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Sacrococcygeal Teratoma : A Tumor at the Center of Embryogenesis

  • Phi, Ji Hoon (Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine)
  • Received : 2021.01.20
  • Accepted : 2021.03.10
  • Published : 2021.05.01

Abstract

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.

Keywords

References

  1. Altman RP, Randolph JG, Lilly JR : Sacrococcygeal teratoma: American academy of pediatrics surgical section survey-1973. J Pediatr Surg 9 : 389-398, 1974 https://doi.org/10.1016/S0022-3468(74)80297-6
  2. Anteby EY, Yagel S : Route of delivery of fetuses with structural anomalies. Eur J Obstet Gynecol Reprod Biol 106 : 5-9, 2003 https://doi.org/10.1016/s0301-2115(02)00033-7
  3. Bader D, Riskin A, Vafsi O, Tamir A, Peskin B, Israel N, et al. : Alphafetoprotein in the early neonatal period--a large study and review of the literature. Clin Chim Acta 349 : 15-23, 2004 https://doi.org/10.1016/j.cccn.2004.06.020
  4. Barksdale EM Jr, Obokhare I : Teratomas in infants and children. Curr Opin Pediatr 21 : 344-349, 2009 https://doi.org/10.1097/MOP.0b013e32832b41ee
  5. Cowles RA, Stolar CJ, Kandel JJ, Weintraub JL, Susman J, Spigland NA : Preoperative angiography with embolization and radiofrequency ablation as novel adjuncts to safe surgical resection of a large, vascular sacrococcygeal teratoma. Pediatr Surg Int 22 : 554-556, 2006 https://doi.org/10.1007/s00383-006-1650-7
  6. Damjanov I, Solter D, Belicza M, Skreb N : Teratomas obtained through extrauterine growth of seven-day mouse embryos. J Natl Cancer Inst 46 : 471-475, 1971
  7. Derikx JP, De Backer A, van de Schoot L, Aronson DC, de Langen ZJ, van den Hoonaard TL, et al. : Long-term functional sequelae of sacrococcygeal teratoma: a national study in the Netherlands. J Pediatr Surg 42 : 1122-1126, 2007 https://doi.org/10.1016/j.jpedsurg.2007.01.050
  8. Ferlay J, Soerjomataram I, Dikshit R, Eser S, Mathers C, Rebelo M, et al. : Cancer incidence and mortality worldwide: sources, methods and major patterns in GLOBOCAN 2012. Int J Cancer 136 : E359-E386, 2015 https://doi.org/10.1002/ijc.29210
  9. Fumino S, Tajiri T, Usui N, Tamura M, Sago H, Ono S, et al. : Japanese clinical practice guidelines for sacrococcygeal teratoma, 2017. Pediatr Int 61 : 672-678, 2019 https://doi.org/10.1111/ped.13844
  10. Gabra HO, Jesudason EC, McDowell HP, Pizer BL, Losty PD : Sacrococcygeal teratoma--a 25-year experience in a UK regional center. J Pediatr Surg 41 : 1513-1516, 2006 https://doi.org/10.1016/j.jpedsurg.2006.05.019
  11. Gross SJ, Benzie RJ, Sermer M, Skidmore MB, Wilson SR : Sacrococcygeal teratoma: prenatal diagnosis and management. Am J Obstet Gynecol 156 : 393-396, 1987 https://doi.org/10.1016/0002-9378(87)90290-0
  12. Hedrick HL, Flake AW, Crombleholme TM, Howell LJ, Johnson MP, Wilson RD, et al. : Sacrococcygeal teratoma: prenatal assessment, fetal intervention, and outcome. J Pediatr Surg 39 : 430-438, 2004 https://doi.org/10.1016/j.jpedsurg.2003.11.005
  13. Izant RJ Jr, Filston HC : Sacrococcygeal teratomas. Analysis of forty-three cases. Am J Surg 130 : 617-621, 1975 https://doi.org/10.1016/0002-9610(75)90523-1
  14. Keslar PJ, Buck JL, Suarez ES : Germ cell tumors of the sacrococcygeal region: radiologic-pathologic correlation. Radiographics 14 : 607-620; quiz 621-622, 1994 https://doi.org/10.1148/radiographics.14.3.8066275
  15. Kochling J, Pistor G, Marzhauser Brands S, Nasir R, Lanksch WR : The Currarino syndrome--hereditary transmitted syndrome of anorectal, sacral and presacral anomalies. Case report and review of the literature. Eur J Pediatr Surg 6 : 114-119, 1996 https://doi.org/10.1055/s-2008-1066487
  16. Kratz CP, Mai PL, Greene MH : Familial testicular germ cell tumours. Best Pract Res Clin Endocrinol Metab 24 : 503-513, 2010 https://doi.org/10.1016/j.beem.2010.01.005
