Giant

巨人
  • 文章类型: Case Reports
    神经纤维瘤,一种罕见的周围神经系统良性肿瘤,可以沿着神经从背神经节到其末端分支的任何地方表现出来。粘液样神经纤维瘤可以表现为孤立的非压痛结节,通常通过S-100蛋白的阳性免疫组织化学染色得到证实。然而,在50%的案例中,神经纤维瘤与神经纤维瘤病有关。
    我们介绍了一例34岁男性左大腿后部轻度疼痛的病例,伴随着一个缓慢增长的肿胀,特别明显的弯曲他的膝盖。它的大小在几个月内逐渐增加,患者观察到膝盖伸展程度降低。最初的活检显示神经鞘瘤,没有恶性肿瘤的证据。四年后,肿胀的大小增加,需要切除手术,露出一个不规则的巨大肿瘤,大小为8*6*4.5厘米,粘附于相邻结构,包括股骨,肌肉,动脉和静脉,坐骨神经的一个分支.病理分析将诊断重新分类为低度粘液样神经纤维瘤。随访3个月后MRI显示完全切除,无肿瘤残留或复发。
    孤立性神经纤维瘤通常体积较小,范围从1到2厘米的最大尺寸。或者,作为遗传性神经纤维瘤病的一部分而发生的肿瘤往往是多发性的,并且经常长成大尺寸。在我们的案例中,尽管患者有约8*6*4.5cm的巨大肿瘤,但患者没有达到神经纤维瘤病的诊断标准。据我们所知,这是除神经纤维瘤病外的大腿巨大粘液样孤立性神经纤维瘤的首次报道。因此,在对该部位肿瘤进行鉴别诊断时,应考虑这种类型的肿瘤.
    UNASSIGNED: Neurofibroma, a rare benign tumor of the peripheral nervous system, can manifest anywhere along a nerve from the dorsal ganglion to its terminal branches. Myxoid neurofibroma can present as a solitary non-tender nodule and is often confirmed by positive immunohistochemical staining for S-100 protein. However, in 50% of cases, neurofibromas are associated with neurofibromatosis.
    UNASSIGNED: We present a case of a 34-year-old male with mild pain in the posterior part of his left thigh, accompanied by a slowly-growing swelling particularly noticeable when flexing his knee. It had gradually increased in size over several months, which the patient observed as a decrease in the degree of knee extension. Initial biopsy indicated schwannoma with no evidence of malignancy. Four years later, the swelling increased in size and necessitated resection surgery, revealing an irregular giant tumor measuring 8 *6 *4.5 cm, adherent to adjacent structures, including the femur, muscles, popliteal artery and vein, and a branch of the sciatic nerve. Pathological analysis reclassified the diagnosis to low-grade myxoid neurofibroma. Follow-up MRI three months later showed gross total resection without residual or recurrence of the tumor.
    UNASSIGNED: Solitary neurofibromas are often small in size, ranging from 1 to 2 cm in the greatest dimension. Alternatively, tumors that occur as a part of genetic neurofibromatosis tend to be multiple and often grow to large sizes. In our case, the patient didn\'t have neurofibromatosis as he didn\'t meet its diagnostic criteria despite having a giant tumor measuring approximately 8*6*4.5 cm. To our knowledge, this is the first report of giant myxoid solitary neurofibroma of the thigh apart from neurofibromatosis. Thus, this type of tumor should be considered in the differential diagnosis of tumors at this location.
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  • 文章类型: Journal Article
    目的:通过对相关异常的分析来确定GO发病率的预测因素,缺陷特征和管理策略。
    方法:从2013年至2023年检索了PubMed和OvidEMBASE数据库。主要终点评估发病率与胎龄(GA)的相关性,出生体重(BW),内脏,缺陷尺寸,相关的异常情况和管理策略。
    结果:共纳入20篇文章进行分析,共1009个GO。中位数GA为37周(27-41周),BW中位数为2700克(900-6000)。143心血管异常,238例肺异常,98肌肉骨骼异常,53泌尿生殖道异常,94个胃肠道异常,鉴定出11种神经系统异常和43种染色体异常。82有未指定的额外异常,其中20和17被定义为主要和次要,分别。123例新生儿接受了初级闭合治疗,206例新生儿分阶段关闭和312例新生儿保守治疗。并发症包括脓毒症,呼吸损害,喂养功能障碍和闭合相关不良事件。长期发病率包括家庭通风(n=72),长期父母营养(n=36),和运动神经发育延迟(n=21)。发病率的主要预测因素是肺动脉高压/发育不全,主要的先天性异常,较大的缺损大小和肝疝。
    结论:不良结局的主要预测因素包括存在额外的先天性异常,缺陷尺寸,肝疝和肺动脉高压/发育不全。
    OBJECTIVE: To identify predictors of morbidity in GO through an analysis of associated anomalies, defect characteristics and management strategies.
