Evaluative Approach

Patients may present with an adnexal mass in a variety of clinical settings. Some may be symptomatic (pelvic pain or pressure) as a result of the mass, others may have a mass identified as part of a workup for another condition (computed tomography [CT] scan performed for back pain), and some have a mass incidentally identified as part of a normal well-woman examination. In addition, masses are occasionally identified during pregnancy as a result of the number of prenatal ultrasounds that are performed. A patient with a mass is approached differently depending on the clinical scenario. The approach of what to do in the setting of adnexal mass can be broken down into two fundamental questions: (1) What is the suspicion that the mass is malignant? and (2) Is the patient symptomatic? Understanding these two issues helps focus treatment planning into a decision for surgery versus observation.

To determine the appropriate intervention for an adnexal mass, a thorough evaluation, including a complete history and physical examination, liberal use of transvaginal ultrasonography, and judicious use of serum tumor markers, should be performed. Pelvic examination should always be performed under optimal circumstances. The patient’s bladder should be empty. It is not unheard of for a patient with 10-cm midline mass to have the mass disappear with catheterization of the bladder. The rare pelvic kidney should always be kept in mind as a possible cause of a pelvic mass. Tragic reports of excision of such a mass in a patient with one kidney are found in the literature. Whenever possible, the rectum and rectosigmoid should also be empty when a pelvic examination is done. This avoids the misdiagnosis of fecal material as an adnexal mass. Detection of an adnexal mass is greatly facilitated by a rectovaginal examination (see Fig. 3.13 ), which allows more complete access to the cul-de-sac and to the more superficial areas of the pelvic basin. Knowledge of the size, shape, contour, and general location of the mass within the pelvis helps the physician arrive at the most likely diagnosis. Benign tumors are commonly smooth walled, cystic, mobile, unilateral, and smaller than 8 cm (7 cm is the exact diameter of a new tennis ball). Malignant tumors are usually solid or semisolid, bilateral, irregular, fixed, and associated with nodules in the cul-de-sac. Ascites usually is found with malignant neoplasms ( Table 10.2 ). Koonings and coworkers showed that the risk of malignant disease was 2.6-fold greater for women with bilateral neoplasms than for women with unilateral neoplasms.

The patient’s symptoms may derive from the physical nature of the mass by producing pressure against the bladder or rectum and increasing abdominal distention. Pain may be acute or chronic in nature. It may be a result of rapid size change and can be caused by torsion, hemorrhage into the mass, or rupture. Pain may also be a result of associated inflammatory processes from tubo-ovarian abscess or diverticular abscess. The nature and severity of the pain and other associated symptoms frequently lead to operative intervention, irrespective of the risk for malignancy.

Classifying the Mass

The complete evaluation of a patient with an adnexal mass requires that the physician assemble and analyze all of the available information from the history, examination, imaging studies, and tumor markers. One goal is to characterize the mass based on its malignant potential. Management then depends on a combination of many factors, including age and menopausal status of the patient, morphologic characteristics of the mass by ultrasonography, clinical findings, and patient desires. Classically, features associated most commonly with benign masses include young age, absence of symptoms (nausea, vomiting, weight loss, changes in urinary frequency, pain), unilateral and unilocular cystic masses, and normal cancer antigen 125 (CA-125) level. Despite these factors, it is clear that no single characteristic guarantees that a mass will be benign. Increasingly, models taking into account all available information have shown the best ability to predict benign versus malignant masses.

Of all variables, however, age is probably the most important factor for predicting the potential for malignancy. For example, Moore and colleagues showed in one large prospective series of 531 patients with adnexal masses for whom surgery was to be performed that 7% of premenopausal versus 39% of postmenopausal patients had a diagnosis of an epithelial ovarian malignancy on final pathology. The differential diagnosis of an adnexal mass varies with the age of the patient. In premenarchal girls and postmenopausal women, an adnexal mass should be considered highly abnormal and must be immediately investigated. In premenarchal patients, most neoplasms are germ cell in origin and require immediate surgical exploration. Stromal, germ cell, and epithelial tumors are seen in postmenopausal women. It was once dogma that a postmenopausal patient with any enlargement of the ovary be considered to have “cancer until proven otherwise.” In an era before pelvic ultrasonography, examining a patient with a “palpable postmenopausal ovary” was thought pathognomonic for ovarian cancer. Today, with common use of imaging studies, it has been shown that adnexal masses in postmenopausal women are more frequent than previously recognized, and many of these are benign. Greenlee and colleagues demonstrated in a prevalence study of more than 15,000 women older than 55 years of age who underwent pelvic ultrasonography that 14% had an adnexal cyst at initial screening. As with patients in all age groups, a complete evaluation can better characterize the risk of malignancy. (See section on postmenopausal ovary.)

