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Pediatric Gynecologic Cancers

  • Gynecologic Cancers (NS Reed, Section Editor)
  • Published:
Current Oncology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Three primary categories of gynecologic cancer are found in pediatric and adolescent patients: stromal carcinomas including juvenile granulosa cell tumors and Sertoli-Leydig cell tumors, rhabdomyosarcomas arising from the vagina and cervix (sarcoma botryoides), and ovarian germ cell tumors which comprise a wide range of histologies. These entities are rare and treatment approaches have focused on decreasing late effects of chemotherapy treatment. Here, we review presentation, histologic classifications, diagnosis, and treatment recommendations for pediatric gynecologic cancers.

Recent Findings

Event-free and overall survival for these cancers is high, and the goals of treatment are minimization of morbidity and preservation of fertility with unilateral salpingo-oophorectomies and limited staging. Surveillance of tumor markers after surgery is helpful in monitoring for disease progression and adjuvant chemotherapy is often reserved for patients at recurrence.

Summary

Recent literature supports avoiding chemotherapy even in high-grade germ cell tumors in the pediatric population.

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References

Papers of particular interest, published recently, have been highlighted as: •• Of major importance

  1. Schultz KAP, Schneider DT, Pashankar F, Ross J, Frazier L. Management of ovarian and testicular sex cord-stromal tumors in children and adolescents. J Pediatr Hematol Oncol. 2012;34(Suppl 2):S55–63.

    Article  PubMed  Google Scholar 

  2. •• Schneider DT, Orbach D, Cecchetto G, Stachowicz-Stencel T, Brummel B, Brecht IB, et al. Ovarian Sertoli Leydig cell tumours in children and adolescents: an analysis of the European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT). Eur J Cancer. 2015;51(4):543–50. Sertoli-Leydig cell tumors can be risk stratified by stage and histopathologic differentiation. Patients with stage IA disease can be monitored with postoperative surveillance while those with stage IC and higher should be treated with adjuvant chemotherapy.

    Article  PubMed  Google Scholar 

  3. Kottarathil VD, Antony MA, Nair IR, Pavithran K. Recent advances in granulosa cell tumor ovary: a review. Indian J Surg Oncol. 2013;4(1):37–47.

    Article  PubMed  Google Scholar 

  4. Sivasankaran S, Itam P, Ayensu-Coker L, Sanchez J, Egler RA, Anderson ML, et al. Juvenile granulosa cell ovarian tumor: a case report and review of literature. J Pediatr Adolesc Gynecol. 2009;22(5):e114–7.

    Article  PubMed  Google Scholar 

  5. Schneider DT, Janig U, Calaminus G, Gobel U, Harms D. Ovarian sex cord-stromal tumors—a clinicopathological study of 72 cases from the Kiel Pediatric Tumor Registry. Virchows Arch. 2003;443(4):549–60.

    Article  PubMed  Google Scholar 

  6. Stine JE, Pierce S, Soper JT. A comprehensive review of diagnostic and treatment options for granulosa cell tumors of the ovary. Obstet Gynecol Surv. 2014;69(1):29–38.

    Article  PubMed  Google Scholar 

  7. Calaminus G, Wessalowski R, Harms D, Gobel U. Juvenile granulosa cell tumors of the ovary in children and adolescents: results from 33 patients registered in a prospective cooperative study. Gynecol Oncol. 1997;65(3):447–52.

    Article  CAS  PubMed  Google Scholar 

  8. Powell JL, Otis CN. Management of advanced juvenile granulosa cell tumor of the ovary. Gynecol Oncol. 1997;64(2):282–4.

    Article  CAS  PubMed  Google Scholar 

  9. Kalfa N, Ecochard A, Patte C, Duvillard P, Audran F, Pienkowski C, et al. Activating mutations of the stimulatory g protein in juvenile ovarian granulosa cell tumors: a new prognostic factor? Journal of Clinical Endocrinology & Metabolism. 2006;91(5):1842–7.

    Article  CAS  Google Scholar 

  10. Ala-Fossi SL, Aine R, Punnonen R, Maenpaa J. Is potential to produce inhibins related to prognosis in ovarian granulosa cell tumors? Eur J Gynaecol Oncol. 2000;21(2):187–9.