    METHODS: PubMed and Ovid EMBASE databases were searched from 2013 to 2023. Primary end points assessed correlation of morbidity with gestational age (GA), birth weight (BW), eviscerated organs, defect size, associated anomalies and management strategy.
    RESULTS: Twenty articles were included for analysis with a total of 1009 GO. Median GA was 37 weeks (27-41), with a median BW of 2700 g (900-6000). 143 cardiovascular anomalies, 238 pulmonary anomalies, 98 musculoskeletal anomalies, 53 urogenital anomalies, 94 gastrointestinal anomalies, 11 neurological anomalies and 43 chromosomal anomalies were identified. 82 had unspecified additional anomalies, of which 20 and 17 were defined as major and minor, respectively. 123 neonates were managed with primary closure, 206 neonates with staged closure and 312 neonates with conservative treatment. Complications included sepsis, respiratory compromise, feeding dysfunction and closure-related adverse events. Long-term morbidity included home ventilation (n = 72), long-term parental nutrition (n = 36), and delayed motor neurodevelopment (n = 21). Main predictors of morbidity were pulmonary hypertension/hypoplasia, major congenital anomalies, greater defect size and liver herniation.
    CONCLUSIONS: Key predictors of poor outcomes include the presence of additional congenital anomalies, defect size, liver herniation and pulmonary hypertension/hypoplasia.
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  • 文章类型: Case Reports
    巨大主动脉瘤,定义为直径超过10厘米,如果不解决,会给生命带来相当大的风险。胸腹主动脉瘤(TAAA)由于其破裂倾向而特别危险。
    方法:我们报告一例由巨大的胸腹主动脉瘤引起的胃肠道梗阻,并伴有一个包含性渗漏和急性破裂。病人出现腹痛,恶心,呕吐,和不耐受口服。
    某些情况和感染使个体容易发生巨大动脉瘤。本报告详细介绍了大量的慢性TAAA,其狭窄的泄漏导致周围的壁血栓和其中的急性破裂。由于上消化道梗阻,患者被复苏并接受了紧急主动脉-肾脏重建手术。
    结论:虽然上消化道梗阻是一种极其罕见的巨大胸腹动脉瘤的表现,应将其视为类似病例的鉴别诊断。
    UNASSIGNED: Giant aortic aneurysms, defined as those exceeding 10 cm in diameter, present considerable risk to life if not addressed. Thoracoabdominal aortic aneurysms (TAAAs) are particularly perilous due to their propensity for rupture.
    METHODS: We report a case of gastrointestinal obstruction manifesting from a giant thoracoabdominal aortic aneurysm with a contained leak and acute rupture. The patient presented with abdominal pain, nausea, vomiting, and intolerance to oral intake.
    UNASSIGNED: Certain conditions and infections predispose individuals to the development and expansion of giant aneurysms. This report details a massive chronic TAAA with a confined leak that led to a circumferential mural thrombus and an acute rupture within it. The patient was resuscitated and underwent urgent aorto-renal reconstruction surgery due to the presentation of upper gastrointestinal obstruction.
    CONCLUSIONS: Though upper gastrointestinal obstruction is an extremely rare manifestation of giant thoracoabdominal aneurysm, it should be considered as a differential diagnosis in similar cases.
