Pediatric Radiology

, Volume 49, Issue 11, pp 1463–1475 | Cite as

Ovarian neoplasms of childhood

  • Shailee V. LalaEmail author
  • Naomi Strubel
Pediatric oncologic imaging


Ovarian neoplasms are rare in children. Although usually asymptomatic, they sometimes present with abdominal pain, abdominal distension or palpable mass. The distribution of neoplasms in the pediatric population is different from in adults; benign mature cystic teratoma is the most common ovarian tumor in children. Radiologists should be familiar with the variable sonographic, CT and MRI findings of ovarian neoplasms. Although the less frequently encountered ovarian malignancies cannot be reliably distinguished by imaging alone, it does play an important role in workup. This review discusses the imaging and relevant clinical manifestations of the more commonly encountered pediatric ovarian neoplasms.


Children Magnetic resonance imaging Malignancy Neoplasm Ovary Ultrasound 


Compliance with ethical standards

Conflicts of interest



  1. 1.
    Skinner MA, Schlatter MG, Heifetz SA et al (1993) Ovarian neoplasms in children. Arch Surg 128:849–853CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Brookfield KF, Cheung MC, Koniaris LG et al (2009) A population-based analysis of 1,037 malignant ovarian tumors in the pediatric population. J Surg Res 156:45–49CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Oltmann SC, Garcia N, Barber R et al (2010) Can we preoperatively risk stratify ovarian masses for malignancy? J Pediatr Surg 45:130–134CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Carcangiu ML (2014) WHO classification of tumours of female reproductive organs. International Agency for Research on Cancer (IARC). United Nations, New YorkGoogle Scholar
  5. 5.
    Zhang M, Jiang W, Li G et al (2014) Ovarian masses in children, and adolescents — an analysis of 521 clinical cases. J Pediatr Adolesc Gynecol 27:e73–e77CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Deprest J, Moerman P, Corneillie P et al (1992) Ovarian borderline mucinous tumor in a premenarchal girl: review on ovarian epithelial cancer in young girls. Gynecol Oncol 45:219–224CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Norris HJ, Jensen RD (1972) Relative frequency of ovarian neoplasms in children and adolescents. Cancer 30:713–719CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Goudie C, Witkowski L, Vairy S et al (2018) Pediatric ovarian tumors and their associated cancer susceptibility syndromes. J Med Genet 55:1–10CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Schultz KAP, Harris AK, Finch M et al (2017) DICER1-related Sertoli-Leydig cell tumor and gynandroblastoma: clinical and genetic findings from the international ovarian and testicular stromal tumor registry. Gynecol Oncol 147:521–527CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    de Kock L, Terzic T, McCluggage WG et al (2017) DICER1 mutations are consistently present in moderately and poorly differentiated Sertoli-Leydig cell tumors. Am J Surg Pathol 41:1178–1187CrossRefGoogle Scholar
  11. 11.
    Schultz KAP, Williams GM, Kamihara J et al (2018) DICER1 and associated conditions: identification of at-risk individuals and recommended surveillance strategies. Clin Cancer Res 24:2251–2261CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Madenci AL, Levine BS, Laufer MR et al (2016) Preoperative risk stratification of children with ovarian tumors. J Pediatr Surg 51:1507–1512CrossRefPubMedPubMedCentralGoogle Scholar
  13. 13.
    Oltmann SC, Fischer A, Barber R et al (2010) Pediatric ovarian malignancy presenting as ovarian torsion: incidence and relevance. J Pediatr Surg 45:135–139CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Papic JC, Finnell SM, Slaven JE et al (2014) Predictors of ovarian malignancy in children: overcoming clinical barriers of ovarian preservation. J Pediatr Surg 49:144–147CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Young RH, Dickersin GR, Scully RE (1984) Juvenile granulosa cell tumor of the ovary. A clinicopathological analysis of 125 cases. Am J Surg Pathol 8:575–596CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    De Backer A, Madern GC, Oosterhuis JW et al (2006) Ovarian germ cell tumors in children: a clinical study of 66 patients. Pediatr Blood Cancer 