sacrococcygeal teratoma

骶尾部畸胎瘤
  • 文章类型: Case Reports
    具有可逆性脾病变(MERS)的轻度脑炎/脑病是一种罕见疾病,其特征是在MRI上观察到的call体(SCC)脾的可逆性病变。MERS的确切病因未知,尽管感染和抗癫痫药物被报道为潜在原因。在这里,我们介绍一名56岁的男性患者,他经历了3天的发烧和头痛。他的初级保健医生对症治疗后未能改善他的症状,他被转诊到我们医院。患者没有精神症状或明显的神经系统表现。头部MRI在弥散加权成像上显示SCC高信号,怀疑MERS确定MERS原因的所有检查均为阴性。使用抗生素和复合维生素B,患者的症状得到改善。一被录取,腹部CT偶然发现直肠背侧表面有一个清晰的肿块,怀疑是尾肠囊肿,保证手术切除。肿瘤的颅缘在第三骶骨的尾部,经骶骨入路切除。切除第五骶骨和尾骨,切除肿瘤时没有损伤直肠.组织病理学检查显示成熟畸胎瘤,无任何恶性肿瘤。术后4个月的CT随访显示,没有证据表明MERS的临床复发。成人发作的MERS相对罕见,没有与肿瘤相关的报道。脑炎和畸胎瘤之间的关联包括卵巢畸胎瘤,引起抗N-甲基-D-天冬氨酸受体脑炎和副肿瘤边缘叶脑炎。尽管在这种情况下MERS的原因尚不清楚,我们报告了骶骨畸胎瘤和MERS的共存,有助于了解它们之间的关联.
    Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is a rare disease characterized by a reversible lesion in the splenium of the corpus callosum (SCC) observed on MRI. The exact etiology of MERS is unknown, although infections and antiepileptic drugs have been reported as potential causes. Herein, we present the case of a 56-year-old male patient who experienced fever and headache for 3 days. He was referred to our hospital after symptomatic treatment by his primary care physician failed to improve his symptoms. The patient had no psychiatric symptoms or significant neurological findings. Head MRI revealed a high signal on SCC on diffusion-weighted imaging, raising the suspicion of MERS. All examinations to determine the cause of MERS were negative. The patient\'s symptoms improved with antibiotics and B complex vitamins. Upon admission, abdominal CT incidentally revealed a well-defined mass on the dorsal surface of the rectum suspected to be a tailgut cyst, warranting surgical resection. The cranial margin of the tumor was caudal to the third sacrum, and a trans-sacral approach was used for resection. The fifth sacrum and the coccyx were resected, and the tumor was resected without damaging the rectum. A histopathological examination revealed a mature teratoma without any malignancy. A follow-up CT at four months postoperatively showed no evidence of clinical recurrence of MERS. Adult-onset MERS is relatively rare, and no association with tumors has been reported. The association between encephalitis and teratomas includes ovarian teratomas, which cause anti-N-methyl-D-aspartate receptor encephalitis and paraneoplastic limbic encephalitis. Although the cause of MERS was unknown in this case, we report the coexistence of a sacral teratoma and MERS to contribute to the knowledge of the association between them.
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  • 文章类型: Journal Article
    背景:具有恶性组织学的骶尾部畸胎瘤(SCT)经常复发并积极治疗,但成熟肿瘤的危险因素和监测方案建立得较少。特别是,先前的研究尚未调查卵黄囊瘤(YST)在其他成熟畸胎瘤中的微观沉积是否会导致更高的复发率。
    方法:我们回顾了2011年至2021年在我们机构切除的成熟SCT患者,并分析了肿瘤特征,治疗,和结果。
    结果:我们确定了56例成熟的SCT患者,其中9人(16%)表现为微观YST。手术后,7/56(13%)患者出现局部复发,平均为1.2±0.7年,而没有患者发生转移。镜下YST患者复发的可能性更大[5/9(56%)与2/47(4%),p=0.021]和正利润率[6/24(35%)与1/32(3.1%),p=0.030]。实体瘤成分也倾向于增加复发风险[6/29(21%)与1/27(4%),p=0.053]。5例患者表现出恶性复发,并且全部通过升高的甲胎蛋白(AFP)检测到,而两名患者表现出成熟畸胎瘤复发,并在磁共振成像(MRI)监测中检测到。
    结论:YST的显微病灶可能会增加成熟SCT患者的复发风险。这些患者可能会受益于通过连续AFP测量和MRI进行更密切的术后监测。
    BACKGROUND: Sacrococcygeal teratomas (SCT) with malignant histology frequently recur and are treated aggressively, but risk factors and surveillance protocols are less established for mature tumors. In particular, prior studies have not investigated whether microscopic deposits of yolk sac tumor (YST) in otherwise mature teratomas lead to higher recurrence rates.