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  • 文章类型: Case Reports
    脂肪瘤很常见,界限清楚的中胚层起源的肿瘤,以缓慢为特征,大部分是皮下的无痛生长,没有侵入性,手术后不会复发.脂肪瘤是最常见的间质瘤,然而巨大的脂肪瘤在颈部和枕骨区是罕见的。我们报告一名46岁的尿崩症女性被转诊为巨大的枕骨颈部肿瘤,她已经注意到了17年,很少给她任何压迫症状的抱怨。临床评估表明,无痛,和移动肿胀,表现出静脉扩张的特征,没有外部溃疡.超声检查和颈部MRI显示,在两种模式下都具有独特的回声特征的皮下脂肪肿瘤,包括T1和T2上的高强度信号,并且在注射钆后没有环增强。由于质量光滑,圆形,没有附着在任何结构上,患者在全身麻醉下接受了手术摘除,完全康复,无并发症。病理显示良性脂肪组织肿瘤无脂肪肉瘤,在两年的随访中没有发现任何困难。
    Lipomas are common, well-circumscribed neoplasms of mesodermal origin, characterized by being slow, painless growths that are mostly subcutaneous, not invasive, and not recurring after surgery. Lipomas are the most prevalent kind of mesenchymal tumor, yet giant lipomas are rare in the cervical region and the occipital area. We report a 46-year-old female with diabetes insipidus was referred with a giant occipital cervical tumor, which she had noticed for 17 years and which had rarely given her any complaints of compressive symptoms. The clinical assessment indicated a firm, painless, and mobile swelling, which demonstrated features of venous ectasia, and there was no external ulceration. Ultrasonography and MRI of the neck revealed a large, subcutaneous fatty tumor with distinctive echographic features on both modalities, including hyperintense signals on T1 and T2 and no ring enhancement after Gadolinium injection. Due to the mass being smooth, round, and not attached to any structure, the patient underwent surgical enucleation under general anesthesia, resulting in full recovery without complications. Pathology revealed a benign adipose tissue tumor without liposarcoma, and there were no difficulties observed during follow-up for two years.
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  • 文章类型: Case Reports
    成熟的囊性畸胎瘤在其病理学中表现出多种组织。在成年人中,畸胎瘤通常起源于性腺。然而,最罕见的起源之一是肺,使肺内畸胎瘤(IPT)非常罕见。在婴儿中,性腺外畸胎瘤更常见,文献中仅报道了两例IPT。虽然婴儿和成人的临床表现相似,发烧似乎是婴儿病例特有的。我们介绍了一例一岁女性,表现出呼吸窘迫和发烧。胸部X光显示右半胸部不透明,最初导致肺炎的诊断。尽管静脉(IV)抗生素治疗,没有任何改善。随后的胸部计算机断层扫描(CT)扫描显示,整个右半胸部有一个密度不均的大肿块,指示IPT。肿块被成功切除,术后第11天患儿出院,无并发症发生。与先前发表的两个病例相比,该病例增加了有关婴儿巨大IPT的有限文献。
    Mature cystic teratomas exhibit a variety of tissues within their pathology. In adults, teratomas typically originate in the gonads. However, one of the rarest origins is the lung, making intrapulmonary teratoma (IPT) exceedingly uncommon. In infants, extragonadal teratomas are more common, with only two cases of IPT reported in the literature. While the clinical presentation in infants and adults is similar, fever appears to be unique to infant cases. We present a case of a one-year-old female who exhibited respiratory distress and fever. A chest X-ray revealed an opaque right hemithorax, initially leading to a diagnosis of pneumonia. Despite intravenous (IV) antibiotic treatment, there was no improvement. A subsequent chest computed tomography (CT) scan revealed a large mass with heterogeneous densities occupying the entire right hemithorax, indicative of IPT. The mass was successfully excised, and the infant was discharged on the 11th postoperative day without complications. This case adds to the limited literature on giant IPT in infants compared to the two previously published cases.
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  • 文章类型: Case Reports
    胃的巨大消化性溃疡被定义为直径超过3厘米。其中约2/3是良性消化性溃疡,并在所有消化性溃疡胃穿孔的1%-2%中穿孔。据报道,术后发病率和死亡率很高。治疗方案包括网膜修补,空肠浆膜补片修复,十二指肠排斥,和胃切除术.据报道,术后网膜补片修复失败,并且与溃疡直径密切相关。该病例报告了一例女性患者的巨大消化性胃溃疡穿孔,成功进行了网膜补片修复,根据Cellan-Jones的说法.
    Giant peptic ulcer of the stomach is defined with a diameter of more than 3 cm. About 2/3 of them are benign peptic ulcers and perforate in 1%-2% of all peptic ulcer stomach perforations. High rates of postoperative morbidity and mortality are reported. The treatment options include omental patch repair, jejunal serosal patch repair, duodenal exclusion, and gastric resection. Postoperative omental patch repair failure is reported and is strongly associated with the ulcer diameter. This case reports a giant peptic stomach ulcer perforation in a female patient treated successfully with omental patch repair, according to Cellan-Jones.
