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Prenatal Diagnosis Nov 2023Several factors associated with poor outcome in patients with prenatally diagnosed sacrococcygeal teratoma (SCT) have been found. However, the prognostic accuracy of... (Meta-Analysis)
Meta-Analysis Review
Several factors associated with poor outcome in patients with prenatally diagnosed sacrococcygeal teratoma (SCT) have been found. However, the prognostic accuracy of these factors has not been well established. Therefore, we aimed to systematically review the prognostic accuracy of factors associated with poor outcome in these patients. We queried Search Premier, COCHRANE Library, EMCARE, EMBASE, PubMed, ScienceDirect, and Web of Science databases to identify studies regarding patients with prenatally diagnosed SCT. Poor outcome was defined as termination of pregnancy (TOP), intrauterine fetal death (IUFD), or perinatal death. We estimated the odds ratio of factors associated with poor outcome. Eleven studies (447 patients) were included. Overall mortality, including TOP, was 34.9%. Factors associated with poor outcome in fetuses with prenatally diagnosed SCT were cardiomegaly, hypervascular tumor, solid tumor morphology, fetal hydrops, and placentomegaly. A tumor volume to fetal weight ratio (TFR) of >0.12 before a gestational age of 24 weeks is predictive of poor outcome. The prognostic accuracy of factors associated with poor outcome in fetuses prenatally diagnosed with SCT seems promising. Factors associated with cardiac failure such as cardiomegaly, hypervascular tumor, solid tumor morphology, fetal hydrops, placentomegaly, and TFR >0.12 were found to be predictive of poor outcome.
Topics: Pregnancy; Female; Humans; Infant; Prognosis; Hydrops Fetalis; Ultrasonography, Prenatal; Teratoma; Cardiomegaly; Sacrococcygeal Region
PubMed: 37964422
DOI: 10.1002/pd.6457 -
European Journal of Surgical Oncology :... Jan 2023Sacrococcygeal teratoma (SCT) is a rare neoplasm affecting 1:35,000 newborns. Long-term follow-up from small observational studies report impaired bladder/bowel... (Review)
Review
Sacrococcygeal teratoma (SCT) is a rare neoplasm affecting 1:35,000 newborns. Long-term follow-up from small observational studies report impaired bladder/bowel function. This current study comprehensively analyses all published studies to better define true long-term functional sequelae. Medline/Embase databases were searched with PRISMA guidelines. Final analysis yielded 37 studies involving 1116 patients (854 female; 77%). Individual datasets were available in 14 studies (222 patients). According to Altman classification - 298/845 (35%) were Type I, 252/845 (30%) Type II, 133/845 (16%) Type III, and 128/845 (15%) Type IV tumours. Most neoplasms were benign (640/858; 75%), 77/858 (9%) immature and 141/858 (16%) malignant. Abnormal bladder function was reported in 7/39 (18%) Altman Type I, 23/61 (37.7%) Type II, 11/34 (32.4%) Type III, and 15/25 (60%) Type IV cases (p = 0.007). Adverse urological outcomes were notably common in immature/malignant neoplasms vs benign tumours and in patients requiring reoperation(s); p = 0.002 and p = 0.01. Bowel dysfunction was evident in 19% index cases and constipation in 26% with no significant association(s) with tumour characteristics. Higher Altman stage, unfavourable tumour biology and reoperation are associated with poor functional outcome(s). Multidisciplinary management from primary diagnosis of SCT is crucially important for all patients to best optimise functional outcomes across surgical specialities.
Topics: Humans; Infant, Newborn; Female; Pelvic Neoplasms; Teratoma; Spinal Neoplasms; Urinary Bladder; Disease Progression; Sacrococcygeal Region; Retrospective Studies
PubMed: 36127201
DOI: 10.1016/j.ejso.2022.09.002 -
Journal of Clinical Medicine Apr 2024This study aims to evaluate the outcomes of fetal sacrococcygeal teratoma (SCT) submitted to prenatal interventions. We performed a systematic literature review of... (Review)
Review
This study aims to evaluate the outcomes of fetal sacrococcygeal teratoma (SCT) submitted to prenatal interventions. 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). 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 ( = 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: = 0.033, PI: < 0.001, NI: < 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 ( < 0.001). 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.
