Case 496 -- A 9-year-old girl with recurrent bilateral ovarian masses

Contributed by Akosua B. Domfeh MBChB and Ronald Jaffe, MB.BCh
Published on line in February 2007


The patient is a 9-year-old girl who presented with a two-year history of abdominal pain, increasing abdominal girth and fatigue. On physical examination she was Tanner stage 0 with palpable abdominal masses.

A CT scan revealed multiloculated, multicystic masses arising from the pelvis to the level of the umbilicus and ovarian in origin. She underwent a laparoscopic decompression of the cysts together with biopsy of the cyst wall. The cytology was benign and the cyst wall was diagnosed as "luteinized follicular cysts" with a diagnostic note that since the cysts were large and bilateral, a non-ovarian endocrine effect was possible. After two months, the ovarian cysts recurred larger than before (CT scan, Figure 1) and were removed laparoscopically. These cysts were diagnosed as multiple luteinized cysts with prominent granulosa cell layer. Outside expert consultation was sought because of the clinical concern of their rapid recurrence. The expert consultant diagnosed the cysts as "multicystic juvenile granulosa cell tumors". The in-house diagnosis was amended accordingly. Four months after her initial presentation, a bilateral salpingo-oophorectomy was performed. Post surgery she was started on chemotherapy (Cisplatin, VP-16 and Bleomycin). At the time of her first chemotherapy administration she was noted on admission to have symptoms of clinical hypothyroidism including dry skin, obesity, cold intolerance, constipation and short stature.

An endocrine panel was performed which confirmed her to be hypothyroid.

She was started on Synthroid.


The specimen from her bilateral salpingo-oophorectomy consisted of a 6.4 x 5.2 x 3.1 cm, 49.0 g, multicystic left ovary (Figure 2) with a 4.7 cm X 1.0 cm tan-pink edematous segment of fallopian tube with edematous fimbriae and a 7.5 x 5.5 x 3.0 cm, 71.0 g, multicystic ovary with a 5.0 cm x 1.0 cm, edematous and congested pink-gray segment of fallopian tube (Figure 3). The surfaces of both ovaries were white, gray and smooth with a few focal hemorrhages. The cut surfaces showed large multiple tense cysts containing light yellow and clear fluid. The walls of the cysts varied from thin to fairly thick. The cut surfaces of the solid component of both specimens showed tan-light gray, glistening tissue and focal dark red hemorrhages (Figures 4 and 5). A few of the cysts had a bright yellow lining.


Both ovaries revealed a thin serosal lining and an expanded and edematous cortex with some stroma with relatively small numbers of primary oocytes (Figure 6). Luteinized stromal cells were seen occasionally throughout the ovary. Many cysts, some of which had a thickened and an expanded granulosa cell layer surrounded by a thicker interna with some luteinization occupied the ovaries (Figure 7 and 8). In some of these areas, there was a relatively prominent apoptotic rate and scattered mitosis (Figure 9 and 10) and the lining stained intensely for CD99 (Figure 11) and inhibin (Figure 12).


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