Tumors of the ovary presenting with diameters greater than 10 cm are referred to as giant ovarian cysts . These are rarely seen in high-income countries and consequently are rarely described in the literature, owing to availability of resources and advanced imaging technologies leading to early diagnosis of small or medium-sized tumors .
These tumors are generally asymptomatic at early stages, causing symptoms only after reaching enormous dimensions, and consequently are diagnosed late in low- and middle-income countries (LMICs) . Compressive symptoms or a visible abdominal mass are the most frequent presenting complaints .
The surgical management of these masses is associated with many life-threatening complications, which arise predominantly after surgery owing to rapid changes in body circulation, and with pulmonary edema. The former include severe hypotension, increased venous return, cardiac failure, respiratory failure, and intestinal distention .
We report a case of a 48-year-old woman with a giant mucinous cystadenoma who presented with massive abdominal distention and whose surgical management resulted in fatal complications. We aim to remind clinicians of the challenges in diagnosing, managing, and precautions to be taken when performing surgery for patients with giant ovarian cysts.
A 48-year-old, para 5, postmenopausal black African woman who was human immunodeficiency virus (HIV)-negative presented to our casualty department with a 5-year history of progressive abdominal swelling. Five years prior to presenting, which was 1 year before she reached menopause, she had noticed that her abdomen was gradually distending. Her symptoms were associated with constipation, feeling of incomplete rectal emptying, early satiety, vomiting, and urinary frequency and urgency. She did not have any chronic illnesses and had a negative personal and family history of ovarian, uterine, bowel, and breast cancers. She was not receiving any medication prior to this presentation. She had delivered five children by cesarean section, and they were all alive and well. She lived in a rural area and was a subsistence farmer. She did not smoke and did not drink alcohol.
2013 Tumour marker results
Lactate dehydrogenase (LDH)
120250 U/L high
Serum -human chorionic gonadotropin
Carcinoembryonic antigen (CEA)
037 U/ml high
Cancer antigen 125 (CA 125)
Results of investigations showed microcytic anemia with hemoglobin of 9.9g/dl, white blood cell count of 7103/l, and platelet count of 250103/l. She had normal urea and electrolytes and hypoalbuminemia of 21g/L, with results of the rest of her liver function tests being normal. Urinalysis did not show abnormalities.
Her case was discussed by a multidisciplinary team (MDT) that included gynecological oncologists, radiation oncologists, general surgeons, anesthetists, and nursing staff. The MDT considered the risks of death following hemodynamic instability and bleeding as well as the postoperative risks of deep vein thrombosis, pulmonary embolism, difficulty weaning off the ventilator, and death. The conclusion of the meeting was to take the patient for staging laparotomy despite these risks.
Preoperatively, the patient and her relatives were counseled on the possible complications. The patient received a transfusion with 2 Units (U) of packed cells (PCs). The team also contemplated drainage of the mass preoperatively but was unable to secure appropriate drains to drain the thick green fluid from the mass.
We report a case of a 48-year-old woman with a giant mucinous cystadenoma who presented with massive abdominal distention and whose surgical management resulted in fatal complications. Benign mucinous cystadenomas make up the majority (81%) of mucinous tumors. These tumors can grow to extremely large sizes and are among the largest of any recorded tumor in the body , as was the case with our patient. They usually occur as large multicystic masses with mucus-containing fluid, presenting commonly in women in their 20s to 40s. The large size can itself be suggestive of mucinous histology. Our patients presentation was pathognomic: massive abdominal distention caused by the huge mass with associated symptoms of compression of adjacent organs, constipation and rectal tenesmus from colonic compression, early satiety, and vomiting from gastric compression, frequency and urgency from increased pressure on the bladder, ureteral obstruction with the hydroureter seen on a USS, and lower limb edema from pressure on the draining veins and lymphatic vessels [4,5].
This patient presented to the tertiary hospital 5years after being referred from the district hospital. In low-resource settings, late presentation is a common feature due to socioeconomic factors as well as cultural beliefs and fear of surgery leading most to present when the symptoms become unbearable . It is therefore imperative that community health workers be involved in scouting and follow-up of community members with unusual abdominal swellings.
Among the tumor markers measured in our patient, lactate dehydrogenase (LDH), carcinoembryonic antigen (CEA), and cancer antigen 125 (CA 125) were elevated. At the time the tests were done, the patient was 43 years old and premenopausal. The CA 125 assay is unreliable in differentiating benign from malignant ovarian masses in premenopausal women because of the increased rate of false-positive results and reduced specificity . When CA 125 is less than 200U/ml as in our patient, further investigations are appropriate. LDH, -human chorionic gonadotrophin, and -fetoprotein should be measured in all women under age 40 with a complex ovarian mass because of the possibility of germ cell tumors . In postmenopausal women, CA 125 should be used for primary evaluation because it allows the Risk of Malignancy Index of ovarian cysts to be calculated . However, CA 125 should not be used in isolation, because it is nonspecific for ovarian cancer. There is not enough evidence to suggest that panels including multiple tumor markers will offer any further advantage in the initial assessment of ovarian cysts in postmenopausal women, because all the markers show low sensitivity and wide variation in specificity when used in isolation or in combination with CA 125; hence, their routine use is not recommended . The elevated CEA in our patient supported the diagnosis because it is more likely to be elevated in 88% of cases vs 19% of nonmucinous ovarian carcinomas .