46:459–464CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Choong CS, Fuller PJ, Chu S et al (2002) Sertoli-Leydig cell tumor of the ovary, a rare cause of precocious puberty in a 12-month-old infant. J Clin Endocrinol Metab 87:49–56CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Spinelli C, Pucci V, Buti I et al (2012) The role of tumor markers in the surgical approach of ovarian masses in pediatric age: a 10-year study and a literature review. Ann Surg Oncol 19:1766–1773CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Stankovic ZB, Djukic MK, Savic D et al (2006) Pre-operative differentiation of pediatric ovarian tumors: morphological scoring system and tumor markers. J Pediatr Endocrinol Metab 19:1231–1238CrossRefPubMedPubMedCentralGoogle Scholar
  20. 20.
    Comerci JT Jr, Licciardi F, Bergh PA et al (1994) Mature cystic teratoma: a clinicopathologic evaluation of 517 cases and review of the literature. Obstet Gynecol 84:22–28PubMedPubMedCentralGoogle Scholar
  21. 21.
    Patel MD, Feldstein VA, Lipson SD et al (1998) Cystic teratomas of the ovary: diagnostic value of sonography. AJR Am J Roentgenol 171:1061–1065CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Sheth S, Fishman EK, Buck J et al (1988) The variable sonographic appearances of ovarian teratomas: correlation with CT. AJR Am J Roentgenol 151:331–334CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Malde HM, Kedar RP, Chadha D et al (1992) Dermoid mesh: a sonographic sign of ovarian teratoma. AJR Am J Roentgenol 159:1349–1350CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Mais V, Guerriero S, Ajossa S et al (1995) Transvaginal ultrasonography in the diagnosis of cystic teratoma. Obstet Gynecol 85:48–52CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Hertzberg BS, Kliewer MA (1996) Sonography of benign cystic teratoma of the ovary: pitfalls in diagnosis. AJR Am J Roentgenol 167:1127–1133CrossRefPubMedPubMedCentralGoogle Scholar
  26. 26.
    Buy JN, Ghossain MA, Moss AA et al (1989) Cystic teratoma of the ovary: CT detection. Radiology 171:697–701CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Peterson WF, Prevost EC, Edmunds FT et al (1995) Benign cystic teratomas of the ovary: a clinico-statistical study of 1,007 cases with a review of the literature. Am J Obstet Gynecol 70:368–382CrossRefGoogle Scholar
  28. 28.
    Stern JL, Buscema J, Rosenshein NB et al (1981) Spontaneous rupture of benign cystic teratomas. Obstet Gynecol 57:363–366PubMedPubMedCentralGoogle Scholar
  29. 29.
    Dalmau J, Gleichman AJ, Hughes EG et al (2008) Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol 7:1091–1098CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Norris HJ, Zirkin HJ, Benson WL (1976) Immature (malignant) teratoma of the ovary: a clinical and pathologic study of 58 cases. Cancer 37:2359–2372CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    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 17:2137–2143CrossRefPubMedPubMedCentralGoogle Scholar
  32. 32.
    Pashankar F, Hale JP, Dang H et al (2016) Is adjuvant chemotherapy indicated in ovarian immature teratomas? A combined data analysis from the Malignant Germ Cell Tumor International Collaborative. Cancer 122:230–237Google Scholar
  33. 33.
    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 181:353–358CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Heifetz SA, Cushing B, Giller R et al (1998) Immature teratomas in children: pathologic considerations: a report from the combined Pediatric Oncology Group/Children's Cancer Group. Am J Surg Pathol 22:1115–1124CrossRefPubMedPubMedCentralGoogle Scholar
  35. 35.
    Alotaibi MO, Navarro OM (2010) Imaging of ovarian teratomas in children: a 9-year review. Can Assoc Radiol J 61:23–28CrossRefPubMedPubMedCentralGoogle Scholar
  36. 36.
    Yanai-Inbar I, Scully RE (1987) Relation of ovarian dermoid cysts and immature teratomas: an analysis of 350 cases of immature teratoma and 10 cases of dermoid cyst with microscopic foci of immature tissue. Int J Gynecol Pathol 6:203–212CrossRefPubMedPubMedCentralGoogle Scholar
  37. 37.
    Liang L, Zhang Y, Malpica A et al (2015) Gliomatosis peritonei: a clinicopathologic and immunohistochemical study of 21 cases. Mod Pathol 28:1613–1620CrossRefPubMedPubMedCentralGoogle Scholar
  38. 38.