One of the most common modalities used for characterizing an adnexal mass is pelvic ultrasonography. Pelvic ultrasonography may be performed transabdominally (better for larger masses extending out of the pelvis) or transvaginally (best for masses in the cul-de-sac or pelvis) to characterize masses. Many adnexal masses have characteristic appearances that define them as benign or suspicious for malignancy. The ultrasonography shows size, mass morphology, unilateral or bilateral involvement, and associated findings such as ascites. Ultrasonography is the most valuable initial tool and should be considered the first choice in assessing an adnexal mass by imaging. In some cases, CT or magnetic resonance imaging (MRI) may augment information on the mass and provide additional relevant anatomic information (eg, description of upper abdominal findings and information on adenopathy and ureteral patency). Masses may be described as purely cystic (so-called simple cyst), solid, or mixed solid–cystic (so-called complex cyst). The risk of malignancy increases with increasing complexity. Large series have shown that simple cysts have a very low risk of malignancy. Cysts with septations and no solid component also have been shown to have a low risk of malignancy. Malignancy is more commonly associated when a cyst wall or septation is thickened or has nodularity or the cyst contains solid components.

Much research has been devoted to developing accurate biomarkers that can detect malignancy and better characterize adnexal masses. To date, no single marker has been shown to be accurate in distinguishing a mass as benign or malignant. Information from tumor markers may be useful in characterizing the potential risk of malignancy so appropriate therapy may be offered (eg, surgery vs. observation), and patients may be appropriately triaged to specialists who are trained to perform complete surgical staging and debulking as needed.

One of the most widely used serum biomarkers for ovarian cancer is CA-125. CA-125 is used to monitor response of therapy and to assess for possible recurrence in patients with a known diagnosis of ovarian cancer. It is elevated in approximately 80% of patients with nonmucinous, epithelial ovarian cancers. It is elevated in only approximately 50% of stage I ovarian cancers, however. In combination with examination and serial pelvic ultrasonography, CA-125 has been evaluated in several large screening studies including asymptomatic women at risk for ovarian cancer to identify those with unrecognized disease. These studies show that CA-125 may augment the information from ultrasonography alone. CA-125 testing has limited sensitivity and specificity, making routine CA-125 testing problematic. Conditions associated with peritoneal or serosal inflammation or irritation of any etiology may be associated with CA-125 level elevations. In addition to ovarian cancer, CA-125 levels may be elevated by a number of other malignancies and benign conditions, including endometriosis, uterine leiomyomata, benign adnexal masses, tubal inflammation or infection, liver disease or hepatitis, congestive heart failure, menses, and pregnancy. Because most of these conditions are seen in younger women, CA-125 testing in premenopausal women is particularly difficult to interpret. A higher sensitivity has been reported in postmenopausal women, in whom CA-125 levels greater than 50 mL have been associated with a high risk of ovarian malignancy. In a prospective study comparing ultrasonography with CA-125 levels to characterize patients with persistent adnexal masses ( n = 1066), Van Calster reported ultrasound pattern classification correctly classified 93% of tumors as benign or malignant compared with 83% by CA-125 testing. Histologic diagnoses most commonly misclassified by CA-125 testing were fibromas, endometriosis, abscess, and borderline tumors. CA-125 information in the setting of an adnexal mass may provide information in conjunction with patient symptoms, examination, and imaging studies as to whether to offer observation or perform surgery, make referral to specialists, or perform additional testing.

The CA-125 to carcinoembryonic antigen (CEA) ratio is now being considered helpful in deciphering the site of neoplastic origin. CEA is generally a marker for gastrointestinal (GI) malignancy but is also a marker for the rare entity of mucinous ovarian cancer. A ratio greater than 25 is generally consistent with ovarian origin.

Differential Diagnosis

The differentiation between benign ( Table 10.3 ) and malignant ovarian enlargements is often the exclusive decision of the pathologist, but a short discussion of these lesions is pertinent even in a textbook of oncology. Although functional ovarian cysts are usually asymptomatic, they can on occasion be accompanied by a minor degree of lower abdominal discomfort, pelvic pain, or dyspareunia. In addition, rupture of one of these fluid-filled structures can result in additional peritoneal irritation and possibly an accompanying hemoperitoneum; however, this is rarely serious. More intense lower abdominal discomfort results when these ovarian tumors undergo torsion or infarction. Similar to functional cysts of the ovary, benign ovarian neoplasms do not produce any symptoms that readily differentiate them from malignant tumors or from various other pelvic diseases. Although these tumors are more likely to twist, resulting in infarction, malignant neoplasms may have the same fate. Indeed, one of the most unfortunate features of benign ovarian neoplasms is that they are indistinguishable clinically from their malignant counterparts. Although it is not known whether malignant ovarian tumors arise de novo or develop from benign tumors, there is strong inferential evidence that at least some benign tumors will become malignant. All too often, the first symptom of cancer in an ovarian tumor is increasing abdominal distention, although benign ovarian neoplasms may become apparent because of increasing abdominal girth, and indeed the “giant tumors” of the ovary are often benign mucinous cystadenomas. Benign mucinous cystadenomas weighing up to 300 lb have been reported.