Our patients case displays the shortfalls of transabdominal USS as an imaging modality for the assessment of suspected ovarian masses. Although a pelvic ultrasound is the single most effective way of evaluating an ovarian mass, transvaginal ultrasonography is preferable because of its increased sensitivity over transabdominal ultrasound. A transabdominal USS should not be used in isolation; it should be used to provide supplementary information to transvaginal ultrasound, particularly when the ovarian cyst is large . Our patients case shows the utility of a CT scan if malignant disease is suspected, because it was able to define the large cystic mass.
As in our patients case, most mucinous tumors (about 79%) are unilateral. An algorithm was proposed in which a tumor greater than 10 cm correctly predicted primary ovarian origin in 82% of cases .
Our team considered aspiration of the tumor prior to surgery. However, some authors state that aspiration of abdominal cysts should be avoided because this could cause complications such as infections, bleeding, cyst rupture, increased peritoneal adhesions, or possible dissemination of malignant cells. They therefore consider preoperative and intraoperative drainage unsafe . In the past, it was thought that pseudomyxoma peritonei resulted from mucinous ovarian tumors, but now it is believed to be derived universally from appendicular low-grade (adenomatous) mucinous tumors; the ovarian involvement is secondary . Other surgeons advocate controlled fluid aspiration intraoperatively to reduce hypotension and low caval reload related to sudden drop of abdominal pressure. Draining the mass is an extreme option for selected unresectable cases with high wall tension .
The gold standard of treatment of any suspected ovarian mass includes intact removal of the involved adnexa with intraoperative pathological evaluation, typically laparotomy, total hysterectomy, bilateral salpingo-oophorectomy, and staging procedure, including lymphadenectomy . Surgery in patients with such huge masses has a high risk, as evidenced by the complications experienced by our patient. Fatal complications described in the literature include pulmonary and cardiac failure, pulmonary embolism, and sepsis. In one case with good prognosis, part of the patient preparation prior to surgery included lung preparation to improve pulmonary function postsurgery . This may involve incentive spirometry to breathe deeply and exercise the lungs before and after surgery [11,12].
Adequate supportive care while resecting giant ovarian masses is of the utmost importance. Postoperatively, reducing ileus, providing respiratory support, supporting abdominal wall tension, and monitoring hemodynamic parameters are key . After tumor excision, an abdominal wall reconstruction might be necessary because of the laxity and redundancy of the skin . We planned reconstructive surgery of the anterior abdominal wall after initial postoperative recovery because the patient was unstable at primary surgery.
When the abdomen is entered surgically, care should be taken to remove the ovary intact without spillage of the cyst contents, because rupture of a stage I mucinous ovarian carcinoma may increase its potential for recurrence. Benign mucinous cystadenomas are by definition confined to the ovary, and no further procedure is required. Therefore, had a frozen section been available, this diagnosis would possibly have enabled an oophorectomy to be performed, and the hysterectomy that contributed to additional theater time could have been avoided. Consideration also needs to be given to the fact that though benign, borderline to invasive disease may exist as a continuum in a cystadenoma. Only a limited number of sections can be evaluated intraoperatively because these tumors are quite large; hence, this may contribute to inaccuracies in diagnosis with frozen sections. Although appendectomy was previously performed for any ovarian tumor with mucinous histology, including benign lesions, current data do not support the practice .
The mass was not weighed, because it ruptured and was drained intraoperatively. A review of 20 cases with lesions exceeding 20kg confirmed a low malignancy rate . Other authors also concur, stating that huge intra-abdominal tumors that almost double a patients body weight can hardly be malignant .
Benign mucinous cystadenomas can grow to massive sizes, particularly in LMICs, and provide a huge surgical management challenge. Transvaginal ultrasound should be employed early to clearly delineate the ovaries, particularly in resource-limited nations. Community health workers in developing countries must be involved in scouting and follow-up of community members with unusual abdominal swellings to avoid delays in care.
- CA 125:Cancer antigen 125
- CEA:Carcinoembryonic antigen
- CT:Computed tomography
- HIV:Human immunodeficiency virus
- ICU:Intensive care unit
- LDH:Lactate dehydrogenase
- LMICs:Low- and middle-income countries
- MDT:Multidisciplinary team
- PCs:Packed cells
- USS:Ultrasound scan