    Wang D, Jia CW, Feng RE et al (2016) Gliomatosis peritonei: a series of eight cases and review of the literature. J Ovarian Res 9:45CrossRefPubMedPubMedCentralGoogle Scholar
  39. 39.
    Mann JR, Gray ES, Thornton C et al (2008) Mature and immature extracranial teratomas in children: the UK Children's Cancer Study Group experience. J Clin Oncol 26:3590–3597CrossRefPubMedPubMedCentralGoogle Scholar
  40. 40.
    Levy AD, Shaw JC, Sobin LH (2009) Secondary tumors and tumorlike lesions of the peritoneal cavity: imaging features with pathologic correlation. Radiographics 29:347–373CrossRefPubMedPubMedCentralGoogle Scholar
  41. 41.
    Gordon A, Lipton D, Woodruff JD (1981) Dysgerminoma: a review of 158 cases from the Emil Novak Ovarian Tumor Registry. Obstet Gynecol 58:497–504PubMedPubMedCentralGoogle Scholar
  42. 42.
    Kawai M, Kano T, Kikkawa F et al (1992) Seven tumor markers in benign and malignant germ cell tumors of the ovary. Gynecol Oncol 45:248–253CrossRefPubMedPubMedCentralGoogle Scholar
  43. 43.
    Shaaban AM, Rezvani M, Elsayes KM et al (2014) Ovarian malignant germ cell tumors: cellular classification and clinical and imaging features. Radiographics 34:777–801CrossRefPubMedPubMedCentralGoogle Scholar
  44. 44.
    Vicus D, Beiner ME, Klachook S et al (2010) Pure dysgerminoma of the ovary 35 years on: a single institutional experience. Gynecol Oncol 117:23–26CrossRefPubMedPubMedCentralGoogle Scholar
  45. 45.
    Patterson DM, Murugaesu N, Holden L et al (2008) A review of the close surveillance policy for stage I female germ cell tumors of the ovary and other sites. Int J Gynecol Cancer 18:43–50CrossRefPubMedPubMedCentralGoogle Scholar
  46. 46.
    Kim SH, Kang SB (1995) Ovarian dysgerminoma: color Doppler ultrasonographic findings and comparison with CT and MR imaging findings. J Ultrasound Med 14:843–848CrossRefPubMedPubMedCentralGoogle Scholar
  47. 47.
    Tanaka YO, Kurosaki Y, Nishida M et al (1994) Ovarian dysgerminoma: MR and CT appearance. J Comput Assist Tomogr 18:443–448Google Scholar
  48. 48.
    Roth LM, Cheng L (2018) Classical gonadoblastoma: its relationship to the 'dissecting' variant and undifferentiated gonadal tissue. Histopathology 72:545–555CrossRefPubMedPubMedCentralGoogle Scholar
  49. 49.
    Patel PR, Pappas J, Arva NC et al (2013) Early presentation of bilateral gonadoblastomas in a Denys-Drash syndrome patient: a cautionary tale for prophylactic gonadectomy. J Pediatr Endocrinol Metab 26:971–974CrossRefPubMedPubMedCentralGoogle Scholar
  50. 50.
    Mueller RF (1994) The Denys-Drash syndrome. J Med Genet 31:471–477CrossRefPubMedPubMedCentralGoogle Scholar
  51. 51.
    Sinha A, Sharma S, Gulati A et al (2010) Frasier syndrome: early gonadoblastoma and cyclosporine responsiveness. Pediatr Nephrol 25:2171–2174CrossRefPubMedPubMedCentralGoogle Scholar
  52. 52.
    Joki-Erkkilä MM, Karikoski R, Rantala I et al (2002) Gonadoblastoma and dysgerminoma associated with XY gonadal dysgenesis in an adolescent with chronic renal failure: a case of Frasier syndrome. J Pediatr Adolesc Gynecol 15:145–149CrossRefPubMedPubMedCentralGoogle Scholar
  53. 53.
    Scully RE (1970) Gonadoblastoma. A review of 74 cases. Cancer 25:1340–1356CrossRefPubMedPubMedCentralGoogle Scholar
  54. 54.
    Ebert KM, Hewitt GD, Indyk JA et al (2018) Normal pelvic ultrasound or MRI does not rule out neoplasm in patients with gonadal dysgenesis and Y chromosome material. J Pediatr Urol 14:154.e1–154.e6CrossRefGoogle Scholar
  55. 55.
    Papaioannou G, Sebire NJ, McHugh K (2009) Imaging of the unusual pediatric ‘blastomas’. Cancer Imaging 9:1–11Google Scholar
  56. 56.
    Seymour EQ, Hood JB, Underwood PB Jr et al (1976) Gonadoblastoma: an ovarian tumor with characteristic pelvic calcifications. AJR Am J Roentgenol 127:1001–1002CrossRefPubMedPubMedCentralGoogle Scholar
  57. 57.
    Fresneau B, Orbach D, Faure-Conter C et al (2015) Sex-cord stromal tumors in children and teenagers: results of the TGM-95 study. Pediatr Blood Cancer 62:2114–2119CrossRefPubMedPubMedCentralGoogle Scholar
  58. 58.
    Schneider D, Calaminus G, Wessalowski R et al (2003) Ovarian sex cord-stromal tumors in children and adolescents. J Clin Oncol 21:2357–2363CrossRefPubMedPubMedCentralGoogle Scholar
  59. 59.
