Sivasankaran Sujatha, Itam Paul, Ayensu-Coker Leslie, Sanchez Judith, Egler Rachel A, Anderson Matthew L, Brandt Mary L, Dietrich Jennifer E
Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX 77030, USA.
J Pediatr Adolesc Gynecol. 2009 Oct;22(5):e114-7. doi: 10.1016/j.jpag.2008.08.001. Epub 2009 Jul 3.
Juvenile granulosa cell tumors (JGCT) are rare ovarian tumors that frequently present with precocious puberty. Presentation in infants less than a year of age is also rare.
We describe a 10-month-old infant who presented with both premature thelarche and adrenarche due to JGCT. Laboratory evaluation revealed classic elevation of estradiol and inhibin B, and less classic elevation of total and free testosterone. Oophorectomy and staging resulted in a diagnosis of Stage IA JGCT.
Survival rates are >95% among patients diagnosed under 10 years of age. Tumor recurrence is rare but can occur as late as 48 months. Therefore, tumor surveillance is warranted for patients with even a Stage IA JGCT and involves monitoring serial inhibin B levels along with intermittent imaging.
青少年颗粒细胞瘤(JGCT)是罕见的卵巢肿瘤,常表现为性早熟。在1岁以下婴儿中出现的情况也很罕见。
我们描述了一名10个月大的婴儿,因JGCT出现乳房过早发育和肾上腺功能初现。实验室检查显示雌二醇和抑制素B典型升高,总睾酮和游离睾酮非典型升高。卵巢切除术和分期诊断为IA期JGCT。
10岁以下诊断的患者生存率>95%。肿瘤复发罕见,但可在48个月时出现。因此,即使是IA期JGCT患者也需要进行肿瘤监测,包括监测连续的抑制素B水平以及间歇性影像学检查。