    METHODS: We reviewed patients with mature SCTs resected at our institution from 2011 to 2021 and analyzed tumor characteristics, treatment, and outcomes.
    RESULTS: We identified 56 patients with mature SCT, of which 9 (16%) demonstrated microscopic YST. Following surgery, 7/56 (13%) patients developed local recurrence at a mean of 1.2 ± 0.7 years, while no patients developed metastases. Recurrence was more likely in patients with microscopic YST [5/9 (56%) vs. 2/47 (4%), p = 0.021] and positive margins [6/24 (35%) vs. 1/32 (3.1%), p = 0.030]. A solid tumor component tended to increase recurrence risk as well [6/29 (21%) vs. 1/27 (4%), p = 0.053]. Five patients demonstrated malignant recurrence and were all detected by a rising alpha-fetoprotein (AFP), while two patients demonstrated recurrence of mature teratoma and were detected on surveillance magnetic resonance imaging (MRI).
    CONCLUSIONS: Microscopic foci of YST may increase recurrence risk for patients with mature SCT. Such patients might benefit from closer postoperative surveillance with serial AFP measurements and MRI.
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  • 文章类型: Journal Article
    骶尾部畸胎瘤是一种罕见的先天性畸形,预后取决于影响胎儿发育的因素。诊断基于超声检查,尤其是对妊娠第20周胎儿的详细形态的评估。因此,继续寻找能够在产前确定胎儿最准确预后的超声标记是至关重要的。现在,我们依赖于少数以病例报告为主的研究.我们提供了有关骶尾部畸胎瘤诊断的基本信息的文献综述,治疗,与产前诊断有关的骶尾部畸胎瘤的并发症。事实证明,根据产前超声检查和分娩后充分的手术治疗,预后良好的病例,这种先天畸形的预后很好。
    : Sacrococcygeal teratoma is a rare congenital malformation, the prognosis depends on factors affecting foetal development. The diagnosis is based on ultrasound examination, especially the evaluation of the detailed morphology of the foetus in the 20th week of pregnancy. Therefore, it is crucial to keep looking for ultrasound markers that would prenatally determine the most accurate prognosis for the foetus. Now, we rely on a small number of studies with a predominance of case reports. We offer a literature review of the essential information concerning sacrococcygeal teratoma diagnostics, therapy, and complications of sacrococcygeal teratomas in connection with prenatal diagnosis. It turns out that in cases with a favourable prognosis according to prenatal ultrasound examination and adequate surgical treatment after childbirth, the prognosis of this congenital malformation is excellent.
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  • 文章类型: Case Reports
    骶尾部畸胎瘤是一种罕见的性腺外生殖细胞肿瘤,发生在背部下端。它是先天性的,可以在子宫内或出生后诊断。这些肿瘤是散发性的,女性比男性更常见。它们可以是固体,囊性的或两者的混合物。作者介绍这种情况是为了分享他们的经验,因为这些先天性肿瘤很少见,并且由于复发的机会和疾病恶性的可能性,适当管理的失误可能是毁灭性的。
    方法:在这种情况下,作者介绍了一个婴儿,该婴儿在阴道分娩后5天被转诊到他们的中心,骶尾部区域有巨大的肿块。MRI显示骶尾部畸胎瘤II型。进行了广泛的局部切除。肿瘤边缘镜下阴性。患者恢复良好并出院。患者不需要辅助治疗。
    常规产科超声诊断骶尾部畸胎瘤的敏感性为100%。肿瘤大小<5cm的胎儿可以通过阴道递送。大于此的肿瘤应在足月通过剖宫产分娩。高危骶尾部畸胎瘤可以在妊娠28周时通过剖宫产,然后进行子宫外治疗。MRI可用于评估肿瘤的骨盆内范围和计划管理。分娩后应进行手术干预。
    结论:宫内诊断不是终止妊娠的指征。切缘阴性的手术切除可治愈。肿瘤的组织学检查是强制性的。对于恶性骶尾部畸胎瘤,手术切除后肿瘤切缘阳性需要辅助化疗。阳性切缘可能需要化疗。如果肿瘤浸润邻近结构或转移,则新辅助化疗可用于手术前的肿瘤减积。
    UNASSIGNED: Sacrococcygeal teratoma is a rare extragonadal germ cell tumor occurring at the lower end of the back. It is congenital, and can be diagnosed in utero or just after birth. These tumors are sporadic in nature, more common in females than in males. They can be solid, cystic or a mixture of the two in consistency. Authors present this case to share their experience because these congenital tumors are rare and a lapse in appropriate management can be devastating because of chances of recurrence and the possibility of a malignant nature of the disease.
