Version May 2024
INTRODUCTION — Ovarian sex cord-stromal tumors (SCSTs (table 1)) are a group of benign and malignant neoplasms that develop from the sex cord (eg, Sertoli cell tumor, granulosa cell tumor) or stromal cells (eg, fibroma, thecoma, Leydig cell tumor) or both (eg, Sertoli-Leydig cell tumor). Some ovarian SCSTs produce steroid hormones, particularly androgens or estrogens, and thus may present with signs of virilization or estrogen excess (table 2) [1,2].
Ovarian SCSTs are less common than tumors of epithelial cell and germ cell origin. Benign ovarian SCSTs account for <4 percent of ovarian benign neoplasms, and malignant ovarian SCSTs account for <8 percent of ovarian malignant neoplasms. In contrast to the more common epithelial ovarian malignant neoplasms, most patients with malignant SCSTs are diagnosed with early-stage disease [3]. Histology is generally low grade, lymph node metastases are rare, and prognosis is good [4-6]. However, some neoplasms are aggressive and have a lethal outcome.
This topic will discuss the epidemiology, clinical features, and diagnosis of ovarian SCSTs. Many patients with SCSTs present with an adnexal mass; the initial approach to the patient who presents with an adnexal mass is discussed separately (see "Approach to the patient with an adnexal mass"). Management of SCSTs is also reviewed separately. (See "Sex cord-stromal tumors of the ovary: Management in adults".)
EPIDEMIOLOGY — In the Surveillance, Epidemiology, and End Results (SEER) United States national cancer database, the incidence of SCSTs was 0.2 per 100,000 females [3]. The average age at diagnosis was 50 years (compared with 61 years for epithelial ovarian cancer); 12 percent of patients were younger than age 30, and 57 percent were between ages 30 and 59. The incidence was higher in Black patients than in White patients (0.44 versus 0.18 per 100,000).
In a separate retrospective study, older age at last birth (≥40 years) and shorter time since last birth (<10 years) were associated with a reduction in risk of SCSTs [7].
Malignant SCSTs account for less than 8 percent of ovarian malignant neoplasms. In contrast to ovarian carcinomas, most SCSTs have no association with the BRCA germline mutations or a genetic predisposition to breast cancer. An exception is granulosa cell tumors, which appear to be more common in patients who have a family history of breast or ovarian cancer [8].
PATHOGENESIS — Ovarian SCSTs arise from the dividing cells that would typically give rise to specialized gonadal stroma surrounding the oocytes, including granulosa cells, theca cells, Sertoli cells, Leydig cells, and fibroblasts (figure 1). In comparison, the two major other types of ovarian neoplasms originate from germ cells or from the dividing cell population that gives rise to müllerian epithelium, ovarian surface epithelium, or fallopian tube epithelium.
Morphologically, SCSTs display at least some areas with unequivocal gonadal stromal differentiation, such as granulosa, theca, Sertoli, or Leydig cell differentiation. They may display indifferent morphologic features, such as fibroblastic differentiation, and may contain areas of other types of stromal differentiation (eg, cartilage or skeletal muscle) or areas of epithelial differentiation ("heterologous elements"). This may happen because the dividing cell population from which these neoplasms arise may still have capacity for various lines of differentiation. Most consist of ovarian cell types, but testicular (Sertoli cell) and mixed ovarian and testicular (gynandroblastoma) cell types can occur.
CLINICAL PRESENTATION AND DIAGNOSTIC CONSIDERATIONS
Nonspecific signs and symptoms — Patients with SCSTs generally present in the same way as patients with epithelial or germ cell ovarian neoplasms: because of abdominal or pelvic symptoms from the mass or as an incidental finding of adnexal mass on examination or imaging. Further details on diagnostic considerations in patients presenting with an adnexal mass are reviewed elsewhere. (See "Adnexal mass: Differential diagnosis".)
Some SCSTs can have characteristic clinical presentations, as described below.