    CAS  PubMed  Google Scholar 

  11. Powell JL, Johnson NA, Bailey CL, Otis CN. Management of advanced juvenile granulosa cell tumor of the ovary. Gynecol Oncol. 1993;48(1):119–23.

    Article  CAS  PubMed  Google Scholar 

  12. Erdreich-Epstein A, Monforte HL, Lavey RS, Joshi S, Phillips JD, Villablanca JG. Successful multimodality therapy of recurrent multifocal juvenile granulosa cell tumor of the ovary. J Pediatr Hematol Oncol. 2002;24(3):229–33.

    Article  PubMed  Google Scholar 

  13. Benesch M, Lackner H, Pilhatsch A, Gurtl-Lackner B, Schwinger W, Urban C. Long-term remission in a female with multiple relapsed juvenile granulosa cell tumor. J Pediatr Hematol Oncol. 2015;37(8):e486–9.

    Article  PubMed  Google Scholar 

  14. Cecchetto G, Ferrari A, Bernini G, Alaggio R, Collini P, Virgone C, et al. Sex cord stromal tumors of the ovary in children: a clinicopathological report from the Italian TREP project. Pediatr Blood Cancer. 2011;56(7):1062–7.

    Article  PubMed  Google Scholar 

  15. Lim S, Kim N, Lee K. A case of successful salvage chemotherapy of recurrent ovarian sertoli-leydig cell tumor. Obstet gynecol sci. 2013;56(3):198–200.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Ghaemmaghami F, Karimi Zarchi M, Ghasemi M. Lower genital tract rhabdomyosarcoma: case series and literature review. Arch Gynecol Obstet. 2008;278(1):65–9.

    Article  PubMed  Google Scholar 

  17. Villella JA, Bogner PN, Jani-Sait SN, Block AMW, Lele S. Rhabdomyosarcoma of the cervix in sisters with review of the literature. Gynecol Oncol. 2005;99(3):742–8.

    Article  PubMed  Google Scholar 

  18. Kim EE, Valenzuela RF, Kumar AJ, Raney RB, Eftekari F. Imaging and clinical spectrum of rhabdomyosarcoma in children. Clin Imaging. 2000;24(5):257–62.

    Article  CAS  PubMed  Google Scholar 

  19. Norman G, Fayter D, Lewis-Light K, Chisholm J, McHugh K, Levine D, et al. An emerging evidence base for PET-CT in the management of childhood rhabdomyosarcoma: systematic review. BMJ Open. 2015;5(1):e006030.

    Article  PubMed  PubMed Central  Google Scholar 

  20. McLean TW, Castellino SM. Pediatric genitourinary tumors. Curr Opin Oncol. 2008;20(3):315–20.

    Article  PubMed  Google Scholar 

  21. •• Harel M, Ferrer FA, Shapiro LH, Makari JH. Future directions in risk stratification and therapy for advanced pediatric genitourinary rhabdomyosarcoma. Urol Oncol. 2016;34(2):103–15. Alveolar rhabdomyosarcomas with fusions proetins caused by translocations t(2;13) and t(1;13) are known to be more aggressive in nature and future studies will likely incorporate this fusion status into risk stratification.

    Article  PubMed  Google Scholar 

  22. Foulkes WD, Clarke BA, Hasselblatt M, Majewski J, Albrecht S, McCluggage WG. No small surprise—small cell carcinoma of the ovary, hypercalcaemic type, is a malignant rhabdoid tumour. J Pathol. 2014;233(3):209–14.

    Article  CAS  PubMed  Google Scholar 

  23. Andrassy RJ, Hays DM, Raney RB, Wiener ES, Lawrence W, Lobe TE, et al. Conservative surgical management of vaginal and vulvar pediatric rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study III. J Pediatr Surg. 1995;30(7):1034–6. discussion 1036-7

    Article  CAS  PubMed  Google Scholar 

  24. Andrassy RJ, Wiener ES, Raney RB, Hays DM, Arndt CA, Lobe TE, et al. Progress in the surgical management of vaginal rhabdomyosarcoma: a 25-year review from the Intergroup Rhabdomyosarcoma Study Group. J Pediatr Surg. 1999;34(5):731–4. discussion 734-5

    Article  CAS  PubMed  Google Scholar 

  25. Arndt CA, Donaldson SS, Anderson JR, Andrassy RJ, Laurie F, Link MP, et al. What constitutes optimal therapy for patients with rhabdomyosarcoma of the female genital tract? Cancer. 2001;91(12):2454–68.

