The majority of Leydig cell tumors are found in males, usually at 5–10 years of age or in middle adulthood (30–60 years). Children typically present with precocious puberty. Due to excess testosterone secreted by the tumour, one-third of female patients present with a recent history of progressive masculinization. Masculinization is preceded by anovulation, oligomenorrhea, amenorrhea and "defeminization". Additional signs include acne and hirsutism, voice deepening, clitoromegaly, temporal hair recession, and an increase in musculature. Serum testosterone level is high.

In men testicular swelling is the most common presenting feature. Other symptoms depend on their age and the type of tumour. If it is secreting androgens the tumour is usually asymptomatic, but can cause precocious puberty in pre-pubertal boys. If the tumour secretes oestrogens it can cause feminisation in young boys. In adults, this causes a number of problems including gynaecomastia, erectile dysfunction, infertility, feminine hair distribution, gonadogenital atrophy, and a loss of libido.

Estrogens are produced by "functioning" tumours, and the clinical presentation depends on the patient's age and sex.

- Female

- If the patient is postmenopausal, she usually presents with abnormal uterine bleeding, and in some cases hemoperitoneum.

- If the patient is of reproductive age, she would present with menometrorrhagia. However, in some cases she may stop ovulating altogether.

- If the patient has not undergone puberty, early onset of puberty may be seen.

- these tumors tend to have late recurrencies ( even after 30 years )

Due to excess testosterone secreted by the tumour, one-third of female patients present with a recent history of progressive masculinization. Masculinization is preceded by anovulation, oligomenorrhoea, amenorrhoea and defeminization. Additional signs include acne and hirsutism, voice deepening, clitoromegaly, temporal hair recession, and an increase in musculature. Serum testosterone level is high.

Thecomas or theca cell tumors are benign ovarian neoplasms composed only of theca cells. Histogenetically they are classified as sex cord-stromal tumours.

They are typically estrogen-producing and they occur in older women (mean age 59; 84% after menopause). (They can, however, appear before menopause.)

60% of patients present with abnormal uterine bleeding, and 20% have endometrial carcinoma.

Granulosa cell tumours (or granulosa-theca cell tumours or folliculoma) are tumours that arise from granulosa cells. These tumours are part of the sex cord-gonadal stromal tumour or non-epithelial group of tumours. Although granulosa cells normally occur only in the ovary, granulosa cell tumours occur in both ovaries and testicles (see Ovarian cancer and Testicular cancer). These tumours should be considered malignant and treated in the same way as other malignant tumours of ovary. The ovarian disease has two forms, juvenile and adult, both characterized by indolent growth, and therefore has high recovery rates.

The staging system for these tumours is the same as for epithelial tumours and most present as stage I. The peak age at which they occur is 50–55 years, but they may occur at any age.

Juvenile granulosa cell tumour is a similar but distinct rare tumour. It too occurs in both the ovary and testis. In the testis it is extremely rare, and has not been reported to be malignant. Although this tumour usually occurs in children (hence its name), it has been reported in adults.

The tumour is subdivided into many different subtypes. The most typical is composed of tubules lined by Sertoli cells and interstitial clusters of Leydig cells.

Leydig cell tumour, also Leydig cell tumor (US spelling), (testicular) interstitial cell tumour and (testicular) interstitial cell tumor (US spelling), is a member of the sex cord-stromal tumour group of ovarian and testicular cancers. It arises from Leydig cells. While the tumour can occur at any age, it occurs most often in young adults.

A Sertoli-Leydig cell tumour is a combination of a Leydig cell tumour and a Sertoli cell tumour from Sertoli cells.

The ovarian fibroma, also fibroma, is a benign sex cord-stromal tumour.

Ovarian fibromas represent 4% of all ovarian neoplasms. They tend to occur mostly during perimenopause and postmenopause, the median age having been reported to be about 52 years, and they are rare in children. Lesions tend to be asymptomatic. If symptoms are present, the most common one is abdominal pain.

On gross pathology, they are firm and white or tan. On microscopic examination, there are intersecting bundles of spindle cells producing collagen.

There may be thecomatous areas (fibrothecoma). The presence of an ovarian fibroma can cause ovarian torsion in some cases.

Variants with edema can be associated with Meigs' syndrome. They may be a part of nevoid basal cell carcinoma syndrome (Gorlin syndrome).

Sex cord–gonadal stromal tumour (or sex cord–stromal tumour) is a group of tumors derived from the stromal component of the ovary and testis, which comprises the granulosa, thecal cells and fibrocytes. In contrast, the epithelial cells originate from the outer epithelial lining surrounding the gonad while the germ cell tumors arise from the precursor cells of the gametes, hence the name germ cell. In humans, this group accounts for 8% of ovarian cancers and under 5% of testicular cancers. Their diagnosis is histological: only a biopsy of the tumour can make an exact diagnosis. They are often suspected of being malignant prior to operation, being solid ovarian tumours that tend to occur most commonly in post menopausal women.

This group of tumours is significantly less common than testicular germ cell tumours in men, and slightly less common than ovarian germ cell tumours in women (see Ovarian cancer).

These tumours are of the following types, characterized by their abnormal production of otherwise apparently normal cells or tissues.

Although each of the cell and tissue types normally occurs in just one sex (male or female), within a tumour they can occur in the opposite sex. Consequently, depending on the specific histology produced, these tumours can cause virilization in women and feminization in men.