Uterine Masses

Pregnancy should always be kept in mind as a cause of uterine enlargement. Most physicians are familiar with the unreliability of a menstrual history, and any patient of reproductive age with a pelvic mass should first have pregnancy ruled out by any of a variety of pregnancy tests or by detection of a fetus with ultrasound examination.

Myomas of the uterus are the most common uterine neoplasms ( Fig. 10.1 ). They are usually discrete, relatively round tumors that are firm to palpation and may be single or multiple. Myomas may be located within the myometrium (intramural), just beneath the endometrial lining (submucosal), or on the surface of the uterus (serosal). A myoma may frequently be found in the broad ligament attached to the lower uterine segment by a thin pedicle. This will often confuse the examiner and suggest that the mass originates in the ovary or tube. In the United States, myomas are found in at least 10% of white women and 30% to 40% of black women older than age 35 years. In the postmenopausal group, it is said that the incidence increases to 30% in white women and 50% in black women. Fortunately, these neoplasms usually shrink after menopause, especially in patients not receiving high doses of exogenous estrogen stimulation. It appears that most of these benign neoplasms are somewhat estrogen dependent. Growth is commonly seen during pregnancy, probably secondary to elevated hormone levels. Degeneration, infarction, and infection can occur in these lesions, and these complications are associated with considerable lower abdominal pain. Sarcomatous elements, associated with myomas less than 0.1% of the time, are most often recognized postoperatively. Symptoms of associated sarcomatous elements may include a rapidly enlarging pelvic mass, often accompanied by pain and tenderness. The mean age at diagnosis of these lesions is 50 to 55 years, with a range of 25 to 85 years.

Myomas can be seen initially in multiple sizes and states of growth, disease, and degeneration.

(Courtesy of Erle Henriksen, MD, Los Angeles, CA.)

A recent enlargement of the uterus in a postmenopausal patient is rarely caused by fibroids, particularly if the enlargement develops in a short time (“rapid” growth). The use of hormone replacement therapy in and of itself is not an explanation for such an enlargement. The most probable diagnosis is a malignant neoplasm, and physicians must prepare to do an appropriate surgical procedure even if the result of dilatation and curettage (D&C) is negative.

Other conditions that can cause enlargement of the uterus are adenomyosis and endometrial carcinoma or sarcoma. Endometrial carcinoma can enlarge the uterus to as much as four times normal size. Most patients with endometrial cancer describe abnormal vaginal bleeding, and the diagnosis can be made appropriate endometrial sampling (office biopsy or D&C).

Tubal Masses

Neoplasms arising from the fallopian tube are rare. More commonly, adnexal masses secondary to tubal disease are inflammatory or represent an ectopic pregnancy. Distinction between tubal and ovarian masses on the basis of examination alone is often difficult. In the acute phase of salpingitis, the fallopian tube is distended by grossly purulent material. This infection may be secondary to gonorrhea or other organisms, including anaerobes. As the salpingitis process progresses, the adjacent ovary may become involved, creating a so-called tubo-ovarian abscess. Although this acute process may resolve, the patient is often subject to reinfection. As a result of repeated chronic infectious processes, the tubal ostia may close or firmly adhere to the adjacent ovary, and the fallopian tube fills with a clear fluid. As the structure distends, it creates a mass that can easily be mistaken for an ovarian cyst. Although the symptoms of acute pelvic inflammatory disease are distinct (pelvic pain, fever, increased vaginal discharge, and abnormal uterine bleeding), the symptoms of chronic pelvic infection may be subtle. Even the traditional elevation of the erythrocyte sedimentation rate or leukocyte count may be absent in as many as 30% of patients with chronic pelvic inflammatory disease and adnexal masses.

A cystic mass in the adnexal region may be neither ovarian nor tubal in origin but caused instead by remnants of embryologic structures. The parovarium, located within the portion of the broad ligament containing the fallopian tube, consists of vestigial remnants of the Wolffian duct. Paraovarian cysts are found as distal remnants of the Wolffian duct system. They are characteristically located between the fallopian tube and the ovary; when large, they are often found with the fallopian tube stretched over the top of the cyst. These paraovarian cysts are most commonly unilocular and filled with clear yellow fluid. They often persist into the postmenopausal period and can appear as cystic structures in the adnexa on imaging studies done for other complaints.

Approximately 98% of ectopic pregnancies are tubal. Unfortunately, a pelvic mass can be found on examination in fewer than half the cases of tubal pregnancy. Rupture usually occurs when the distended fallopian tube reaches a diameter of 4 cm. Tubal pregnancy must be distinguished from pelvic inflammatory disease, torsion of the adnexa, and bleeding corpus luteum cysts because all produce pain or abnormal bleeding.

