Summary

The ovarian remnant syndrome, a complication of bilateral salpingo-oophorectomy, has progressively received more attention in gynecological surgery literature, in recent years. The syndrome is manifested by pelvic pain, and a palpable or sonographic pelvic mass. In rare cases, patients may present with large masses. Cystic masses or carcinomas are reported to develop in some of the ovarian remnant syndromes after surgery.

We hereby present a 69-year-old white female, complaining of abdominal pain, an enlarging abdominal mass, pollakuria, and urinary incontinence with duration of 1 year, 25 years after bilateral salpingo-oopherectomy. Clinical and radiological evidence of a mesenteric cyst was discovered. The final pathological diagnosis was mucinous cystadenoma in an ovarian remnant. Curiously, this patient had no history of endometriosis, previous pelvic or abdominal surgery excl. hysterectomy, pelvic inflammatory disease, inflammatory bowel disease, dense pelvic adhesions, or difficulty encountered during the previous hysterectomy. This tumor is the largest among all other ovarian remnants published in the international literature.

Women with complaints of abdominal or pelvic mass and /or pain with a history of total abdominal hysterectomy- bilateral salpingo-oophorectomy must be searched carefully and it must be kept in mind that ovarian remnant syndrome can develop without predisposing potential risk factors.

Introduction

Ovarian remnant syndrome (ORS) refers to a condition occuring in women who have undergone bilateral salpingo-oophorectomy, with or without hysterectomy, in which a remnant ovarian tissue is left behind[1].

Ovarian remnant syndrome is not the same entity as residual ovarian syndrome in which an ovary intentionally left in place during gynecologic surgery, eventually causes pelvic pain[2].

An ovary can usually be removed without difficulty. However when it is attached to other pelvic organs, or the pelvic wall, some of the cortex may adhere to peritoneal surfaces of these structures[3]. Risk factors associated with incomplete removal of an ovary and subsequent development of ORS include a history of endometriosis, pelvic inflammatory disease, previous multiple abdominal or pelvic surgeries and pelvic adhesive disease[2,4]. In addition, intraoperative conditions (eg, intraoperative bleeding, anatomical variation, or deviation from proper surgical principles) may contribute to incomplete removal of the ovaries[1]. Cortical tissue, which has been separated from its major blood supply, may undergo necrosis, cystic degeneration, or neoplastic changes, or it may remain functional, as Shemwell and Weed demonstrated in their experiments with cats in 1970[5]. The condition in which the detached ovarian tissue remains functional is called the ovarian remnant syndrome[3]. Patients most frequently present with chronic pelvic pain, pelvic pain associated with a pelvic mass, or an asymptomatic pelvic mass[1].

Case Presentation

A 69-year-old woman, Gravida: 3, Para: 2, and D&C: 1, comprised the study case. She had a clinical history of total abdominal hysterectomy and bilateral salpingo-oophorectomy which was performed for leiomyoma uteri, 25 years earlier. She had menopausal symptoms (vasomotor symptoms) for only two years after total abdominal hysterectomy and bilateral oophorectomy (TAH-BSO). After that period, the symptoms subsided spontaneously, despite the fact that she had not received hormone replacement therapy (HRT). She had no clinical history of endometriosis, pelvic inflammatory disease, previous pelvic and/or abdominal surgeries, inflammatory bowel disease, or pelvic adhesive disease. She presented to the gynecology clinic in July 2003 with complaints of abdominal pain, an enlarging abdominal mass, pollakuria, and also urinary incontinence, which had occurred in the previous year.

Laboratory findings: Normal blood biochemistry and a slight degree of anemia (Hb: 11.3 mg/dl, Htc: 34.1%) was detected. Tumor markers were not searched for.

Computerized tomography revealed a 28x35x20 cm, thin walled cystic mass between the portal hilus and symphysis pubis which filled the entire peritoneal cavity. This cystic mass had resulted in compression of the abdominal organs posteriorly. The urinary bladder was particularly compressed infero-posteriorly.

