February 22, 2017
6 min read

Evolution of surgical management optimizes care for patients with rare gynecologic tumors

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Editor’s Note: This column is a three-part series intended to offer oncologists practical advice on the treatment of rare gynecologic malignancies. Part 1, published here, outlines advances in surgical management. Part 2 will address adjuvant therapy for rare ovarian tumors and part 3 will address rare diseases of the uterus and cervix. They will be published in subsequent issues.

Physicians tasked with treating patients with rare gynecologic cancers face a formidable challenge in that they must provide the most current diagnostic and therapeutic solutions based on limited information.

Significant progress has been made in the treatment of rare gynecologic cancers over the past decade, and clinicians should be aware of these advances in order to provide optimal patient care.

Jubilee Brown, MD
Jubilee Brown
Erin K. Crane, MD
Erin K. Crane
R. Wendel Naumann, MD
R. Wendel Naumann

An estimated 107,470 new gynecologic cancers will be diagnosed in 2017, and approximately 31,600 women will die of these malignancies this year. Rare gynecologic cancers have been variably defined, but they tend to be the histologies responsible for less than 5% to 10% of cancers in a given anatomic site.

Rare tumors may have a predilection for certain age groups. For example, germ cell tumors of the ovary — which represent less than 10% of all ovarian cancers — are the predominant histologic subtype of cancer in adolescents and young adults aged younger than 25 years, but they almost never occur in women aged older than 35 years.

Risk factors for some rare gynecologic tumors may be identified in certain circumstances, such as a history of pelvic radiation for uterine sarcoma. Most often, there are no specific risk factors, and diagnosis is made when symptoms or signs prompt physician consultation. Diagnosis may be difficult on intraoperative evaluation, necessitating understanding of these rare tumors by both clinicians and pathologists.

Evolution of surgical approaches

Surgical treatment of gynecologic malignancies has evolved over the last decade.

The principles of surgical treatment of rare ovarian tumors include en bloc removal. Because the histology often is not known until after removal, this can be safely accomplished through minimally invasive surgery in most cases, with removal in containment bags to prevent spill.

Patients with apparent early-stage ovarian cancers who desire fertility are candidates for fertility-sparing surgery, in which the involved adnexa is removed and staging is performed, but the normal-appearing uterus and contralateral adnexa are preserved. Outcomes data support the safety of this approach, which is especially important in women with germ cell or sex cord–stromal ovarian tumors, as they tend to occur in women of reproductive age.

Minimally invasive surgery — either through a robotic or laparoscopic platform — has become the standard of care for women with other rare gynecologic malignancies, including rare histologies of early cervical and uterine cancer. Patients who undergo minimally invasive surgery have shorter recovery time, less blood loss, less pain and reduced hospital stay with equivalent outcomes.

The surgical approach to certain rare ovarian tumors has been refined. Sex cord–stromal tumors of the ovary — such as granulosa cell tumors and Sertoli-Leydig cell tumors — and mucinous tumors of the ovary do not require routine lymphadenectomy as a component of surgical staging, as the incidence of lymphatic metastases approaches zero. It should be noted that this exception applies only to these specific histologies.

There also has been robust discussion surrounding the extent of surgery required for malignant ovarian germ cell tumors, as pediatric oncologists only perform a limited resection due to the extreme chemosensitivity of these tumors. Gynecologic oncologists caring for adults continue to perform full staging procedures — including peritoneal biopsy, omentectomy, and pelvic and para-aortic lymphadenectomy — but this practice is being studied. It has become acceptable in patients with an incomplete staging procedure at the initial surgery to forgo a second surgery, instead opting for radiologic and serum marker evaluation followed by chemotherapy or surveillance, and early outcomes support this approach.


Minimization of surgery

Other advances in the surgical arena include the utility of lymphatic mapping with sentinel node identification to limit the morbidity of complete bilateral lymphadenectomy in patients with vulvar, cervical and endometrial cancers.

The advent of indocyanine green (ICG-10) has allowed a more user-friendly mapping technique that can be performed open, laparoscopically or with robotic assistance. Although this has not achieved status as standard of care, data are accumulating that promote this as a safe approach for these disease entities.

The process of uterine morcellation has come under scrutiny due to the risk for morcellating an undetected leiomyosarcoma. The FDA released a black box warning stating that the power morcellator should not be used in peri- or postmenopausal women, when malignancy is known or suspected, or when the uterus could be removed through a mini-laparotomy incision. This has spurred multiple publications quantifying the risk of morcellation and prompted innovative design to develop contained extraction systems.

Surgical modalities for the primary treatment of early cervical cancer have focused on minimizing the extent of surgery and preserving fertility, including robotic radical trachelectomy. In addition, two international randomized trials are evaluating the safety and outcomes of cone biopsy and pelvic lymphadenectomy with uterine preservation in early cervical cancers.

Finally, an improvement in the understanding of ovarian carcinogenesis has modified the approach to preinvasive lesions of the fallopian tube, serous tubal intraepithelial carcinoma, or serous tubal in situ carcinoma lesions. Previously thought to be a rare tumor, fallopian tube carcinoma may be the forerunner of most serous ovarian cancers. Therefore, patients with known BRCA mutations are recommended to undergo removal of their tubes and ovaries at age 40 years or when childbearing is complete.

To prevent ovarian, peritoneal or tubal cancer in women of population risk, the American Congress of Obstetricians and Gynecologists and Society of Gynecologic Oncology endorsed routine salpingectomy once childbearing is complete at the time of hysterectomy, in lieu of tubal ligation, or at the time of other pelvic surgery.


Minimally invasive surgery through laparoscopic or robotic platforms has become the standard of care for the treatment of most patients with early cervical and uterine cancers, and for select patients with early ovarian cancer. Fertility-sparing surgery plays a role in patients of reproductive age with early gynecologic malignancies.

Further, routine lymphadenectomy is not required for patients with sex cord–stromal or mucinous ovarian cancers. Prophylactic salpingectomy and salpingo-oophorectomy may prevent a significant portion of ovarian, peritoneal and tubal cancers.

Patient outcomes depend directly on appropriate referrals to gynecologic oncologists, who specialize in the surgical and medical management of women with gynecologic cancers and women at high risk for gynecologic cancers. Improved patient outcomes depend on patient care in high-volume centers and care by gynecologic oncologists.


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For more information:

Jubilee Brown, MD, is professor and associate director in the division of gynecologic oncology at Levine Cancer Institute at Carolinas HealthCare System. She can be reached at 1021 Morehead Medical Drive, Suite 2100, Charlotte, NC 28204; email: jubilee.brown@carolinashealthcare.org.

Erin K. Crane, MD, is a gynecologic oncologist at Levine Cancer Institute at Carolinas HealthCare System. She can be reached at erin.crane@carolinashealthcare.org.

R. Wendel Naumann, MD, is a gynecologic oncologist at Levine Cancer Institute at Carolinas HealthCare System. He can be reached at wnaumann@mac.com.

Disclosure: Brown, Crane and Naumann report no relevant financial disclosures.