Carcinoma limited to the fallopian tube is rare and accounts for fewer than 0.5% of all female genital tract malignant neoplasms. Historically, most of these neoplasms were discovered by serendipity, with a preoperative diagnosis of ovarian neoplasm being most common. On gross evaluation, the fallopian tube is usually enlarged, smooth walled, and sausage shaped. On occasion, patients present with the symptom of several weeks of profuse watery vaginal discharge, the so-called hydrops tubae profluens. However, it is now known that the fallopian tube and, more specifically, the fimbriae is the origin of most high-grade serous carcinomas of the ovary. This has become apparent over recent years as women with hereditary risk underwent risk-reducing surgery and represents a shift away from incessant ovulation as the leading hypothesis of epithelial ovarian carcinogenesis.

Adnexal Masses of Nongynecologic Origin

Functional Cysts

The cyclic ovarian function of a reproductive-age women continually produces follicular growth and resorption. Among the most frequently found “masses” involving the adnexa are the nonneoplastic cysts related to the process of ovulation that are sometimes referred to as functional cysts. They are by far the most common clinically detectable enlargements of the ovary occurring during the reproductive years. If ovulation does not occur, a clear fluid-filled follicular cyst ( Fig. 10.2 ) lined by granulosa cells may result that can reach a size as large as 10 cm in diameter. These cysts usually resolve spontaneously within a few days to 2 weeks but can persist longer. When ovulation occurs, a corpus luteum is formed that may become abnormally enlarged through internal hemorrhage or cyst formation. The masses are of great significance because young women will have cysts on their ovaries at essentially all times (in varying sizes, numbers) during the reproductive years, and occasionally the cysts can produce pain and discomfort, and a mass will be noted on examination or ultrasonography. Clinical sequelae of these benign physiologic processes can also bring patients to the physician’s attention because the masses may undergo torsion; acutely hemorrhage into the mass; or grow to sufficient size to produce pain, pressure, or bloating symptoms. The clinical challenge is that these functional cysts cannot be readily distinguished from true neoplasms on clinical grounds alone. Ultrasound evaluation typically shows a thin-walled, simple-appearing, anechoic cyst without solid component or septations. A hemorrhagic cyst may also show more complexity on ultrasonography given the presence of blood and fibrin stranding.

A, Follicular cyst (8 cm) with fimbria. B, Microscopic view of a follicular cyst as seen in A.

(Courtesy of Ibrahim Ramzy, MD, University of California, Irvine, CA.)

In addition, such cysts are often associated with variable delays in the onset of menses and confusion regarding the possibility of pregnancy. Theca-lutein cysts result from overstimulation of the ovary by hCG and on histologic examination are characterized by extensive luteinization of the stroma surrounding the follicle. Although theca-lutein cysts do not commonly occur in a normal pregnancy, they are often associated with hydatidiform moles and choriocarcinoma. Gross examination of the ovary containing theca-lutein cysts shows a structure almost completely replaced by lobulated thin-walled cysts that vary in size and are smooth and yellow.

Corpus luteum, follicular, and theca-lutein cysts are benign and represent an exaggerated physiologic response of the ovary. In most instances, they involute over time, but they do need to be included in the differential diagnosis. Luteomas of pregnancy are often large and solid but are not neoplastic masses originating in the ovary during pregnancy. Most cases are discovered at cesarean section in the last 2 months of pregnancy. The exact origin of luteomas have not been established. It is probably a hCG-dependent nonneoplastic hyperplasia and usually regresses after delivery. Therefore, surgical excision of these lesions at the time of cesarean section is not necessary.

Polycystic (sclerocystic) ovaries contain multiple follicle cysts with hyperplasia and luteinization of theca interna surrounding the cysts and atretic follicles. The ovaries are two to five times normal size with a thickened capsule. The condition is found most frequently in association with the Stein–Leventhal or polycystic ovarian syndrome.

Endometriotic Cysts

Endometriosis is a condition in which implants of normal-appearing endometrial glands and stroma are found outside their normal location in the uterine cavity. Presumptive evidence of endometriosis is also supported by significant scarring in the pelvis and the presence of hemosiderin-laden macrophages in the cyst wall or peritoneum. The most common sites for endometriosis are the ovaries, the supporting ligaments of the uterus, and the peritoneum of the cul-de-sac and bladder. Endometriosis is most common in women 35 to 45 years of age and is more common in white and nulliparous women. Endometriotic cysts sometimes may be distinguished from ovarian neoplasms by patient history (history of endometriosis or symptoms suggestive of endometriosis, including dysmenorrhea, pelvic pain, or infertility) and examination findings (nodularity of the uterosacral ligaments). Pelvic pain is by far the most usual symptom of endometriosis. Although physical activity and sexual intercourse usually increase the discomfort, the amount of endometriosis present does not seem to correlate with the intensity of the symptoms. For some, pain produced from small peritoneal implants appears to be incapacitating. Ultrasonography may show a homogenous echo, sometimes referred to as having a “ground-glass appearance,” and on occasion may be misinterpreted as a solid mass. Septations and wall nodularity are also seen commonly. Of note, CA-125 levels are frequently elevated in patients with endometriosis. The morphologic complexity on ultrasonography coupled with CA-125 level elevations make endometriotic cysts among the most common masses incorrectly thought to be suspicious for malignancy on workup. During surgery, the ovary may be enlarged and cystic. The lumen contains blood of varying color, depending on the amount and age of the hemorrhage. In most instances, it is dark brown (chocolate cyst). In at least 50% of cases, both ovaries are involved. These cysts rarely exceed a diameter of 12 cm. They are frequently adherent to surrounding structures.

