• Journal of Korean Neurosurgical Society
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• v.41 no.1
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• pp.57-60
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• 2007

The incidence of diastematomyelia associated with teratoma is extremely rare. We present a case of sacrococcygeal teratoma in a neonate with split spinal cord malformation[SSCM]. Magnetic resonance imaging[MRI] showed a heterogenous mass lesion with cyst in the sacrococcygeal region and multiple spinal anormalies [diastematomyelia, tethered cord, hydromyelia, and hemivertebrae]. The mature teratoma was confirmed on histopathological examination. In SSCMs, the potential for coexisting congenital anomalies at separate levels of the spinal cord must be considered in radiological investigations.

• Journal of Korean Neurosurgical Society
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• v.64 no.3
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• pp.406-413
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• 2021

Sacrococcygeal teratoma (SCT) is an extragonadal germ cell tumor (GCT) that develops in the fetal and neonatal periods. SCT is a type I GCT in which only teratoma and yolk sac tumors arise from extragonadal sites. SCT is the most common type I GCT and is believed to originate through epigenetic reprogramming of early primordial germ cells migrating from the yolk sac to the gonadal ridges. Fetal SCT diagnosed in utero presents many obstetrical problems. For high-risk fetuses, fetal interventions (devascularization and debulking) are under development. Most patients with SCT are operated on after birth. Complete surgical resection is the key for tumor control, and the anatomical location of the tumor determines the surgical approaches. Incomplete resection and malignant histology are risk factors for recurrence. Approximately 10-15% of patients have a tumor recurrence, which is frequently of malignant histology. Long-term surveillance with monitoring of serum alpha fetoprotein and magnetic resonance imaging is required. Survivors of SCT may suffer anorectal, urological, and sexual sequelae later in their life, and comprehensive evaluation and care are required.

• Childhood Kidney Diseases
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• v.21 no.1
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• pp.26-30
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• 2017

Newborn infants with huge and highly vascular sacrococcygeal teratoma (SCT) are frequently subjected to renal hypoperfusion secondary to high-output cardiac failure. Any underlying renal dysfunction is a significant risk factor for the development of contrast-induced nephropathy (CIN). However, reports on CIN in infants are rare. I report here a case of a premature infant born at 28 weeks and 3 days of gestation with a huge SCT who survived preoperative embolization and surgical resection but presented with persistent non-oliguric renal failure that was suggestive of CIN. During radiological intervention, a contrast medium had been administered at about 10 times the manufacturer-recommended dose for pediatric patients. Despite hemodynamic stabilization and normalization of urine output immediately following surgery, the patient's serum creatinine and cystatin-C levels did not return to baseline until 4 months after birth. No signs of reflux nephropathy were observed in follow-up imaging studies. Dosing guidelines for the use of a contrast medium in radiological interventions should be provided for infants or young patients.

• Advances in pediatric surgery
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• v.5 no.1
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• pp.1-14
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• 1999

Among 60 children with teratoma, forty-three (71.7 percent) were girls and 17 (28.3 percent) boys, Primary sites were sacrococcygeal in 30 patients (50 percent), retroperitoneal in 12 (20 percent), ovarian in 11 (18.3 percent), testicular in 3 (5 percent), and one in each of nasopharyngeal, gastric, hepatic and pancreatic (1.6 percent, respectively). Fifty-five (91.7 percent) teratomas were benign and 5 (8.3 percent) malignant. Malignant teratomas W8,re detected only in the sacrococcygeal region (16.7 percent). Age greater than 2 mouths at diagnosis, presence of urinary or colonic obstructive symptoms, multiple masses and elevated serum alpha-fetoprotein were indicators of malignancy in the sacrococcygeal region. Tumor size, presence of calcification, and gross apperance (cystic or solid) did not correlate with malignancy. Thirteen (21.7 percent) cases were associated with other anomalies. For the immature teratoma, operative resection without adjuvant chemotherapy was adequate treatment. Three patients with malignant tumors survived, one who received chemotherapy survived 3 years and the others without chemotherapy survived for 5 and 10 years.