  17. Kremer MEB, Althof JF, Derikx JPM, van Baren R, Heij HA, Wijnen MHWA, et al. : The incidence of associated abnormalities in patients with sacrococcygeal teratoma. J Pediatr Surg 53 : 1918-1922, 2018 https://doi.org/10.1016/j.jpedsurg.2018.01.013
  18. Lahdes-Vasama TT, Korhonen PH, Seppanen JM, Tammela OK, Iber T : Preoperative embolization of giant sacrococcygeal teratoma in a premature newborn. J Pediatr Surg 46 : e5-e8, 2011
  19. Litchfield K, Summersgill B, Yost S, Sultana R, Labreche K, Dudakia D, et al. : Whole-exome sequencing reveals the mutational spectrum of testicular germ cell tumours. Nat Commun 6 : 5973, 2015 https://doi.org/10.1038/ncomms6973
  20. Lobo J, Gillis AJM, Jeronimo C, Henrique R, Looijenga LHJ : Human germ cell tumors are developmental cancers: impact of epigenetics on pathobiology and clinic. Int J Mol Sci 20 : 258, 2019 https://doi.org/10.3390/ijms20020258
  21. Lynch SA, Wang Y, Strachan T, Burn J, Lindsay S : Autosomal dominant sacral agenesis: Currarino syndrome. J Med Genet 37 : 561-566, 2000 https://doi.org/10.1136/jmg.37.8.561
  22. Mamsen LS, Brochner CB, Byskov AG, Mollgard K : The migration and loss of human primordial germ stem cells from the hind gut epithelium towards the gonadal ridge. Int J Dev Biol 56 : 771-778, 2012 https://doi.org/10.1387/ijdb.120202lm
  23. Mann JR, Gray ES, Thornton C, Raafat F, Robinson K, Collins GS, et al. : Mature and immature extracranial teratomas in children: the UK Children's Cancer Study Group Experience. J Clin Oncol 26 : 3590-3597, 2008 https://doi.org/10.1200/JCO.2008.16.0622
  24. Masahata K, Ichikawa C, Makino K, Abe T, Kim K, Yamamichi T, et al. : Long-term functional outcome of sacrococcygeal teratoma after resection in neonates and infants: a single-center experience. Pediatr Surg Int 36 : 1327-1332, 2020 https://doi.org/10.1007/s00383-020-04752-7
  25. Monk D, Mackay DJG, Eggermann T, Maher ER, Riccio A : Genomic imprinting disorders: lessons on how genome, epigenome and environment interact. Nat Rev Genet 20 : 235-248, 2019 https://doi.org/10.1038/s41576-018-0092-0
  26. Murphy JJ, Blair GK, Fraser GC : Coagulopathy associated with large sacrococcygeal teratomas. J Pediatr Surg 27 : 1308-1310, 1992 https://doi.org/10.1016/0022-3468(92)90282-C
  27. Oosterhuis JW, Looijenga LHJ : Human germ cell tumours from a developmental perspective. Nat Rev Cancer 19 : 522-537, 2019 https://doi.org/10.1038/s41568-019-0178-9
  28. Oosterhuis JW, Stoop H, Honecker F, Looijenga LH : Why human extragonadal germ cell tumours occur in the midline of the body: old concepts, new perspectives. Int J Androl 30 : 256-263; discussion 263-264, 2007 https://doi.org/10.1111/j.1365-2605.2007.00793.x
  29. Padilla BE, Vu L, Lee H, MacKenzie T, Bratton B, O'Day M, et al. : Sacrococcygeal teratoma: late recurrence warrants long-term surveillance. Pediatr Surg Int 33 : 1189-1194, 2017 https://doi.org/10.1007/s00383-017-4132-1
  30. Partridge EA, Canning D, Long C, Peranteau WH, Hedrick HL, Adzick NS, et al. : Urologic and anorectal complications of sacrococcygeal teratomas: prenatal and postnatal predictors. J Pediatr Surg 49 : 139-142; discussion 142-143, 2014 https://doi.org/10.1016/j.jpedsurg.2013.09.042
  31. Pauniaho SL, Heikinheimo O, Vettenranta K, Salonen J, Stefanovic V, Ritvanen A, et al. : High prevalence of sacrococcygeal teratoma in Finland - a nationwide population-based study. Acta Paediatr 102 : e251-e256, 2013 https://doi.org/10.1111/apa.12211
  32. Phi JH, Wang KC, Kim SK : Intracranial germ cell tumor in the molecular era. J Korean Neurosurg Soc 61 : 333-342, 2018 https://doi.org/10.3340/jkns.2018.0056
