复发.在怀孕或分娩的任何数量上,组间没有显着差异,全身性疾病状态,或血象参数(均p>0.05)。
    结论:子宫内膜样1级EC(通过子宫内膜活检显示)的绝经前妇女在MRI上缺乏肌层浸润的可能性更大。
    OBJECTIVE: We asked why endometrial cancer sometimes vanishes.
    METHODS: A total of 454 patients diagnosed with endometrioid-type endometrial cancer (EC) (via endometrial sampling) and treated in our clinic over the past 5 years were enrolled. The patients were divided into two groups: vanishing and residual, depending on whether a tumor was detected in the postoperative hysterectomy specimen. Patient age, numbers of pregnancies and deliveries, menopausal status, systemic disease status, hemogram parameters, International Federation of Gynecology and Obstetrics (FIGO) grade, and invasion status (evident on magnetic resonance imaging [MRI]) were compared between the groups.
    RESULTS: ECs vanished in 42 (9.25%) patients. The vanishing rates were 19.7% (37/187) in FIGO grade 1 patients, 2.1% (5/238) in grade 2 patients, and 0% (0/29) in grade 3 patients. The average age was lower in the vanishing than the residual group, but the premenopausal status and grade 1 tumor rates were higher (both p < 0.001). An absence of invasion (as revealed by MRI) was more common in the vanishing group (p < 0.001). No recurrence developed in the vanishing group, but recurrences were noted in 3.3% (14/412) of the residual group. There were no significant between-group differences in any of the numbers of pregnancies or births, systemic disease status, or hemogram parameters (all p > 0.05).
    CONCLUSIONS: Vanishing EC is more likely in premenopausal women with endometrioid grade 1 EC (as revealed by endometrial biopsy) who lack myometrial invasion on MRI.
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  • 文章类型: Case Reports
    未经批准:Gorham-Stout病(GSD),一种纤维淋巴血管实体,其中组织取代骨骼,导致大量骨质溶解及其后遗症,很少导致脊柱畸形/不稳定和神经功能缺损。这里,我们报告了一名12岁女性,她被诊断并接受了GSD治疗.
    未经证实:一名12岁女性背痛,不能走路,坐下,或归因于三个MR/CT记录的L2-L4腰椎塌陷。闭合活检为阴性。然而,开放性活检诊断为GSD.她接受了背侧腰椎至骨盆融合(即,T5-T12至L5/S1/S2)使用多级椎弓根螺钉/杆稳定和人白细胞抗原(HLA)匹配的同种异体移植物(即来自她的父亲)。术后,她接受了“标签外”特立帕肽注射治疗,双膦酸盐,还有西罗莫司.四年后,在继续双膦酸盐治疗的同时,她保持稳定。
    UASSIGNED:从T5到S2的手术多杆稳定,辅以HLA兼容的同种异体移植,和多种医疗“标签外”疗法(即,特立帕肽,西罗莫司,和双膦酸盐)导致一名12岁女性GSD的良好4年结局。
    UNASSIGNED: Gorham-Stout disease (GSD), a fibro-lymphovascular entity in which tissue replaces the bone leading to massive osteolysis and its sequelae, rarely leads to spinal deformity/instability and neurological deficits. Here, we report a 12-year-old female who was diagnosed and treated for GSD.
    UNASSIGNED: A 12-year-old female presented with back pain, and the inability to walk, sit, or stand attributed to three MR/CT documented L2-L4 lumbar vertebral collapses. Closed biopsies were negative. However, an open biopsy diagnosed GSD. She underwent a dorsal-lumbar-to-pelvis fusion (i.e., T5-T12 through L5/S1/S2) using multilevel pedicle screw/rod stabilization and human leukocyte antigens (HLAs) matched allograft (i.e. from her father). Postoperatively, she was treated with \"off-label\" teriparatide injections, bisphosphonates, and sirolimus. Four years later, while continuing the bisphosphonate therapy, she remained stable.
    UNASSIGNED: Surgical multirod stabilization from T5 to S2, supplemented with HLA compatible allograft, and multiple medical \"off-label\" therapies (i.e., teriparatide, sirolimus, and bisphosphonates) led to a good 4-year outcome in a 12-year-old female with GSD.