    METHODS: Authors in this case present a baby that was referred to their center 5 days post vaginal delivery with a huge mass on the sacrococcygeal region. MRI revealed sacrococcygeal teratoma type II. Wide local excision was done. Tumor margins were microscopically negative. The patient recovered well and was discharged. The patient did not require adjuvant treatment.
    UNASSIGNED: Routine obstetric ultrasound can diagnose sacrococcygeal teratoma with 100 % sensitivity. Fetuses with tumors <5 cm in size can be delivered vaginally. Tumors larger than that should be delivered at term through cesarean section. High risk sacrococcygeal teratomas can be delivered at 28 weeks of gestation by cesarean section followed by ex-utero intrapartum therapy. MRI is useful to assess the intrapelvic extent of the tumor and to plan management. Surgical intervention should be done after delivery.
    CONCLUSIONS: Intrauterine diagnosis is not an indication for termination of pregnancy. Surgical resection with negative margins provides a cure. Histological examination of the tumor is mandatory. For malignant sacrococcygeal teratomas, adjuvant chemotherapy is necessary for positive tumor margin after surgical resection. Positive margins may require chemotherapy. Neoadjuvant chemotherapy may be used for tumor debulking prior to surgery if the tumor infiltrates neighboring structures or is metastatic.
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  • 文章类型: Case Reports
    背景:巨大骶尾部畸胎瘤(SCT)由于其高血管分布而具有围产期发病率和死亡率的风险。术前栓塞供血动脉,在完成手术切除之前,可以通过阻塞这些供血动脉来帮助减少术中失血。
    方法:我们介绍一例新生儿高度血管巨大的SCT,手术前通过血管内途径成功栓塞。选择股动脉入路,使用显微穿刺导引器作为护套建立通路。使用微线圈的组合进行栓塞,明胶海绵浆,和聚乙烯醇颗粒。患者术后出现股动脉痉挛,通过应用三硝酸甘油酯贴剂解决了。
    结论:对巨大骶尾部畸胎瘤进行术前血管内栓塞治疗具有特殊的挑战,主要是由于难以评估小血管和与此手术相关的潜在并发症。然而,这种技术在帮助外科医生减少手术过程中的失血方面非常有价值,从而降低发病率和死亡率的风险。栓塞过程的全面规划至关重要,包括识别潜在的血管接入点和替代方案,仔细选择合适的导管。
    BACKGROUND: Giant sacrococcygeal teratomas (SCTs) are at risk of perinatal morbidity and mortality due to their high vascularity. Pre-operative embolization of the feeding arteries, prior to complete surgical resection, may assist in minimizing the intraoperative blood loss by occluding these feeding arteries.
    METHODS: We present a case of a highly vascular giant SCT in a neonate, which was successfully embolized through an endovascular approach prior to surgery. The femoral artery approach was chosen, with access established using a Micropuncture introducer as a sheath. Embolization was performed using a combination of microcoils, Gelfoam slurry, and polyvinyl alcohol particles. The patient developed femoral artery spasm post-procedure, which resolved with the application of a glyceryl trinitrate patch.
    CONCLUSIONS: Performing pre-operative endovascular embolization on a giant sacrococcygeal teratoma presents particular challenges, primarily due to the difficulty in assessing small vessels and the potential complications associated with this procedure. Nevertheless, this technique proves exceptionally valuable in helping the surgeon minimize blood loss during surgery, thereby reducing the risks of morbidity and mortality. Comprehensive planning for the embolization procedure is essential, encompassing the identification of potential vascular access points and alternatives, along with careful selection of the appropriate catheter.
