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Last updated: 7/18/2024
Years published: 2024
NORD gratefully acknowledges Lauren Walters and Nikita Baral, MD candidates, Creighton University School of Medicine and Adrienne Perfilio, MD, Department of Obstetrics and Gynecology, Creighton University School of Medicine, for assistance in the preparation of this report.
Summary
Ovarian remnant syndrome (ORS) is seen in patients who have previously had both ovaries or fallopian tubes removed (salpingo-oophorectomy). It is caused when ovarian tissue is unintentionally left behind in surgery. This leftover tissue may still act like an ovary and produce hormones but can also develop ovarian diseases like cysts or other benign or cancerous growths. This tissue may also enlarge, putting pressure on surrounding structures. Symptoms of ORS might include pelvic pain, a pelvic mass, painful intercourse, abnormal uterine bleeding, or bladder/bowel problems. The diagnosis is based on hormonal evaluation and pelvic imaging. Treatment usually consists of surgically removing the remnant tissue.
The remnant ovarian tissue may grow into and interfere with surrounding structures. If this occurs, symptoms depend on which structure is affected. Some patients have a painless pelvic mass found during routine pelvic examination or imaging, but most patients have one or more of the following symptoms:
It is also possible for the ovarian tissue to develop cancerous growths. Symptoms of ovarian cancer include abdominal fullness, pelvic pain, early satiety, weight loss and/or vaginal bleeding.
ORS typically occurs within the first five years after a salpingo-oophorectomy but can happen at any time. It is caused by the incomplete removal of the ovary during surgery. The remnant tissue can continue to grow from hormonal stimulation, leading to a pelvic mass and associated symptoms.
Current research illustrates that laparoscopic (minimally invasive surgical approach involving a camera and multiple, smaller incisions) salpingo-oophorectomies are associated with a lower risk of developing ORS compared to a transvaginal (incision made through the vagina) or laparotomy (large, open abdominal incision) approaches. This is most likely due to better visualization of the reproductive structures with laparoscopic cameras.
Patients with a past medical history of endometriosis, pelvic inflammatory disease, inflammatory bowel disease, or gynecologic cancers, as well as patients with a history of prior abdominal or gynecological surgery are at greater risk for developing ORS. These factors predispose patients to the formation of adhesions. Adhesions make it more challenging to completely visualize and remove the ovaries during surgery.
Ovarian remnant syndrome is an extremely rare diagnosis with an unknown incidence.
Those affected by ORS are typically middle aged or elderly females with a history of a total hysterectomy, bilateral salpingo-oophorectomy, or unilateral salpingo-oophorectomy.
Diagnosis may be difficult because the remnant ovarian tissue is never intentionally left behind. Because of this, the physician may not initially think to look for ovarian causes of symptoms. It is important for the physician to first take a careful patient history, so they are aware of patient risk factors for ORS.
Laboratory Evaluation
Premenopausal patients
Patients who are premenopausal are usually started on estrogen replacement therapy after a bilateral salpingo-oophorectomy to prevent symptoms of early menopause. Estrogen replacement therapy makes it impossible to evaluate patient hormone levels accurately, so supplemental hormones must be stopped for at least 10 days prior to hormone analysis. Once the patient has stopped taking hormone replacement therapy for an adequate period of time, the physician will evaluate estradiol and follicle stimulating hormone (FSH) levels. If ORS is the cause of symptoms, these levels will reflect those of someone who still has ovarian tissue.
Postmenopausal patients
Patients who are postmenopausal should no longer have hormone production from the remnant ovary, so laboratory evaluation would not be useful. Please see the imaging evaluation section.
All patients
It is important to rule out malignancy in patients with remnant ovarian tissue. Prior to surgery a physician may order a blood sample to test for certain chemicals that are specifically released by tumor cells.
Imaging Evaluation
A pelvic US, CT scan, or MRI, or a combination of these, will likely be obtained. These images will show a pelvic mass corresponding to the leftover ovarian tissue.
Pharmacologic Evaluation
In premenopausal patients, if ORS is suspected but imaging studies are inconclusive, a physician may prescribe a course of clomiphene citrate. This medication stimulates ovarian tissue to grow, therefore, increasing the likelihood of seeing the remnant tissue on imaging. This method is not used in people who have undergone menopause.
Treatment
The treatment of choice for ORS is surgery to remove the remnant tissue. However, depending on the patientโs age and other medical conditions, it may be reasonable to manage ORS with medication.
Surgery to remove the remaining ovarian tissue may be laparoscopic or open, depending on the patientโs specific case and the surgeonโs preference. Laparoscopic surgery typically has a faster recovery time, and some research suggests more favorable outcomes with a laparoscopic approach.
Medication therapy may be used in patients who are not good candidates for surgery (i.e. those with significant heart or lung disease). There are a variety of medications that can be prescribed, but all of them work to suppress ovarian function. Unfortunately, many patients do not have much improvement with medication therapy alone.
Radiation therapy and ovarian artery embolization have sparingly been used to treat ORS. There is currently not much data on these treatment methods, but there may be more information on their efficacy in the future.
It is important for the physician to rule out cancerous growth of the remnant tissue. If cancer is found in the ovary, it should be treated accordingly.
Information on current clinical trials is posted on the Internet at https://clinicaltrials.gov/ All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
http://www.centerwatch.com/
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
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Fu SC, Su HY. Residual ovarian syndrome: A case report with classic symptoms, imaging and pathology findings, and treatment. Taiwan J Obstet Gynecol. 2018;57(5):753-754. doi:10.1016/j.tjog.2018.08.027
Benton A, Deimling T, Pacis M. et al. Ovarian remnant syndrome: a retrospective evaluation of surgical management. Gynecol Surg. 2016; 13, 353โ357. https://doi.org/10.1007/s10397-016-0988-7.
Kho RM, Abrao MS. Ovarian remnant syndrome: etiology, diagnosis, treatment and impact of endometriosis. Curr Opin Obstet Gynecol. 2012;24(4):210-214. doi:10.1097/GCO.0b013e3283558539
Kho RM, Magrina JF, Magtibay PM. Pathologic findings and outcomes of a minimally invasive approach to ovarian remnant syndrome. Fertil Steril. 2007;87(5):1005-1009. doi:10.1016/j.fertnstert.2006.12.075
Kho RM, Magrina JF, Magtibay PM. Pathologic findings and outcomes of a minimally invasive approach to ovarian remnant syndrome. Fertil Steril. 2007;87(5):1005-1009. doi:10.1016/j.fertnstert.2006.12.075
Magtibay PM, Magrina JF. Ovarian remnant syndrome. Clin Obstet Gynecol. 2006;49(3):526-534. doi:10.1097/00003081-200609000-00012
Allen DG. The retained ovary and the residual ovary syndrome. Aust N Z J Obstet Gynaecol. 1998;38(4):446-447. doi:10.1111/j.1479-828x.1998.tb03108.x
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