Estrogenic and androgenic effects — Some SCSTs secrete androgens, estrogens, or other steroid hormones that can cause clinical manifestations related to the hormonal profile (table 2).
Signs of estrogen excess include abnormal uterine bleeding, endometrial neoplasm, or, in a child, precocious puberty. The differential diagnosis in patients who present with both an adnexal mass and abnormal uterine bleeding includes SCSTs as well as an ovarian metastasis from a primary uterine cancer, an endometrial metastasis from a primary ovarian malignant neoplasm, and separate primary ovarian and endometrial carcinomas. (See "Abnormal uterine bleeding in nonpregnant reproductive-age patients: Terminology, evaluation, and approach to diagnosis" and "Endometrial carcinoma: Epidemiology, risk factors, and prevention" and "Definition, etiology, and evaluation of precocious puberty".)
Signs of androgen excess (virilization) include hirsutism, acne, alopecia (male pattern baldness), menstrual abnormalities (oligomenorrhea, amenorrhea), clitoromegaly, and deepening voice [1,9]. (See "Evaluation of premenopausal women with hirsutism".)
Ascites with or without other abdominal symptoms
●Patients with ovarian fibroma and ascites and/or pleural effusion may have Meigs syndrome [10]. Fluid accumulation is probably related to substances like vascular endothelial growth factor that raise capillary permeability [11,12]. Removal of the neoplasm results in elimination of ascites and pleural effusion [13]. Several cases of Meigs syndrome have been reported in association with elevated serum cancer antigen 125 (CA 125) levels [14]. Thus, neither ascites nor pleural effusion, nor an elevated CA 125, is necessarily indicative of an advanced epithelial ovarian carcinoma in a patient with a pelvic mass. (See 'Fibroma' below.)
Pseudo-Meigs syndrome (a clinical syndrome of pleural effusion, ascites, and an ovarian mass that is not a fibroma or fibroma-like mass/tumor) has been reported from a number of sources, such as leiomyomas, struma ovarii, mucinous cystadenoma, teratoma, and malignancies that are metastatic to the ovary (particularly colorectal cancer) [15].
●Patients with luteinized thecoma associated with sclerosing peritonitis commonly have massive ascites, which cause acute abdominal distension and pain. Some patients may have bowel obstruction. (See 'Luteinized thecoma associated with sclerosing peritonitis' below.)
DIAGNOSTIC EVALUATION — An SCST is typically suspected in those patients who present with both an adnexal mass and endocrine effects since the vast majority of adnexal masses do not cause these effects. In such instances, the work-up typically includes a combination of laboratory tests (eg, total testosterone if virilization is present, estradiol if signs of excess estrogen, and possibly SCST tumor markers such as inhibin A and B and alpha-fetoprotein (table 2)). As with other adnexal masses, imaging studies are also used, as discussed elsewhere. (See "Approach to the patient with an adnexal mass", section on 'Role of tumor markers and multimodal tests' and "Approach to the patient with an adnexal mass", section on 'Role of additional imaging'.)
Endometrial sampling should be performed in premenopausal patients with an adnexal mass and abnormal uterine bleeding and in postmenopausal patients with a thickened (>4 to 5 mm) endometrial stripe on transvaginal ultrasound examination since these signs/symptoms can be due to excess estrogen production and affect management. For example, endometrial sampling will detect endometrial hyperplasia/intraepithelial neoplasia in 25 to 50 percent of patients with granulosa cell tumors and carcinoma in 5 to 10 percent [16-18]. In patients with thecoma, carcinoma is present in approximately 20 to 25 percent of patients, and another 15 percent have a precursor lesion. (See "Overview of the evaluation of the endometrium for malignant or premalignant disease".)