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  • 文章类型: Journal Article
    背景:在产前超声检查中诊断出的先天性肺畸形(CLM)随后可能在以后的扫描中无法检测到,“消失”CLM。
    目的:我们研究的目的是描述在我们机构治疗的“消失”病变的产前自然史和产后结局。
    方法:我们对107例产前诊断为CLM的患者进行了回顾性分析。使用Kruskal-Wallis或t检验对连续变量进行比较,Fisher精确检验或卡方检验对分类变量进行比较。采用logistic回归进行多变量分析。
    结果:在104例患者中,59(56.7%)的病变在连续超声扫描中无法通过超声检查检测到。产前病变消失的患者出生时往往需要较少的新生儿重症监护病房(NICU)入院(持续CLM:54.8%vs消失CLM:28.8%),出生时对补充O2的需求减少(持续CLM:31.0%vs消失CLM:11.9%),与持久性CLM相比,饲料延迟减少(持久性CLM:26.2%与消失的CLM:8.5%)。在多变量分析控制母体类固醇给药和性别后,入住NICU保持轻微的统计学意义,消失的CLM组患者入院NICU的可能性降低2.5倍。在产前病变消失的患者中,没有一个需要机械通气。86例患者接受了产后计算机断层扫描(CT)胸部检查。只有2例患者的病变在产后CT上消退。
    结论:在产前影像学上消失的病变可能与改善的临床结局相关。我们机构的真实回归率低至2.3%。
    BACKGROUND: A congenital lung malformation (CLM) that is diagnosed on prenatal ultrasound exam may subsequently become undetectable on later scans, a \"vanishing\" CLM.
    OBJECTIVE: The purpose of our study is to characterize the prenatal natural history and postnatal outcomes of \"vanishing\" lesions treated at our institution.
    METHODS: We performed a retrospective chart review of 107 patients diagnosed prenatally with CLM at our institution. Comparisons were made using Kruskal-Wallis or t-test for continuous variables and Fisher\'s exact test or Chi-Square test for categorical variables. Multivariable analysis using logistic regression was performed.
    RESULTS: Of the 104 patients, 59 (56.7%) had lesions that became sonographically undetectable on serial ultrasound scans. Patients with lesions that vanished prenatally tended to need less Neonatal Intensive Care Unit (NICU) admission at birth (persistent CLM: 54.8%vs vanished CLM: 28.8%), decreased need for supplemental O2 at birth (persistent CLM: 31.0%vs vanished CLM: 11.9%), and decreased delay in feeds (persistent CLM: 26.2%vs vanished CLM: 8.5%) compared to those with persistent CLM. After multivariate analysis controlling for maternal steroid administration and sex, admission to NICU maintained a slight statistical significance, with patients in the vanishing CLM group 2.5 times less likely to be admitted to the NICU. None of our patients whose lesions vanished prenatally required mechanical ventilation. Eighty-six patients underwent postnatal computed tomography (CT) chest. Only 2 patients had lesions that regressed on postnatal CT.
    CONCLUSIONS: Lesions that vanish on prenatal imaging may be associated with improved clinical outcomes. The rate of true regression at our institution was as low as 2.3%.
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  • 文章类型: Journal Article
    BACKGROUND: Vanishing brain tumor is defined as spontaneously disappearing or decreasing of the initial brain mass volume to ≤ 70% before establishing the definitive diagnosis. The condition is rare and can be attributed to different factors. The exact mechanism is under debate, but the increasing rate and accuracy of neuroimaging studies and occurrence of similar scenario in other pathologies rather than brain tumors can be of particular importance in finding vanishing brain lesions.
    METHODS: We present two unusual cases of congenital brain masses which underwent spontaneous shrinkage within the first months of life.
    CONCLUSIONS: The condition is scarcely observed in congenital brain masses. As congenital brain lesions are distinct entities with peculiar characteristics, this rare phenomenon may reflect different aspects in this age group.
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  • 文章类型: Journal Article
    BACKGROUND: Approximately 30% of patients with colorectal cancer develop colorectal liver metastases (CRLM). CRLM that become undetectable by imaging after chemotherapy are called disappearing liver metastases (DLM). But a DLM is not necessarily equal to cure. An increasing incidence of patients with DLM provides surgeons with a difficult dilemma: to resect or to not resect the original sites of DLM? The aim of this review was to investigate to what extent a DLM equates a complete response (CR) and to compare outcomes.
    METHODS: This review was conducted in accordance with the PRISMA guidelines and registered in Prospero (registration number CRD42017070441). Literature search was made in the PubMed and Embase databases. During the process of writing, PubMed was repeatedly searched and reference lists of included studies were screened for additional studies of interest for this review. Results were independently screened by two authors with the Covidence platform. Studies eligible for inclusion were those reporting outcomes of DLM in adult patients undergoing surgery following chemotherapy.