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  • 文章类型: Journal Article
    目的:我们分析了巨大脐膨出的闭合技术。缺乏共识的具有挑战性的病理学。
    方法:Cochrane,在1992年1月1日至2022年12月31日之间使用术语和变体搜索了MEDLINE和EMBASE:exomphalos,巨人,关闭和结果。论文是使用2020年系统评价和荟萃分析标准的首选报告项目选择的。收集的数据包括人口统计,手术修复的时机和技术,发病率和死亡率。
    结果:我们确定了342篇论文;34篇符合纳入标准,共有356例新生儿。26篇论文描述了初始非手术治疗(14篇敷料,八个筒仓,四个连续囊结扎)。论文的手术技术如下:早期闭合:无贴片的九种主要缝合闭合,两种主要的补片封闭和四种混合方法。延迟关闭:五个简单,四组分分离技术,四个组织扩张器,一个肉毒杆菌/气腹和两个带贴片。手术的中位数在早期组为2(1-6),在延迟组为3(1-4)。最有利的是早期用生物贴片进行初级闭合。最不利的是用补丁延迟关闭。累计报告死亡率仍然很高,主要是由于非手术原因。
    结论:文献中巨大脐膨出的定义与所描述的各种管理方法不同。
    OBJECTIVE: We analysed closure techniques in the treatment of giant omphalocele. A challenging pathology where there lacks consensus.
    METHODS: Cochrane, MEDLINE and EMBASE were searched between 1 January 1992 and 31 December 2022 using terms and variations: omphalocele, exomphalos, giant, closure and outcome. Papers were selected using Preferred Reporting Items for Systematic review and Meta-Analyses 2020 criteria. Data collected included demographics, timing and technique of surgical repair, morbidity and mortality.
    RESULTS: We identified 342 papers; 34 met inclusion criteria with a total 356 neonates. Initial non-operative management was described in 26 papers (14 dressings, eight silo, four serial sac-ligation). Operative techniques by paper were as follows: Early closure: nine primary suture closure without patch, two primary closure with patch and four mixed methods. Delayed closure: five simple, four-component separation technique, four tissue expanders, one Botox/pneumoperitoneum and two with patch. Median number of procedures was two (1-6) in the early group versus three (1-4) in the delayed. The most favourable was early primary closure with biological patch. The most unfavourable was delayed closure with patch. Cumulative reported mortality remained high, mostly due to non-surgical causes.
    CONCLUSIONS: Definitions of giant omphalocele in the literature were heterogeneous with a variety of management approaches described.
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  • 文章类型: Case Reports
    眼睑皮脂腺癌是一种罕见的恶性肿瘤。手术切除通常是标准的治疗方法。然而,在由于局部晚期肿瘤而无法进行手术的情况下,明确的放射治疗可以被认为是一种选择。一名71岁的男子,有两年前的眼睑皮脂腺癌病史。肿瘤,测量93x55x56毫米,位于右上眼睑,没有手术切除.然后他接受了调强放疗,在35个部分中接受70Gy的总剂量。24个月后,患者无局部复发。
    Eyelid sebaceous carcinoma is a rare malignant tumor. Surgical excision is generally the standard curative treatment. However, in cases where surgery is not possible due to locally advanced tumors, definitive radiotherapy can be considered an option. A 71-year-old man presented with a history of eyelid sebaceous carcinoma dating back two years. The tumor, measuring 93 x 55 x 56 mm and located on the right upper eyelid, was not surgically resected. He was then treated with intensity-modulated radiotherapy, receiving a total dose of 70 Gy in 35 fractions. After 24 months, the patient shows no local recurrence.
