TY - JOUR
T1 - Malignant transformation and tumour recurrence in sacrococcygeal teratoma
T2 - a global, retrospective cohort study
AU - Van Heurn, Lieke J.
AU - Derikx, Joep P.M.
AU - Hall, Nigel
AU - Aldrink, Jennifer H.
AU - Bailez, Maria M.
AU - Chirdan, Lohfa B.
AU - Fumino, Shigehisa
AU - Hesse, Afua
AU - Soyer, Tuktu
AU - StPeter, Shawn
AU - Twisk, Jos
AU - Yang, Tianyou
AU - Van Heurn, Ernst L.W.
AU - Ignacio, Siffredi Juan
AU - Lobos, Pablo
AU - Till, Holger
AU - Mitul, Ashrarur Rahman
AU - Govorukhina, Olga
AU - Prokopenya, Natalya
AU - De Backer, Antoine
AU - Reusens, Helena
AU - De Campos Vieira Abib, Simone
AU - Stefanova-Peeva, Penka Peneva
AU - Tolekova, Nadezhda
AU - Faustin, Mouafo Tambo
AU - Laberge, Jean Martin
AU - Zani, Augusto
AU - Walker, Richard J.B.
AU - Pérez, Maricarmen Olivos
AU - Valenzuela, Marco Andrés
AU - Shen, Yuanchao
AU - Zou, Yan
AU - Cavar, Stanko
AU - Pogorelic, Zenon
AU - Pos, Lucie
AU - Skaba, Richard
AU - Jørgensen, Peter Hjorth
AU - AbouZeid, Amr Abdelhamid
AU - Elfiky, Mahmoud
AU - Taher, Heba
AU - Märtson, Matis
AU - Derbew, Miliard
AU - Tigabie, Workye Molla
AU - Koivusalo, Antti
AU - Pakarinen, Mikko
AU - Abbo, Olivier
AU - Arnaud, Alexis Pierre Emmanuel
AU - Ballouhey, Quentin
AU - Bastard, Francois
AU - Dariel, Anne
AU - SCT-study consortium
AU - van Gemert, Wim
AU - Kremer, Marijke
N1 - Funding Information:
This study received funding from Kika Children Cancer-free Foundation. Kika Children Cancer-free Foundation had no involvement in collection, analysis and interpretation of data or in writing and decision to submit the manuscript for publication. The authors thank the EUPSA Network Office for their help in raising awareness for the study and contacting centres for possible participation. We would like to thank Mikhail Povarov for helping with the Russian translation of the study documentation.
Publisher Copyright:
Copyright © 2024 The Author(s).
PY - 2024/1/1
Y1 - 2024/1/1
N2 - Introduction: Sacrococcygeal teratoma (SCT) is a rare congenital tumour. The risk of malignancy and recurrence is not well defined. Previous studies are small and report differing conclusions about the timing of surgery and the duration of follow-up. The authors studied the risk of malignant transformation and SCT recurrence after surgery to address these gaps. Methods: This was a global retrospective cohort study. Data of consecutive SCT patients was obtained from 145 institutes in 62 countries. Malignant transformation, defined as malignancy at initial resection, malignant recurrence or death due to malignancy, and its risk factors were analysed. Results: Of the 3612 included patients, 3407 entered analysis. The risk of malignant transformation of the initial tumour was 3.3, 5.1, 10.1, and 32.9% at age 3 months, 6 months, 1 year, and 2 years, respectively. After 6 years, the censored risk of malignancy (64%) did not further increase. Recurrent SCT was diagnosed in 349 (10.2%) children with 126 (36.1%) malignant recurrences. Risk factors for recurrence were Altman type II [odds ratio (OR): 1.6, 95% confidence interval (CI): 1.2–2.2], Altman type III (OR: 1.6, 95% CI: 1.2–2.3), initial immature histology (OR: 1.9, 95% CI: 1.4–2.6), and initial malignant histology (OR: 4.0, 95% CI: 2.9–5.4). Conclusion: The risk of malignancy at initial resection in SCT increases with age reaching a plateau at 6 years of age. Recurrence after resection occurred in 10% of patients and 36% of these were malignant at that time. Altman type II or type III, and immature or malignant histology were associated with recurrence.
AB - Introduction: Sacrococcygeal teratoma (SCT) is a rare congenital tumour. The risk of malignancy and recurrence is not well defined. Previous studies are small and report differing conclusions about the timing of surgery and the duration of follow-up. The authors studied the risk of malignant transformation and SCT recurrence after surgery to address these gaps. Methods: This was a global retrospective cohort study. Data of consecutive SCT patients was obtained from 145 institutes in 62 countries. Malignant transformation, defined as malignancy at initial resection, malignant recurrence or death due to malignancy, and its risk factors were analysed. Results: Of the 3612 included patients, 3407 entered analysis. The risk of malignant transformation of the initial tumour was 3.3, 5.1, 10.1, and 32.9% at age 3 months, 6 months, 1 year, and 2 years, respectively. After 6 years, the censored risk of malignancy (64%) did not further increase. Recurrent SCT was diagnosed in 349 (10.2%) children with 126 (36.1%) malignant recurrences. Risk factors for recurrence were Altman type II [odds ratio (OR): 1.6, 95% confidence interval (CI): 1.2–2.2], Altman type III (OR: 1.6, 95% CI: 1.2–2.3), initial immature histology (OR: 1.9, 95% CI: 1.4–2.6), and initial malignant histology (OR: 4.0, 95% CI: 2.9–5.4). Conclusion: The risk of malignancy at initial resection in SCT increases with age reaching a plateau at 6 years of age. Recurrence after resection occurred in 10% of patients and 36% of these were malignant at that time. Altman type II or type III, and immature or malignant histology were associated with recurrence.
KW - malignant transformation
KW - recurrence
KW - sacrococcygeal teratoma
U2 - 10.1097/JS9.0000000000002045
DO - 10.1097/JS9.0000000000002045
M3 - Article
SN - 1743-9191
VL - 110
SP - 7177
EP - 7186
JO - International Journal of Surgery
JF - International Journal of Surgery
IS - 11
ER -