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Most ovarian masses in children are not malignant.[
Most malignant ovarian tumors occur in girls aged 15 to 19 years.[
The Fédération Internationale de Gynécologie et d'Obstétrique (FIGO) staging system has been used for ovarian cancers (see Table 1).
|FIGO = Fédération Internationale de Gynécologie et d'Obstétrique.|
| a Adapted from Berek et al.[
|I||Tumor confined to the ovary.|
|IA||Tumor limited to one ovary (capsule intact); no tumor on surface of the ovary; no malignant cells in the ascites or peritoneal washings.|
|IB||Tumor limited to both ovaries (capsules intact); no tumor on surface of the ovary; no malignant cells in the ascites or peritoneal washings.|
|IC||Tumor limited to one or both ovaries, with any of the following:|
|IC2||Capsule ruptured before surgery or tumor on the surface of the ovary.|
|IC3||Malignant cells in the ascites or peritoneal washings.|
|II||Tumor involves one or both ovaries with pelvic extension (below pelvic brim) or primary peritoneal cancer.|
|IIA||Extension and/or implants on uterus and/or fallopian tubes.|
|IIB||Extension to other pelvic intraperitoneal tissues.|
|III||Tumor involves one or both ovaries or primary peritoneal cancer, with cytologically or histologically confirmed spread to the peritoneum outside the pelvis and/or metastasis to the retroperitoneal lymph nodes.|
|IIIA1||Positive retroperitoneal lymph nodes only (cytologically or histologically proven):|
|IIIA1(i)||Lymph nodes ≤10 mm in greatest dimension.|
|IIIA1(ii)||Lymph nodes >10 mm in greatest dimension.|
|IIIA2||Microscopic extrapelvic (above the pelvic brim) peritoneal involvement with or without positive retroperitoneal lymph nodes.|
|IIIB||Macroscopic peritoneal metastasis beyond the pelvis ≤2 cm in greatest dimension, with or without metastasis to the retroperitoneal lymph nodes|
|IIIC||Macroscopic peritoneal metastasis beyond the pelvis >2 cm in greatest dimension, with or without metastasis to the retroperitoneal lymph nodes (includes extension of tumor to capsule of liver and spleen without parenchymal involvement of either organ).|
|IV||Distant metastasis excluding peritoneal metastases.|
|IVA||Pleural effusion with positive cytology.|
|IVB||Parenchymal metastases and metastases to extra-abdominal organs (including inguinal lymph nodes and lymph nodes outside of the abdominal cavity).|
Clinical Presentation, Histology, and Prognosis
The most common presenting symptoms of ovarian tumors in children are dysmenorrhea and abdominal pain.
Ovarian tumors derived from malignant epithelial elements include the following types:
There are subtypes within each tumor type. The subtypes include benign tumors, tumors with low malignant potential or borderline tumors, and adenocarcinomas. Most ovarian tumors in the pediatric age range are benign and borderline,[
Girls with ovarian carcinoma (epithelial ovarian neoplasia) fare better than do adults with similar histology, probably because girls usually present with low-stage disease.[
The potential association with genetic predisposition (e.g., BRCA mutation) in pediatric patients has not yet been studied.
Treatment of Childhood Epithelial Ovarian Neoplasia
Treatment options for nonmalignant childhood epithelial ovarian neoplasia include the following:
Treatment of epithelial ovarian neoplasia is based on stage and histology. Most pediatric and adolescent patients have stage I disease. In the TREP study,[
Treatment options for childhood malignant ovarian epithelial cancer include the following:
Treatment of malignant ovarian epithelial cancer is stage-related and follows adult protocols; it may include surgery, radiation therapy, and chemotherapy. For more information, see Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment.