Benign Ovarian Neoplasms

The ovary is composed of tissue derived from coelomic epithelium, germ cells, and mesenchyme, and any one of these components may undergo neoplastic change (benign or malignant). Morphologically, neoplasms can be divided into solid and cystic types based on ultrasonography and gross appearances. The most common benign cystic neoplasms of the ovary are serous and mucinous cystadenomas and cystic teratomas (dermoids). Benign cystadenomas may vary in size from 5 to 50 cm and are thin walled, ovoid, and frequently unilocular. Benign neoplasms do not spontaneously regress, nor do they have the potential to disseminate or metastasize. Whether some begin neoplasms are premalignant is a source of controversy. Intraepithelial neoplasia has been reported in otherwise benign serous cystadenomas; in early stage invasive epithelial cancer, several authors have described the presence of transitional changes from normal epithelium to intraepithelial neoplasia to invasive cancer.

Serous Cystadenoma

Serous cystadenomas are more common than the mucinous type of tumor, but as a rule, they do not attain the large size characteristic of their mucinous counterparts. On gross evaluation, the cyst fluid is usually thin, watery, and yellow tinged. The surface of the cyst is usually smooth and frequently unilocular ( Fig. 10.3 ). Septations dividing the cyst can be seen, however. Some serous tumors may have small papillary projections on the surface of the cyst wall, which at times are so numerous that a cauliflower pattern is produced. Large, frondlike solid projections or nodules or areas of necrosis should raise the concern for malignancy. On microscopic examination, the epithelium is usually of the low columnar type with cilia. Particularly characteristic of this type of cyst are small calcific granules, the so-called psammoma bodies, which are an end product of degeneration of the papillary implants. Associated fibrosis may lead to the so-called cystadenofibroma.

A, Gross photograph of an opened serous cyst adenoma that contained 500 mL of clear straw-colored fluid. Note the papillary projections on the inner surface, especially on the right. B, Microscopic view of a serous cystadenoma with a few papillary projections from the surface.

Many serous cystadenomas of the ovary are asymptomatic and are discovered as an incidental finding during routine pelvic examination or through imaging studies performed for another reason. Symptoms may develop because of the size of the mass (pain, pressure on the bladder or rectum) or as a result of rupture or torsion. Ultrasound appearance is frequently similar to a large functional cyst, unilocular and simple.

Mucinous Cystadenoma

Mucinous cystadenomas ( Fig. 10.4 ) may become huge, with some ( Fig. 10.5 ) reported to weigh more than 300 lb. On gross evaluation, the masses are round or ovoid with smooth capsules that are usually translucent or bluish to whitish gray. The interior is divided by a number of discrete septa into loculi containing in general a clear, viscid fluid. Papillae are rarely noted. However, on microscopic examination, the lining of the epithelium is of a tall, pale-staining secretory type with nuclei at the basal pole; the presence of goblet cells is common. The cells are found to be rich in mucin if suitable stains are obtained. It is believed that this type of cyst usually arises from simple metaplasia of the germinal epithelium. It may occasionally arise from a teratoma in which all the other elements have been lost. It rarely occurs from a Brenner tumor in which there has been mucinous transformation of the epithelium.

Barium enema film of a patient with a large mucinous cystadenoma of the right ovary filling the pelvis and lower abdomen.

Mucinous cystadenoma. A, Gross appearance. B, Histologic section showing tall epithelial lining with pale-staining nuclei at basal pole.

(A, Courtesy of Ibrahim Ramzy, MD, University of California, Irvine, CA.)

Bilaterality may be found in as many as 10% of patients with serous cystadenomas, in contrast with mucinous cystadenomas, for which there is essentially no significant incidence of bilaterality. This information is helpful when the surgeon needs to make a judgment about surgical inspection of the opposite ovary in a young woman desirous of further childbearing. If the other ovary is of normal size, shape, and configuration, surgical evaluation is not needed.

