• Journal of Korean Neurosurgical Society
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• 제64권3호
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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.

• Advances in pediatric surgery
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• 제19권1호
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• pp.25-31
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• 2013

성선 외 생식세포종양은 비교적 드물어 모든 생식세포종양의 5% 미만을 차지한다. 주로 천미부생식세포종양이 골반부나 후복막으로 확장된 경우가 대부분이며, 후복막이나 복강 내에 발생하는 생식세포종양은 매우 드물다. 저자는 복부 종괴를 주소로 내원한 18개월 된 여아에서 간 전이를 동반한 복강 내 혼합 생식세포종양을 치료하였다. 복부 초음파 및 전산화 단층촬영에서 복부 좌상사분역에 석회화, 고형 및 지방 성분을 포함하고 있는 낭성 종괴와 간에 1cm 크기의 결절이 관찰되었다. 종괴는 개복 후 제거되었으며, 병리조직학적으로 대부분의 내배엽동 종양과 적은 성숙 기형종으로 구성된 혼합 생식세포종양으로 진단되었으며, 간의 전이병변도 동일하게 진단되었다. 수술 후 항암화학요법을 시행하였으며, 환자는 현재 수술 후 10년이 경과하였으나 재발의 소견은 없다.

• Investigative Magnetic Resonance Imaging
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• 제23권4호
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• pp.367-373
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• 2019

Yolk sac tumors are rare malignant germ cell neoplasms that usually arise from the gonads. Extragonadal yolk sac tumors (EGYSTs) frequently occur in the mediastinum in post-pubertal females. EGYSTs in the pelvis are extremely rare, and to date, only thirteen cases have been reported in the English literature. Among them, the primary EGYST of the pelvic peritoneum in post-pubertal females has only been reported in ten cases. The present case describes a 26-year-old female diagnosed with primary peritoneal yolk sac tumor located in the rectouterine pouch. We report clinical and tumor imaging features, including ultrasound, computed tomography (CT), magnetic resonance images (MRI), positron emission tomography-computed tomography (PET-CT), and present a review of the literature.

• Asian Pacific Journal of Cancer Prevention
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• 제15권2호
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• pp.831-835
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• 2014

Background: Predictor factors determining complete response to treatment are still not clearly defined. We aimed to evaluate clinicopathological features, risk factors, treatment responses, and survival analysis of patient with advanced nonseminomatous GCTs (NSGCTs). Materials and Methods: Between November 1999 and September 2011, 140 patients with stage II and III NSGCTs were referred to our institutions and 125 patients with complete clinical data were included in this retrospective study. Four cycles of BEP regimen were applied as a first-line treatment. Salvage chemotherapy and/or high-dose chemotherapy (HDCT) with autologous stem cell transplantation were given in patients who progressed after BEP chemotherapy. Post-chemotherapy surgery was performed in selected patients with incomplete radiographic response and normal tumor markers. Results: The median age was 28 years. For the good, intermediate and poor risk groups, compete response rates (CRR) were, 84.6%, 67.9% and 59.4%, respectively. Extragonadal tumors, stage 3 disease, intermediate and poor risk factors, rete testis invasion were associated with worse outcomes. There were 32 patients (25.6%) with non-CR who were treated with salvage treatment. Thirty-one patients died from GCTs and 94% of them had stage III disease. Conclusions: Even though response rates are high, some patients with GCTs still need salvage treatment and cure cannot be achieved. Non-complete response to platinium-based first-line treatment is a negative prognostic factor. Our study confirmed the need for a prognostic and predictive model and more effective salvage approaches.

• Clinical and Experimental Pediatrics
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• 제58권10호
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• pp.386-391
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• 2015

Purpose: To evaluate the outcomes and prognostic factors in children with extracranial germ cell tumors (GCTs) treated at a single institution. Methods: Sixty-six children diagnosed with extracranial GCTs between 1996 and 2012 were included in the study. Primary treatment was surgical excision, followed by six cycles of cisplatin-based chemotherapy. The survival rates were compared according to the International Germ Cell Cancer Cooperative Group classification used for GCTs in adults to validate the classification guidelines for GCTs in children. Results: The median patient age was 4.4 years. In 34 patients (51.5%), the primary tumor site was the gonad. Extragonadal GCTs were detected in 32 patients. The 5-year overall survival and event-free survival (EFS) were $92.0%{\pm}3.5%$ and $90.4%{\pm}3.7%$, respectively. In univariate analysis, tumor histology, metastasis, and elevated alpha-fetoprotein were not prognostic factors in children with extracranial GCTs. However, EFS was poorer in patients with mediastinal disease (n=12, $66.7%{\pm}13.6%$) than in those with nonmediastinal disease (n=54, $96.0%{\pm}2.8%$) (P=0.001). The 5-year EFS was lower in patients older than 10 years, (n=21, $80.0%{\pm}8.9%$) compared with those younger than 10 years (n=45, $95.2%{\pm}3.3%$) (P=0.04). Multivariate analysis identified the mediastinal tumor site as the only independent prognostic factor. Conclusion: The prognosis of children with extracranial GCTs was favorable. However, nongerminomatous mediastinal tumors were associated with poor survival in children. Further research is needed to improve the prognosis of children with malignant mediastinal GCTs.

• Archives of Plastic Surgery
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• 제45권6호
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• pp.578-582
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• 2018

Teratomas can occur in almost any region of the body and are the most common extragonadal germ cell childhood tumors. However, craniofacial teratomas are rare. Craniofacial teratomas can present unique features and cause significant functional and aesthetic concerns. There are complex lesions that can have components intra-cranially and extra-cranially. Therefore, their management requires significant multi-stage multidisciplinary surgical procedures. Herein, we present a case of craniofacial teratoma in a child with the phenotype of proboscis lateralis that highlights some of the pertinent point of the diagnosis and management of congenital neonatal teratomas.