    Article  CAS  PubMed  Google Scholar 

  26. de Lambert G, Haie-Meder C, Guerin F, Boubnova J, Martelli H. A new surgical approach of temporary ovarian transposition for children undergoing brachytherapy: technical assessment and dose evaluation. J Pediatr Surg. 2014;49(7):1177–80.

    Article  PubMed  Google Scholar 

  27. Ognjanovic S, Linabery AM, Charbonneau B, Ross JA. Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975-2005. Cancer. 2009;115(18):4218–26.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Pappo AS, Anderson JR, Crist WM, Wharam MD, Breitfeld PP, Hawkins D, et al. Survival after relapse in children and adolescents with rhabdomyosarcoma: A report from the Intergroup Rhabdomyosarcoma Study Group. J Clin Oncol. 1999;17(11):3487–93.

    Article  CAS  PubMed  Google Scholar 

  29. Vasquez R, Collini P, Meazza C, Favini F, Casanova M, Ferrari A. Late relapse of embryonal rhabdomyosarcoma, botryoid variant, of the vagina. Pediatr Blood Cancer. 2008;51(1):140–1.

    Article  PubMed  Google Scholar 

  30. Mixon BA, Eckrich MJ, Lowas S, Engel ME. Vincristine, irinotecan, and temozolomide for treatment of relapsed alveolar rhabdomyosarcoma. J Pediatr Hematol Oncol. 2013;35(4):e163–6.

    Article  CAS  PubMed  Google Scholar 

  31. Winter S, Fasola S, Brisse H, Mosseri V, Orbach D. Relapse after localized rhabdomyosarcoma: evaluation of the efficacy of second-line chemotherapy. Pediatr Blood Cancer. 2015;62(11):1935–41.

    Article  CAS  PubMed  Google Scholar 

  32. Mosbech CH, Rechnitzer C, Brok JS, Rajpert-De Meyts E, Hoei-Hansen CE. Recent advances in understanding the etiology and pathogenesis of pediatric germ cell tumors. J Pediatr Hematol Oncol. 2014;36(4):263–70.

    Article  CAS  PubMed  Google Scholar 

  33. Poynter JN, Amatruda JF, Ross JA. Trends in incidence and survival of pediatric and adolescent patients with germ cell tumors in the United States, 1975 to 2006. Cancer. 2010;116(20):4882–91.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Cushing B, Perlman EJ, Marina NM, Castleberry RP. Germ cell tumors. In: Pizzo P, Poplack D, editors. Principles and practice of pediatric oncology. 5th ed. Philadelphia: Lippincott, Williams and Wilkins; 2006. p. 1116–38.

    Google Scholar 

  35. •• Pashankar F, Hale JP, Dang H, Krailo M, Brady WE, Rodriguez-Galindo C, et al. Is adjuvant chemotherapy indicated in ovarian immature teratomas? A combined data analysis from the Malignant Germ Cell Tumor International Collaborative. Cancer. 2016;122(2):230–7. Pooled analysis of pediatric and adult patients with ovarian immature teratomas revealed that grade is the most important risk factor for disease relapse and adjuvant chemotherapy did not decrease the relapse risk in the pediatric cohort.

    Article  PubMed  Google Scholar 

  36. •• Mangili G, Sigismondi C, Lorusso D, Cormio G, Candiani M, Scarfone G, et al. The role of staging and adjuvant chemotherapy in stage I malignant ovarian germ cell tumors (MOGTs): the MITO-9 study. Ann Oncol. 2017 Feb 1;28(2):333–338. A large retrospective review in adults with stage I dysgerminoma and immature teratoma found that reserving adjuvant chemotherapy for patients with relapsed disease resulted in high cure rates and spared many patients the morbidity and mortality associated with chemotherapy. Those patients with yolk sac histology had worse prognosis.

  37. Gershenson DM, Frazier AL. Conundrums in the management of malignant ovarian germ cell tumors: toward lessening acute morbidity and late effects of treatment. Gynecol Oncol. 2016;143(2):428–32.

