and Jyothi G Seshadri2
This section is written with a general/laparoscopic gynecologist in mind, most of whom would not want to operate on malignancies, but come across malignant ovarian tumors occasionally and unexpectedly, usually as a case of an adnexal mass or a suspected leiomyoma which turns out to be malignant. Ovarian tumors with ascites and/or pleural effusion, omental cake, and very high CA-125 levels are always malignant (unless, it is a rare case of Meig’s syndrome where there is pleural effusion, ascites, and it is due to a benign ovarian fibroma [1]), and such instances are usually not associated with unexpected findings on the operating table. Benign conditions with extensive adhesions or those cases where a benign appearing tumor turns out to be malignant on table are the situations which can be a challenge for a gynecologist.
A skilled surgeon may be capable of operating on a complicated case very easily and confidently, but to get the best results during surgery, every surgeon, whether skilled or a beginner, must keep in mind a few points during the preoperative evaluation of the patient. A few “simple” mistakes can ruin the best of intentions.
One of the commonest on table surprises a gynecologist can have is a leiomyoma (suspected and confirmed on imaging) turning out to be an ovarian tumor. The possibility of an ovarian tumor being malignant or borderline should be kept in mind till the definitive histopathology report becomes available even if other features are suggestive of a benign tumor. Borderline tumors are a separate entity with a unique behavior of their own, they can recur after removal, and can uncommonly undergo malignant transformation [2]. Even small ovarian tumors with no sold cystic areas, or ascites, can turn out to be malignant. Tumor marker levels can be normal preoperatively in a good number of malignant ovarian tumors. Frozen section should be kept ready before operating on every case of ovarian tumor, though even in the best of centers the correlation between frozen section and final histopathology is not 100% [3]. If the presence of an ovarian tumor is a surprise finding (e.g., if the preoperative diagnosis was leiomyoma), then one can go ahead with the procedure even if there is no frozen section available. But inform the patient (once she is out of anesthesia and is feeling better) and relatives that restaging might be necessary should the final histopathology report show malignancy or a borderline tumor. This can be the case even when frozen section is available, since there can be a discrepancy between frozen section and the final histopathology reports.
If there are features of malignancy on table, or if consent for loss of fertility has not been taken, close the abdomen and plan a staging procedure at a later date. Remember that surgery is a diagnostic tool, and one should not be embarrassed to have opened the abdomen just to find out what the disease is. Explain this confidently and patients will understand.
For this reason, whenever an ovarian/adnexal mass is encountered by a gynecologist in outpatient consultation, a per rectal examination to assess the nodularity and fixity is a must. Upper GI endoscopy to rule out any possibility of GI malignancy and the ovarian mass being a metastatic tumor should be considered if clinical features are suggestive. Krukenberg tumors of the ovary are rare [4]. They are metastatic ovarian tumors with stomach carcinoma, colorectal carcinoma, breast carcinoma, being the commonest primary malignancies. Metastatic tumors of the ovary can also be due to hematogenous malignancies. They are usually bilateral with the ovaries being moderately enlarged with an irregular surface and generally free of adhesions. Ascites is present in nearly half of the cases. CA-125 levels are raised though not very grossly. Thus they can be mistaken to be a simple case of an ovarian tumor. Though ovarian metastasectomy may have a role in certain cases, Krukenberg tumors have a poor prognosis [4, 5]. Therefore, it would be wise to do a thorough preoperative assessment and not be in a hurry to operate. It is important to do a breast examination as a part of routine gynecological examination since breast lesions are common and can actually be the primary in the rare event of the ovarian tumor being a metastatic tumor. Also, peripheral smear is important and should be done as a part of CBC. The ovarian tumor can be a metastatic tumor due to a primary hematogenous malignancy.
A normal CA-125 can be a feature of early malignancy before the tumor has spread in a good number of patients [6]. Germ cell tumors are more likely in young girls and ideally all tumor markers-CA-125, alpha-fetoprotein, LDH, and beta hCG, should be done in all patients with an adnexal mass [7]. If a granulosa cell tumor is suspected, then an endometrial biopsy to rule out endometrial hyperplasia and carcinoma endometrium due to hyperestrogenic state should be done preoperatively [8, 9]. And if the granulosa cell tumor is detected for the first time on table by frozen section, then one should be prepared to expect endometrial hyperplasia or carcinoma endometrium also to be present. If the patient has completed her family and if the surgery planned includes hysterectomy, then one must immediately proceed to do the staging and completion of surgery. Granulosa cell tumors of the ovary are low-grade malignancies with a slow indolent growth and are known to secrete estrogen [8]. Carcinoma endometrium which can arise due to unopposed estrogen can be more aggressive than the granulosa cell tumor itself. If the patient is young and desires fertility, or if the consent has not been recorded before the start of the surgery, then one must close the abdomen and inform the patient about the need for repeat surgery for staging and completion of surgery. Fertility conserving options can be explored depending on the stage and grade.
