Granulosa theca cell tumors in premenarchal girls: A clinical and pathologic study of ten cases

Abstract

Granulosa theca cell tumor (GTCT) in the premenarche accounted for 7% of all ovarian tumors treated at the Children's Hospital Medical Center from 1928 through 1979. The average age of the ten girls at the time of diagnosis was 5 years (range 11 months-121/2 years) with precocious "pseudopuberty" and an abdominal mass being the most common presentation. GTCTs were solitary (five right, five left) with an average diameter of 12.1 cm. Histologic examination showed a predominantly diffuse or solid pattern with prominent luteinization; Call-Exner bodies and folded ("coffee-bean") nuclei were inconspicuous. Ultrastructural examination of one GTCT confirmed the presence of both granulosa and theca components with the latter showing extensive luteinization; estradiol, testosterone and prolactin were demonstrated in the same tumor using immunologic techniques. The average follow-up time was 21 years with nine of the ten patients being followed for more than ten years. Salpingo-oophorectomy resulted in cures despite the occurrence of tumor spillage in two patients. The prognosis for GTCTs in the premenarche appears more favorable than for those occurring in adulthood, but further study is needed to completely define their full biologic potential; the significance of subsequent breast cancer in two long-term survivors merits further investigation as well.

Tumeurs juvéniles de la granulosa : expression clinique et moléculaire

“…Microscopiquement, cinq sous-types sont décrits [42,43], dont le plus fréquent est microfolliculaire caractérisé par des corps de Call-Exner [44], des noyaux en « grain de café », des atypies cellulaires et des mitoses [32]. Ces éléments sont cependant plus fréquemment rencontrées dans la forme adulte de la maladie et ne sont pas présents dans la forme juvénile, ce qui permet d'en établir le diagnostic différentiel [5,7,45,46]. Les tumeurs de la granulosa expriment la vimentine, la cytokératine et l'antigène EMA [32].…”

Prognostic significance of FOXL2 mutation and mRNA expression in adult and juvenile granulosa cell tumors of the ovary

“…1,2 In both types, stage at the time of diagnosis is thought to be the most powerful factor predicting outcome. [3][4][5][6][7][8] Whereas the adult type requires long-term follow-up due to its tendency to late recurrences, juvenile granulosa cell tumors tend to be confined to the ovary and are associated with favorable prognosis. Only juvenile tumors extending beyond the ovary are associated with poor outcome.…”

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

“…Androgen secretion by some GCT tumors is associated with very dramatic clinical virilization in affected females. 4,7,8 In addition, anthropometric measurements may be significantly advanced. Other presenting symptoms or signs may be present.…”

“…0.1% of cases. 4,8 At the time of presentation, approximately 90% of JGCTs correspond to stage IA or IB , whereas approximately 3% present at stage II. Extension beyond the ovarian capsule and ascites is encountered in about 10% of cases.…”