The pre-operative diagnosis was a mesenteric cyst.

Intraoperative findings: Status post-TAHBSO. The pedunculated cystic mass originated from the rectosigmoid junction, and was not attached to visceral organs. The cyst contained 13 liters of yellow unclear fluid. A portion of the fluid was sent to the pathology laboratory for intra- operative cytological examination. Cytological examination revealed macrophages filled with mucinous material.

Pathological findings:
Macroscopical findings: A pedunculated, oligolocular, smooth, and thin walled cyst measuring 35x25x20 cm. Its content was drained during the surgery, and its residual content was mucoid material (Figure 1).

Figure 1: Macroscopic appearance of a cystic mass. Thin walled, unilocular cyst, 35 cm in its largest diameter.

Microscopical findings: Mucinous cystadenoma with a single layer of mucinous epithelium. In the epithelium no crowding, stratification, hyperchromasia, or mitotic activity were seen (Figure 2). Mucin lakes were seen in luminal spaces. Focally ovarian stroma and corpus albicans were seen (Figure 3). The final histological diagnosis was a mucinous cystadenoma derived from an ovarian remnant tissue.

Figure 2: Single layer of columnar cells with abundant intracellular mucin and small basilar nuclei. There is no stratification, hyperchromasia, or mitotic activity (HE x40).

Figure 3: Ovarian tissue and corpus albicans, within wall of a cyst. Single layer of columnar cells with small basilar nuclei (HE x10).

Discussion

Ovarian remnant syndrome is a fairly uncommon condition. However its incidence has probably increased in the past four decades[2].

The pre-operative and operative diagnosis of our case was mesenteric cyst. Primary tumors and cysts of the mesentery are quite rare[6]. Reports of mesenteric cysts originated from ovarian tissue have been discussed under various titles. Wharton reported that in 1875 and 1887, reports had been published of women who continued to menstruate after bilateral oopherectomy[6]. In 1903, Malcolm attributed this phenomenon to incomplete removal of the ovaries. In 1953, a case of intestinal obstruction due to the presence of a corpus luteal cyst within the mesentery of the terminal ileum was reported. This patient had undergone a bilateral oopherectomy[6].

Hormonally active remnants manifest with variable presentations, including pelvic and/or flank pain, dyspareunia, hydronephrosis due to ureteral compression, dysuria, bowel obstruction, painful defecation, and a palpable, tender pelvic mass.

The clinicopathological features of ovarian remnants reported to date in the literature are summarized in Table 1 [7,9,10,11,12,13,14]. The largest series of ovarian remnants was reported from Mayo Clinic by Magtibay MP, et al[1]. One hundred and eighty-six ORS patients were evaluated. Their mean age was 37.6 years (range, 20-73 years). The most common indications for BSO were endometriosis (57%), an ovarian mass (9%), PID (pelvic inflammatory disease) (7%), or other pathological conditions (28%) which were surgically managed during hysterectomy. The mean numbers of laparotomies and laparoscopies patients had undergone before BSO were 1.4 (range, 0-8) and 0.77 (range 0-10), respectively. In previously mentioned series 147 (79%) of the 186 patients, at least 1 abdominal surgery had been performed before BSO. Histologically, remnant ovarian tissue was associated with corpus luteum in 78 (42%), endometriosis in 54 (29%), follicular cyst in 12 (7%), simple cyst in 45 (24%), cystadenofibroma in 3 (2%), serous cystadenoma in 2 (1%), and cystadenoma in 1 (0.5%) case respectively.

Table 1: Clinicopathological features of ovarian remnants reported in the literature.

Symptoms commonly present within 5 years after extirpative surgery[7]. Our patient began to experience these symptoms 24 years after extirpative surgery. The majority of women have identifiable cystic structures on ultrasonography, commonly measuring between 3 and 4 cm in their largest diameters (6). To date the largest tumor reported in the literature was 26 cm which was conclusively diagnosed as invasive mucinous carcinoma[7,8]. Our patient’s tumor was 35 cm in its largest diameter.

