What is the role of surgery in the treatment of granulosa cell tumors (GCTs) of the ovaries?

Updated: Aug 30, 2018
  • Author: David C Starks, MD, MPH; Chief Editor: Warner K Huh, MD  more...
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Answer

Standard of care for initial management of GCTs remains surgical. [14, 15] Surgical management allows for staging and tissue diagnosis.

Surgical management of patients who present with signs and symptoms concerning for GCTs begins with a thorough preoperative evaluation.

Preoperative imaging and laboratory studies are helpful for measuring the extent of disease permitting proper patient counseling (see Lab Studies and Imaging Studies).

Appropriate staging with intact removal of the tumor and optimal cytoreduction are the main goals of surgical therapy. Several studies have shown that FIGO stage is the most prognostic factor for granulosa cell tumors.

In a 2003 study, Uygun et al showed a definite survival benefit for patients with lower-stage tumors and for patients who had no residual disease at surgery (mean overall survival 108 mo) versus those with residual disease at the end of surgery (mean 42 mo, p = 0.001). [16]

Prepare patients for the possibility of bowel resection and/or ostomy placement if diffuse spread is suggested following the preoperative assessment. A mechanical bowel preparation, with or without antibiotics, should be used in all patients undergoing surgery for a pelvic mass.

Complete surgical staging consists of a thorough examination of the pelvic and intra-abdominal structures. If disease is identified outside the ovary, optimal debulking should be performed so that all remaining tumor nodules are smaller than 1 cm, but goal should still be complete resection of all visible tumor. Optimal tumor debulking improves overall survival and decreases recurrences.

In younger patients who desire future fertility, a unilateral salpingo-oophorectomy almost always provides sufficient treatment because most of these tumors are stage I (see Staging). Zanagnolo et al, in a review of 63 cases of sex cord stromal tumors, reported that conservative surgical management was performed in 23% of early stage tumors. No recurrences were noted and 5 out of 11 patients became pregnant. [17]

Staging should generally be performed and consists of pelvic washings, selective ipsilateral pelvic and bilateral periaortic lymph node sampling, peritoneal biopsies, partial omentectomy, and biopsy of the contralateral ovary (only if it appears abnormal). Previously, biopsy of the contralateral ovary was considered a routine part of the staging procedure but now is not required because only approximately 2% of tumors are bilateral and biopsy may lead to adhesion formation and subsequent problems with pain and/or fertility.

A retrospective study from MD Anderson has called into question the need for lymphadenectomy to be routinely performed as part of the standard staging procedure for GCTs due to the low risk of lymph node metastasis even in cases of advanced stage disease. Because hormone overproduction is common with GCTs, dilatation and curettage should be considered to help rule out a neoplastic process of the endometrium in younger patients undergoing fertility-sparing surgery, especially if abnormal uterine bleeding was part of their clinical presentation. [18]

A more recent study by Thrall et al supports the concept of avoiding lymphadenectomy. In their study, there were no lymph node metastases in 47 patients who had at least some lymph nodes removed, with a median lymph node count of 14 in 36 of these patients. However, 2 of 18 patients who recurred did not undergo initial nodal dissection. Moreover, they noted that 60% of patients who were stage II or higher had only microscopic extraovarian disease. [19]

Although data on the clinical utility of lymphadenectomy in sex cord stromal tumors is mounting, there are no uniform recommendations and there remains an important role for surgical staging/biopsy based on incidence of microscopic extraovarian disease.

For patients in whom future fertility is not a concern, surgical therapy should consist of bilateral salpingo-oophorectomy and total abdominal hysterectomy, in addition to the staging procedures.

Treatment of recurrent GCTs is not as uniform as it is for the primary tumors. Surgical debulking can be of value if the tumor appears to be focal on imaging studies. Chemotherapy, radiotherapy, and hormonal treatments have been used with variable success. All appear to have some benefit for improving long-term survival and the progression-free interval. Mean survival after a recurrence has been diagnosed is approximately 5 years for adult GCTs. [20]


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