    Zaloudek C, Norris HJ (1982) Granulosa tumors of the ovary in children: a clinical and pathologic study of 32 cases. Am J Surg Pathol 6:503–512CrossRefPubMedPubMedCentralGoogle Scholar
  60. 60.
    Kitamura Y, Kanegawa K, Muraji T et al (2000) MR imaging of juvenile granulosa cell tumour of the ovary: a case report. Pediatr Radiol 30:360CrossRefPubMedPubMedCentralGoogle Scholar
  61. 61.
    Tanaka Y, Sasaki Y, Nishihira H et al (1992) Ovarian juvenile granulosa cell tumor associated with Maffucci's syndrome. Am J Clin Pathol 97:523–527CrossRefPubMedPubMedCentralGoogle Scholar
  62. 62.
    Sampagar AA, Jahagirdar RR, Bafna VS et al (2016) Juvenile granulosa cell tumor associated with Ollier disease. Indian J Med Paediatr Oncol 37:293–295CrossRefPubMedPubMedCentralGoogle Scholar
  63. 63.
    Young RH, Perez-Atayde AR, Scully RE (1984) Ovarian Sertoli-Leydig cell tumor with retiform and heterologous components. Report of a case with hepatocytic differentiation and elevated serum alpha-fetoprotein. Am J Surg Pathol 8:709–718CrossRefPubMedPubMedCentralGoogle Scholar
  64. 64.
    Young RH, Scully RE (1985) Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases. Am J Surg Pathol 9:543–569CrossRefPubMedPubMedCentralGoogle Scholar
  65. 65.
    Schneider DT, Orbach D, Cecchetto G et al (2015) 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 51:543–550CrossRefPubMedPubMedCentralGoogle Scholar
  66. 66.
    Jung SE, Rha SE, Lee JM et al (2005) CT and MRI findings of sex cord–stromal tumor of the ovary. AJR Am J Roentgenol 185:207–215CrossRefPubMedPubMedCentralGoogle Scholar
  67. 67.
    Young RH, Welch WR, Dickersin GR et al (1982) Ovarian sex cord tumor with annular tubules: review of 74 cases including 27 with Peutz-Jeghers syndrome and four with adenoma malignum of the cervix. Cancer 50:1384–1402CrossRefPubMedPubMedCentralGoogle Scholar
  68. 68.
    Nosov V, Park S, Rao J et al (2009) Non-Peutz-Jeghers syndrome associated ovarian sex cord tumor with annular tubules: a case report. Fertil Steril 92:1497.e5–1497.e8CrossRefGoogle Scholar
  69. 69.
    Scully RE (1970) Sex cord tumor with annular tubules a distinctive ovarian tumor of the Peutz-Jeghers syndrome. Cancer 25:1107–1121CrossRefPubMedPubMedCentralGoogle Scholar
  70. 70.
    Renaud EJ, Sømme S, Islam S et al (2019) Ovarian masses in the child and adolescent: an American Pediatric Surgical Association outcomes and evidence-based practice committee systematic review. J Pediatr Surg 54:369–377CrossRefPubMedPubMedCentralGoogle Scholar
  71. 71.
    Hazard FK, Longacre TA (2013) Ovarian surface epithelial neoplasms in the pediatric population: incidence, histologic subtype, and natural history. Am J Surg Pathol 37:548–553CrossRefPubMedPubMedCentralGoogle Scholar
  72. 72.
    Morowitz M, Huff D, von Allmen D (2003) Epithelial ovarian tumors in children: a retrospective analysis. J Pediatr Surg 38:331–335CrossRefPubMedPubMedCentralGoogle Scholar
  73. 73.
    Song T, Choi CH, Park HS et al (2011) Fertility-sparing surgery for borderline ovarian tumors: oncologic safety and reproductive outcomes. Int J Gynecol Cancer 21:640–646CrossRefPubMedPubMedCentralGoogle Scholar
  74. 74.
    Young RH, Oliva E, Scully RE (1984) Small cell carcinoma of the ovary, hypercalcemic type. A clinicopathological analysis of 150 cases. Am J Surg Pathol 18:1102–1116CrossRefGoogle Scholar
  75. 75.
    Callegaro-Filho D, Gershenson DM, Nick AM et al (2016) Small cell carcinoma of the ovary-hypercalcemic type (SCCOHT): a review of 47 cases. Gynecol Oncol 140:53–57CrossRefPubMedPubMedCentralGoogle Scholar
  76. 76.
    Korivi BR, Javadi S, Faria S et al (2018) Small cell carcinoma of the ovary, hypercalcemic type: clinical and imaging review. Curr Probl Diagn Radiol 47:333–339CrossRefPubMedPubMedCentralGoogle Scholar
  77. 77.
    Witkowski L, Carrot-Zhang J, Albrecht S (2014) Germline and somatic SMARCA4 mutations characterize small cell carcinoma of the ovary, hypercalcemic type. Nat Genet 46:438–443CrossRefPubMedPubMedCentralGoogle Scholar
  78. 78.
    Witkowski L, Goudie C, Ramos P et al (2016) The influence of clinical and genetic factors on patient outcome in small cell carcinoma of the ovary, hypercalcemic type. Gynecol Oncol 141:454–460CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of RadiologyNew York University School of MedicineNew YorkUSA

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