Most patients with ORS are generally young and menopausal at the time of BSO. The ovarian tissue remnants in these women often remain functional and continue to secrete substantial concentrations of hormones, including estradiol. Therefore, a young woman with ORS who is not immediately started on estrogen replacement therapy (ERT) after a BSO, often lacks the typical menopausal symptoms observed after abrupt estrogen withdrawal. This clinical information may provide the clinician with clues to the diagnosis of ORS. In the recent series of Mayo Clinic, 37% of patients have not showed any symptoms of estrogen deprivation despite no ERT[1]. Our patient had menopausal symptoms for only two years after TAH-BSO and those symptoms had resolved spontaneously, without receiving ERT.

In our case, the mucinous cystadenoma that developed from an ovarian remnant had the largest dimensions among all the tumors developed in ovarian remnant syndrome reported in the literature.

Tumors that have a preoperative diagnosis of mesenteric cyst in women with a history of TAH-BSO could be ovarian remnants. Therefore, these cysts must be examined carefully in the pathology laboratory and proper sampling is necessary to show residual ovarian tissue.

Women with complaints of an abdominal or pelvic mass and/or pain with a history of TAH-BSO, must be evaluated carefully and it must be kept in mind that the ovarian remnant syndrome can develop without predisposing potential risk factors.

Reference

1) Magtibay PM, Nyholm JL, Hernandez JL, Podratz KC. Ovarian remnant syndrome. Am J Obstet Gynecol 2005;193:2062-2066.

2) Magtibay PM, Magrina JF. Ovarian remnant syndrome. Clin Obstet Gynecol 2006;49:526-534.

3) Muram D, Drouin P. Ovarian remnant syndrome. Can Med Assoc J 1982;127:399-400.

4) Price FV, Edwards R, Buchsbaum HJ. Ovarian remnant syndrome: difficulties in diagnosis and management. Obstet Gynecol Surv 1990;45:151-156.

5) Shemwell RE, Weed JC. Ovarian remnant syndrome. Obstet Gynecol 1970;36:299-303.

6) Payan HM, Gilbert EF. Mesenteric cyst-ovarian implant syndrome. Arch Pathol Lab Med 1987;111:282-

7) Dereska NH, Cornella J, Hibner M, Magrina JF. Mucinous adenocarcinoma in an ovarian remnant. Int J Gynecol Cancer 2004;14:683-686.

8) Narayansingh G, Cumming G, Parkin D, Miller I. Ovarian cancer developing in the ovarian remnant syndrome. A case report and literature review. Aust N Z J Obstet Gynaecol 2000;40:221-223.

9) Steege JF. Ovarian remnant syndrome. Obstet Gynecol 1987;70:64-67.

10) Nezhat CH, Seidman DS, Nezhat FR, Mirmalek SA, Nezhat CR. Ovarian remnant syndrome after laparoscopic oopherectomy. Fertil Steril 2000;74:1024-1028.

11) Lafferty HW, Angioli R, Rudolph J, Penalver MA. Ovarian remnant syndrome: Experience at Jackson Memorial Hospital, University of Miami, 1985 through 1993. Am J Obstet Gynecol 1996;174:641-645.

12) Hamid R, May D. Ovarian malignancy in remnant ovarian tissue. Int J Gynecol Obstet 1997;58:319-320.

13) Burke M, Talerman A, Carlson JA, Bibbo M. Residual ovarian tissue mimicking malignancy in a patient with mucinous carcinoid tumor of the ovary. Acta Cytol 1997;41:1377-1380.

14) Mahdavi A, Kumtepe Y, Nezhat F. Laparoscopic management of benign serous neoplasia arising from persistent ovarian remnant. J Min Inv Gynecol 2007;14:654-656.