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  • 文章类型: Journal Article
    背景:本研究旨在评估接受产前干预的胎儿骶尾部畸胎瘤(SCT)的结局。方法:我们对胎儿SCT患者进行了系统的文献回顾,并比较了开放胎儿手术和经皮介入治疗的结局。此外,我们还比较了未接受任何手术干预(NI)的SCT胎儿的结果.结果:我们确定了16例胎儿开放性手术(OS),经皮胎儿介入术(PI)48例,93名NI患者。OS生存率为56.2%,PI为45.8%(p=0.568),NI患者为71.0%。与所有评估队列中胎儿确实存活的情况相比,在没有存活的情况下,分娩时的胎龄更早(OS:p=0.033,PI:p<0.001,NI:p<0.001)。OS和PI胎儿分娩时的孕周更相似;然而,OS倾向于在怀孕后期进行,受影响的胎儿有更严重的表现。在我们的评价中,我们确定,在SCT病例中,胎儿积液和心力衰竭的存在对生存率没有显著影响.在NI患者中,与存活组相比,未存活的胎儿羊水过多的发生率要高得多(p<0.001).结论:总之,分娩时的胎龄可影响骶尾部畸胎瘤受累胎儿的短期预后。无论分娩方式或在胎儿期进行干预的必要性,监测并发症,包括羊水过多,可以防止早产。
    Background: This study aims to evaluate the outcomes of fetal sacrococcygeal teratoma (SCT) submitted to prenatal interventions. Methods: We performed a systematic literature review of fetal SCT patients and compared the outcomes between open fetal surgery and percutaneous intervention. In addition, we also compared the results of SCT fetuses who did not undergo any surgical intervention (NI). Results: We identified 16 cases of open fetal surgery (OS), 48 cases of percutaneous fetal intervention (PI), and 93 NI patients. The survival rate was 56.2% in OS, 45.8% in PI (p = 0.568), and 71.0% in NI patients. The gestational age at delivery was earlier in cases where there was no survival compared to cases where the fetuses did survive across all evaluated cohorts (OS: p = 0.033, PI: p < 0.001, NI: p < 0.001). The gestational weeks at delivery in OS and PI fetuses were more similar; however, OS tended to be performed later on in pregnancy, and the affected fetuses had more severe presented findings. In our evaluation, we determined that the presence of fetal hydrops and cardiac failure had no significant impact on survival in SCT cases. In NI patients, polyhydramnios was much higher in fetuses who did not survive compared to their surviving cohorts (p < 0.001). Conclusions: In conclusion, gestational age at delivery can affect the short-term prognosis of fetuses affected with sacrococcygeal teratomas. Regardless of the mode of delivery or the necessity for intervention during the fetal period, monitoring for complications, including polyhydramnios, can prevent premature delivery.
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  • 文章类型: Journal Article
    背景:血清甲胎蛋白(AFP)通常用作复发性骶尾部畸胎瘤(SCT)的肿瘤标志物。我们旨在评估初次切除后血清AFP水平的正常动态以及对SCT复发的随访中血清AFP水平的诊断准确性。
    方法:这项回顾性研究包括1980年至2018年在荷兰六个儿科外科中心接受SCT治疗的57例患者。
    结果:57例患者被纳入研究,其中19名儿童在初次切除后中位数为14.0个月时复发20例。初次切除后复发组和非复发组之间的血清AFP水平动态没有显着差异(p=0.950)。与同时无复发儿童的血清AFP水平相比,复发前血清AFP水平没有显着增加(p=0.106)。然而,与无复发患者相比,恶性复发患者血清AFP水平显著升高(n=7)(p=0.03).发现55μg/L的截止值可预测具有0.636的曲线下面积(AUC)的复发性SCT,灵敏度为50%,特异性为100%。
    结论:SCT初次切除后有复发和无复发的患者血清AFP水平动态没有差异。血清AFP水平不能预测成熟或未成熟的复发性SCT,正常的AFP水平不能排除复发性SCT。然而,血清AFP水平超过55μg/L可提示SCT复发,尤其是恶性复发.
