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Ovarian neoplasms in children
M A Skinner1, M G Schlatter, S A Heifetz
1Department of Surgery, James Whitcomb Riley Hospital for Children, Indiana University School of Medicine, Indianapolis.
Insights
Pediatric ovarian neoplasms are rare, often germ-cell tumors. While most are benign, aggressive malignant tumors, particularly sex cord/stromal types, can be unpredictable despite early-stage diagnosis and treatment.
Area of Science:
- Pediatric Oncology
- Gynecologic Oncology
- Tumor Biology
Background:
- Ovarian neoplasms are uncommon in children.
- Germ-cell tumors are the predominant type in pediatric patients, differing from adult populations.
- Clinical presentation varies, including abdominal pain, mass, or hormonal disturbances.
Purpose of the Study:
- To review the clinical presentation, treatment, and outcomes of ovarian neoplasms in pediatric patients.
- To analyze the types, stages, and survival rates of these tumors.
- To identify prognostic factors and treatment responses.
Main Methods:
- Retrospective review of medical records for 29 girls with ovarian neoplasms treated between 1976 and 1992.
- Analysis of patient demographics, tumor characteristics, treatment modalities, and outcomes.
- Follow-up duration ranged from 6 months to 7.5 years, averaging 3.0 years for malignant cases.
Main Results:
- Seventeen tumors were benign and 12 were malignant; germ-cell tumors predominated (n=17).
- Most malignant neoplasms (7/10) were diagnosed at stage I.
- Overall survival was high (100% for benign, 70% for malignant), but aggressive behavior was noted in some malignant types, including sex cord/stromal tumors.
Conclusions:
- Ovarian tumors in children are rare and typically germ-cell in origin.
- While many are benign, some malignant pediatric ovarian tumors exhibit aggressive behavior and poor response to adjuvant therapy.
- Malignant sex cord/stromal cell tumors warrant particular attention due to their unpredictable nature, even at early stages.
Objective:
To review the clinical presentation, treatment, and outcome in a series of children with ovarian neoplasms.
Design:
A retrospective review of the medical records in a case series of 29 girls with ovarian neoplasms. The length of follow-up ranged from 6 months to 7 1/2 years and averaged 3.0 years in the girls with malignant tumors.
Setting:
The patients were treated at a large referral children's hospital.
Patients:
Twenty-nine girls with ovarian neoplasms were treated from 1976 to 1992. The average age of the patients was 10 years and ranged from 2 to 16 years.
Main Outcome Measures:
The principal outcomes examined were mortality and surgical morbidity.
Results:
The most common presenting symptoms for these ovarian tumors in pediatric patients included chronic abdominal pain, an abdominal mass, or distention. Three girls presented with precocious puberty or hirsutism. In 27 cases, the tumor was a primary ovarian lesion. In two patients, the ovarian mass was the presenting finding for a stage IV non-Hodgkin's lymphoma. Seventeen tumors were benign and 12 were malignant. Tumors originating from the germ-cell line predominated (n = 17). Seven of the 10 ovarian malignant neoplasms were stage I at the time of diagnosis. All but one of the girls with malignant tumors received either adjunctive radiation therapy or multiple-agent chemotherapy. Two girls with sex cord/stromal cell tumors who presented with stage I disease ultimately developed widespread metastases. Both girls with large epithelial tumors survived. All of the girls with benign tumors and seven (70%) of 10 with malignant lesions survived.
Conclusion:
Ovarian tumors are unusual lesions in the pediatric population. Unlike in adults, such neoplasms generally originate from the germ-cell line. Whereas most ovarian tumors in girls are benign, some children have malignant tumors that are very aggressive and do not respond well to adjuvant therapy. In particular, malignant sex cord/stromal cell tumors, even when they present at an early stage, may behave unpredictably.