Ovarian androblastoma is a rare hormone-producing ovarian tumor. It is diagnosed mainly at a young age. It is manifested by the appearance of acne, the disappearance of menstruation, a change in the figure of the male type, hirsutism, coarsening of the voice and an increase in the clitoris. Less often there is premature isosexual development (in adolescents) or the appearance of bloody discharge from the vagina (in menopausal women). Ovarian androblastoma is more often highly differentiated and proceeds benign. In some cases, a malignant course is possible. The diagnosis is made on the basis of complaints, general and gynecological examination data and the results of additional studies. The treatment is operative.
ICD 10
C56 D27
General information
Ovarian androblastoma is a tumor of the ovary that secretes male sex hormones. According to various data, it is 0.2-0.4% of the total number of ovarian tissue tumors. It is usually found in teenage girls and young women. It can be highly differentiated, intermediate or low-differentiated.
Highly differentiated ovarian androblastomas with a relatively benign course are more often detected. Less often, usually at an older age or with a low level of cell differentiation, a malignant course is observed. After removal of the ovarian androblastoma, excessive hair growth on the body stops, the ovulatory cycle is restored, the figure again acquires female outlines. In highly differentiated tumors, the prognosis is favorable. Diagnosis and treatment are carried out by specialists in the field of oncogynecology.
Causes of ovarian androblastoma
The causes of ovarian androblastoma are unknown. It is believed that such tumors are formed from the embryonic remains of the male part of the gonad under the influence of certain changes in the hypothalamic-pituitary system or in connection with the peculiarities of the interaction of luteinizing hormone with the corresponding receptors in the ovaries, however, what changes cause the growth of such neoplasms has not yet been established. Hereditary predisposition was not revealed.
Classification
Ovarian androblastoma belongs to the group of virilizing tumors developing from mesenchymal tissue. It is usually single and unilateral, bilateral tumors occur in less than 1% of cases. Taking into account the level of cell differentiation, three variants of ovarian androblastomas are distinguished:
- Highly differentiated.
- Intermediate.
- Low-grade.
There are four types of highly differentiated androblastomas:
- tubular adenoma;
- androblastoma with lipid accumulation;
- leidigoma;
- a tumor from Leydig and Sertoli cells.
Tubular adenoma and androblastoma of the ovary with lipid accumulation consist of Sertoli cells. The effect of virilization is largely provided by Leydig cells, the effect of feminization (with premature isosexual development) is provided by Sertoli cells.
Symptoms of ovarian androblastoma
The disease is usually diagnosed before the age of 40, the peak incidence occurs in the third decade of life. In some cases, children suffer. The development of ovarian androblastoma is accompanied by the appearance of signs of musculinization. Hirsutism occurs in 2/3 of cases, amenorrhea occurs in 1/3 of cases. For about six months, the patient becomes lean, and then menstruation stops, the figure changes, acne and excessive hair loss occur.
Usually the disease occurs in two stages. At the first stage of ovarian androblastoma (the stage of defeminization), oligomenorrhea develops, and then amenorrhea. Mammary glands atrophy, fat deposits disappear, giving a woman’s figure a characteristic roundness and softness. At the second stage of ovarian androblastoma (the stage of masculinization), the clitoris increases, excessive hair loss occurs. The voice becomes rougher. Some patients have receding hairlines in the parietal and frontal areas.
Cases of ovarian androblastoma combined with hyperestrogenemia have been described. In girls, premature puberty begins according to gender. Menstrual cycle disorders are noted in women of reproductive age, bloody discharge from the genital tract appears in postmenopausal patients. This variant of the disease is not typical for androblastoma and is quite rare.
Diagnostics
The diagnosis of ovarian androblastoma is established on the basis of complaints, anamnesis, external and gynecological examination data and the results of instrumental studies. Usually, patients turn to a gynecologist or endocrinologist with complaints about the absence of menstruation, excessive hair loss and a change in appearance. A general examination of patients with ovarian androblastoma reveals atrophy of the mammary glands, hirsutism and a male-type figure change.
- Examination on the chair. Gynecological examination reveals hypertrophy of the clitoris and a painless, mobile, smooth tumor-like formation of dense consistency, located to the right or left of the uterus.
- Ultrasound of the genitals. Sonography indicates the presence of a tumor-like formation of a cystic, solid or cystic-solid type with a clearly defined capsule and an inhomogeneous internal structure, characterized by alternating hyper- and hypoechoic sites.
- Hormonal studies. When conducting blood tests for hormones in patients with ovarian androblastoma, an increase in testosterone levels, a decrease in the level of luteinizing and follicle-stimulating hormone are detected. The level of dehydroepiandrosterone is normal or slightly elevated.
Treatment of ovarian androblastoma
The treatment is surgical. The volume of surgical intervention is determined taking into account the age of the patient. Patients of reproductive age suffering from ovarian androblastoma usually undergo unilateral adnexectomy. During the operation, the second ovary must be audited. With an increase in the size of the ovary, the organ is dissected, an intraoperative histological examination is performed to exclude a second tumor. In post-menopausal age, a pangisterectomy is usually performed – a hysterectomy with the removal of appendages.
With a malignant course, a low level of differentiation and rupture of the capsule of an ovarian androblastoma of a moderate degree of differentiation, radical surgical intervention with removal of the uterus is indicated regardless of the patient’s age. In the postoperative period, chemotherapy and radiation therapy are prescribed, but the effectiveness of combination therapy for malignant ovarian androblastomas is still difficult to assess due to the small number of observations.
Forecast
The prognosis is usually favorable. 90-95% of patients with highly differentiated ovarian androblastoma overcome the ten-year survival threshold. With a malignant tumor diagnosed at stage I and the use of radiation therapy in the postoperative period, the five-year survival rate according to some data is 75%. At the beginning of treatment at stages II and III, there is a decrease in the five-year survival rate to 50%. A characteristic feature of ovarian androblastoma is early recurrence. 60% of relapses are detected within the first year after surgery. Signs of virilization disappear within 1-1.5 years after surgery. Hypertrophy of the clitoris persists throughout life.