PubMed: 38731178
DOI: 10.3390/jcm13092649 -
Ultrasound in Obstetrics & Gynecology :... Jun 2014Large solid sacrococcygeal teratomas (SCT) can cause high-output cardiac failure and fetal or neonatal death. The aim of this study was to describe the outcomes of... (Review)
Review
OBJECTIVE
Large solid sacrococcygeal teratomas (SCT) can cause high-output cardiac failure and fetal or neonatal death. The aim of this study was to describe the outcomes of minimally invasive antenatal procedures for the treatment of fetal SCT.
METHODS
A case review was performed of five fetuses with a large SCT treated antenatally using minimally invasive techniques, and a systematic literature review on fetal therapy for solid SCTs was carried out.
RESULTS
Five women were referred between 17 + 5 and 26 + 4 weeks' gestation for a large fetal SCT with evidence of fetal cardiac failure. Vascular flow to the tumors was interrupted by fetoscopic laser ablation (n = 1), radiofrequency ablation (RFA; n = 2) or interstitial laser ablation ± vascular coiling (n = 2). There were two intrauterine fetal deaths. The other three cases resulted in preterm labor within 10 days of surgery. One neonate died. Two survived without procedure-related complications but had long-term morbidity related to prematurity. The systematic literature review revealed 16 SCTs treated minimally invasively for (early) hydrops. Including our cases, six of 20 hydropic fetuses survived after minimally invasive therapy (30%). Survival after RFA or interstitial laser ablation was 45% (5/11). Of 12 fetuses treated for SCT without obvious hydrops and for which perinatal survival data were available, eight (67%) survived. Mean gestational age at delivery after minimally invasive therapy was 29.7 ± 4.0 weeks. Survival after open fetal surgery in hydropic fetuses was 6/11 (55%), with a mean gestational age at delivery of 29.8 ± 2.9 weeks.
CONCLUSIONS
Fetal therapy can potentially improve perinatal outcomes for hydropic fetuses with a solid SCT, but is often complicated by intrauterine death and preterm birth.
Topics: Adult; Child, Preschool; Embolization, Therapeutic; Female; Fetal Death; Fetal Diseases; Fetoscopy; Heart Failure; Humans; Infant; Infant, Newborn; Laser Therapy; Perinatal Death; Pregnancy; Pregnancy Outcome; Prenatal Care; Sacrococcygeal Region; Spinal Neoplasms; Teratoma
PubMed: 24488859
DOI: 10.1002/uog.13315 -
Fetal Diagnosis and Therapy 2022Although maternal-fetal surgery to treat fetal anomalies such as spina bifida continues to grow more common, potential health disparities in the field remain relatively... (Review)
Review
BACKGROUND/PURPOSE
Although maternal-fetal surgery to treat fetal anomalies such as spina bifida continues to grow more common, potential health disparities in the field remain relatively unexamined. To address this gap, we identified maternal-fetal surgery studies with the highest level of evidence and analyzed the reporting of participant sociodemographic characteristics and representation of racial and ethnic groups.
METHODS
We conducted a systematic review of the scientific literature using biomedical databases. We selected randomized control trials (RCTs) and cohort studies with comparison groups published in English from 1990 to May 5, 2020. We included studies from across the globe that examined the efficacy of fetal surgery for twin-twin transfusion syndrome (TTTS), obstructive uropathy, congenital diaphragmatic hernia (CDH), myelomeningocele (MMC), thoracic lesions, cardiac malformations, or sacrococcygeal teratoma. We determined the frequency of reporting of age, gravidity/parity, race, ethnicity, education level, language spoken, insurance, income level, and relationship status. We identified whether sociodemographic factors were used as inclusion or exclusion criteria. We calculated the racial and ethnic group representation for studies in the USA using the participation-to-prevalence ratio (PPR).