A gonadoblastoma is a complex neoplasm composed of a mixture of gonadal elements, such as large primordial germ cells, immature Sertoli cells or granulosa cells of the sex cord, and gonadal stromal cells. Most gonadoblastomas are benign.

Gonadoblastoma is most often associated with an abnormal chromosomal karyotype, gonadal dysgenesis, or the presence of a Y chromosome in over 90% of cases. Gonadoblastoma has been found in association with androgen insensitivity syndrome, mixed gonadal dysgenesis and Turner syndrome, especially in the presence of Y chromosome-bearing cells.

A cystic nephroma, also known as multilocular cystic nephroma, mixed epithelial stromal tumour (MEST) and renal epithelial stromal tumour (REST), is a type of rare benign kidney tumour.

On gross pathological examination, they are solid, sharply circumscribed and pale yellow-tan in colour. 90% are unilateral (arising in one ovary, the other is unaffected). The tumours can vary in size from less than to . Borderline and malignant Brenner tumours are possible but each are rare.

TCC of the ovary is diagnosed by examination of the tissue by a pathologist. It has a characteristic appearance under the microscope and distinctive pattern of immunostaining.

Benign and borderline variants of this neoplasm are rare, and most cases are malignant.

These tumors may have a worse prognosis than serous tumors.

Despite their name, germ cell tumors occur both within and outside the ovary and testis.

- head

- inside the cranium — pineal and suprasellar locations are most commonly reported

- inside the mouth — a fairly common location for teratoma

- neck

- mediastinum — account for 1% to 5% of all germ cell neoplasms

- pelvis, particularly sacrococcygeal teratoma

- ovary

- testis

In females, germ cell tumors account for 30% of ovarian tumors, but only 1 to 3% of ovarian cancers in North America. In younger women germ cell tumors are more common, thus in patients under the age of 21, 60% of ovarian tumors are of the germ cell type, and up to one-third are malignant. In males, germ cell tumors of the testis occur typically after puberty and are malignant (testicular cancer). In neonates, infants, and children younger than 4 years, the majority of germ cell tumors are sacrococcygeal teratomas.

Males with Klinefelter syndrome have a 50 times greater risk of germ cell tumors (GSTs). In these persons, GSTs usually contain nonseminomatous elements, present at an earlier age, and seldom are gonadal in location.

Brenner tumors are an uncommon subtype of the surface epithelial-stromal tumor group of ovarian neoplasms. The majority are benign, but some can be malignant.

They are most frequently found incidentally on pelvic examination or at laparotomy. Brenner tumours very rarely can occur in other locations, including the testes.

Cystic nephromas are often asymptomatic. They are typically discovered on medical imaging incidentally (i.e. an incidentaloma).

Benign tumors of the ovary include ovarian cysts, such as borderline tumor cysts.

Mixed germ cell tumors occur in many forms. Among these, a common form is teratoma with endodermal sinus tumor.

Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both. This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or choriocarcinoma, or simply by ignoring the teratoma component and referring only to its malignant component: embryonal carcinoma and/or choriocarcinoma. They can present in the anterior mediastinum.

Epithelial-stromal tumors are classified on the basis of the epithelial cell type, the relative amounts of epithelium and stroma, the presence of processes, and the location of the epithelial elements. Microscopic pathological features determine whether a surface epithelial-stromal tumor is benign, borderline, or malignant (evidence of malignancy and stromal invasion). Borderline tumors are of uncertain malignant potential.

This group consists of serous, mucinous, endometrioid, clear cell, and brenner (transitional cell) tumors, though there are a few mixed, undifferentiated and unclassified types.

Ovarian cancer is classified according to the histology of the tumor, obtained in a pathology report. Histology dictates many aspects of clinical treatment, management, and prognosis.

- Surface epithelial-stromal tumours, also known as ovarian epithelial tumors, are the most common type of ovarian cancer. It includes serous tumour, endometrioid tumor, and mucinous tumour. They can be benign (cystadenoma) or malignant (cystadenocarcinoma). Less common tumors are malignant Brenner tumor and transitional cell carcinoma of the ovary.

- Sex cord-stromal tumor, including estrogen-producing granulosa cell tumor and virilizing Sertoli-Leydig cell tumor or arrhenoblastoma, accounts for 8% of ovarian cancers.

- Germ cell tumor accounts for approximately 30% of ovarian tumors but only 5% of ovarian cancers, because most germ cell tumors are teratomas and most teratomas are benign. Germ cell tumors tend to occur in young women (20's-30's) and girls. Whilst overall the prognosis of germ cell tumors tend to be favourable, it can vary substantially with specific histology: for instance, the prognosis of the most common germ cell tumor (dysgerminomas) tends to be good, whilst the second most common (endodermal sinus tumor) tends to have a poor prognosis. In addition, the cancer markers used vary with tumor type: choriocarcinomas are monitored with beta-HCG; dysgerminomas with LDH; and endodermal sinus tumors with alpha-fetoprotein.

- Mixed tumors, containing elements of more than one of the above classes of tumor histology.

Surface epithelial-stromal tumors are a class of ovarian neoplasms that may be benign or malignant. Neoplasms in this group are thought to be derived from the ovarian surface epithelium (modified peritoneum) or from endometrial or Fallopian tube (tubal) tissue. Tumors of this type are also called ovarian adenocarcinoma. This group of tumors accounts for 90% to 95% of all cases of ovarian cancer. Serum CA-125 is often elevated but is only 50% accurate so it is not a useful tumor marker to assess the progress of treatment.