Juvenile granulosa cell ovarian tumor-a rare presentation

Juvenile granulosa cell tumor is a rare gynecologic malignancy. A fourteen year old girl was brought to the emergency with acute abdominal pain with distension of abdomen and admitted in surgical unit, clinically suspected perforation of gut. Transabdominal ultrasonography revealed a right adenexal irregular mass with a moderate amount of ascitic fluid. On laparotomy, it was suspected malignant ovarian tumor stage Ic, and then ipsilateral salphingoophorectomy was done considering the age of the patient. It was juvenile Granulosa cell tumor which confirmed by histo-pathologically. The epidemiology, natural history, presentation, histological and imaging appearances, prognosis and treatment of this malignancy were reviewed.


Introduction
Juvenile granulosa cell tumors (JGCT) are rare sex cord stromal tumors.Granulosa cell tumors (GCT) are encompassing 1.5% of all ovarian tumor and 6% of its malignant tumors. 1 The juvenile type accounts for only about 5% of all GCT. 2 Only 5% of GCT cases are observed before puberty and more than 60% cases occurring after menopause. 2he terms are adult and JGCT because the tumors are not defined by the age groups in which they primarily occur, rather they are distinguished by their histological appearance, that correlates to large extent with the age of the patient. 2 This patient aged 14 years with presentation of acute abdomen needed high index of suspicion to have a such tumor and correct diagnosis will be necessary for selection of proper treatment.

The case
A fourteen year old unmarried and school going young girl was presented to the emergency with acute abdominal pain with distension of abdomen for 5 days.She got admitted in surgery unit.She had been previously healthy and with history of regular menstruation.On physical examination, she had below average body build, mildly anaemic, temperature normal, no palpable lymph node.Abdomen was hugely distended, no palpable lump.Clinically, perforation of gut was suspected at first.Transabdominal ultrasound of whole abdomen was performed-there was a

Mediscope
The Journal of GMC  4 Clinically, these patients typically present with sign of hyperestrogenism-precocious puberty, menstrual irregularities, post menopausal bleeding.In addition, patient may complain of abdominal pain or an abdominal mass. 1 G r oss appearance of these tumors-bilateral at 2%, ruptured in initial discovery in 10%, ascities in 10% of cases, size ranges 3-32 cm, appearance may be solid and cystic neoplasm, cysts containing hemorrhagic fluid, solid is of yellow-gray color with extensive necrosis or hemorrhage. 1 Fig. 1 shows some histological changes of ovarian follicles in JGCT such as presence of lutienization, cells with immature nuclei, atypia and sign of high mitotic rate.Call-Exner bodies grooved with pale, round nuclei are the classic features of the more common adult type tumor, and the lack of those features also helps to distinguish the juvenile type from adult type. 10,11 ging characteristics of adult and juvenile GCT are non-specific and these tumors can not be reliably distinguished from other ovarian neoplasm on imaging alone.They have multiple septations which are thick and irregular. 11Metastasis, though rare at initial presentation, appears as cystic liver masses or peritoneal implants, similar to epithelial ovarian neoplasm. 11 The staging system used for GCT is that applied to other ovarian epithelial tumors, the FIGO staging.Unfavorable prognostic factor may include nuclear atypia, high mitotic rate, and extra capsular extension of tumor within ovary, tumor rupture and presence of residual disease after surgery. 1,12,13Tumor markers such as inhibin can be used to assess for recurrences.
As the majority of these tumors are diagnosed at stage I, their treatment remains surgical.The role of chemotherapy and/or radiation therapy remains unclear.Given the 1.

Fig. 1 .
Fig. 1.Histopathological slide of juvenile granulosa cell tumor of ovary shows nuclei are immature show atypia and a high mitotic rate.
Staging determines which patients are at risk for recurrence and those that require chemotherapy.At presentation, 90% of JGCT are FIGO stage I, confined to possibility for recurrence, long term follow up and surveillance is needed for all patients.theovary.This stage caries very favorable prognosis and a 5 year survival rate of 90-100%.
11JGCT is less likely to occur after resection of tumor in stage I, but if recurs, is more likely rapid & aggressive with early relapses and poor outcomes. 11