Pediatric Surgery International

, Volume 23, Issue 4, pp 315–322

Mature and immature teratomas: results of the first paediatric Italian study

  • Margherita Lo Curto
  • Paolo D’Angelo
  • Giovanni Cecchetto
  • Catherine Klersy
  • Patrizia Dall’Igna
  • Antonia Federico
  • Fortunato Siracusa
  • Rita Alaggio
  • Gabriella Bernini
  • Massimo Conte
  • Tina De Laurentis
  • Andrea Di Cataldo
  • Alessandro Inserra
  • Nicola Santoro
  • Paolo Tamaro
  • Paolo Indolfi
Original Article


Teratoma is the most common germ cell tumour in childhood; mature (MT) and immature teratomas (IT) are benign tumours, but if they recur, they can be in some cases malignant. The aim of this paper is to evaluate Italian patients with MT and IT enrolled from 1991 to 2001, in a prospective multicentric study. One hundred and eighty-three patients, observed in 15 Italian Centers of Paediatric Oncology and three Paediatric Surgical Units were enrolled. Clinical data, treatment and results were all analysed. Initial evaluation and subsequent follow up included clinical examination, tumour markers and imaging procedures. Surgical resection was recommended for all the tumours. Histology was centrally reviewed and IT was classified as grading 1–3. Chemotherapy (CT) with Vinblastine, D-actinomycin and cyclophosphamide was indicated for extra-testicular IT grade 2 or 3. MT was diagnosed in 127 patients (93 F and 34 M, age 1–192 months, median 24): 58 patients had gonadic tumour (23 testicular, 35 ovaric), 69 extragonadic (45 sacrococcygeal, 11 mediastinic, 7 retroperitoneal, 6 in other sites). A complete resection was performed in 117 patients, a partial resection in eight patients and biopsy in one. IT was diagnosed in 56 patients (34 F, 22 M, age 1–168 months, median 7). The T grading was 1 in 14 cases, 2 in 26, 3 in 16; 28 had gonadic T (17 ovary, 11 testis), 28 extragonadic (sacrococcygeal 19, mediastinic 3, retroperitoneal 2, other sites 4). CT was administered in eight patients; 15/182 patients relapsed (1 in a metastatic site) and in 5/15 the relapse showed malignant histology. Seven MT (5.5%) relapsed (five sacrococcygeal, one retroperitoneal, one mediastinic): surgery at diagnosis had been complete in five and with residual in two; the relapse was malignant in two patients with sacrococcygeal (sc) tumours, who had a complete resection and a partial resection respectively. Eight IT (14.2%) relapsed (four ovary, three sc, one retroperitoneal). The initial surgical resection had been complete in one, with residual in six, and a biopsy had been performed in one. A malignant recurrence occurred in two patients with sc tumours (after partial resection in one and after biopsy + CT in one) and in one patient with ovarian IT after a partial resection. All the patients underwent surgical excision of the recurred mass; CT according to Protocol for Malignant GCT was administered to those who had malignant recurrence; 122/126 patients with MT and 53/56 with IT are alive without disease with a follow up of 8–144 months (median 56). Two patients with malignant relapse (one with sc MT, one with sc IT) died because of the progression of the disease. Another two died due to severe malformations (one MT, one IT) and three were lost to follow up (two MT, one IT). The overall survival (OS) at 10 years is 98% (95% CI 93.9–99.4); the event free survival (EFS) is 90.4% (95 CI 84.8–94.0). At Cox analysis no significant difference in EFS was found regarding age and site of the primary tumour, while females (P = 0.011), patients with grade 1–3 histology (P = 0.025) and patients with incomplete resection appeared at higher risk of death or relapse (P < 0.001), with a seven, three and eightfold increase in risk, respectively. Our data showed that incomplete resection and female gender are important risk factors for relapse or death, more so than IT histology. The number of patients treated with CT is not sufficient to evaluate the efficacy of CT in avoiding malignant relapse.