    Article  PubMed  Google Scholar 

  38. Olson TA, Murray MJ, Rodriguez-Galindo C, Nicholson JC, Billmire DF, Krailo MD, et al. Pediatric and adolescent extracranial germ cell tumors: the road to collaboration. J Clin Oncol. 2015;33(27):3018–28.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. •• Billmire DF, Cullen JW, Rescorla FJ, Davis M, Schlatter MG, Olson TA, et al. Surveillance after initial surgery for pediatric and adolescent girls with stage I ovarian germ cell tumors: report from the Children's Oncology Group. J Clin Oncol. 2014;32(5):465–70. Pediatric patients with stage I malignant ovarian germ cell tumor can be monitored after surgery with surveillance alone. Chemotherapy treatment can be reserved for patients who have recurrent disease and overall survival remains preserved.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Baranzelli MC, Bouffet E, Quintana E, Portas M, Thyss A, Patte C. Non-seminomatous ovarian germ cell tumours in children. Eur J Cancer. 2000;36(3):376–83.

    Article  CAS  PubMed  Google Scholar 

  41. Mann JR, Raafat F, Robinson K, Imeson J, Gornall P, Sokal M, et 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(22):3809–18.

    Article  CAS  PubMed  Google Scholar 

  42. De Vuyst H, Clifford GM, Nascimento MC, Madeleine MM, Franceschi S. Prevalence and type distribution of human papillomavirus in carcinoma and intraepithelial neoplasia of the vulva, vagina and anus: a meta-analysis. Int J Cancer. 2009;124(7):1626–36.

    Article  CAS  PubMed  Google Scholar 

  43. Viens LJ, Henley SJ, Watson M, Markowitz LE, Thomas CC, Thompson TD, et al. Human papillomavirus-associated cancers—United States, 2008-2012. MMWR Morb Mortal Wkly Rep. 2016;65(26):661–6.

    Article  PubMed  Google Scholar 

  44. Kahn JA, Widdice LE, Ding L, Huang B, Brown DR, Franco EL, et al. Substantial decline in vaccine-type human papillomavirus (HPV) among vaccinated young women during the first 8 years after HPV vaccine introduction in a community. Clin Infect Dis. 2016;63(10):1281–7.

    Article  PubMed  Google Scholar 

  45. McNamara M, Batur P, Walsh JME, Johnson KM. HPV update: vaccination, screening, and associated disease. J Gen Intern Med. 2016;31(11):1360–6.

    Article  PubMed  Google Scholar 

  46. Drolet M, Benard E, Boily M, Ali H, Baandrup L, Bauer H, et al. Population-level impact and herd effects following human papillomavirus vaccination programmes: a systematic review and meta-analysis. Lancet Infect Dis. 2015;15(5):565–80.

    Article  PubMed  PubMed Central  Google Scholar 

  47. Reagan-Steiner S, Yankey D, Jeyarajah J, Elam-Evans LD, Singleton JA, Curtis CR, et al. National, regional, state, and selected local area vaccination coverage among adolescents aged 13-17 years—United States, 2014. MMWR Morb Mortal Wkly Rep. 2015;64(29):784–92.

    Article  PubMed  Google Scholar 

  48. Holman DM, Benard V, Roland KB, Watson M, Liddon N, Stokley S. Barriers to human papillomavirus vaccination among US adolescents: a systematic review of the literature. JAMA Pediatr. 2014;168(1):76–82.

    Article  PubMed  PubMed Central  Google Scholar 

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Acknowledgements

The authors would like to acknowledge Jason A. Jarzembowski, MD, PhD, for providing the figure in this article.

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Authors and Affiliations

  1. Children’s Hospital of Wisconsin, Department of Pediatrics, Hematology/Oncology/BMT, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI, 53226, USA

    Lauren Pommert

  2. Department of Obstetrics and Gynecology, Froedtert and the Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI, 53226, USA

    William Bradley

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Correspondence to William Bradley.

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Lauren Pommert and William Bradley declare they have no conflict of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Gynecologic Cancers

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Pommert, L., Bradley, W. Pediatric Gynecologic Cancers. Curr Oncol Rep 19, 44 (2017). https://doi.org/10.1007/s11912-017-0604-7

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