Shigehisa Fumino1, Tatsuro Tajiri1, Noriaki Usui2, Masanori Tamura3, Haruhiko Sago4, Shigeru Ono5, Shunsuke Nosaka6, Akihiro Yoneda7, Ryota Souzaki8, Mayumi Higashi1, Kohei Sakai1, Ken Takahashi9, Takahiro Sugiura10, Tomoaki Taguchi8. 1. Department of Pediatric Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan. 2. Department of Pediatric Surgery, Osaka Women's and Children's Hospital, Osaka, Japan. 3. Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Saitama, Japan. 4. Department of Maternal-Fetal and Neonatal Medicine, National Center for Child Health and Development, Tokyo, Japan. 5. Department of Pediatric Surgery, Jichi Children's Medical Center Tochigi, Jichi Medical University, Tochigi, Japan. 6. Division of Radiology, National Center for Child Health and Development, Tokyo, Japan. 7. Department of Pediatric Surgery, Osaka City General Hospital, Osaka, Japan. 8. Department of Pediatric Surgery, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. 9. Department of Obstetrics and Gynecology, Jikei University School of Medicine, Tokyo, Japan. 10. Department of Pediatrics, Toyohashi Municipal Hospital, Aichi, Japan.
Abstract
BACKGROUND: Sacrococcygeal teratoma (SCT) is the most common extragonadal germ cell tumor in neonates and infants. Although most cases of infantile SCT are benign tumors by nature, some develop into extremely large lesions, leading to massive bleeding, high-output heart failure, disseminated intravascular coagulation, and even fatal outcomes during the neonatal period. In addition, some patients may present with tumor recurrence, malignant transformation, long-term sequelae (including bladder and bowel dysfunction) and lower leg palsy during the long-term follow up. SCT, however, is very rare, and there are few opportunities to encounter this disease, therefore general physicians without expert credentials currently lack information relevant to clinical practice. For this reason, the research project committee has compiled guidelines concerning SCT. METHODS: The purpose of these guidelines was to share information concerning the treatment and follow up of infantile SCT. The guidelines were developed using the methodologies in the Medical Information Network Distribution System. A comprehensive search of the English- and Japanese-language articles in PubMed and Ichu-Shi Web identified only case reports or case series, and the recommendations were developed through a process of informal consensus. RESULTS: The clinical questions addressed the risk factors, the efficacy of cesarean section, the initial devascularization of tumor feeding vessels, interventional radiology, recommended clinical studies for follow up and possible long-term complications. CONCLUSIONS: These are the first guidelines for SCT to be established in Japan, and they may have huge clinical value and significance in terms of developing therapeutic strategies and follow up, potentially contributing to the improvement of the prognosis and quality of life of SCT patients.
BACKGROUND:Sacrococcygeal teratoma (SCT) is the most common extragonadal germ cell tumor in neonates and infants. Although most cases of infantile SCT are benign tumors by nature, some develop into extremely large lesions, leading to massive bleeding, high-output heart failure, disseminated intravascular coagulation, and even fatal outcomes during the neonatal period. In addition, some patients may present with tumor recurrence, malignant transformation, long-term sequelae (including bladder and bowel dysfunction) and lower leg palsy during the long-term follow up. SCT, however, is very rare, and there are few opportunities to encounter this disease, therefore general physicians without expert credentials currently lack information relevant to clinical practice. For this reason, the research project committee has compiled guidelines concerning SCT. METHODS: The purpose of these guidelines was to share information concerning the treatment and follow up of infantile SCT. The guidelines were developed using the methodologies in the Medical Information Network Distribution System. A comprehensive search of the English- and Japanese-language articles in PubMed and Ichu-Shi Web identified only case reports or case series, and the recommendations were developed through a process of informal consensus. RESULTS: The clinical questions addressed the risk factors, the efficacy of cesarean section, the initial devascularization of tumor feeding vessels, interventional radiology, recommended clinical studies for follow up and possible long-term complications. CONCLUSIONS: These are the first guidelines for SCT to be established in Japan, and they may have huge clinical value and significance in terms of developing therapeutic strategies and follow up, potentially contributing to the improvement of the prognosis and quality of life of SCT patients.
Authors: Lieke J van Heurn; Audrey B C Coumans; Joep P M Derikx; Mireille N Bekker; Katia M Bilardo; Leonie K Duin; Maarten F C M Knapen; Eva Pajkrt; Esther Sikkel; L W Ernest van Heurn; Dick Oepkes Journal: Prenat Diagn Date: 2021-08-05 Impact factor: 3.242