Here we present a case of a premenarchal girl in whom an elevated CA-125 reflected ovarian torsion. This case demonstrates several interesting issues in the management of pediatric ovarian masses, including conservative surgery, appropriate tumor markers and elevation of CA-125 in the presence of pediatric ovarian torsion.
In a pediatric patient with a pelvic mass, gynecologic malignancy must first be ruled out. In children, oophorectomy is the most common ovarian surgery, even for benign conditions such as ovarian cysts and torsion.3
Since removal of the entire ovary has significant implications for future fertility, every effort should be made to avoid unnecessary oophorectomy. In this case, rather than removing the entire ovary, a biopsy was performed in an open fashion to prevent intra-abdominal spillage and obtain a frozen section. Once malignancy had been ruled out, the necrotic component within the ovary was excised and the ovarian cortex was preserved. Long term follow up is necessary to assess ovarian function.
Multiple serum markers for ovarian tumors are available including HCG, LDH, AFP, CEA and CA-125. Elevated AFP, LDH and HCG are associated with germ cell tumors, while CA-125 can indicate an ovarian tumor of epithelial origin. CA-125 is perhaps the most widely studied marker for ovarian cancer in adults and has sensitivities for stage I and II disease of 50 and 90%, respectively.5
However, the specificity of CA-125 is limited. In addition to ovarian malignancy, other gynecologic states such as adenomyosis, uterine fibroids, pelvic inflammatory disease, pregnancy, menstruation, endometriosis and ovarian torsion can cause elevations in serum CA-125.4
In a premenarchal girl, many of the common benign etiologies of elevated CA-125 are absent and therefore, an elevated CA-125 and a persistent adnexal mass raises concern for malignancy. Two thirds of ovarian malignancies in girls under 18 years of age are of germ cell origin.1
Epithelial tumors, which account for most ovarian neoplasms in adults, are rare in children.6
In general , for children with adnexal masses, CA-125 should not be routinely obtained, as it will not affect the indication for surgical exploration of persistent masses and elevations in CA-125 may discourage ovarian conservation even in benign conditions such as ovarian torsion.
Ovarian torsion is most commonly seen in menstruating women, but cases have been described in neonates and premenarchal girls.7
In a prior study, torsion was the most common diagnosis leading to ovarian surgery in premenarchal girls. Misdiagnosis of this condition can result in unnecessary oophorectomy. The predominant cause of ovarian torsion in children is thought to be functional cysts and benign neoplasms. Only two cases of malignancy have been described associated with ovarian torsion.7
Detorsion, with or without concominant oophoropexy has been used successfully to preserve ovarian function in both adults and children, even when the ovary appears necrotic on gross examination.8
CA-125 can be highly elevated with ovarian torsion. In one report of two adult torsion patients with endometriosis the CA-125 value rose from the 40’s to the 500–600 range.4
Expected CA-125 levels in children with torsion have not been reported.
In conclusion this case represents the first reported association of an elevated CA-125 and ovarian torsion in a pediatric patient. In addition, this benign finding allowed attempting a conservative ovarian-sparing approach during the surgery even in the presence of a highly elevated CA-125.