Granulosa cell tumors are a sex cord-stromal tumor that represents one of the less common types of primary ovarian cancers. They can be divided into two types: adult onset or juvenile onset. Symptoms are related to excess hormonal production, the predominant hormone being estrogen. In adults, this commonly results in abnormal uterine bleeding or postmenopausal bleeding, and in the juvenile population can result in precocious puberty and abnormal uterine bleeding. In the case above, you have an infant with abnormal uterine bleeding and breast buds in the presence of an adnexal mass. The most likely diagnosis is a granulosa cell tumor for which the primary tumor marker is inhibin, which is produced by the granulosa cells. Granulosa cell tumors are typically confined to one ovary and present prior to metastasis. In the juvenile population or women who desire to maintain their fertility potential, a unilateral oophorectomy would be recommended with appropriate staging.

Alpha-fetoprotein (A) is more commonly elevated in germ cell tumors including endodermal sinus tumor, immature teratoma, and embryonal carcinoma. Germ cell tumors are more common in younger populations but given the infant’s symptoms above, granulosa cell tumor is most likely. CA 125 (B) is the tumor marker for non-mucinous epithelial ovarian cancers and is most useful when obtained in the postmenopausal population. Benign pathology such as endometriosis and fibroids can also result in an elevated CA 125. Lactate dehydrogenase (D) is classically associated with a dysgerminoma. It can also be elevated with an immature teratoma.