Lacourt Patricia, Soto Julio, Rumié Hana, Gejman Roger, Pattillo Juan Carlos, García Cristián, García Hernán
Hospital Dr. Sótero del Río, Chile.
Universidad de Concepción, Concepción, Chile.
Rev Chil Pediatr. 2017 Dec;88(6):792-797. doi: 10.4067/S0370-41062017000600792.
Juvenile granulosa cell tumors (JGCT) are very rare, especially in infants under the age of one. The most frequent presentation is with signs of precocious puberty.
Present an in fant with peripheral precocious puberty, diagnosis of JGCT and follow up.
10-month-old female infant with thelarche, pubic hair and palpable abdominal mass accompanied with eleva ted levels of estradiol, very low gonadotrophins and images that show a very large ovarian mass. A sapingooforectomy was carried out with full regression of symptoms and signs and improvement of laboratory exams. The biopsy showed TCGJ so inhibin B (InB) was taken as tumoral marker after surgery. This hormone was high initially, but rapidly declined. Follow-up was based on InB, antimu-llerian Hormone (AMH) and estradiol as described in this type of tumors.
Juvenil gra nulosa cell tumors are very infrequent in pediatric age, but should be suspected in girl with peripheral precocious puberty. The majority of cases improve with surgery, but strict surveillance of tumoral markers is needed. The most specific markers are inhibin B and anti mullerian hormone (AMH), followed by estradiol levels.
青少年颗粒细胞瘤(JGCT)非常罕见,尤其是在1岁以下的婴儿中。最常见的表现是性早熟迹象。
介绍一名患有外周性性早熟的婴儿,诊断为JGCT并进行随访。
一名10个月大的女婴出现乳房发育、阴毛生长及可触及的腹部肿块,伴有雌二醇水平升高、促性腺激素水平极低,影像学检查显示卵巢有一个非常大的肿块。进行了患侧附件切除术,症状和体征完全消退,实验室检查结果改善。活检显示为JGCT,因此术后将抑制素B(InB)作为肿瘤标志物。该激素最初较高,但迅速下降。按照此类肿瘤的描述,基于InB、抗苗勒管激素(AMH)和雌二醇进行随访。
青少年颗粒细胞瘤在儿童期非常罕见,但外周性性早熟的女孩应怀疑患有此病。大多数病例通过手术可改善,但需要严格监测肿瘤标志物。最具特异性的标志物是抑制素B和抗苗勒管激素(AMH),其次是雌二醇水平。