In all patients with an adnexal mass, surgery is performed to resect and determine the histology of an ovarian mass if ultrasound findings suggest the mass has some reasonable chance of being malignant (table 3). Surgery to resect the mass is also performed if there are other risks associated with the mass (eg, torsion) or the mass is causing symptoms. Patients with SCSTs generally meet one or more of these criteria. When a malignant neoplasm is suspected, the entire ovary must be removed. Biopsy of the ovary or cystectomy results in potential spread of neoplastic cells and upstaging that impacts prognosis.
DIAGNOSIS — The diagnosis of ovarian SCST is a histologic diagnosis. While the diagnosis may be suspected preoperatively based on the presence of an adnexal mass combined with signs of estrogen or androgen excess or elevated levels of serum tumor markers, the specific histologic type and risk of malignancy require examination of a surgical specimen. In addition, many expert gynecologic pathologists will not provide a definite diagnosis of SCSTs based upon intraoperative frozen section examination because they are rare and can be mimicked by epithelial ovarian neoplasms (particularly endometrioid neoplasm) or vice versa. (See "Approach to the patient with an adnexal mass".)
Genomic analysis of SCSTs does not have a clear clinical role in diagnosis or targeted treatment of these tumors but is under investigation [19,20].
HISTOPATHOLOGY AND CLINICAL BEHAVIOR
General features — The World Health Organization (WHO) classification of ovarian SCSTs and other ovarian neoplasms is shown in the table (table 1) [21,22]. Selected SCSTs, particularly those that may display malignant behavior, are discussed in the sections below and in the table (table 4).
The pattern of spread and likelihood of distant metastases vary greatly by histologic subtype, but nodal metastases are rare [4,5]. As an example, in a retrospective series of 87 patients with mostly granulosa cell or Sertoli-Leydig cell neoplasms, there were no patients with positive lymph nodes [6]. Among patients with stage III and IV disease, regional spread was abdominal (eg, diaphragm and omentum).
In contrast to the more common ovarian carcinomas, malignant SCSTs are often diagnosed at an early stage, and histology is generally low grade; however, some tumors are aggressive and have a lethal outcome. A study using Surveillance, Epidemiology, and End Results (SEER) United States national cancer database data reported the following distribution of stage at diagnosis: confined to the ovary (57 percent), spread to surrounding organs or tissues (15 percent), and distant metastases (22 percent) [3].
Pure stromal tumors
Fibroma — Fibromas are the most common of the benign SCSTs and account for 4 percent of all ovarian neoplasms. Clinical characteristics are described in the table (table 4).
●Microscopic appearance – Fibromas are made almost entirely of fibroblasts. Cellular fibromas are a type of fibroma characterized by mildly increased cellular density, mild nuclear atypia, and an average of three or fewer mitotic figures per 10 high power fields (HPF).
●Genomics – Ovarian fibromas associated with basal cell cancers are called nevoid basal cell carcinoma syndrome or Gorlin syndrome. Whether the inherited germline abnormality responsible for Gorlin syndrome (a mutation in the patched or PTCH1 gene on chromosome 9) is related to the development of ovarian fibromas as well is unclear [23]. (See "Nevoid basal cell carcinoma syndrome (Gorlin syndrome)".)
●Behavior – Fibromas are benign and have neither estrogenic nor androgenic effects. Approximately 10 percent of fibromatous tumors of the ovary demonstrate increased cellularity and varying degrees of mitotic activity [24,25]. Cellular tumors are classified as cellular fibromas; those with mitotic activity are considered tumors of low malignant potential.
Thecoma — Thecomas are rare. Clinical characteristics are described in the table (table 4).
●Microscopic appearance – Thecomas are composed of theca cells but may also contain granulosa cell components; such tumors are designated granulosa-theca cell tumors [26]. If granulosa cells predominate, then they are considered granulosa cell tumors. (See 'Granulosa cell tumors' below.)
●Behavior – Pure thecomas are benign and commonly display estrogenic effects. Malignant behavior is rare. (See 'Other' below and 'Granulosa cell tumors' below.)
