Recent Advances in Granulosa Cell Tumor Ovary: A Review

Abstract

Granulosa cell tumors constitute less than 5 % of all ovarian tumors. Unlike epithelial ovarian tumors, they occur in a younger age group, are usually detected in an early stage and often have features of hyperestrogenism. The presenting symptoms are usually nonspecific with abdominal pain or distension. They follow an indolent course and are characterized by a long natural history. Mutation of FOXL2 (402C->G) seen in 97 % of adult GCT may be pathognomonic for adult GCT. Only stage of the disease has been consistently shown in various studies to affect survival of patients with GCT. The initial management of patients, for whom fertility is not an issue, is total abdominal hysterectomy, bilateral salpingo-oophorectomy and removal of all gross disease. Nodal dissection is not a significant factor for survival and is not recommended in surgical staging of GCT. Fertility preserving surgery with unilateral salpingooophorectomy is feasible in young patients with stage Ia GCT. Patients with early stage disease (stage I and II) have a very good prognosis with 5 year DFS and OS of 89 % and 99 % respectively and these groups of patients usually don't require any postoperative treatment. Patients with stage Ic disease associated with poor prognostic factors like large tumor size or high mitotic index and stage II, have a higher chance of relapse, and may benefit with postoperative treatment but role of chemotherapy is still debatable. In advanced stage disease (stage III and IV) the 5 year DFS and OS disease was 72 % and 80 % respectively hence the option of postoperative treatment with 6 cycles of BEP should be considered in this group. Recently paclitaxel is being investigated as an effective tool in GCT. The efficacy of radiation in GCT is not well defined but in optimally debulked cases postoperative radiation is a viable option. Due to high chance of recurrence even years after apparent clinical cure of the primary tumor, lifelong follow up with clinical examination and tumor markers like inhibin B is recommended. About 25 % GCT develop recurrence and the median time to recur is usually 4-5 years. Most recurrences are intraperitoneal and usually a complete debulking of the disease is feasible even in the recurrent setting. Postoperative chemotherapy (platinum based) is usually given after surgery more so in cases with widespread disease or after suboptimal cytoreduction. Recurrent chemoresistant, progressive non-responding GCT or patients with high surgical risk are ideal candidates for targeted therapy.

Parameters for Predicting Granulosa Cell Tumor of the Ovary: A Single Center Retrospective Comparative Study

“…There are two different clinical and histopathological types of GCTO including juvenile and adult type. The adult type forms more than 95% of all GCTO and is usually seen in perimenopausal and postmenopausal women with a peak incidence between 50-55 years (Kottarathil et al, 2013).…”

May Lymphadenectomy Be Omitted in Granulosa Cell Tumors of the Ovary?

“…Any type of hyperplasia could be detected in 25-50% of cases and also endometrial adenocarcinoma in 5-10% of cases; these endometrial carcinomas are generally welldifferentiated, early stage carcinomas [8]. For our patient group no endometrial carcinoma had been detected.…”

Single tertiary care centre experience of ovarian granulosa cell tumour in Chennai, India: a retrospective analysis

“…However, late relapse often occurs due to an indolent disease course, and the prognosis of advanced disease is poor, with a 5-year survival rate of 0% to 20%, comparable to that for epithelial ovarian cancer. 4 Prognostic factors for Granulosa Cell tumours have been reported previously and include age, tumor size, tumor rupture, tumor stage, bilaterality, postoperative residual tumour status and high mitotic index (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15). However, prognostic factors for Granulosa Cell tumour and it's recurrent nature remain unclear, due to rarity and indolent course of the disease.…”

“…Investigational agents in relapse setting include antiangiogenic therapy (Bevacizumab), LHRH agonists (Leuprolide) and mTOR inhibitors. 14 The Overall survival is good with approximately 90 % at 5 years for early stage disease. The most important prognostic variable is Stage.…”