Mature teratoma Immature teratoma Childhood tumours Treatment and outcome 


  1. 1.
    Norris HJ, Zirken HI, Benson WL (1976) Immature teratoma of the ovary: a clinical and pathologic study of 58 cases. Cancer 37:2359–2372PubMedCrossRefGoogle Scholar
  2. 2.
    Gonzales-Crussi (1982) Extragonadal teratoma. Atlas of tumor pathology, 2nd serie, fascicle 18. Armed Forces Inst of Pathology I, Washington, 44–129Google Scholar
  3. 3.
    Mostofi FK, Sobin LH (1973) Histologic typing of testis tumors. World Health Organisation, GeneveGoogle Scholar
  4. 4.
    Lo Curto M, Lumia F, Alaggio R et al (2003) Malignant germ cell tumors in childhood: results of the first Italian cooperative study “TCG 91”. Med Pediatr Oncol 41:417–425PubMedCrossRefGoogle Scholar
  5. 5.
    Cushing B, Giller R, Ablin A et al (1999) Surgical resection alone is effective treatment for ovarian immature teratoma in children and adolescents: a report of the pediatric oncology group and the children’s cancer group. Am J Obstet Gynecol 181:353–358PubMedCrossRefGoogle Scholar
  6. 6.
    Schropp KP, Lobe TE, Rao B et al (1992) Sacrococcygeal teratoma: the experience of four decades. J Pediatr Surg 27:1075–1078PubMedCrossRefGoogle Scholar
  7. 7.
    Mann JR, Raafat F, Robinson K et al (1998) Mature and immature extracranial teratomas in children’s cancer study group’s experience. In: Jones WG, Appleyard I, Harnden P, Joffe JK (eds) Germ cell tumours IV. John Libbey, pp 237–246Google Scholar
  8. 8.
    Lo Curto M, Zingone A, Provenzano G et al (1989) Teratomi infantili: osservazioni su 87 casi. Riv Ital Ped (IJP) 15:268–274Google Scholar
  9. 9.
    Altman RP, Randolph JG, Lilly JR (1974) Sacrococcygeal teratoma American academy of pediatric surgical section survey. J Pediatr Surg 9:389–398PubMedCrossRefGoogle Scholar
  10. 10.
    Marina NM, Cushing B, Giller R, et al (1999) Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: a pediatric oncology group/children’s cancer group intergroup study. J Clin Oncol 17:2137–2143PubMedGoogle Scholar
  11. 11.
    Gobel U, Calaminus G, Engert J et al (1998) Teratomas infancy and childhood. Med Pediatr Oncol 31:8–15PubMedCrossRefGoogle Scholar
  12. 12.
    Schneider DT, Calaminus G, Koch S et al (2004) Epidemiologic analysis of 1442 children and adolescents registered in the German germ cell tumor protocols. Pediatr Blood Cancer 42:169–175PubMedCrossRefGoogle Scholar
  13. 13.
    Asheraft KW, Holder TM (1965) Congenital anal stenosis with presacral teratoma. Ann Surg 162:1091–1095CrossRefGoogle Scholar
  14. 14.
    Currarino G, Coln D, Votteler T (1981) Trial of anorectal, sacral and presacral anomalies. Am J Roentgenol 137:395–398Google Scholar
  15. 15.
    Rescorla FJ, Sawin RS, Coran AG et al (1998) Long-term outcome for infants and children with sacrococcygeal teratoma: a report from the childrens cancer group. J Pediatr Surg 33:171–176PubMedCrossRefGoogle Scholar
  16. 16.
    Gobel U, Calaminus G, Waag KL et al (1998) Extracranial non-testicular teratoma in childhood and adolescence: the German experience. In Jones WG, Appleyard I, Harnden P, Joffe JK (eds) Germ cell tumours IV. John Libbey, pp 233–236Google Scholar
  17. 17.
    Rescorla F, Billmire D, Stolar C et al (2001) The effect of cisplatinum dose and surgical resection in children with malignant germ cell tumors at the sacrococcygeal region: a pediatric intergroup trial (POG9049/CCG 8882). J Pediatr Surg 36:12–17PubMedCrossRefGoogle Scholar
  18. 18.
    Dewan PA, Davidson PM, Campbell PE et al (1987) Sacrococcygeal teratoma: has chemotherapy improved survival? J Pediatr Surg 22:274–277PubMedCrossRefGoogle Scholar
  19. 19.
    Dark CG, Bower M, Newlands ES (1997) Surveillance policy for stage I ovarian germ cell tumors. J Clin Oncol 15:620–624PubMedGoogle Scholar
  20. 20.
    Flamant F, Baranzelli MC, Kalifa C et al (1990) Treatment of malignant germ cell tumors in children: experience of the Institut gustave roussy and the French society of pediatric oncology. Crit Rev Oncol Hematol 10:99–110PubMedGoogle Scholar
  21. 21.
    Baranzelli MC, Flamant F, De Lumley L et al (1993) Treatment of non-metastatic, non-seminomatous malignant germ cell tumors in childood: experience of the SFOP MGCT 1985–1989 study. Med Pediatr Oncol 21:395–401PubMedCrossRefGoogle Scholar
  22. 22.
    Gobel U, Haas G, Calaminus G et al (1990) Treatment of the germ cell tumors in children: results of the European trials for testicular and non testicular primary sites. Crit Rev Oncol Hematol 10:89–98PubMedGoogle Scholar
  23. 23.
    Mann JR, Pearson D, Barrett A et al (1989) Results of the United Kingdoom children cancer study study group’s malignant germ cell tumors studies. Cancer 63:1657–1667PubMedCrossRefGoogle Scholar
  24. 24.
    Gobel U, Calaminus G, Blohm M et al (1997) Extracranial non-testicular teratoma in childhood and adolescence: introduction of a risk score for stratification of therapy. Klin Padiatr 209:228–234PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2007