Fibrothecoma — The term "fibrothecoma" is used by some experts to refer to a rare neoplasm with features that are intermediate between a fibroma and a thecoma, but fibrothecoma is not part of the WHO classification of tumors of the ovary [27]. There is no universal agreement on which neoplasms should be classified as a fibrothecoma rather than either a fibroma or a thecoma; however, many neoplasms have mixtures of these cell types. Most of these tumors are benign [28,29]. The risk of malignant neoplasm is difficult to predict due to inconsistent classification and the paucity of data regarding these neoplasms. Hormonal activity depends upon the extent to which they resemble fibromas (lipid poor, hormonally inert) or thecomas (lipid containing, hormonally [estrogen] active) [27,30]. Patients with a significant amount of hormonally active thecoma elements are at risk for endometrial neoplasia, similar to pure thecomas. On ultrasound, the tumors tend to be large and solid tumors, and may have areas of edema and cystic degeneration.
Luteinized thecoma associated with sclerosing peritonitis — Luteinized thecoma associated with sclerosing peritonitis is rare. Clinical characteristics are described in the table (table 4).
●Microscopic appearance – A large tumor may replace the entire ovary, but smaller lesions may circumferentially involve the ovarian cortex with sparing of the medullary region of the ovary. Other microscopic features may include a high mitotic count, prominent cellular lobules, hemorrhage, edema, and cytologically bland spindle-shaped cells. There is no pathognomonic immunohistochemical staining pattern. Luteinized cells may be positive for alpha-inhibin, calretinin, and CD56, and spindled cells may be alpha-smooth muscle actin positive or desmin positive [31]. A proportion of tumors may be estrogen receptor or progesterone receptor positive.
●Behavior – Luteinized thecoma associated with sclerosing peritonitis is a benign tumor. Hormonal manifestations are usually absent, but one patient with an elevated testosterone level and one patient with menorrhagia have been reported [31].
At surgery, there is significant thickening of the peritoneum (sclerosing peritonitis) with involvement of the omentum, peritoneal surfaces, and small intestine. The sclerosing peritonitis may cause significant morbidity, including severe adhesions, intestinal obstruction, fistulas, and death [31,32]. The cause of the sclerosing peritonitis is unknown, but the most popular theories involve either some type of inciting agent that causes both the ovarian pathology and the peritoneal fibrosis, or the secretion of some type of substance by the ovaries that results in the associated peritoneal fibrosis. There have been reports of an association between this entity and antiseizure medications, beta-adrenergic blocking agents, or peritoneal dialysis [33-35].
Fibrosarcoma — Fibrosarcomas are extremely rare. Clinical characteristics are described in the table (table 4).
●Microscopic appearance – Fibrosarcomas have four or more mitotic figures per 10 HPF plus marked cellular density and nuclear atypia.
●Behavior – Fibrosarcomas are very rare malignant ovarian sarcomas. Aggressiveness correlates with the number of mitoses and the degree of anaplasia. Hormonal effects are generally absent.
Pure sex cord tumors
Granulosa cell tumors — Granulosa cell tumors are the most common type of potentially malignant ovarian SCST; they comprise 2 to 5 percent of all ovarian malignant neoplasms and 90 percent of malignant SCSTs [36]. Clinical characteristics are described in the table (table 4).
●Microscopic appearance – There are two subtypes: adult (95 percent of cases) and juvenile (5 percent of cases). Granulosa cells of the adult subtype appear round to oblong, pale, with scant cytoplasm, and classic "coffee-bean" grooved nuclei; atypia and mitoses are typically not frequent but do occur (picture 1). The cells may arrange themselves in small clusters or rosettes around a central cavity. These arrangements, which are termed "Call-Exner bodies," resemble primordial follicles and, when diffusely present, constitute a microfollicular pattern [37]. Lack of Call-Exner bodies is not infrequent.
Juvenile subtype has a macrofollicular or cystic pattern and is composed of immature granulosa cells with frequent mitoses; Call-Exner bodies and coffee-bean grooved nuclei are infrequent.