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  • 文章类型: Case Reports
    一名74岁的女性,一般健康状况良好,有5年的进行性脱发史,解释为女性雄激素性脱发(AGA),并且用局部5%米诺地尔治疗而没有改善。患者的相关病史显示浸润,四年前的右乳腺三阴性大汗腺癌,通过四肢切除术治疗,辐射,淋巴结清扫和化疗,在最后一次随访时没有复发。在检查中,在中央头皮上有一个无症状的15×15厘米坚硬和发白的疤痕脱发区域。皮肤镜检查显示多个树干化血管和许多毛细血管扩张。临床考虑主要包括乳腺癌的皮肤转移(脱发瘤),Broque和恶性基底细胞癌(BCC)的假泡沫。组织病理学检查显示morpheapeformBCC的特征性变化,具有基底岛状岛和非典型基底细胞的绳索扩散浸润真皮,包埋在硬化和血管过度化的基质中。由于头皮上的恶性BCC引起的继发性脱发是一种极为罕见的实体,具有微妙的临床特征,可以模拟瘢痕和非瘢痕性脱发。延迟识别可能会导致攻击行为和广泛的局部破坏。用刺猬抑制剂治疗头皮局部晚期BCC,在辅助和新辅助模式中,很有希望。
    A 74-year-old woman in good general health presented with a 5-year history of progressive hair loss over several years, interpreted as female androgenetic alopecia (AGA), and was treated with topical 5% Minoxidil without improvement. The patient\'s relevant medical history revealed infiltrating, triple-negative apocrine carcinoma of the right breast four years before, treated by quadrantectomy, radiation, lymphadenectomy and chemotherapy, with no recurrence at the last follow-up. On examination, there was an asymptomatic 15 × 15 cm firm and whitish area of scarring alopecia on the central scalp. Dermoscopy revealed multiple arborizing vessels and many telangiectasia. The clinical considerations included mainly cutaneous metastasis of breast carcinoma (alopecia neoplastica), pseudopelade of Broque and morpheaform basal cell carcinoma (BCC). A histopathologic examination revealed characteristic changes of morpheaform BCC with basaloid islands and cords of atypical basaloid cells diffusely infiltrating the dermis, embedded in a sclerotic and hypervascularized stroma. Secondary alopecia neoplastica due to morpheaform BCC on the scalp is an exceedingly rare entity, possessing subtle clinical features that may mimic both scarring and non-scarring alopecia. Delayed recognition may contribute to aggressive behavior and extensive local destruction. Treatment with hedgehog inhibitors in locally advanced BCC of the scalp, both in adjuvant and neoadjuvant modalities, is promising.
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  • 文章类型: Journal Article
    背景颅内海绵状畸形(CMs),通常被称为海绵状血管瘤或海绵状血管瘤,是低流量的,界限清楚的血管病变由正弦空间组成,由单层内皮衬里,并由无弹性蛋白的胶原基质隔开,平滑肌,或其他血管壁元素。对于CM,直径大于3cm是不可能的。这些病变在磁共振成像(MRI)上可能具有非典型外观。MRI采用先进技术,如磁敏感加权图像或T2梯度回波,扩散加权图像和相应的表观扩散系数图,和弥散张量纤维束成像彻底改变了这些病变的诊断方法。材料与方法本研究综述了病因,临床表现,MRI策略,和CM的MRI外观,下面是我们档案中的几个巨型CM的例子。结果颅内巨大CM可能有意想不到的位置,尺寸,数字,以及因反复出血而出现的各种影像学表现,不寻常的增强模式,强烈的病灶周围水肿,和不寻常的联想,使鉴别诊断变得困难。结论熟悉巨大颅内CMs的MRI表现和鉴别诊断,提高了诊断的准确性和患者管理。
    Background  Intracranial cavernous malformations (CMs), commonly known as cavernomas or cavernous angiomas, are low-flow, well-circumscribed vascular lesions composed of sinusoidal spaces lined by a single layer of endothelium and separated by a collagenous matrix without elastin, smooth muscle, or other vascular wall elements. A diameter greater than 3 cm for a CM is unlikely. These lesions may have atypical appearances on magnetic resonance imaging (MRI). MRI with advanced techniques such as a susceptibility-weighted image or T2-gradient echo, a diffusion-weighted image and corresponding apparent diffusion coefficient map, and diffusion tensor tractography have revolutionized the diagnostic approach to these lesions. Materials and Method  The present study reviews the etiopathogenesis, clinical manifestations, MRI strategy, and MRI appearances of the CMs, with a few examples of the giant CMs from our archive. Results  Intracranial giant CMs may have unexpected locations, sizes, numbers, and varied imaging appearances due to repeated hemorrhages, unusual enhancement patterns, intense perifocal edema, and unusual associations, making the differential diagnosis difficult. Conclusion  Familiarity with the MRI appearances of the giant intracranial CMs and the differential diagnosis improves diagnostic accuracy and patient management.
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