Juvenile granulosa cell tumor associated with Maffucci syndrome in pregnancy: A case report


      • Juvenile granulosa cell tumor (JGCT) is extremely rare in the general population, but it is a diagnosis that must be considered in patients with Maffucci syndrome and Ollier disease.
      • JGCT is hormonally sensitive and exhibit rapid growth during pregnancy, therefore, early diagnosis is vital.
      • Tumor markers have to be interpreted with caution during pregnancy. As a result, imaging plays an important role in the diagnosis of JGCT.


      Juvenile granulosa cell tumor (JGCT) is an extremely rare ovarian tumor that has been associated with Maffucci syndrome. It both secretes hormone and has been postulated to grow in response to hormone. We present a case of a 33-year-old G1P0 asymptomatic woman with a history of Maffucci syndrome found to have a left adnexal mass on routine ultrasonography at 13 weeks gestation. This case demonstrates the sonographic and magnetic resonance imaging (MRI) features of JGCT, as well as the natural progression of the tumor during pregnancy. A follow-up ultrasound 3 weeks after initial diagnosis demonstrated marked growth in size and vascularity of the tumor, prompting unilateral salpingo-oophorectomy. Histopathological findings confirmed the diagnosis of JGCT.


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