Theca cells, which are luteinized cells within the stroma, are present in approximately 70 percent of cases. Theca cells produce androstenedione, a weak androgen, and granulosa cells convert the androstenedione to estradiol.
●Genomics – FOXL2 encodes a transcription factor that is expressed as a nuclear protein and is critically important in the development of granulosa cells [38,39]. Somatic mutations in FOXL2 have been identified in 97 percent of adult subtype granulosa cell tumors [38,40]. By contrast, the mutation was identified in only 1 of 10 juvenile subtype granulosa cell tumors and 3 of 14 thecomas (21 percent), while it was absent in SCSTs of other types and in other ovarian neoplasms. In a retrospective study including 423 adult granulosa cell tumor profiles, the most common mutations identified included TERT (54 percent), KMT2D (17 percent), and TP53 (8 percent) [41].
●Behavior – Most granulosa cell tumors have an indolent growth pattern. Estrogen effects are common; androgenic effects are also possible. The prognosis depends upon the stage of disease at diagnosis and the presence of residual disease after surgery (table 5) [17,26,42-45]. Outcomes tend to be less favorable in the presence of a large tumor size (10 to 15 cm) or (in many but not all series [46]) tumor rupture [43,47-50]. These tumors have metastatic potential and a tendency for late relapse. In one report of 37 patients with stage I disease, survival rates at 5, 10, and 20 years were 94, 82, and 62 percent, respectively [51]. The median time to relapse is approximately four to six years after initial diagnosis; however, late recurrences have been reported after as many as 40 years [12,43,47,51-53].
A number of histologic features have also been examined for their prognostic significance. In adult neoplasms, cellular atypia, high mitotic index (4 to 10 mitoses per 10 HPF), and the absence of Call-Exner bodies are the only significant histologic predictors of early recurrence [43,46,52]. Abnormal karyotype, p53 overexpression, and ploidy do not appear to be of prognostic value [54,55].
Sertoli cell — Sertoli cell tumors are rare. Clinical characteristics are described in the table (table 4).
●Microscopic appearance – Closely packed hollow or solid tubules with no or rare Leydig cells [56]. The tumor cells forming the tubules have abundant eosinophilic or vacuolated cytoplasm. Some tumors have marked atypia and increased mitotic index (>5 per 10 HPF), which correlates with more aggressive behavior.
●Genomics – Patients with Peutz-Jeghers syndrome (PJS) appear to be predisposed to Sertoli cell tumors, although sex cord tumors with annular tubules (SCTATs) are more common. In one series of 54 patients with Sertoli cell tumors, 6 had PJS [57]; others have reported a similar association [58-60].
●Behavior – Most Sertoli cell tumors are stage I at diagnosis and clinically benign, but higher stage cases have been reported, and approximately 11 percent of stage I tumors have atypical features (size >5 cm, >5 mitoses per 10 HPF, nuclear atypia, or necrosis) with the potential for malignant behavior [36,57].
Sex cord tumors with annular tubules — SCTATs are rare. Clinical characteristics are described in the table (table 4).
●Microscopic appearance – SCTATs are characterized by simple and complex annular (ring-shaped) tubules. They have been described as having morphologic features intermediate between those of Sertoli cell and granulosa cell tumors [61]. It has been suggested that they arise from granulosa cells but grow in a pattern more characteristic of Sertoli cells [62].
●Genomics – Approximately 30 percent of SCTATs are associated with PJS, an autosomal dominant disorder [63,64]. PJS is caused by germline-inactivating variants of STK11, a tumor suppressor gene. Loss of heterozygosity is also identified in SCTATs associated with PJS. (See "Peutz-Jeghers syndrome: Clinical manifestations, diagnosis, and management", section on 'Epidemiology and genetics'.)
Sporadic type (most common) SCTATs have no evidence of somatic STK11 mutations and only rare cases of loss of heterozygosity.