RESULTS
We included 112 studies (10 RCTs, 102 cohort) published from 1990-1999 (8%), 2000-2009 (30%), and 2010-2020 (62%). Most studies were conducted in the USA (47%) or Europe (38%). The median sample size was 58. TTTS was the most common disease group (37% of studies), followed by MMC (23%), and CDH (21%). The most frequently reported sociodemographic variables were maternal age (33%) and gravidity/parity (20%). Race and/or ethnicity was only reported in 12% of studies. Less than 10% of studies reported any other sociodemographic variables. Sociodemographic variables were used as exclusion criteria in 13% of studies. Among studies conducted in the USA, White persons were consistently overrepresented relative to their prevalence in the US disease populations (PPR 1.32-2.11), while Black or African-American, Hispanic or Latino, Asian, American-Indian or Alaska-Native, and Native-Hawaiian or other Pacific Islander persons were consistently underrepresented (PPR 0-0.60).
CONCLUSIONS
Sociodemographic reporting quality in maternal-fetal surgery studies is poor and inhibits examination of potential health disparities. Participants enrolled in studies in the USA do not adequately represent the racial and ethnic diversity of the population across disease groups.
Topics: Black People; Ethnicity; Female; Hispanic or Latino; Humans; Maternal Age; Pregnancy; United States
PubMed: 35272297
DOI: 10.1159/000523867 -
Journal of Pediatric Surgery Sep 2020The incidence of children developing recurrent sacrococcygeal teratoma (SCT) is 2-35%. Serum alpha-fetoprotein (AFP) is often used as a tumor marker for (malignant)...
BACKGROUND
The incidence of children developing recurrent sacrococcygeal teratoma (SCT) is 2-35%. Serum alpha-fetoprotein (AFP) is often used as a tumor marker for (malignant) recurrences of SCT and could potentially be used during routine follow-up after SCT resection. However, the diagnostic accuracy of serum AFP levels during follow-up has not been well established. Therefore, we aimed to systematically review the diagnostic accuracy of serum AFP levels in recurrent SCT.
METHODS
We queried Search Premier, COCHRANE Library, EMCARE, EMBASE, PubMed, ScienceDirect and Web of Science databases to identify studies regarding patients with SCT with follow-up using serum AFP levels postoperative. We estimated sensitivity and specificity of serum AFP levels.
RESULTS
Fifteen studies (613 patients, 121 recurrences) were included and these mainly described serum AFP levels in patients with recurrent SCT (n = 111); 83 (75%) patients with recurrent SCT had elevated serum AFP levels. A subgroup analysis of articles that measured serum AFP levels in all patients (n = 6, 136 patients, 14 recurrences) showed a sensitivity and specificity of 79% and 95%, respectively. The sensitivity of AFP levels to detect malignant recurrence was 96%.
CONCLUSION
Diagnostic accuracy of serum AFP levels to detect recurrent SCT seems promising, though sensitivity could be overestimated since serum AFP levels are mainly described in patients with elevated AFP levels or at recurrent SCT. Furthermore, serum AFP levels could be helpful to detect malignant recurrences.
TYPE OF STUDY
Systematic review of level 2-4 studies.
LEVEL OF EVIDENCE
Level 2-4 (mostly level 2).
Topics: Biomarkers, Tumor; Child; Humans; Neoplasm Recurrence, Local; Pelvic Neoplasms; Sacrococcygeal Region; Sensitivity and Specificity; Spinal Neoplasms; Teratoma; alpha-Fetoproteins
PubMed: 32376010
DOI: 10.1016/j.jpedsurg.2020.03.014 -
Ultrasound in Obstetrics & Gynecology :... Jun 2016The objectives of this study were to evaluate the efficacy of minimally invasive ablation of high-risk large sacrococcygeal teratomas (SCT) and to compare the efficacy... (Review)
Review
OBJECTIVES
The objectives of this study were to evaluate the efficacy of minimally invasive ablation of high-risk large sacrococcygeal teratomas (SCT) and to compare the efficacy of vascular and interstitial tumor ablation.
METHODS
This was a retrospective multicenter study including a cohort of fetuses with high-risk large SCTs between 2004 and 2010. In addition, we performed a systematic literature review of all cases that underwent tumor ablation in order to compare the survival rates after 'vascular' and 'interstitial' ablation. Statistical analysis was conducted using Bayesian methods.
