Ovarian neoplasms in children
M. A. Skinner, M. G. Schlatter, S. A. Heifetz and J. L. Grosfeld
Department of Surgery, James Whitcomb Riley Hospital for Children, Indiana University School of Medicine, Indianapolis.
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.