●Behavior – Almost all patients have signs/symptoms of excess estrogen.
PJS-associated SCTATs are usually benign and do not have malignant potential, with rare exceptions (three case reports of malignant transformation in SCTATs associated with PJS [65-67]). However, patients with PJS are at risk for a variety of other gynecologic and nongynecologic malignancies. (See "Peutz-Jeghers syndrome: Clinical manifestations, diagnosis, and management", section on 'Cancer risk' and "Peutz-Jeghers syndrome: Clinical manifestations, diagnosis, and management", section on 'Cancer screening'.)
Patients with sporadic SCTATs have a 20 percent incidence of metastasis at initial surgery [64,68-70].
Mixed sex cord-stromal tumors
Sertoli-Leydig cell tumors — Sertoli-Leydig cell tumors are rare. There are four subtypes: well differentiated, moderately differentiated (formerly intermediate differentiation), poorly differentiated, and retiform. The moderately differentiated, poorly differentiated, and retiform types may have heterologous elements. Clinical characteristics are described in the table (table 4).
●Microscopic appearance – Well differentiated neoplasms are composed of hollow or solid tubules surrounded by a fibrous stroma (picture 2); Sertoli cells line the tubules, and Leydig cells are observed within the stroma.
In moderately or poorly differentiated neoplasms, dense cellular areas with increasing numbers of mitoses are seen.
The retiform pattern is characterized by a network of irregularly branching, narrow, slit-like tubules and cysts with papillary or polypoid projections resembling the rete testis and rete ovarii. This pattern has been associated with hepatocytic differentiation and increased serum levels of alpha-fetoprotein [71].
Mucinous epithelium is the most common element in heterologous tumors, but rhabdomyoblasts, cartilage, hepatoid elements, carcinoid tumor, and neuroblastoma have also been observed [72].
●Genomics – Two gene mutations associated with Sertoli-Leydig cell tumors have been reported and can help with diagnosis since SCSTs can exhibit unusual or overlapping features and may mimic non-SCSTs.
•FOXL2 is a somatic missense point mutation expressed as a nuclear protein mainly in the adult ovary. In one study, FOXL2 immunostaining was a relatively sensitive (80 percent) and highly specific (99 percent) marker for SCSTs of the ovary overall, with more modest sensitivity for Sertoli-Leydig cell tumors (50 percent) because of poor immunoexpression in the retiform and poorly differentiated subtypes [19]. Molecular testing for the FOXL2 (402C-G) mutation was less sensitive than immunostaining, detecting 3 of 40 cases (7.5 percent) of Sertoli-Leydig cell tumors in the same study. Together with alpha-inhibin and calretinin, FOXL2 can be used as an immunomarker panel to aid in distinguishing SCSTs from non-SCSTs when morphology alone is equivocal.
•DICER1 is an endoribonuclease in the RNase III family that is essential for processing microRNAs. Somatic DICER1 mutations are common in Sertoli-Leydig cell tumors. In one report, DICER1 mutations in the RNase IIIb domain were found in 26 of 43 (60 percent) Sertoli-Leydig cell tumors, including 4 tumors with additional germline DICER1 mutations [73].
DICER1 germline mutations have also been associated with pleuropulmonary blastoma (PPB), a childhood cancer. In a series of 325 children with PPB, 3 children and 3 of their family members had Sertoli-Leydig cell tumors, suggesting that Sertoli-Leydig cell tumors may be a manifestation of the familial PPB syndrome and may be the initial presentation of DICER1 mutations within a family [74].
Other clinical conditions that have been associated with germline DICER1 mutations include other nonepithelial ovarian cancers, lung cysts, cystic nephroma, thyroid nodular hyperplasia and differentiated carcinomas, and sarcoma botryoides [75].
●Behavior – Sertoli-Leydig cell tumors are typically benign, but malignant behavior is more likely with higher grade disease. Androgenic effects are common; estrogenic effects are less common.