    RESULTS: Fifteen studies were included with a total of 2955 patients with CRLM. They had 4742 CRLM altogether. Post-chemotherapy, patients presented with 1561 DLM. Patients with one or more DLM ranged from 7 to 48% (median 19%). Median DLM per patient was 3.4 (range 0.4-5.6). Patients were predominantly evaluated by contrast-enhanced computed tomography (CE-CT) before and after chemotherapy, with some exceptions and with addition of magnetic resonance imaging (MRI) in some studies. Intraoperative ultrasound (IOUS) was universally performed in all but two studies. If a DLM remained undetectable by IOUS, this DLM represented a CR in 24-96% (median 77.5%). Further, if a DLM on preoperative CE-CT remained undetectable by additional workup with MRI and CE-IOUS, this DLM was equal to a CR in 75-94% (median 89%). Patients with resected DLM had a longer disease-free survival compared to patients with DLM left in situ but statistically significant differences in overall survival could not be found.
    CONCLUSIONS: Combination of CE-CT, MRI, and IOUS showed promising results in accurately identifying DLM with CR. This suggests that leaving DLM in situ could be an alternative to surgical resection when a DLM remains undetectable by MRI and IOUS.
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  • 文章类型: Journal Article
    随着时间的推移,全身化疗的进展导致结直肠癌肝转移(CRLM)的反应率显着增加。尽管放射学反应通常是预后良好的结果,化疗后CRLM完全收缩,即“肝转移消失”(DLMs)带来了重大的治疗困境。对文献进行了系统回顾,以评估使用PubMed(Medline)对DLM患者进行成像和管理的现有证据,Embase和Cochrane图书馆到12月21日,2018.使用以下算法:“(消失或消失或缺失或(残留微小))和((肝或肝)和(转移或转移或继发))。\"从检索到的225条记录中,15项研究最终被认为是合格的。共有479例DLM患者,中位年龄为59.5岁(范围,30-83)被确定。每位患者的DLM中位数为1至8.8。化疗前LMs的中位大小为1.07cm(范围0.3-3.5)。用于实现DLM的全身治疗包括单独的全身化疗(仅2项研究)或与靶向药物组合(11项研究)。纳入研究的化疗周期中位数为7.8(范围6-12)。确定的诱发DLM发展的因素是小尺寸(<2cm),增加治疗周期数,基于奥沙利铂的治疗,增加CRLM(≥3)和同步CRLM的数量。基线和术前MRI静脉造影显示DLM检测的灵敏度最高。基准放置有助于DLM的术前和术中识别。虽然DLM的切除降低了局部复发的风险,在切除所有消失的病灶部位后,无明显的生存获益.未来的随机临床试验被高度鼓励提供严格的,DLM患者的循证治疗建议。
    Advances in systemic chemotherapy have resulted in a significant increase in the reported response rates of colorectal liver metastases (CRLM) over time. Although radiologic response is usually prognostic of favorable outcomes, complete shrinkage of CRLM after chemotherapy, namely \"disappearing liver metastases\" (DLMs) poses significant therapeutic dilemmas. A systematic review of the literature was conducted to evaluate the existing evidence on the imaging and management of patients with DLMs using the PubMed (Medline), Embase and Cochrane library through December 21st, 2018. The following algorithm was used: \"(disappearing OR vanishing OR missing OR (residual tiny)) AND ((liver OR hepatic) AND (metastasis OR metastases OR metastatic OR secondary)).\" From the 225 records retrieved, 15 studies were finally deemed eligible. A total of 479 patients with DLMs with a median age of 59.5 years (range, 30-83) were identified. Median number of DLM per patient ranged from 1 to 8.8. Median size of LMs prior to chemotherapy was 1.07 cm (range 0.3-3.5). The systemic treatment used to achieve DLMs included systemic chemotherapy alone (only 2 studies) or in combination with targeted agents (11 studies). The median number of chemotherapy cycles in the included studies was 7.8 (range 6-12). Identified factors predisposing to the development of DLM were small size (<2 cm), increased number of treatment cycles, oxaliplatin-based therapy, increased number of CRLM (≥3) and synchronous CRLM. Baseline and preoperative MRI with iv contrast showed the highest sensitivity for DLM detection. Fiducial placement facilitated pre- and intra-operative identification of DLM. Although resection of DLM decreased the local recurrence risk, there was no clearly demonstrated survival benefit after resecting all sites of disappearing lesions. Future randomized clinical trials are highly encouraged to provide strict, evidence-based recommendations for the treatment of patients with DLM.