Authors and Affiliations

  • Margherita Lo Curto
    • 1
    • 15
  • Paolo D’Angelo
    • 2
  • Giovanni Cecchetto
    • 3
  • Catherine Klersy
    • 4
  • Patrizia Dall’Igna
    • 3
  • Antonia Federico
    • 1
  • Fortunato Siracusa
    • 5
  • Rita Alaggio
    • 6
  • Gabriella Bernini
    • 7
  • Massimo Conte
    • 8
  • Tina De Laurentis
    • 9
  • Andrea Di Cataldo
    • 10
  • Alessandro Inserra
    • 11
  • Nicola Santoro
    • 12
  • Paolo Tamaro
    • 13
  • Paolo Indolfi
    • 14
  1. 1.Paediatric DepartmentUniversity of PalermoPalermoItaly
  2. 2.Unit of Paediatric Hematology and Oncology“G. Di Cristina” Children HospitalPalermoItaly
  3. 3.Paediatric Surgery DepartmentUniversity of PaduaPaduaItaly
  4. 4.Biometry and Clinical EpidemiologyIRCCS San Matteo HospitalPaviaItaly
  5. 5.Paediatric Surgery DepartmentUniversity of PalermoPalermoItaly
  6. 6.Section of Pathology, Department of Oncologic SciencesUniversity of PaduaPaduaItaly
  7. 7.Unit of Paediatric OncologyMeyer HospitalFlorenceItaly
  8. 8.Unit of Paediatric OncologyGaslini InstituteGenoaItaly
  9. 9.Unit of Paediatric Oncology“Bambin Gesù” HospitalRomeItaly
  10. 10.Department of Paediatric Hematology and OncologyUniversity of CataniaCataniaItaly
  11. 11.Paediatric Surgery Department“Bambin Gesù” Children HospitalRomeItaly
  12. 12.Department of Paediatric Hematology and OncologyUniversity of BariBariItaly
  13. 13.Paediatric Hematology and Oncology DepartmentUniversity of TriesteTriesteItaly
  14. 14.Paediatric Hematology and Oncology DepartmentUniversity of NaplesNaplesItaly
  15. 15.Dipartimento di PediatriaIstituto Materno-InfantilePalermoItaly

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