Ovarian Borderline Tumors Treatment (PDQ®)–Health Professional Version

Incidence and Mortality

Ovarian borderline tumors (i.e., tumors of low malignant potential) account for 15% of all epithelial ovarian cancers. Nearly 75% of borderline tumors are stage I at the time of diagnosis.[1] Recognizing these tumors is important because their prognosis and treatment is different from the frankly malignant invasive carcinomas. Ovarian borderline tumors can also become low-grade serous tumors. For more information, see the Treatment of Low-Grade Serous Carcinoma section in Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment.

A review of 22 series (which included 953 patients), with a mean follow-up of 7 years, revealed a survival rate of 92% for patients with advanced-stage ovarian borderline tumors, if patients with so-called invasive implants were excluded. The causes of death in these patients were determined to be benign complications of disease (e.g., small bowel obstruction), complications of therapy, and only rarely (0.7% of patients), malignant transformation.[2] In one series, the 5-, 10-, 15-, and 20-year survival rates of patients with borderline tumors (all stages), as demonstrated by clinical life table analysis, were 97%, 95%, 92%, and 89%, respectively.[3] In this series, mortality rates were stage dependent: 0.7% of patients with stage I tumors, 4.2% of patients with stage II tumors, and 26.8% of patients with stage III tumors died of disease.[3] The Fédération Internationale de Gynécologie et d’Obstétrique reported an extremely good prognosis for ovarian borderline tumors, with a 10-year survival rate of approximately 95%.[1] These survival rates are clearly in contrast with the 30% survival rate for patients with invasive tumors (all stages).

Another large retrospective study showed that early stage, serous histology, and younger age were associated with more favorable prognoses in patients with ovarian borderline tumors.[4]

Endometrioid Tumors

Endometrioid borderline tumors are less common and should not be regarded as malignant because they seldom, if ever, metastasize. However, malignant transformation can occur and may be associated with a similar tumor outside of the ovary. Such tumors are the result of either a second primary or rupture of the primary endometrial tumor.[5]

Fédération Internationale de Gynécologie et d’Obstétrique (FIGO) Staging

FIGO and the American Joint Committee on Cancer have designated staging to define ovarian borderline tumors; the FIGO system is most commonly used.[1,2]

Table 1. Definitions of FIGO Stage Ia

Stage	Definition	Illustration
FIGO = Fédération Internationale de Gynécologie et d’Obstétrique.
aAdapted from FIGO Committee for Gynecologic Oncology.[1]
I	Tumor confined to ovaries or fallopian tube(s).	Enlarge
IA	Tumor limited to one ovary (capsule intact) or fallopian tube; no tumor on ovarian or fallopian tube surface; no malignant cells in the ascites or peritoneal washings.
IB	Tumor limited to both ovaries (capsules intact) or fallopian tubes; no tumor on ovarian or fallopian tube surface; no malignant cells in the ascites or peritoneal washings.
IC	Tumor limited to one or both ovaries or fallopian tubes, with any of the following:
	IC1: Surgical spill.
	IC2: Capsule ruptured before surgery or tumor on ovarian or fallopian tube surface.
	IC3: Malignant cells in the ascites or peritoneal washings.

Table 2. Definitions of FIGO Stage IIa

Stage	Definition	Illustration
FIGO = Fédération Internationale de Gynécologie et d’Obstétrique.
aAdapted from FIGO Committee for Gynecologic Oncology.[1]
II	Tumor involves one or both ovaries or fallopian tubes with pelvic extension (below the pelvic brim) or primary peritoneal cancer.	Enlarge
IIA	Extension and/or implants on uterus and/or fallopian tubes and/or ovaries.
IIB	Extension to other pelvic intraperitoneal tissues.

