Tumor de las células de granulosa: pubertad precoz en lactante menor de 1 año. Caso clínico

Introduction: 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. Objective: Present an infant with peripheral precocious puberty, diagnosis of JGCT and follow up. Clinical case: 10-monthold female infant with thelarche, pubic hair and palpable abdominal mass accompanied with elevated 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, antimullerian Hormone (AMH) and estradiol as described in this type of tumors. Conclusions: Juvenil granulosa 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.