Dermoid Cyst (Benign Cystic Teratoma)

Dermoid cysts are rarely large (typically <10 cm), are often bilateral (15%–25%), and occur with disproportionate frequency in younger patients. On gross evaluation, there is a thick, opaque, whitish wall; on opening of the cyst, one frequently finds hair, bone, cartilage, and a large amount of greasy fluid, which rapidly becomes sebaceous on cooling. On microscopic examination, various amounts of mature ectoderm, mesoderm, and endoderm may be present. Stratified squamous epithelium, hair follicles, sebaceous and sudoriferous glands, cartilage, neural and respiratory elements, and indeed all elements normally seen in fetal life may be present. Sequelae include growth of the mass, rupture ( Fig. 10.6 ), and malignant degeneration. Malignant degeneration occurs in benign cystic teratomas in 1% to 3% of these tumors, and it is usually of a squamous type. These neoplasms are thought to arise from early ova that have been triggered by some type of parthenogenetic process. Imaging evaluation of teratomas can be useful. For example, abdominal radiographs may demonstrate calcifications (teeth, bone), and CT imaging is excellent at showing fat densities commonly seen with dermoids. Ultrasound appearance is variable, and complex morphology with hyperechoic areas is common.

A, Ruptured dermoid cyst with sebaceous material seen. B, Photomicrograph of a mature teratoma (dermoid) of the ovary.

Removal of the ovary or cyst is recommended, and given the young age of many patients with mature teratoma, cystectomy (open or laparoscopically) in a patient desirous of further childbearing is standard. In most instances, a significant portion of normal ovary can be preserved and reconstituted. Care must be taken to remove the entire capsule of the neoplasm to avoid recurrence. In our experience, bilaterality is relatively rare when the opposite ovary is normal in appearance and is usually identified by preoperative ultrasonography. We do not recommend bivalving or opening a normal-appearing contralateral ovary in these circumstances. Care should be taken to prevent spillage of the contents of the dermoid cyst because this material can cause a chemical peritonitis. Because many of the masses are found in pregnant patients undergoing routine prenatal ultrasonography, conservative management until delivery is reasonable. Caspi reported on 49 women with ultrasonographically diagnosed ovarian cystic teratoma smaller than 6 cm in pregnancy who were followed for change in size. A total of 68 pregnancies resulted. None of the classic complications of dermoid cysts such as torsion, dystocia, or rupture occurred. The authors concluded that such small lesions are safe to just follow, especially in pregnancy.

Fibroma

Benign solid tumors of the ovary are usually of connective tissue origin (fibromas, thecomas, or Brenner tumors). They vary in size from small nodules found on the surface of the ovary to large neoplasms weighing several thousand grams. On physical examination, these neoplasms are usually firm, slightly irregular in contour, and mobile. The occurrence of fibromas ( Fig. 10.7 ) is not at all infrequent. Sometimes they are first noted as small nodules on the ovarian cortex. In other instances, they can be extremely large, filling the entire pelvis and lower abdomen. The tumors are characterized by their firmness and resemblance to myomas, and they are frequently misdiagnosed as such. The cut surface has a homogeneous grayish-white and firm appearance, although areas of cystic degeneration are common in larger tumors. On microscopic examination, one finds stellate or spindle-shaped cells arranged in fusiform fashion. The cells are uniformly well differentiated with nothing to suggest malignancy. Hyalinization is frequent, particularly in the larger tumors, and if fat stains are done, admixtures of theca cells may be seen. Meigs syndrome is characterized by ascites, hydrothorax, and an ovarian tumor that was originally believed to be specifically a fibroma; however, many other types of ovarian tumors are now known to be associated with this syndrome, such as Brenner tumors and Krukenberg tumors. The cause of Meigs syndrome is not completely understood, but it seems that the hydrothorax occurs by certain lymphatics through the diaphragm. After removal of the ovarian neoplasm, there is a prompt resolution of both abdominal and pleural fluid.

Ovarian fibroma. A, Gross appearance: solid white lesion with a firm cut surface and fallopian tube. B, Histologic section: whorls of fibromatous matrix.

(Courtesy of Ibrahim Ramzy, MD, University of California, Irvine, CA.)

Brenner Tumor

Brenner tumor ( Fig. 10.8 ), an uncommon type of ovarian neoplasm, is grossly identical to a fibroma. Frequently, the tumor is an incidental finding in an otherwise unremarkable ovary. On microscopic examination, one finds a markedly hyperplastic fibromatous matrix interspersed with nests of epithelioid cells. The epithelioid cells under high magnification show a “coffee bean” pattern caused by the longitudinal grooving of the nuclei. The cell nests show a frequent tendency toward central cystic degeneration, producing a superficial resemblance to a follicle. Although it was originally believed that Brenner tumors arise from simple Walthard cell rests, it has been conclusively demonstrated that Brenner tumors can arise from diverse sources, including the surface epithelium, rete ovarii, and ovarian stroma itself. It was originally stressed that Brenner tumors were uniformly benign, but there have been scattered reports in the past several decades of a number of malignant Brenner tumors. Brenner tumors are generally thought to be hormonally inert, but several cases in recent years have been associated with postmenopausal endometrial hyperplasia, and a frequent estrogen effect has been attributed to this neoplasm . These lesions are managed by simple excision.