Table 3. Definitions of FIGO Stage IIIa

Stage	Definition	Illustration
FIGO = Fédération Internationale de Gynécologie et d’Obstétrique.
aAdapted from FIGO Committee for Gynecologic Oncology.[1]
III	Tumor involves one or both ovaries or fallopian tubes, or primary peritoneal cancer, with cytologically or histologically confirmed spread to the peritoneum outside the pelvis and/or metastasis to the retroperitoneal lymph nodes.
IIIA1	Positive retroperitoneal lymph nodes only (cytologically or histologically proven):	Enlarge
	IIIA1(I): Metastasis ≤10 mm in greatest dimension.
	IIIA1(ii): Metastasis >10 mm in greatest dimension.
IIIA2	Microscopic extrapelvic (above the pelvic brim) peritoneal involvement with or without positive retroperitoneal lymph nodes.
IIIB	Macroscopic peritoneal metastasis beyond the pelvis ≤2 cm in greatest dimension, with or without metastasis to the retroperitoneal lymph nodes.	Enlarge
IIIC	Macroscopic peritoneal metastasis beyond the pelvis >2 cm in greatest dimension, with or without metastasis to the retroperitoneal lymph nodes (includes extension of tumor to capsule of liver and spleen without parenchymal involvement of either organ).	Enlarge

Table 4. Definitions of FIGO Stage IVa

Stage	Definition	Illustration
FIGO = Fédération Internationale de Gynécologie et d’Obstétrique.
aAdapted from FIGO Committee for Gynecologic Oncology.[1]
IV	Distant metastasis excluding peritoneal metastases.	Enlarge
IVA	Pleural effusion with positive cytology.
IVB	Parenchymal metastases and metastases to extra-abdominal organs (including inguinal lymph nodes and lymph nodes outside of the abdominal cavity).

Treatment Options for Early-Stage Ovarian Borderline Tumors

Treatment options for early-stage ovarian borderline tumors include:

Importance of Staging for Treatment

The value of complete staging has not been demonstrated for patients with early-stage disease, but the opposite ovary should be carefully evaluated for evidence of bilateral disease. The impact of surgical staging on therapeutic management has not been defined. However, in a study of 29 patients with presumed localized disease, 7 patients were upstaged after complete surgical staging.[2]

In two other studies, 16% and 18% of patients with presumed localized borderline tumors were upstaged as a result of a staging laparotomy.[3,4] In one of these studies, the yield for serous tumors was 30.8%, compared with 0% for mucinous tumors.[3]

In another study, patients with localized intraperitoneal disease and negative lymph nodes had a low incidence of recurrence (5%), whereas patients with localized intraperitoneal disease and positive lymph nodes had a statistically significant higher incidence of recurrence (50%).[5]

Fertility Preservation

When a patient wishes to retain childbearing potential, a unilateral salpingo-oophorectomy is adequate therapy.[6,7] In the presence of bilateral ovarian cystic neoplasms, or for patients with a single ovary, a partial oophorectomy can be used to preserve fertility.[8] Some physicians stress the importance of limiting ovarian cystectomy to patients with stage IA disease whose cystectomy specimen margins are tumor free.[9]

In a large series, the relapse rate was higher for patients who underwent more conservative surgery (cystectomy > unilateral oophorectomy > total abdominal hysterectomy and bilateral salpingo-oophorectomy [TAHBSO]). However, differences were not statistically significant, and the survival rates were nearly 100% for all groups.[5,10] When childbearing is not a consideration, a TAHBSO is appropriate therapy. Once a patient's family is complete, most, but not all,[9] physicians favor removal of remaining ovarian tissue as there is risk of recurrence of a borderline tumor or, rarely, a carcinoma.[3,6]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Treatment Options for Advanced-Stage Ovarian Borderline Tumors

Treatment options for advanced-stage ovarian borderline tumors include:

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of ovarian borderline tumors. It is intended as a resource to inform and assist clinicians in the care of their patients. It does not provide formal guidelines or recommendations for making health care decisions.

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PDQ® Adult Treatment Editorial Board. PDQ Ovarian Borderline Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/ovarian/hp/ovarian-borderline-tumors-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389466]

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