Ascites as a Sole Presentation of Juvenile Granulosa Cell Tumor

A two years old girl presented with history of urinary retention since 2 months. It was associated with decrease urinary output and painful micturation since 1 month. She also had a history of gradual abdominal distention with mild abdominal pain. She is a product of consanguineous marriage with death 2 of her preceding sibling due to unknown reasons at age of 3 months and 2 years. Her general physical examination was normal. Her abdominal examination showed massive abdominal distention with positive fluid thrill and shifting dullness. A rounded, fixed mass felt at the pelvic area with smooth surface. Her rectal examination showed tenderness at the anterior rectal wall. She had normal breast and genetalia. Normal blood & urine tests. Abdominal ultrasound showed a mixed echogenicity mass measuring 11.5x6.7cm with both solid and cystic components arising from the pelvic area with gross ascites. Contrast enhanced CT scan shows massive ascites and a large, heterogenous, solid cystic mass in lower abdomen & false pelvis (11x8.1x7.5cm); originating from the left adenxa with no internal calcification. There was associated with abdomen-pelvic ascites. Rest of abdominal viscera, peritoneium and lymph nodes were unremarkable. Her Alfha fetoprotein and bHCG were normal. On exploratory Laprotomy 1 liter of Ascitic fluid was drained with samples sent for analysis. Left salpingoopherctomy was done as the mass was originating from the left ovary figure 1. Uterus, right ovary and lymph nodes were grossly normal. Peritoneal fluid cytology was negative for malignant cells. Grossly the mass was having mixed cystic and solid areas, weighting 1.3kg with some focal areas of hemorrhage figure 2. Histopathological examination Microscopic features show diffuse sheets and macrofollicular pattern of tumor cells. The malignant cells were polygonal and small. The cytoplasm was abundant and amphophilic. The nuclei were atypical without grooves and show conspicuous nucleoli at places. Mitotic activity was more than 10 cells/10 hpf. Large tumor cells with extensive lutenization were seen focally, presenting the cal component of ovarian stroma that is compatible with Juvenile granulosa cell tumor figure 3. Immuno-histo-chemistry was positive for Inhibin and CD99 and negative for EMA, CD117 and alpha feto-protein. Bone scan and chest radiographs were unremarkable. Based on FIGO staging system this patient is stage IA. On 3 months follow up patient is doing well and her urinary retention relieved, however she is on long term follow up due to late recurrence. Case Report Abstract