Year: 2024
Type: review
Study design: Systematic Review
Abstract: Introduction Sacrococcygeal teratoma (SCT) is a rare embryonal tumor that occurs in the sacrococcygeal region, with an incidence of about 1 in 35,000 to 40,000 live births. This study presents a systematic review of giant SCT greater than 10 cm. Methods A systematic review of published studies regarding giant SCT in infants was conducted. The studies included met the following criteria: 1) the teratoma was situated in the sacrococcygeal region; 2) all case reports involved infants with a teratoma larger than 10 cm, and 3) the size of the teratoma was verified to exceed 10 cm through diagnostic methods. Results The current study included 31 studies that met the inclusion criteria. The studies included patients aged 6.1 to 9.3 months, with a median age of 7.6 months, predominantly female (72.7%). Diagnoses were primarily made in the first and second trimesters (39%) or after birth (33.3%), with cesarean delivery being the most common method (66.7%). Tumors weighed between 1.5 and 5 kg, with an average diameter of 15.6 cm. Surgical resection was performed in 93.9% of cases. The most common complication was respiratory failure (30.3%), and histopathology revealed that 39.4% of tumors were immature teratomas, while 33.3% were mature teratomas. The overall survival rate was 66.7%, with 18.2% of survivors experiencing tumor recurrence. Most complications occurred in the second trimester; however, no significant associations were found concerning the timing of diagnosis. Additionally, tumor size did not significantly impact outcomes. Conclusion Routine ultrasound and MRI are essential for the antenatal diagnosis of SCT. Due to the high risk of morbidity with larger tumors, cesarean delivery is advised for tumors over 10 cm. Coccygectomy is the most effective approach to prevent recurrence, highlighting the importance of timely surgical intervention and ongoing follow-up. Introduction Sacrococcygeal teratoma (SCT) is an uncommon embryonal tumor form in the sacrococcygeal region. It affects around one among every 35,000 to 40,000 live births [1]. The condition is significantly more common in females, with a female-to-male ratio of 3:1 to 4:1. Teratomas consist of tissues originating from all three germ layers: ectoderm, endoderm, and mesoderm [2,3]. Although their exact embryonic origin remains uncertain, SCTs are believed to arise early in gestation from totipotent cells in Hensen’s node, a remnant of the primitive streak in the coccygeal region [2]. SCTs can vary significantly in size, with some growing large enough to cause noticeable anatomical changes, such as the anterior displacement of the anus and resulting in clinical symptoms like anal displacement, tightening of the anal canal, and constipation, often due to tumor compression of the bladder or rectum [1,3]. Obstetric ultrasound during the second trimester is a crucial tool for making antenatal diagnoses of tumors, helping to prevent perinatal and neonatal complications such as fetal hydrops, tumor rupture, placentomegaly, and high-output cardiac failure, all of which are linked to a higher risk of mortality [4,5]. In 1973, according to the American Academy of Pediatrics Surgical Section (AAPSS), Altman classified SCTs into four types based on the tumor's intrapelvic and intra-abdominal extension and external components. SCTs observed at birth are typically classified as Altman Type I or II, with Type III being rare, while Type IV is usually identified later in life [2]. The management of SCT involves surgical excision, and the likelihood of recurrence after complete removal is minimal [5], leading to a favorable prognosis. However, long-term follow-up is essential to monitor for recurrence [3]. While there have been reports of large SCTs, to the best of our knowledge, based on an extensive literature review and previous case reports, no systematic reviews specifically address SCTs greater than 10 cm in infants. All included references were confirmed for eligibility [6]. Methods Study design The present systematic review adhered to the preferred reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Data sources and search strategy A comprehensive review of all published studies on SCT was conducted by searching databases such as Google Scholar, PubMed/MEDLINE, Cochrane Library, ScienceDirect, CINAHL, Web of Science, and EMBASE. The search used the following keywords: (sacrococcygeal) AND (teratoma OR tumor) in combination with (antenatal OR neonate OR infancy OR infant OR child OR children OR newborn OR pediatric). Eligibility criteria The non-English studies and those unrelated to humans were excluded