  33. Poynter JN, Hooten AJ, Frazier AL, Ross JA : Associations between variants in KITLG, SPRY4, BAK1, and DMRT1 and pediatric germ cell tumors. Genes Chromosomes Cancer 51 : 266-271, 2012 https://doi.org/10.1002/gcc.20951
  34. Rattan KN, Singh J : Neonatal sacrococcygeal teratoma: our 20-year experience from a tertiary care centre in North India. Trop Doct : 49475520973616, 2020
  35. Rescorla FJ, Sawin RS, Coran AG, Dillon PW, Azizkhan RG : Long-term outcome for infants and children with sacrococcygeal teratoma: a report from the Childrens Cancer Group. J Pediatr Surg 33 : 171-176, 1998 https://doi.org/10.1016/S0022-3468(98)90426-2
  36. Robertson FM, Crombleholme TM, Frantz ID 3rd, Shephard BA, Bianchi DW, D'Alton ME : Devascularization and staged resection of giant sacrococcygeal teratoma in the premature infant. J Pediatr Surg 30 : 309-311, 1995 https://doi.org/10.1016/0022-3468(95)90579-0
  37. Sananes N, Javadian P, Schwach Werneck Britto I, Meyer N, Koch A, Gaudineau A, et al. : Technical aspects and effectiveness of percutaneous fetal therapies for large sacrococcygeal teratomas: cohort study and literature review. Ultrasound Obstet Gynecol 47 : 712-719, 2016 https://doi.org/10.1002/uog.14935
  38. Schneider DT, Wessalowski R, Calaminus G, Pape H, Bamberg M, Engert J, et al. : Treatment of recurrent malignant sacrococcygeal germ cell tumors: analysis of 22 patients registered in the german protocols MAKEI 83/86, 89, and 96. J Clin Oncol 19 : 1951-1960, 2001 https://doi.org/10.1200/jco.2001.19.7.1951
  39. Schropp KP, Lobe TE, Rao B, Mutabagani K, Kay GA, Gilchrist BF, et al. : Sacrococcygeal teratoma: the experience of four decades. J Pediatr Surg 27 : 1075-1078; discussion 1078-1079, 1992 https://doi.org/10.1016/0022-3468(92)90563-M
  40. Shen H, Shih J, Hollern DP, Wang L, Bowlby R, Tickoo SK, et al. : Integrated molecular characterization of testicular germ cell tumors. Cell Rep 23 : 3392-3406, 2018 https://doi.org/10.1016/j.celrep.2018.05.039
  41. Sobis H, Vandeputte M : Yolk sac-derived rat teratomas are not of germ cell origin. Dev Biol 51 : 320-323, 1976 https://doi.org/10.1016/0012-1606(76)90146-9
  42. Teilum G, Albrechtsen R, Norgaard-Pedersen B : The histogenetic-embryologic basis for reappearance of alpha-fetoprotein in endodermal sinus tumors (yolk sac tumors) and teratomas. Acta Pathol Microbiol Scand A 83 : 80-86, 1975
  43. van Heurn LJ, Knipscheer MM, Derikx JPM, van Heurn LWE : Diagnostic accuracy of serum alpha-fetoprotein levels in diagnosing recurrent sacrococcygeal teratoma: a systematic review. J Pediatr Surg 55 : 1732-1739, 2020 https://doi.org/10.1016/j.jpedsurg.2020.03.014
  44. Van Mieghem T, Al-Ibrahim A, Deprest J, Lewi L, Langer JC, Baud D, et al. : Minimally invasive therapy for fetal sacrococcygeal teratoma: case series and systematic review of the literature. Ultrasound Obstet Gynecol 43 : 611-619, 2014 https://doi.org/10.1002/uog.13315
  45. Yao W, Li K, Zheng S, Dong K, Xiao X : Analysis of recurrence risks for sacrococcygeal teratoma in children. J Pediatr Surg 49 : 1839-1842, 2014 https://doi.org/10.1016/j.jpedsurg.2014.09.036
  46. Yoneda A, Usui N, Taguchi T, Kitano Y, Sago H, Kanamori Y, et al. : Impact of the histological type on the prognosis of patients with prenatally diagnosed sacrococcygeal teratomas: the results of a nationwide Japanese survey. Pediatr Surg Int 29 : 1119-1125, 2013 https://doi.org/10.1007/s00383-013-3384-7
  47. Yoon HM, Byeon SJ, Hwang JY, Kim JR, Jung AY, Lee JS, et al. : Sacrococcygeal teratomas in newborns: a comprehensive review for the radiologists. Acta Radiol 59 : 236-246, 2018 https://doi.org/10.1177/0284185117710680
  48. Yoshida M, Tanaka M, Gomi K, Ohama Y, Kigasawa H, Iwanaka T, et al. : Malignant steroidogenic tumor arising from sacrococcygeal mature teratoma. Hum Pathol 42 : 1568-1572, 2011 https://doi.org/10.1016/j.humpath.2010.12.022