RESULTS
In our cohort, a total of 13 fetuses had high-risk large SCT and five of them underwent tumor ablation. The estimated difference in hydrops resolution rate between the fetal intervention and the no fetal intervention groups was 44.6% (95% credibility interval, 1.5 to 81.0%; Pdiff> 0 = 97.9%). The estimated difference in survival rate between the fetal intervention and the no fetal intervention groups was 31.0% (13.9 to 48.1%; Pdiff> 0 = 99.9%). We analyzed our five cases together with 28 cases from the literature and estimated the difference in survival rate between the vascular and interstitial ablation groups as 19.8% (-13.1 to 50.1%; Pdiff> 0 = 88.3%). The estimated difference in hydrops resolution rate between the vascular and the interstitial ablation groups was 36.7% (-5.7 to 72.7%; Pdiff> 0 = 95.5%).
CONCLUSION
Minimally invasive surgery seems to improve perinatal outcome in cases of high-risk large fetal SCT. Our findings suggest that 'vascular' ablation may improve outcome and may be more effective than 'interstitial' tumor ablation, but this hypothesis needs further investigation in a larger multicenter prospective study. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.
Topics: Ablation Techniques; Cohort Studies; Female; Fetal Therapies; Humans; Minimally Invasive Surgical Procedures; Pregnancy; Retrospective Studies; Sacrococcygeal Region; Spinal Neoplasms; Survival Analysis; Teratoma; Treatment Outcome
PubMed: 26138446
DOI: 10.1002/uog.14935 -
Frontiers in Pain Research (Lausanne,... 2022The anesthetic management of fetal operative procedures (FOP) is a highly debated topic. Literature on fetal pain perception and response to external stimuli is rapidly... (Review)
Review
OBJECTIVE
The anesthetic management of fetal operative procedures (FOP) is a highly debated topic. Literature on fetal pain perception and response to external stimuli is rapidly expanding. Nonetheless, there is no consensus on the fetal consciousness nor on the instruments to measure pain levels. As a result, no guidelines or clinical recommendations on anesthesia modality during FOP are available. This systematic literature review aimed to collect the available knowledge on the most common fetal interventions, and summarize the reported outcomes for each anesthetic approach. Additional aim was to provide an overall evaluation of the most commonly used anesthetic agents.
METHODS
Two systematic literature searches were performed in Embase, Medline, Web of Science Core Collection and Cochrane Central Register of Controlled Trials up to December 2021. To best cover the available evidence, one literature search was mostly focused on fetal surgical procedures; while anesthesia during FOP was the main target for the second search. The following fetal procedures were included: fetal transfusion, laser ablation of placental anastomosis, twin-reversed arterial perfusion treatment, fetoscopic endoluminal tracheal occlusion, thoraco-amniotic shunt, vesico-amniotic shunt, myelomeningocele repair, resection of sacrococcygeal teratoma, ligation of amniotic bands, balloon valvuloplasty/septoplasty, ex-utero intrapartum treatment, and ovarian cyst resection/aspiration. Yielded articles were screened against the same inclusion criteria. Studies reporting anesthesia details and procedures' outcomes were considered. Descriptive statistical analysis was performed and findings were reported in a narrative manner.
RESULTS
The literature searches yielded 1,679 articles, with 429 being selected for full-text evaluation. A total of 168 articles were included. Overall, no significant differences were found among procedures performed under maternal anesthesia or maternal-fetal anesthesia. Procedures requiring invasive fetal manipulation resulted to be more effective when performed under maternal anesthesia only. Based on the available data, a wide range of anesthetic agents are currently deployed and no consistency has been found neither between centers nor procedures.
CONCLUSIONS
This systematic review shows great variance in the anesthetic management during FOP. Further studies, systematically reporting intraoperative fetal monitoring and fetal hormonal responses to external stimuli, are necessary to identify the best anesthetic approach. Additional investigations on pain pathways and fetal pain perception are advisable.
PubMed: 36246050
DOI: 10.3389/fpain.2022.935427 -
Prenatal Diagnosis Nov 2015To describe the epidemiology and outcomes of sacrococcygeal teratoma (SCT) and identify the factors affecting prognosis in a population-based cohort. (Review)
Review
OBJECTIVES
To describe the epidemiology and outcomes of sacrococcygeal teratoma (SCT) and identify the factors affecting prognosis in a population-based cohort.