before or during the initial screening. Studies on SCT were included if they met the following criteria: 1) the teratoma was located in the sacrococcygeal region; 2) all case reports involved infants with a teratoma larger than 10 cm, and 3) the teratoma size was confirmed to be over 10 cm through diagnostic methods. Studies published in predatory journals (inappropriately peer-reviewed) and those not meeting the inclusion criteria were excluded. Study selection and data extraction The titles and abstracts of the identified studies were initially screened. This was followed by a comprehensive review of the full text to assess eligibility. Data points collected from the included studies included the year and country of the study, patient age and gender, age at delivery, trimester of detection, postnatal diagnosis, mode of delivery, tumor size and weight, imaging technique, complications, and recurrence rates. Statistical analysis The data were first utilized in a qualitative synthesis, followed by quantitative re-analysis using the Chi-square test and Fisher's exact test for categorical variables and the independent sample t-test for numerical data, conducted with SPSS software version 25.0. A significance level of 0.05 was not established. Results Study selection and characteristics A systematic search initially identified 157 articles. Before screening, eight were excluded due to duplication and non-English language. During the initial title and abstract screening, 55 studies were excluded as they did not meet the inclusion criteria. Subsequently, 94 studies underwent full-text screening, and 32 were further assessed for eligibility. Ultimately, 31 studies [1-4, 7-33] were compatible with the inclusion criteria and included in the final analysis (Figure 1). All the studies included were case reports (Table 1). Table 1. Raw data of each included study. Author [reference] Year Country Gender Age of delivery (days) Diagnosis time Mode of delivery Weight (kg) Size of tumor (cm) Altman classification Complication Histopathology Survival Recurrence Chamberlin et al. [7] N/A USA N/A 224 1st trimester CS 3.7 13.9 N/A Near cardiac arrest & hypoxia N/A Yes N/A Sabir et al. [1] 2023 Iraq F 266 2nd trimester CS 5.0 17.5 1 None Immature teratoma Yes No Dey et al. [8] 2023 USA F 206 Prenatal (Uknown trimester) CS 2.4 11.6 3 Poor respiratory effort Immature teratoma Yes No Abou-Bekr et al. [4] 2022 Algeria F N/A Postnatal CS N/A 10 1 None Mature teratoma Yes N/A Koc et al. [9] 2022 Turkey F 209 N/A N/A 2.2 10 1 None Teratoma (unknown type) Yes N/A Meshram et al. [2] 2021 India M 259 2nd trimester CS 2.2 24.5 2 Polyhydramnios Mature teratoma No N/A Zlatan et al. [10] 2021 Bosnia and Herzegovina M 238 2nd trimester CS 2.3 15.5 2 Cardiac arrest Immature teratoma Yes N/A Guitart et al. [11] 2020 Spain N/A 245 2nd trimester CS 4.0 18 1 Premature rupture of the membranes & abnormal cardiotocography Immature teratoma Yes N/A Savitri et al. [12] 2019 Indonesia F 266 2nd trimester CS 4.2 11.2 1 Resuscitation needed Teratoma (unknown type) Yes No Singhal et al. [13] 2018 India F 196 Postnatal NVD 3.6 16 N/A Infection Mature teratoma N/A N/A Konoplitskyi et.al [14] 2018 Ukraine F 280 3rd trimester CS 4.3 14.9 1 N/A Immature teratoma Yes Yes Sop Lee et al. [15] 2017 Korea M 199 2nd trimester CS 2.9 12.3 3 Poor respiratory distress Immature teratoma Yes No Bechtel et.al [16] 2014 USA N/A 226 Prenatal (Uknown trimester) CS 3.7 13.9 2 Lower limb flaccid paralysis, scoliosis Immature teratoma Yes Yes Mondal et al. [17] 2014 India F 245 Postnatal NVD 3.5 16.7 1 Bleeding N/A No N/A Mbumba et al. [18] 2010 France F 266 1st trimester CS N/A 16 N/A None Mature teratoma N/A N/A Roka et al. [3] 2010 Nepal F N/A Postnatal N/A N/A 22.4 N/A None Mature and yolk sac components (Schiller-Duval bodies) Yes N/A Lahdes-Vasama et al. [19] 2010 Finland F 210 2nd trimester CS 3.4 16.9 2 Bleeding Immature teratoma with a malignant component in a small area Yes No Abraham et al. [20] 2010 USA F 238 2nd trimester CS N/A 18.9 3 Premature rupture of membranes & poor respiratory effort N/A Yes N/A Den Otter et al. [21] 2007 Netherlands F 192 2nd trimester CS 2.2 11 2 Respiratory failure Mixed teratoma Yes Yes Howman-Giles et al. [22] 2007 Australia F N/A Postnatal N/A N/A 16 N/A Left iliac lymph node enlargement, colostomy needed Immature teratoma No Yes Hosono et al. [23] 2004 Japan F 252 3rd trimester CS 4.0 13.1 1 Poor respiratory efforts Immature teratoma Yes No Ribeiro et al. [24] 1999 France F 266 Postnatal CS 2.7 11.4 1 None Mature teratoma Yes No Jona et al. [25] 1999
Source: Barw Medical Journal
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4406367187