Brenner tumor. A, Gross appearance: solid, firm, white cut surface. B, Histologic section: hyperplastic fibromatous matrix interspersed with nests of epithelioid cells.

(Courtesy of Ibrahim Ramzy, MD, University of California, Irvine, CA.)

Observation versus Surgery

Optimal management for patients with adnexal masses can be achieved by combining all available information from the history, examination, and diagnostic studies to create an accurate differential diagnosis. The presence and severity of symptoms are other important considerations. For example, a patient with a 6-cm unilocular cyst may be initially observed expectantly, but a similar patient with severe pain may need urgent surgery. In addition, consultation with additional specialists (gynecologic oncologist, urologist, general surgeon) may be required based on suspicion of malignancy or the differential diagnosis being considered.

Decisions that must be considered are as follows:

• 1.
  

  Does the patient require surgical evaluation (vs. observation)?

  
  
• 2.
  

  After a decision for surgery has been made, is a specialist referral or consultation required, and what type of surgical procedure is planned (open vs. minimally invasive)?

  
  
• 3.
  

  If observation has been elected, at what intervals and with what thresholds for changing course would be considered?

Deciding which patients need surgical exploration can best be done by considering the characteristics of the adnexal mass. In reproductive-age women, 95% of ovarian cysts smaller than 5 cm in diameter are nonneoplastic. In addition, functional cysts are seldom larger than 7 cm in diameter and are usually unilateral and freely mobile. A physician can presume that during the reproductive years, an adnexal mass as described earlier is a functional or hyperplastic change of the ovary rather than a true neoplasm. The transitory existence of functional cysts is of prime importance in distinguishing them from true neoplasms. Tradition and clinical experience have shown that functional cysts usually persist for only a few days to a few weeks, and reexamination during a later phase of the menstrual cycle has been a reliable procedure in confirming this diagnosis. Many gynecologists prescribe oral contraceptives to accelerate the involution of the functional cyst on the presumption that these cysts are gonadotropin dependent. The unconfirmed theory is that the inhibitory effect of the contraceptive steroids on the release of pituitary gonadotropins shortens the lifespan of these cysts, hastening their identification as functional or nonneoplastic lesions. In an era before the use of ultrasonography, Spanos conducted a careful study of 286 patients who had adnexal cysts. Combination oral contraceptives were prescribed, and the women were reexamined in 6 weeks. In 72% of the women, the masses disappeared during the observation period. Of the 81 patients whose masses persisted, none were found to have a functional cyst at laparotomy. The fact that five of the removed tumors were malignant underscores the importance of avoiding an unnecessary delay in the operative investigation of these patients. In general, masses larger than 10 cm in diameter should be surgically explored regardless of concerns for malignancy because these masses are not likely to be functional and seldom resolve spontaneously. Our practice is to reassess young women with suspected functional cysts with a repeat examination and ultrasonography in 6 to 8 weeks. If the cyst resolves, no further therapy is indicated. If the cyst persists, the patient is counseled regarding surgical evaluation ( Fig. 10.9 ).

Management of a premenopausal woman with an adnexal mass.

Data also have accumulated showing that some patients with nonfunctional cyst adnexal masses may be conservatively followed with serial observation and not undergo surgery. For example, postmenopausal women with unilocular cysts smaller than 5 cm and normal CA-125 levels may be considered for observation because the risk for malignancy is very low. In addition, many of the masses will resolve spontaneously. In some older patients with concurrent medical comorbidities, avoiding surgery may be a safer strategy. Patients undergo serial repeat pelvic ultrasonography every 3 to 6 months with CA-125 testing. Surgical intervention is recommended if the morphology becomes complex, the size increases, or the CA-125 level increases outside of normal range. Saunders and colleagues at the University of Kentucky evaluated a series of 1319 patients (25% premenopausal, 75% postmenopausal) with 2870 septated ovarian cysts without solid or papillary projections. On serial follow-up, 39% of cysts resolved (mean duration to resolution, 12 months). Of 1756 cysts that persisted, 128 surgeries were performed, and no case of ovarian cancer was identified. The most common histopathologies were serous cystadenoma, mucinous cystadenoma, and endometrioma. The authors suggested that patients with septated cysts without solid components are at low risk for ovarian cancer and may be followed.

Masses that raise a suspicion for malignancy require surgical intervention ( Table 10.4 ). In addition, consultation with a specialist trained in the management of ovarian cancer should be considered. Patients with associated hallmarks of ovarian cancer, including peritoneal or omental implants, ovarian caking, and markedly elevated CA-125 levels, frequently have advanced ovarian cancer and require care from physicians specialized in surgical debulking techniques. In other tumors with the potential for malignancy, surgical evaluation should be considered, and consultation is advised. Conditions raising the concern of possible malignancy include the following:

• 1.
  