METHODS
Analyses of fetal SCTs from a population-based congenital anomaly register between 1995 and 2012, linked to regional datasets. A systematic literature review was performed for published studies on perinatal SCT (1995 to 2012).
RESULTS
Thirty-six confirmed SCT cases were identified, giving a total prevalence of 0.30 per 10 000 births (95%CI 0.20-0.39). Twenty-three cases (63.9%) were diagnosed prenatally. There were six false positive prenatal diagnoses, and the positive predictive value of ultrasound for SCT was 79.3%. Secondary complications in prenatally diagnosed cases were polyhydramnios (27.2%), fetal hydrops (9.1%) and rapidly growing tumour (54.0%). The perinatal (PNMR) and infant mortality rates were 333.3 per 1000 births and 285.7 per 1000 live births, respectively. All stillbirths and infant deaths occurred in cases diagnosed prenatally. Factors associated with higher PNMR in registerable births were solid, vascular tumour composition (1000), polyhydramnios (667), premature delivery (667) and rapidly growing tumour (454). In the systematic review, prenatal hydrops fetalis and prematurity were the most morbid association in SCT.
CONCLUSION
Prenatal ultrasound was relatively sensitive and specific in diagnosing SCT with good survival rates in live-born cases.
Topics: Adult; Cohort Studies; England; Female; Humans; Hydrops Fetalis; Infant, Newborn; Infant, Premature; Perinatal Mortality; Polyhydramnios; Predictive Value of Tests; Pregnancy; Prevalence; Retrospective Studies; Sacrococcygeal Region; Sensitivity and Specificity; Stillbirth; Teratoma; Ultrasonography, Prenatal
PubMed: 26114890
DOI: 10.1002/pd.4641 -
Frontiers in Pediatrics 2021The use of robotics-assisted surgery in oncology has been proved effective and safe in adults. Despite these results, the use of robotics has been rarely reported for...
AIM
The use of robotics-assisted surgery in oncology has been proved effective and safe in adults. Despite these results, the use of robotics has been rarely reported for pediatric oncology. Our review aims to evaluate the safety and feasibility of robotics-assisted surgery in this field, analyzing our experience and performing a systematic review of the most recent studies.
METHODS
We reviewed all patients affected by an oncological disease who underwent a robotics-assisted procedure at our institute. We performed a systematic review of the literature from 2012 to 2021 on the subjects.
FINDINGS
A total of 14 patients underwent robotics-assisted tumor resection. Eleven procedures (median age 13.2-years old) were carried out in children with adnexal lesions (seven tumor excision and four ovariectomies). Histological diagnosis was mature teratoma (six), serous papillary cystadenofibromas of the fallopian tube (two), ovarian serous cystadenoma (one), ovarian mucinous cystadenoma (one), and ovarian seromucinous cystadenoma. The median length of stay was 2 days. No recurrences or complications at a median follow-up of 2.1-years were observed. A 5-year-old girl underwent a complete posterior resection of a type 3 sacrococcygeal tumor with a robotics-assisted approach for the dissection of a possible intraabdominal residual component of the lesion. No intra- and postoperative complications were recorded. Complete excision of a recurrent differentiating neuroblastoma of the left para-renal region was performed on a 9-year-old girl. An idiopathic anaphylactic shock occurred 1 day after the procedure. At 9 months' follow-up, no local recurrences of the lesion were observed. Overall, we reported no conversion to open surgery. Lastly, a robotic excision of a growing left superior mediastinal intermixed ganglioneuroblastoma was performed on an 8-year-old girl with no postoperative complications. Follow-up was uneventful (7 months). In the literature, the rate of complications ranges from 0 to 28%, mainly related to difficult dissection and impaired anatomy. Conversion is reported in 5% of all oncological procedures, due to more invading tumors and altered anatomical features. No robotics-related complications were reported.
CONCLUSION
Robotics-assisted surgery in pediatric oncology has proven to be feasible. Nevertheless, its use should be limited to selected cases and performed by highly trained oncological surgeons. Preparation and patient positioning, alongside a correct port placement, are crucial to carrying out these procedures. Further innovations in robotics may allow a wider application of this technology in pediatric oncology.
PubMed: 35118030
DOI: 10.3389/fped.2021.780830