Growing teratoma syndrome of the ovary: review of literature and first report of a carcinoid tumor arising in a growing teratoma of the ovary.

We report the first case of a secondary tumor arising from a peritoneal nodule of mature teratoma in a patient with growing teratoma syndrome (GTS) of the ovary. The patient originally presented 19 years ago with an immature teratoma of the ovary and positive retroperitoneal lymph nodes. After surgery and chemotherapy, mature teratomas recurred as abdominal and pelvic masses after 1, 6, and 19 years. Upon the last recurrence, a trabecular carcinoid tumor developed in a mature teratoma associated with the liver. This case illustrates the importance of long-term follow-up for patients with GTS of the ovary, where the recurrent masses can appear many years after the primary tumor, compress the abdominal and pelvic structures and give rise to secondary neoplasms. In addition, we present a literature review of GTS of the ovary and some novel observations about this entity. On the basis of our review of ovarian GTS cases in the literature, we have found that ovarian GTS nodules tend to appear for the first time within 2 years of the initial primary. They remain confined almost exclusively to the pelvis, abdomen, and the retroperitoneum and do not venture to distant systemic sites. This new information may help identify and screen women with germ cell tumors of the ovary at risk for GTS.