Juvenile Granulosa Cell Ovarian Tumor: A Case Report and Review of Literature

Abstract Background 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. Case We describe a 10-month-old infant who presented with both premature thelarche and adrenarche due to JG...

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Bibliographic Details
Published in:Journal of pediatric & adolescent gynecology 2009-10, Vol.22 (5), p.e114-e117
Main Authors: Sivasankaran, Sujatha, MD, Itam, Paul, MD, Ayensu-Coker, Leslie, MD, Sanchez, Judith, MD, Egler, Rachel A., MD, Anderson, Matthew L., MD, PhD, Brandt, Mary L., MD, Dietrich, Jennifer E., MD, MSc
Format: Article
Language:English
Subjects:
Adrenarche
Age Determination by Skeleton
Female
Granulosa cell
Granulosa Cell Tumor - diagnosis
Granulosa Cell Tumor - diagnostic imaging
Granulosa Cell Tumor - pathology
Granulosa Cell Tumor - surgery
Humans
Immunohistochemistry
Infant
Obstetrics and Gynecology
Ovarian Neoplasms - diagnosis
Ovarian Neoplasms - diagnostic imaging
Ovarian Neoplasms - pathology
Ovarian Neoplasms - surgery
Ovariectomy
Pediatrics
prepubertal
Puberty, Precocious - etiology
tumor marker
Ultrasonography
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Summary:Abstract Background 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. Case 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. Summary and Conclusion 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.
ISSN:1083-3188
1873-4332
DOI:10.1016/j.jpag.2008.08.001