If granulosa cell tumor is detected for the first time after the final histopathology report is ready, then again, the patient should be counseled about the need for staging and completion of surgery.
In fact, during preoperative evaluation, one must suspect the possibility of endometrial hyperplasia and carcinoma endometrium if there is a history of irregular and excessive or postmenopausal bleeding, or if there is an associated hyperestrogenic state like PCOD or anovulatory cycles. The imaging report should be carefully studied. Apart from noting findings like enlarged lymph nodes, loss of fat planes, etc., one must look for ET, and if this is significant for patient’s age, then a preoperative endometrial biopsy is indicated. Patients taking tamoxifen should also be counseled to report any vaginal bleeding promptly to rule out the possibility of endometrial hyperplasia and carcinoma endometrium [10].
The ovarian tumor (or whatever the gynecological condition which has been detected preoperatively) could be benign, but there could be complex atypical endometrial hyperplasia or carcinoma endometrium, which has been missed in the preoperative assessment.
All these measures will greatly help the gynecologist to avoid an intraoperative disaster. Remember that many times a fertility sparing surgery is what is being planned.
If the patient is a clear case of ovarian malignancy, then refer to or call a gynecologic/surgical oncologist to scrub in for surgery. Fertility-sparing surgeries in a patient with malignancy require a greater amount of documentation. It is better to close the abdomen after careful explanation to relatives than proceed with what might be a confused and a suboptimal surgery, with the patient herself not being involved in the decision-making.
Aspiration or FNAC is strongly discouraged since it will only upstage the tumor. It can however be done in a clear case of advanced ovarian tumor for the purpose of tissue diagnosis for neoadjuvant therapy [11].
Do the staging. Collect free fluid for cytology to rule out malignant cells. Flush the pouch of Douglas, the two paracolic gutters, and the two subdiaphragmatic spaces with saline and aspirate it for cytology. Take generous peritoneal biopsies and biopsies from any suspicious area or adhesions. This has to be done even if the tumor is certain to be benign. This can be done laparoscopically as well. If one is unable to remove all the visible evidence of the tumor, the residual disease can be removed by a repeat surgery at a later date (after neoadjuvant chemotherapy in cases of malignant ovarian tumor, or if the patient becomes symptomatic or has an increase in growth of the residual disease as in cases of endometriosis, leiomyoma, PID, etc.), but remember staging is something that is done only during the first surgery. So if staging is not done or done inadequately, then subsequent treatment will always be suboptimal if the tumor turns out to be malignant.
Do not cause rupture of an intact and well-circumscribed tumor. It might lead to upstaging should the tumor be malignant.
In case of ovarian torsion, it was earlier believed that once an ovarian tumor undergoes torsion, the blood supply and venous return get cut off. There will be anaerobic metabolism in cells and accumulation of lactic acid and toxic metabolites. Untwisting will release this into circulation. The more congested and purplish the tumor looks, more likely it is for it to contain toxic metabolites. However, recent studies suggest that detorsion can be safely done if ovarian function is desired. It can be done even if the twisted ovary looks swollen and congested [12]. However, if there is a tumor which needs to be removed, then there is no point in untwisting it. It should be removed intact and sent for frozen section.
Divide the round ligament, open the folds of broad ligament, locate the ureter, and then clamp, cut, and ligate the infundibulopelvic ligament. Ovarian tumors are particularly known for their proximity to the ureter, and one should not underestimate the importance of this step [13]. Remove the specimen and send it for frozen section, if available. And then proceed with the surgery (hysterectomy, if the patient is elderly/postmenopausal). If the frozen section report and the cytology report are positive for malignancy, then proceed with completion of surgery. If frozen section facility is not available, then it will take a week for the staging report and the definitive histopathology reports to arrive. Inform the patient and relatives, should the definitive histopathology show malignancy; then restaging with completion of surgery will be required.
Now let us study some photographs taken during surgery for ovarian tumor. In some cases, it was certain that the patient has a malignant ovarian tumor. In some other cases, it was a complete surprise on table. Sometimes even the presence of ovarian torsion can turn out to be a complete surprise.
Malignant Ovarian Tumor: A Surprise Finding (Fig. 5.1)
Fig. 5.1
Ovarian Tumors Can Be Huge! (Fig. 5.2a, b)
Fig. 5.2a
Burst abdomen is a serious complication with an incidence less than 5% in most centers [14]. It can occur even with small vertical and transverse incisions. It is associated with raised intra-abdominal pressures in the postoperative period and wound infection. The predisposing factors which cause postoperative increase in intra-abdominal pressure and wound infection need to be controlled in order to prevent burst abdomen.
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