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  • 文章类型: Case Reports
    BACKGROUND: Meningiomas are common intracranial neoplasms of undetermined etiology. Accelerated growth during episodes of elevated serum estrogen and progesterone have been demonstrated in pregnant patients, as exhibited by an overexpression of estrogen or progesterone on immunohistochemical analysis. This case report and literature review describe a case of complete meningioma regression in a postpartum patient.
    METHODS: A 23-year-old female presented at 37 weeks of pregnancy with 1-month history of fluctuating severe left-sided headaches, periodic blurry vision, nausea, and vomiting. She had 2 previous pregnancies without complication. Magnetic resonance imaging revealed a dural-based, heterogeneously enhancing mass along the left tentorium, just posterior to the transverse sinus, with supratentorial extension and surrounding edema. Differential diagnoses included meningioma versus hemangioma versus hemangiopericytoma. The patient followed up with neurosurgery 1 month after delivery. She had continued left-sided headaches but no longer complained of visual changes. A postpartum surgical resection via left occipital and suboccipital craniotomy was planned. Approximately 1 month later (now about 3 months after delivery) a repeat magnetic resonance imaging demonstrated a marked decrease in meningioma size, and the previously seen edema had resolved. In light of the sudden disappearance of the meningioma, no further surgical intervention was pursued.
    CONCLUSIONS: Because meningioma shrinkage or disappearance may occur after pregnancy, repeat imaging is advised as part of a preoperative evaluation. In addition, it is possible that an undetermined amount of meningioma removal surgeries may be avoided with further research into monitoring hormone levels connected to meningioma growth.
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    文章类型: Journal Article
    UNASSIGNED: Of all parotid gland tumors, only oncocytoma has been reported to appear isointense to the parotid gland, namely vanishing, on fat-saturated T2 and T1 postcontrast gadolinium-enhanced magnetic resonance imaging (MRI). The purpose of this study was to evaluate vanishing of parotid tumors on conventional MRI with and/or without postcontrast gadolinium-enhancement and on diffusion weighted imaging (DWI).
    UNASSIGNED: In 8 of 51 patients, ten parotid gland tumors had homogeneously enhanced lesions and were retrospectively analysed. Comparisons of signal intensity between those parotid tumors and parotid glands and evaluations of vanishing were performed on T1-weighted imaging (T1WI), T2-weighted imaging (T2WI), fat-suppressed T2WI (FS-T2WI), postcontrast gadolinium-enhanced T1WI (CE-T1WI) and fat-suppressed CE-T1WI (FS-CE-T1WI), DWI as well as apparent diffusion coefficient (ADC).
    UNASSIGNED: Ten parotid gland tumors consisted of five Warthin tumors, two pleomorphic adenomas, two parotid carcinomas (small cell carcinoma and adenoid cystic carcinoma) and one oncocytoma. All tumors showed hypointensity on T1WI and hyperintensity on DWI. Nine of ten tumors showed vanishing on the other MR sequences. All Warthin tumors showed vanishing on FS-T2WI, FS-CE-T1WI and the ADC map. One oncocytoma showed vanishing on FS-T2WI and the ADC map and hyperintensity on FS-CE-T1WI. All pleomorphic adenomas showed vanishing on T2WI and CE-T1WI. One adenoid cystic carcinoma showed vanishing only on CE-T1WI.
    UNASSIGNED: Vanishing of parotid tumors can be observed not only on FS-T2WI and FS-CE-T1WI but also on T2WI, CE-T1WI and ADC mapping.
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  • 文章类型: Case Reports
    We present a severe case of vanishing gastroschisis resulting in intrauterine death with post mortem examination findings. Gastroschisis is defined as a full thickness paraumbilical abdominal wall defect associated with evisceration of fetal intestine. It is almost always right-sided. Vanishing gastroschisis is an extremely rare form of gastroschisis that results in short bowel syndrome due to exteriorized bowel disconnected from the lumen of the rest of the bowel proximally as well as distally in association with partial or complete closure of the abdominal wall. This case is only the second published case of vanishing gastroschisis resulting in intrauterine fetal death including post mortem examination findings. It highlights the importance of being aware of this rare form of gastroschisis and provides insights regarding pathogenesis, ultrasound surveillance, and antenatal counseling.
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