  Bilateral adnexal masses

  
  
• 2.
  

  Complex masses, especially including solid components, thick septations, or mural nodules

  
  
• 3.
  

  Premenopausal patients with complex masses that persist or grow after period of observation

  
  
• 4.
  

  Postmenopausal patients with simple masses larger than 5 cm or complex masses of any size

  
  
• 5.
  

  Masses associated with elevated tumor markers

  
  
• 6.
  

  Symptomatic masses

In a joint publication, the American College of Obstetrics and Gynecology (ACOG) and the SGO suggested referral guidelines to triage patients with adnexal masses to specialists in the care of ovarian cancer. The guidelines are shown in Table 10.5 . The SGO performed a multisite, retrospective validation trial assessing the guidelines in 1035 women undergoing surgery for an adnexal mass. The prevalence of ovarian cancer was 31%. When using the referral guidelines, the positive predictive values were 34% for premenopausal and 60% for postmenopausal women. The negative predictive value was approximately 92% for each group. Others have criticized the guidelines for largely detecting cases of advanced cancers and missing many cases of early stage cancers (when appropriate staging would be desired).

Minimally Invasive Surgery for Adnexal Masses

Minimally invasive surgery (MIS), either laparoscopic or robotic, is the preferred approach for a presumptively benign adnexal mass. With the use of specific ultrasound criteria, benign masses can be predicted with a high degree of certainty, especially in premenopausal women. Careful selection of patients is critical for the appropriate use of MIS for removal of adnexal masses. The patient’s age, the clinical examination, tumor markers, and the ultrasound findings provide important information that helps determine the appropriate operative approach. MIS should be used cautiously in the presence of any mass with clinical or ultrasonographic characteristics suggestive of malignancy, especially an adnexal mass in a postmenopausal woman associated with an elevated serum CA-125 value.

All patients scheduled for MIS with an adnexal mass should also consent to possible laparotomy. If the preoperative risk for finding malignancy is high enough, referral to a surgeon prepared to proceed with staging or surgical debulking if malignant disease is uncovered is warranted. Cell washings are obtained from the pelvis and upper abdomen and saved for proper staging if a malignant neoplasm is found. Any lesions or suspicious areas are sampled and sent for frozen section analysis. If an unexpected malignancy is found, appropriate biopsies and photo documentation can be taken, and referral can be made to a cancer specialist. The main reasons for conversion from laparoscopy to laparotomy are extensive adhesions or intraoperative complications.

Nezhat and colleagues reported their experience with 1209 patients with adnexal masses who were managed laparoscopically. Of 1011 cases with surgical management, ovarian cancer was discovered intraoperatively in four. The management of a cystic mass included aspiration of fluid, which was sent for cytologic examination, followed by opening of the cyst and inspection of the wall for any irregular thickening. Frozen section biopsy specimens were obtained if the surgeon thought any surfaces were suspicious. An ovarian cystectomy–oophorectomy was then performed, and tissue was sent for permanent histologic examination. The Nezhat et al. study suggests that experienced surgeons using intraoperative histologic sampling may safely evaluate adnexal masses laparoscopically.

Hasson reported another series of 102 women with ovarian cysts who were managed laparoscopically. In 83 of the women, laparoscopic fenestration and biopsy were done, with or without coagulation or removal of the cyst lining. Only 1 of 56 functional, simple, or paraovarian cysts recurred during the study. Two of the 18 ovarian endometriomas treated with fenestration and coagulation or removal of the lining recurred, and eight of nine such lesions recurred with treatment by fenestration alone. There were no surgical complications.

Canis et al. reported on their group’s experience with 247 adnexal masses suspicious at ultrasound examination; 17 patients were evaluated by laparotomy and 230 by laparoscopy. Of the 230 women triaged to laparoscopy, the procedure was completed laparoscopically in 204 women. Women who were treated by laparoscopy included 7 of 37 malignant tumors and 191 of 210 benign masses. One case of tumor dissemination occurred after a laparoscopic adnexectomy and morcellation of a grade 1 immature teratoma. This study demonstrated the feasibility of laparoscopy for management of a suspicious adnexal mass, thus sparing the patient the known morbidity of a laparotomy. Some surgeons prefer to manage cystic ovarian masses with a mini laparotomy. A small incision (3–5 cm) is made over the mass, and the cyst fluid is aspirated with a long needle, trocar, or suction cannula after draping the field with dry laparotomy pads ( Fig. 10.10, A ). The collapsed mass can then be removed through the small incision ( Fig. 10.10, B ) and submitted to pathology. Spill is minimal with this approach, but thorough irrigation of the surgical field is recommended before closure. Our preferred approach is to place the mass into a bag inserted laparoscopically to enable aspiration and extraction of the mass without intraperitoneal spill and with use of a truly minimally invasive approach.

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