In a series of 207 patients with Sertoli-Leydig cell tumors, the level of differentiation was well, intermediate, and poor in 11, 54, and 13 percent, respectively, and 22 percent contained heterologous elements [76]. With prolonged follow-up, the tumor was clinically malignant (ie, it recurred and/or metastasized) in 18 percent of patients. All of the well differentiated tumors had benign behavior, while malignant behavior occurred in 11 percent of those with intermediate differentiation, 59 percent of poorly differentiated tumors, and 19 percent of those with heterologous elements.
Other
●Gynandroblastoma is no longer considered an entity in the 2014 WHO classification system. Nevertheless, senior expert gynecologic pathologists still believe that gynandroblastoma exists but is exceedingly rare.
Patient age at diagnosis ranges from 16 to 65 years (average 30 years) [77]. These tumors can be associated with androgen and (less commonly) estrogen production.
The current classification system divides this category into:
•Pure stromal tumors
•Pure sex cord tumors
•Mixed SCSTs (table 1)
Gynandroblastomas are usually benign, small neoplasms with Sertoli-Leydig and granulosa cell differentiation [78]. The diagnosis should be reserved for neoplasms containing at least 10 percent of the minor component (Sertoli-Leydig or granulosa).
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Ovarian, fallopian tube, and peritoneal cancer".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)
●Basics topics (see "Patient education: Sertoli-Leydig cell tumor (The Basics)")
SUMMARY AND RECOMMENDATIONS
●Ovarian sex cord-stromal tumors (SCSTs) (table 1) are a rare group of benign and malignant neoplasms. (See 'Introduction' above and 'Epidemiology' above and 'Pathogenesis' above.)
●Patients with SCSTs generally present similarly to patients with epithelial or germ cell ovarian neoplasms: because of abdominal or pelvic symptoms from the mass or as an incidental finding of adnexal mass on examination or imaging. Because some SCSTs produce androgens and/or estrogen, patients with these histologic types may present with signs of virilization or estrogen excess. (See 'Clinical presentation and diagnostic considerations' above.)
●Patients with ovarian fibromas or luteinized thecoma associated with sclerosing peritonitis may present with ascites. Sclerosing peritonitis can also cause bowel obstruction. (See 'Ascites with or without other abdominal symptoms' above.)
●In contrast to the more common epithelial ovarian cancer, malignant SCSTs are often diagnosed at an early stage, histology is generally low grade, lymph node metastases are rare, and prognosis is good. However, some tumors are aggressive and have a lethal outcome (eg, fibrosarcoma). (See 'General features' above.)
●If an SCST is suspected because of the combination of an adnexal mass and endocrine effects, the diagnostic work-up includes laboratory tests (eg, testosterone, estradiol, tumor markers) and imaging studies. Endometrial sampling should be performed in premenopausal patients with an adnexal mass and abnormal uterine bleeding and in postmenopausal patients with a thickened (>4 to 5 mm) endometrial stripe on transvaginal ultrasound examination since these signs/symptoms can be due to excess estrogen production and affect management. (See 'Diagnostic evaluation' above.)
●In all patients with an adnexal mass, surgery is performed to resect and determine the histology of an ovarian mass if ultrasound findings suggest the mass has some reasonable chance of being malignant (table 3). Surgery to resect the mass is also performed if there are other risks associated with the mass (eg, torsion) or the mass is causing symptoms. Patients with SCSTs generally meet one or more of these criteria. (See 'Diagnostic evaluation' above.)
●The diagnosis of ovarian SCST is a histologic diagnosis. The World Health Organization classification of ovarian SCSTs and other ovarian neoplasms is shown in the table (table 1). (See 'Diagnosis' above.)
●Fibromas are the most common of the benign SCSTs. Granulosa cell tumors are the most common type of potentially malignant ovarian SCST. Comparative characteristics of several types of SCSTs are shown in the table (table 4). (See 'Histopathology and clinical behavior' above.)
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