Leydig cell tumour
Encyclopedia
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
Sex cord-stromal tumour
Sex cord-gonadal stromal tumour is a group of tumours of sex cord-derived tissues of the ovary and testis. 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...

 group of ovarian
Ovarian cancer
Ovarian cancer is a cancerous growth arising from the ovary. Symptoms are frequently very subtle early on and may include: bloating, pelvic pain, difficulty eating and frequent urination, and are easily confused with other illnesses....

 and testicular cancer
Testicular cancer
Testicular cancer is cancer that develops in the testicles, a part of the male reproductive system.In the United States, between 7,500 and 8,000 diagnoses of testicular cancer are made each year. In the UK, approximately 2,000 men are diagnosed each year. Over his lifetime, a man's risk of...

s. While the tumour can occur at any age, it occurs most often in young adults.

A Sertoli-Leydig cell tumour
Sertoli-Leydig cell tumour
Sertoli-Leydig cell tumour is a group of tumours composed of variable proportions of Sertoli cells, Leydig cells, and in the case of intermediate and poorly differentiated neoplasms, primitive gonadal stroma and sometimes heterologous elements....

 is a combination of a Leydig cell tumour and a Sertoli cell tumour
Sertoli cell tumour
A Sertoli cell tumour, also Sertoli cell tumor , is a Sex cord-gonadal stromal tumor of a specific type that produces Sertoli cells...

.

Presentation

The majority of Leydig cell tumors are found in men, usually at 5–10 years of age or in middle adulthood (30–60 years). Children typically present with precocious puberty. Due to excess testosterone
Testosterone
Testosterone is a steroid hormone from the androgen group and is found in mammals, reptiles, birds, and other vertebrates. In mammals, testosterone is primarily secreted in the testes of males and the ovaries of females, although small amounts are also secreted by the adrenal glands...

 secreted by the tumour, one-third of female patients present with a recent history of progressive masculinization. Masculinization is preceded by anovulation
Anovulation
An anovulatory cycle is a menstrual cycle during which the ovaries do not release an oocyte. Therefore, ovulation does not take place. However, a woman who does not ovulate at each menstrual cycle is not necessarily going through menopause...

, oligomenorrhea
Oligomenorrhea
Oligomenorrhea is infrequent menstruation. More strictly, it is menstrual periods occurring at intervals of greater than 35 days, with only four to nine periods in a year. Also, menstrual periods should have been regularly established previously before the development of infrequent flow...

, amenorrhea and defeminization
Defeminization
Defeminization is a term in developmental biology, especially zoology, referring to an aspect of the process of sexual differentiation by which a potential female-specific structure, function, or behavior is prevented from developing by one of the processes of male development...

. Additional signs include acne
Acne vulgaris
Acne vulgaris is a common human skin disease, characterized by areas of skin with seborrhea , comedones , papules , pustules , Nodules and possibly scarring...

 and hirsutism
Hirsutism
Hirsutism or frazonism is the excessive hairiness on women in those parts of the body where terminal hair does not normally occur or is minimal - for example, a beard or chest hair. It refers to a male pattern of body hair and it is therefore primarily of cosmetic and psychological concern...

, voice deepening, clitoromegaly
Clitoromegaly
Clitoromegaly is an abnormal enlargement of the clitoris ....

, temporal hair recession, and an increase in musculature. Serum testosterone level is high.

Diagnosis

Presence of an ovarian tumour plus hormonal disturbances suggests a Leydig cell tumour, granulosa cell tumour
Granulosa cell tumour
Granulosa cell tumours are tumours that arise from granulosa cells. These tumours are part of the sex cord-gonadal stromal tumouror non-epithelial group of tumours. Although granulosa cells normally occur only in the ovary, granulosa cell tumours occur in both ovaries and testicles...

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

. However, hormonal disturbances, in Leydig tumours, is present in only 2/3 of cases. A conclusive diagnosis is made via histology
Histology
Histology is the study of the microscopic anatomy of cells and tissues of plants and animals. It is performed by examining cells and tissues commonly by sectioning and staining; followed by examination under a light microscope or electron microscope...

, as part of a pathology report made during or after surgery. See also Sex cord-stromal tumour
Sex cord-stromal tumour
Sex cord-gonadal stromal tumour is a group of tumours of sex cord-derived tissues of the ovary and testis. 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...

.

Immunohistochemical markers of Leydig cell tumours include inhibin-alpha, calretinin
Calretinin
Calretinin also known as 29 kDa calbindin is a vitamin D-dependent calcium-binding protein involved in calcium signaling. In humans, the calretinin protein is encoded by the CALB2 gene.- Function :...

, and melan-A.

Treatment

The usual treatment is surgery. The surgery usually is a fertility-sparing unilateral salpingo-oophorectomy. For malignant tumours, the surgery may be radical and usually is followed by adjuvant chemotherapy, sometimes by radiation therapy. In all cases, initial treatment is followed by surveillance. Because in many cases Leydig cell tumour does not produce elevated tumour marker
Tumor marker
A tumor marker is a substance found in the blood, urine, or body tissues that can be elevated in cancer, among other tissue types. There are many different tumor markers, each indicative of a particular disease process, and they are used in oncology to help detect the presence of cancer...

s, the focus of surveillance is on repeated physical examination and imaging.

The prognosis
Prognosis
Prognosis is a medical term to describe the likely outcome of an illness.When applied to large statistical populations, prognostic estimates can be very accurate: for example the statement "45% of patients with severe septic shock will die within 28 days" can be made with some confidence, because...

 is generally good as the tumour tends to grow slowly and usually is benign
Benign
A benign tumor is a tumor that lacks the ability to metastasize. Common examples of benign tumors include moles and uterine fibroids.The term "benign" implies a mild and nonprogressive disease. Indeed, many kinds of benign tumors are harmless to human health...

: 25% are malignant
Malignant
Malignancy is the tendency of a medical condition, especially tumors, to become progressively worse and to potentially result in death. Malignancy in cancers is characterized by anaplasia, invasiveness, and metastasis...

. For malignant tumours with undifferentiated histology, prognosis is poor.

It is usually benign.

See also

  • Androgen-dependent syndromes
    Androgen-dependent syndromes
    Androgen-dependent syndromes are caused by the excessive production of adrenal or gonadal androgens by adrenal adenomas, carcinomas, or hyperplasia, Leydig cell tumors in men, and arrhenoblastomas and polycystic ovarian syndrome in women....

  • Sertoli cell tumour
    Sertoli cell tumour
    A Sertoli cell tumour, also Sertoli cell tumor , is a Sex cord-gonadal stromal tumor of a specific type that produces Sertoli cells...

  • Sertoli-Leydig cell tumour
    Sertoli-Leydig cell tumour
    Sertoli-Leydig cell tumour is a group of tumours composed of variable proportions of Sertoli cells, Leydig cells, and in the case of intermediate and poorly differentiated neoplasms, primitive gonadal stroma and sometimes heterologous elements....

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