• Advances in pediatric surgery
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• v.2 no.2
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• pp.102-109
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• 1996

Pediatric surgeons are familiar with the posterior sagittal approach to the rectum at sacrococcygeal area and well oriented with the anatomy because of the Penal procedure for imperforate anus. The author utilized the posterior vertical elliptical incisions in 12 cases of sacrococcygeal teratoma since 1987. For presacral tumor(type IV)$^2$, the incision was exactly same as the posterior sagittal procedure for imperforate anus. But the out-growing(type I) or dumbbell-shaped(type II & III) tumors, a vertical elliptical incision was required. For the laterally deviated tumors, a vertical and half-chevron incision was utilized in one case, but an unbalanced vertical elliptical incision was acceptable for the remaining two cases, with shrinkage of the overlying skin. In dumbbell-shaped tumors(type II & III), the narrow waist of the tumor was at the level of the levator muscle, which formed a muscle-belt on the tumor waist. A careful dissection to save the muscle-belt seemed to be the most important point during this procedure, utilizing the nerve-stimulator. After complete removal of the tumor and the coccyx, the levator muscles and the skin were closed in vertical fashion along the midline. For the caudally extending tumors in 3 cases, the muscle complex was divided in midline. Nothing by mouth and total parenteral nutrition was maintained for 1 week and then laxatives were given for 2 weeks in order to give the sphincters rest. Operative scars were acceptable resembling natural vertical midline folds, and the sphincter function was continent in all cases. In conclusion, vertical elliptical incision in sacrococcygeal teratoma is recommended because of the acceptable scar, functional restoration, and because it is a familiar procedure particularly for the pediatric surgeons who are accustomed performing posterior sagittal approach for imperforate anus.

• Advances in pediatric surgery
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• v.19 no.2
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• pp.81-89
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• 2013

The purposes of this study was to describe the clinical correlation of mass size and gestational age, prognostic factors in sacrococcygeal teratoma (SCT) at a tertiary pediatric surgery, University of Ulsan College of Medicine and Asan Medical Center (AMC), Seoul, Korea. Fifty five patients admitted to the AMC with a SCT between May 1989 and April 2013 were included in this retrospective review. Mean follow up was 861 days. Mean maternal age at delivery was $30{\pm}2.7$ year, mean gestational age (GA) was $36.9{\pm}3.6$ wks, and preterm delivery was 21.8%. Birth body weight was $3182{\pm}644$ g and male vs. female ratio was 1:2.05. We can't find significant difference between Caesarean section and maternal age at delivery (p =0.817). But, caesarean section was favored by gestational age (p = 0.002), larger tumor size (p =0.029) or higher tumor weight fraction rate to birth body weight (p =0.024). Type I was 13, II 21, III 17, and IV 3 according to Altman et al. classification. The tumor component was predominantly cystic(> 50%) in 73.1 %. And the majority histological classification of tumors were mature teratoma (70.3%). The motality rate was 5.5%. Three patients expired because of postpartum bleeding, post-op bleeding related complication such as DIC. SCT recurred in four patients. The interval between first and second operation was $206.2{\pm}111.0$ d (range 53~325 d). In two patients, serum AFP levels were elevated at a regular checkup without any symptom, and subsequent imaging studies revealed SCT. The most common cause of death was bleeding and bleeding related complication. So Caesarean section and active peripartum and perioperative management will be needed for huge solid SCT. In the case of Yolk sac tumor or huge immature teratoma, possibility of recurrence have to be always considered, so follow up by serial AFP and MRI is important for SCT management.

• Advances in pediatric surgery
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• v.9 no.2
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• pp.94-97
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• 2003

The teratoma is a unique complex neoplasm and is one of the most frequent pediatric tumors originated from the extragonadal germ cells. Mature teratoma is composed of mature differentiated tissues, while immature teratoma always contains embryonic tissues of variable degrees of immaturity, especially in the neuroepithelial elements. Diagnosis of teratoma is relatively easy by conventional radiologic study, but the immaturity can be identified only by histopathological examination. Between January 1993 to December 2002, 63 cases of teratoma were operated and analysed retrospectively at the Chonnam University Hospital Female to male ratio was about 3:1 and age distribution was relatively even. Among 63 cases, gonadal teratoma was the most common (52.4%), followed by sacrococcygeal (25.4%), retroperitoneal (9.5%) and mediastinal teratoma (9.5%). Fifty-six cases were mature teratomas and seven were immature teratomas. Alpha-fetoprotein (AFP) was elevated in 4 of 6 immature cases, but in 2 of 51 mature ones Elevated AFP progressively returned to normal range by 1 month after operation in all. Complete excision of the mass was performed, and major complication was not noticed. In five immature cases, PEB chemotherapy (Cisplatin, Etoposide, Bleomycin) was performed. Two of 2 cases in histological grade II were well tolerated to the aggressive chemotherapy. One of three cases in grade III expired due to severe bone marrow depression, and two of them expired by tumor recurrences. In conclusion, immature teratoma in histological grade III showed high potentiality of recurrence. Therefore, postoperative chemotherapy has to be applied to the high graded immature tumors.

