Adult Granulosa Cell Tumor in Pregnancy: A New Case and a Review of the Literature

Abstract

Granulosa cell tumors are rare ovarian tumors that can arise during pregnancy. We present a new case of recurrent adult granulosa cell tumor (AGCT) in pregnancy and a systematic review of the literature. The new case described is a 41-year-old woman G5P1122 with a prior history of AGCT that was referred to our center at 29 weeks because of a symptomatic abdominal mass, compatible with a possible recurrence of AGCT. At 36 + 3 weeks, she underwent a cesarean delivery for preterm labor and a total hysterectomy with a radical surgical staging. A healthy female infant was delivered. The patient received a platinum-based chemotherapy, with a 26-month follow-up negative for recurrence. Analyzing our case with the four identified by the literature review, three were recurrent and two were primary AGCT. Only one required surgery for AGCT at 15 weeks, while another underwent chemotherapy in pregnancy. In the other three cases, surgery for AGCT was done at the time of cesarean delivery. There were three cases of preterm delivery. All five pregnancies resulted in the birth of live babies with weight adequate for gestational age. In conclusion, AGCT diagnosed in pregnancy is rare, reported in only five cases. All gave birth to live babies in the third trimester, and maternal outcome at up to 18 months showed no recurrence.

Keywords: fetal outcome; granulosa cell tumor; gynecology; maternal outcome; ovarian cancer; pregnancy; recurrence.

Figure 1

(A) Abdominal MRI axial T1-weighted spoiled gradient-echo post-Gd administration image showing a multiloculated anterior swelling of the uterine fundus, with a partially hematic liquid content. (B) Abdominal MRI sagittal T2-weighted image showing the mass extending cranio-caudally and the fetus in the gravid uterus.

Figure 2

The histology of the AGCT shows a diffuse-trabecular pattern with areas of oedema and luteinisation (A) composed by monotonous small cells with scant cytoplasm and nuclei with occasional grooves (B). At immunohistochemistry neoplastic cells are positive for inhibin (C), CD56 (D) and they exhibit a significant expression of estrogen (E) and progesterone (F) receptors. H&E (A,B) immunoperoxidase (C–F).

Figure 3

Cases of AGCT presenting or recurring in pregnancy were included, 4 from review of the literature [5,6,7,8] and our new case. Patients’ age ranged from 23 to 41. Three of the 4 cases reporting gravidity and parity were primigravid women. Three were primary, and 2 recurrent AGCT. In the 3 recurrent, previous surgery involved at least a salpingo-oophorectomy (Table 1).