87. Small Case Series of Neonatal Autoamputated Ovary Presenting as Enlarging Complex Ovarian Mass with Increasingly Elevated AFP

Abstract

<h3>Background</h3> We present a case series of two girls with enlarging complex ovarian masses associated with increasing levels of alpha-fetoprotein (AFP). Both girls were found to have an autoamputated ovary. Tumor markers associated with benign ovarian masses are usually within the normal range. Abnormally elevated AFP levels in the neonates with large complex ovarian masses should be surgically evaluated. This case series could suggest an association between elevated AFP and ovarian autoamputation. <h3>Case</h3> The first patient was a term female baby delivered without complications and known to have a complex cyst in utero. She was a thriving baby girl and her physical exam was unremarkable. Post-natal pelvic ultrasound revealed a 62 × 40 mm enlarging right ovarian cyst with a vascularized nodule. Imaging was suggestive of a mature teratoma or a germinal tumor. Tumor markers were significant for elevated levels of AFP up to 373 mcg/L. Repeat ultrasound showed a persistent ovarian cyst. The second patient was a 7-month-old female with a known history of a right neonatal ovarian cyst followed with serial ultrasounds. She appeared healthy and her exam was unremarkable. A pelvic ultrasound revealed a 44 × 53 mm ovarian cyst with echogenic debris. Tumor markers were significant for an elevated AFP level of 695 mcg/L. Given the elevated AFP and persistence of the cysts, surgical excision were performed. Both diagnostic laparoscopies revealed autoamputated right ovary and fallopian tube and right salpingoophorectomies were performed. Surgeries were uneventful. The pathology exam of the first specimen showed necrotic ovarian tissue with benign cystic lesion of unknown origin, without neoplasia. The second pathology specimen analysis showed a torsed hemorrhagic cyst with no evidence of malignancy. The AFP levels normalized subsequently. <h3>Comments</h3> Prenatal ovarian cysts are frequent and tend to regress in the first months of life. Complex cysts require follow-up with serial ultrasounds and tumor marker measurements. Large or complex cysts may require surgical intervention due to suspicion of ovarian torsion or malignancy. Perinatally, increased AFP levels are associated with hepatic or germ cell tumors. Suggested reference level of AFP is 15.7 to 146.5 mcg/L. Although autoamputated ovaries are thought to be normal, on pathology, it is difficult to identify ovarian tissue. Elevated AFP levels found in this case series are somewhat difficult to interpret since pathology analysis was limited by necrosis. Nonetheless, abnormally elevated AFP levels in the neonates with large complex ovarian masses should be surgically evaluated. Further research is needed to investigate the possible association of elevated AFP levels and ovarian autoamputation.