• Advances in pediatric surgery
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• v.1 no.2
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• pp.122-132
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• 1995

Teratoma contains elements derived from all three embryonic germ layers and mature teratomas are entirely composed of mature differentiated tissues, while immature types always contain additional embryonic tissues of variable degree of immaturity, especially neuroepithelial elements. Twenty cases of teratoma in infancy and childhood were reviewed and the clinical features and pathologic findings including PCNA expression were studied. Sacrococcygeal teratomas were the most common type(45%), followed by retroperitoneal and ovarian teratomas. There was a predilection of females in a ratio of 4:1 and age distribution was most prevalent below the first year of age(45%). But in ovarian teratomas, the age at diagnosis was above 4 years of age in all cases. Serum alpha-fetoprotein levels were checked in 18 cases. In all mature teratomas and 1 of 5 immature teratomas, the levels were normal. But in 4 of 5 immature teratomas, the serum levels were elevated and progressively declined to normal range after mass excision. Radiologically, calcifications in tumor were found in 60.0% of teratomas and was higher in mature teratomas(69.2%) than immature teratomas(42.9%). Immunohistochemical staining for PCNA(proliferating cell nuclear antigen) was done in 16 cases and PCNA expression was higher in grade III immature teratomas than grade I and II. The operative modes were complete mass excisions. Five immature teratomas were treated with multiagent PEB(Bleomycin, Etoposide, Cisplatin) adjuvant chemotherapy, 3 tolerated well without significant complications, but in one case, severe bone marrow suppression was developed and died of sepsis. In conclusion, grade III immature teratoma showed higher PCNA expression than mature or lower grade immature teratoma, which suggests that chemotherapy after surgical excision may be effective modality for grade III immature teratoma. We think, however, multicenter study is necessary because of low incidence of immature teratoma.

• Advances in pediatric surgery
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• v.13 no.2
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• pp.169-178
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• 2007

Teratomas arising from totipotential primitive germ cell are composed of 2 or 3 germ cell layers. We reviewed the records of 166 children who underwent the operation for teratoma from Jan, 1990 through April, 2007. There were 40 boys and 126 girls (average age $6.93{\pm}5.83$ years). Primary sites were ovary (n = 88), sacrococcygeum (n = 24), testis (n = 16), retroperitoneum (n = 16), mediastinum (n = 8), brain (n = 4), thymus (n = 3) and a single tumor involved the adrenal gland, neck, middle ear, oropharynx, stomach, pelvis, omentum, chest wall and scalp. Teratomas were mature in 141 patients, and immature in 25. Six patients who had ovarian immature teratomas grade 2 or 3 with peritoneal gliomatosis underwent adjuvant chemotherapy. During follow up period, 6 mature teratomas recurred at sacrococcygeal area (n = 1) and contralateral ovary (n = 5). Five patients of them underwent reoperation and diagnosed as a mature teratoma, but one who had underwent a left salpingooophrectomy with right ovary cystectomy at initial operation was observed carefully. Teratomas were dominant in female patients and developed at various organs. Complete excision was needed for good prognosis. In case of immature teratoma, complete excision and appropriate chemotherapy according to grading can contribute to favorable results.

• Korean Journal of Cleft Lip And Palate
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• v.11 no.2
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• pp.49-58
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• 2008

The development of fetal surgery has led to promising options for many congenital malformations, such as congenital diaphragmatic hernia (CDH), obstructive uropathy, twin-to-twin transfusion syndrome (TTTS), and sacrococcygeal teratoma. However, preterm labor (PTL) and premature rupture of membranes continue to be uniquitous risks for both mother and fetus. To reduce maternal morbidity and the risk of prematurity, minimal access techniques were developed and are increasingly employed recently. Lift-threatening diseases as well as severely disabling but not life-threatening conditions are potentially amenable to treatment. Recently, improvement of video-endoscopic technology has boosted the development of operative techniques for feto-endoscopic surgery, which has been demonstrated to be less invasive than the open approach. Fetal surgery for repair of cleft lip and palate, a congenital anomaly which is not life threatening, is inappropriate until such time that the benefits are shown to outweigh the risks of both the procedure itself and preterm delivery. Further animal studies will be needed before intrauterine surgery for humans should be considered. For the better understanding of recent techniques and complications associated with fetal intervention of congenital facial defect patients, we reviewed recent related articles about the current knowledge and new perspectives of experimental fetal fetal surgery in the cleft lip and palate defects.