General Information About Sex Cord–Stromal Tumors
The clinical presentation and prognosis of patients with sex cord–stromal tumors vary by histology. In all entities, metastatic spread occurs rarely and if present, is usually limited to the peritoneal cavity.[
In the United States, these tumors may be registered in the International Testicular and Ovarian Stromal Tumor Registry.[
The European Cooperative Study Group for Pediatric Rare Tumors within the PARTNER project (Paediatric Rare Tumours Network - European Registry) has published comprehensive recommendations for the diagnosis and treatment of sex cord–stromal tumors in children and adolescents.[
Histology and molecular features
Ovarian sex cord–stromal tumors are a heterogeneous group of rare tumors that derive from the gonadal non–germ cell component.[
Ovarian Sertoli-Leydig cell tumors in children and adolescents are commonly associated with the presence of germline DICER1 mutations and may be a manifestation of the familial pleuropulmonary blastoma syndrome.[
Prognostic factors related to stage and high mitotic count have been identified. In a report from the German Maligne Keimzelltumoren (MAKEI) study, 54 children and adolescents with prospectively registered sex cord–stromal tumors were analyzed. Forty-eight patients presented with stage I tumors, and six patients had peritoneal metastases. While overall prognosis was favorable, patients at risk could be identified by stage (stage Ic, preoperative rupture, stages II and III) and histological criteria such as high mitotic count.[
A study of 44 patients from the European Cooperative Study Group on Pediatric Rare Tumors showed that prognosis of Sertoli-Leydig cell tumors was determined by stage and histopathologic differentiation.[
Treatment of childhood sex cord–stromal tumors
Treatment options for childhood sex cord–stromal tumors include the following:
A French registry identified 38 girls younger than 18 years with ovarian sex cord tumors.[
Childhood Juvenile Granulosa Cell Tumors
The most common histological subtype of sex cord–stromal tumors in girls younger than 18 years is juvenile granulosa cell tumor (median age, 7.6 years; range, birth to 17.5 years).[
Juvenile granulosa cell tumors have been reported in children with Ollier disease and Maffucci syndrome.[
Patients with juvenile granulosa cell tumors present with the following symptoms:[
Treatment of childhood juvenile granulosa cell tumors
Treatment options for childhood juvenile granulosa cell tumors include the following:
As many as 90% of children with juvenile granulosa cell tumors will have low-stage disease (stage I) by Fédération Internationale de Gynécologie et d'Obstétrique (FIGO) criteria. These patients are usually curable with unilateral salpingo-oophorectomy alone. In one series, 15 of 17 patients underwent fertility-sparing surgery and only two patients received adjuvant chemotherapy. No recurrences were reported.[
Patients with spontaneous tumor rupture or malignant ascites (FIGO stage IC2, IC3), advanced disease (FIGO stages II–IV), and those with high mitotic activity tumors have a poorer prognosis and require chemotherapy.[
Childhood Sertoli-Leydig Cell Tumors
Clinical presentation and risk factors
Sertoli-Leydig cell tumor is a common histological subtype of sex cord–stromal tumors. It is rare in young girls and more frequently seen in adolescents. The tumor may secrete androgens and, thus, present with virilization, secondary amenorrhea,[
These tumors may be associated with Peutz-Jeghers syndrome, but more frequently are a part of the DICER1-tumor spectrum.[
Treatment and outcome of childhood Sertoli-Leydig cell tumors
Treatment options for childhood Sertoli-Leydig cell tumors include the following:
Surgery is the primary treatment for Sertoli-Leydig cell tumors and is the only treatment for low-stage disease (FIGO stage IA). The event-free survival rate for these patients is approximately 100%.[
Patients with Sertoli-Leydig cell tumors with abdominal spillage during surgery, spontaneous tumor rupture, or metastatic disease (FIGO stages IC, II, III, and IV) are treated with cisplatin-based combination chemotherapy, although the impact of chemotherapy has not been studied in clinical trials in children.[
One study reported on 40 women (average age, 28 years) with FIGO stage I or IC Sertoli-Leydig cell tumors of the ovary.[
Small cell carcinomas of the ovary are exceedingly rare and aggressive. The prognosis is poor for these patients. This cancer may be associated with hypercalcemia.[
Somatic and germline SMARCA4 mutations are characteristic of these tumors, putting these in a group with similar molecular features as rhabdoid tumors.[
Treatment of Childhood Small Cell Carcinoma of the Ovary, Hypercalcemia-Type
Treatment options for childhood small cell carcinoma of the ovary include the following:
Aggressive multimodality therapy
Successful treatment has been reported in a few cases using aggressive therapy, including surgery and high-dose chemotherapy with stem cell rescue.[
Tazemetostat is an EZH2 inhibitor that demonstrates activity against preclinical models of small cell carcinoma of the ovary with SMARCA4 loss.[
Information about National Cancer Institute (NCI)–supported clinical trials can be found on the NCI website. For information about clinical trials sponsored by other organizations, see the ClinicalTrials.gov website.
