Prognostic factors in children with extracranial malignant germ cell tumors: a monocentric pediatric Tunisian study

##plugins.themes.academic_pro.article.main##

Faten Fedhila
Samar Rhayem
Habiba Hafsi
Wiem Douira
Raoudha Doghri
Monia Khemiri
Karima Mrad
Ibtissem Bellagha
Bechir Zouari
Sihem Barsaoui

Abstract

ABSTRACT:
Background: Extracranial Germ cell tumors (GCT) are a rare and a heterogeneous group of pediatric cancers but highly curable.
Aim: We aimed to review management, outcome and prognostic factors that influence overall survival (OS) in a pediatric Tunisian oncologic unit.
Methods: We retrospectively evaluated between January 1998 and December 2012, 33 patients affected by extracranial germ cell tumors and treated according to TGM95 protocol established by the SFOPin a pediatric Tunisian oncologic unit.
Results: Patients had a mean age of 57 months (ranges: 1 day-13 years). There were 19 girls and 14 boys. Primary sites included 12 sacrococcygeal, 11 ovarian, 6 testicular, 3retro peritoneal and 1 mediastinal site. After a mean follow up of 26.1 months (ranges: 0-96 months), OSat 2 years and 5 years were respectively 82% and 75%. Event-free survival were respectively 79% at 2 years and 74% at 5 years. Various prognostic factors have been studied according to Kaplan-Meier. Univariate analyses identified significant factors which influence strongly OS: the stage (p=0.04), the completeness of surgery (p<0.001) and the relapse (p = 0, 0001). A multivariate study showed that only the quality of resection and the clinical stage remained strong significant prognostic factors (p=0,021) for 5-year OS.
Conclusion: Disease stage, completeness of surgery and relapse have been established as the most powerful prognostic parameter in our analysis. The improvement of survival of patients affected by extracranial germ cell tumors in Tunisia is a real achievement mainly due to the success of salvage treatments.

Keywords:

germ cell tumors; children; prognosis.

##plugins.themes.academic_pro.article.details##

References

  1. Kaatsch P, Hafner C, Calaminus G, Blettner M, Tulla M. Pediatric germ cell tumors from 1987 to 2011: incidence rates, time trends, and survival. Pediatrics. 2015;135:136-43.
  2. Billmire DF, Grosfeld JL. Teratomas in childhood: analysis of 142 cases. J Pediatr Surg. 1986;21:548-51.
  3. International Germ Cell Consensus Classification: a prognostic factor-based staging system for metastatic germ cell cancers. International Germ Cell Cancer Collaborative Group. J Clin Oncol. 1997;15:594-603.
  4. Lopes LF, Macedo CR, Pontes EM, Dos Santos Aguiar S, Mastellaro MJ, Melaragno R and al. Cisplatin and etoposide in childhood germ cell tumor: brazilian pediatric oncology society protocol GCT-91. J Clin Oncol. 2009; 27:1297-303.
  5. De Backer A, Madern GC, Pieters R, Haentjens P, Hakvoort-Cammel FG, Oosterhuis JW and al. Influence of tumor site and histology on long-term survival in 193 children with extracranial germ cell tumors. Eur J Pediatr Surg. 2008; 18:1-6.
  6. Isaacs H. Jr. Perinatal (fetal and neonatal) germ cell tumors. J Pediatr Surg. 2004; 39:1003-13.
  7. Mann JR, Gray ES, Thornton C, Raafat F, Robinson K, Collins GS and al. Mature and immature extracranial teratomas in children: the UK Children's Cancer Study Group Experience. J Clin Oncol. 2008; 26: 3590-7.
  8. Marina N, London WB, Frazier AL, Lauer S, Rescorla F, Cushing B and al. Prognostic factors in children with extragonadal malignant germ cell tumors: a pediatric intergroup study. J Clin Oncol. 2006; 24: 2544-8.
  9. Billmire D, Vinocur C, Rescorla F, Colombani P, Cushing B, Hawkins E and al. Malignant mediastinal germ cell tumors: an intergroup study. J Pediatr Surg. 2001; 36:18-24.
  10. Mann JR, Raafat F, Robinson K, Imeson J, Gornall P, Sokal M and al. The United Kingdom Children's Cancer Study Group's second germ cell tumor study: carboplatin, etoposide, and bleomycin are effective treatment for children with malignant extracranial germ cell tumors, with acceptable toxicity. J Clin Oncol. 2000;18: 3809-18.
  11. Faure-Conter C, Orbach D, Cropet C, Baranzelli MC, Martelli H, Thebaud E and al. Salvage therapy for refractory or recurrent pediatric germ cell tumors: the French SFCE experience. Pediatr Blood Cancer. 2014; 61: 253-9.
  12. Baranzelli MC, Kramar A, Bouffet E, Quintana E, Rubie H, Edan C and al. Prognostic factors in children with localized malignant nonseminomatous germ cell tumors. J Clin Oncol. 1999; 17: 1212.
  13. Gobel U, Schneider DT, Calaminus G, Jurgens H, Spaar HJ, Sternschulte W and al. Multimodal treatment of malignant sacrococcygeal germ cell tumors: a prospective analysis of 66 patients of the German cooperative protocols MAKEI 83/86 and 89. J Clin Oncol. 2001;19: 1943-50.
  14. Calaminus G, Schneider DT, Bokkerink JP, Gadner H, Harms D, Willers R and al. Prognostic value of tumor size, metastases, extension into bone, and increased tumor marker in children with malignant sacrococcygeal germ cell tumors: a prospective evaluation of 71 patients treated in the German cooperative protocols Maligne Keimzelltumoren (MAKEI) 83/86 and MAKEI 89. J Clin Oncol. 2003; 21:781-6.
  15. De Giorgi U, Rosti G, Slavin S, Yaniv I, Harousseau JL, Ladenstein R and al. Salvage high-dose chemotherapy for children with extragonadal germ-cell tumours. Br J Cancer. 2005; 93: 412-7.
  16. Frazier AL, Hale JP, Rodriguez-Galindo C, Dang H, Olson T, Murray MJ and al. Revised risk classification for pediatric extracranial germ cell tumors based on 25 years of clinical trial data from the United Kingdom and United States. J Clin Oncol. 2015; 33:195-201.