    BACKGROUND: Serum alpha-fetoprotein (AFP) is often used as tumour marker for recurrent sacrococcygeal teratoma (SCT). We aimed to assess the normal dynamics of serum AFP levels after initial resection and diagnostic accuracy of serum AFP levels the follow-up for recurrence in SCT.
    METHODS: This retrospective study included 57 patients treated for SCT in the six pediatric surgical centers in the Netherlands from 1980 to 2018.
    RESULTS: 57 patients were included in the study of whom 19 children developed 20 recurrences at a median of 14.0 months after initial resection. No significant difference was found in serum AFP level dynamics between the recurrence and non-recurrence group after initial resection (p = 0.950). Serum AFP levels did not significantly increase before recurrence (p = 0.106) compared to serum AFP levels of children without recurrence at the same time. However, serum AFP levels did significantly increase in malignant recurrences (n = 7) (p = 0.03) compared to patients without recurrence. A cut-off value of 55 μg/L was found to be predictive for recurrent SCT with an Area Under the Curve (AUC) of 0.636 with sensitivity of 50% and specificity of 100%.
    CONCLUSIONS: Dynamics of serum AFP levels are not different between patients with and without recurrence after initial resection of SCT. Serum AFP levels are not predictive for mature or immature recurrent SCT and normal AFP levels do not rule out recurrent SCT. However, serum AFP levels exceeding 55 μg/L can indicate recurrent SCT, especially malignant recurrences.
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  • 文章类型: Letter
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  • 文章类型: Case Reports
    未成熟骶尾部畸胎瘤代表了一种具有快速肿瘤生长的组织学形式,早产的风险,并发症发生率提高,复发的风险增加,死亡率高于成熟型。因此,未成熟形式的产前诊断将显著改善这些病例的预后。为此,我们对诊断进行了广泛的文献回顾,治疗管理,以及未成熟畸胎瘤的随访。关于这种医疗行为,我们还介绍了我们的情况。总之,早期识别有或没有其他相关结构异常的未成熟骶尾部畸胎瘤及其正确的治疗方法是这些病例有利发展的基本原则。
    Immature sacrococcygeal teratoma represents a histological form with rapid tumor growth, a risk of premature birth, an enhanced rate of complications, an increased risk of recurrence, and a higher mortality rate than the mature type. Thus, prenatal diagnosis of immature forms would significantly improve the prognosis of these cases. To this end, we performed an extensive literature review on the diagnosis, therapeutic management, and follow-up of immature teratomas. Regarding this medical conduct, we also presented our case. In conclusion, the early identification of immature sacrococcygeal teratomas with or without other associated structural abnormalities and their correct therapeutic approach are basic principles for a favorable evolution of these cases.
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  • 文章类型: Case Reports
    头状骶尾部畸胎瘤(同体)是成熟囊性畸胎瘤的高度分化亚组,类似于畸形胎儿。这些肿瘤起源于尾骨的底部,并且可能在其肾盂内和肾盂外的范围和位置上有所不同。重要的是要将这种异常与具有更高结构组织程度的胎儿区分开来。一名5天大的新生儿,表现为II型骶尾囊型畸胎瘤。肿块包含囊性和实性成分。在手术切除和尾骨切除术后,在肿块内发现完全形成的肠,以及骨骼和其他定义明确的结构。证实肿瘤已完全切除,在组织病理学检查中未发现恶性或未成熟特征。患者最后一次见到生长良好,甲胎蛋白为3.5μg/L,切除后14个月。
    A fetiform sacrococcygeal teratoma (homunculus) is a highly differentiated subgroup of mature cystic teratoma that resembles a malformed fetus. These tumors originate at the base of the coccyx and may vary in their intrapelvic and extrapelvic extent and location. It is important to differentiate this anomaly from fetus-in-fetu which has a higher degree of structural organization. A 5-day-old neonate presented with a type II sacrococcygeal fetiform teratoma. The mass contained both cystic and solid components. Upon surgical excision and coccygectomy, fully formed bowel was found inside the mass, as well as bones and other well-defined structures. The tumor was confirmed to be fully excised and no malignant or immature features were found on histopathological examination. The patient was last seen growing well with an alpha-fetoprotein of 3.5 μg/L, 14 months after resection.
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