The following are examples of national and/or institutional clinical trials that are currently being conducted:
Patients with tumors that have molecular variants addressed by open treatment arms in the trial may be enrolled in treatment on Pediatric MATCH. Additional information can be obtained on the NCI website and ClinicalTrials.gov website.
Cancer in children and adolescents is rare, although the overall incidence has been slowly increasing since 1975.[
For information about supportive care for children and adolescents with cancer, see the summaries on Supportive and Palliative Care.
The American Academy of Pediatrics has outlined guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer.[
Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2020, childhood cancer mortality decreased by more than 50%.[
Childhood cancer is a rare disease, with about 15,000 cases diagnosed annually in the United States in individuals younger than 20 years.[
The designation of a rare tumor is not uniform among pediatric and adult groups. In adults, rare cancers are defined as those with an annual incidence of fewer than six cases per 100,000 people. They account for up to 24% of all cancers diagnosed in the European Union and about 20% of all cancers diagnosed in the United States.[
Most cancers in subgroup XI are either melanomas or thyroid cancers, with other cancer types accounting for only 2% of the cancers in children aged 0 to 14 years and 9.3% of the cancers in adolescents aged 15 to 19 years.
These rare cancers are extremely challenging to study because of the low number of patients with any individual diagnosis, the predominance of rare cancers in the adolescent population, and the lack of clinical trials for adolescents with rare cancers.
Information about these tumors may also be found in sources relevant to adults with cancer, such as Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment.
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Stage Information for Ovarian Cancer
Added this new section.
Childhood Sex Cord–Stromal Tumors
Added text to state that in one series, 15 of 17 patients underwent fertility-sparing surgery and only two patients received adjuvant chemotherapy. No recurrences were reported (cited Bergamini et al. as reference 16).
Childhood Small Cell Carcinoma of the Ovary, Hypercalcemia-Type
Revised text to state that successful treatment has been reported in a few cases using aggressive therapy, including surgery and high-dose chemotherapy with stem cell rescue.
Treatment Options Under Clinical Evaluation for Childhood Ovarian Cancer
Added text about the PEPN2121 clinical trial as a treatment option under clinical evaluation for patients with small cell carcinomas of the ovary, hypercalcemic type.
Special Considerations for the Treatment of Children With Cancer
Revised text to state that between 1975 and 2020, childhood cancer mortality decreased by more than 50% (cited National Cancer Institute as reference 4 and Surveillance Research Program, National Cancer Institute as reference 5).
Revised text to state that rare pediatric cancers account for about 5% of the cancers diagnosed in children aged 0 to 14 years and about 27% of the cancers diagnosed in adolescents aged 15 to 19 years. Also revised text to state that most cancers in subgroup XI are either melanomas or thyroid cancers, with other cancer types accounting for only 2% of the cancers in children aged 0 to 14 years and 9.3% of cancers in adolescents aged 15 to 19 years.
This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® Cancer Information for Health Professionals pages.
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of pediatric ovarian cancer. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.
Reviewers and Updates
This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewers for Childhood Ovarian Cancer Treatment are:
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.
Levels of Evidence
Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.
Permission to Use This Summary
PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary]."
The preferred citation for this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Ovarian Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/hp/child-ovarian-treatment-pdq. Accessed <MM/DD/YYYY>.
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Last Revised: 2022-12-09
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