Tumors of the Stroma of the Genital Cord (Hormone-active)

What are Tumor Stroma Tumors (Hormone-active)?

Tumor stroma tumors include granulosa-stromal cell tumors (granulosa cell tumor and tecomphibroma group) and androblastomas, tumors that originate from granulosa cells, tech cells, Sertoli cells, Leydig cells and ovarian stroma fibroblasts. In some cases, female and male tumor components are combined (ginandroblastomas). Hormone-dependent tumors are divided into feminizing (granulosa cell and tekoma) and masculinizing (androblastoma).

Most neoplasms contain ovarian cells (granulosa-stromal cell tumors). A smaller part is represented by derivatives of testicular cells (Sertoli – stromal cell tumors). If it is impossible to differentiate female and male types of tumors, the term “unclassified tumors of the sex cord and ovarian stroma” can be used.

Tumors of stroma of the genital cord make up about 8% of all ovarian formations.

Feminizing tumors are found at any age: granulosa cell disease is more common in children and at a young age, tecoma in pre- and postmenopausal women and extremely rare in children. A granulosa cell tumor (folliculoma) develops from granulosa tissue, similar in structure to the granular epithelium of a ripening follicle. Tecoma consists of cells similar to theca cells of atretic follicles. Granulosa cell tumors make up 1-2% of all ovarian neoplasms. Tecoms are 3 times less common.

Symptoms of Tumors of the Stroma of the Genital Cord (Hormone-active)

Clinical manifestations are associated with the hormonal activity of feminizing tumors. Granulosa cell tumor of the “juvenile type” causes premature puberty, which is more correctly considered false due to the lack of ovulation. In girls, irregular spotting from the genital tract appears with a slight development of secondary sexual characteristics; features of estrogenic influence are determined – a symptom of the “pupil”, cyanosis of the vulva, folding of the vagina, and an increase in the body of the uterus. Somatic development is not accelerated. Bone age corresponds to the calendar. In reproductive age, dysfunctional uterine bleeding is possible. Feminizing tumors in old age are usually manifested by metrorrhagia, which is a particularly significant symptom of a neoplasm. In the postmenopausal period, the level of estrogen hormones with the “rejuvenation” of the patient is increased. Proliferative processes can be detected in the endometrium: glandular-cystic hyperplasia, often with atypia of varying degrees, endometrial polyps, the development of endometrial adenocarcinoma is possible.

Diagnosis of Tumors of the Stroma of the Genital Cord (Hormone-active)

The diagnosis is established on the basis of a pronounced clinical picture, data from a general examination and gynecological examination, tests of functional diagnostics, the level of hormones, ultrasound with CDC, laparoscopy.

A hormone-producing feminizing tumor of the ovary with a two-handed abdominal-abdominal examination is defined as a unilateral formation with a diameter of 4 to 20 cm (average 10-12 cm), dense or tight-elastic consistency (depending on the proportion of fibrous or tecmatous stroma), mobile, smooth, painless.

The granulosa cell tumor has a clear capsule, in the section pronounced lobation and yellow color, focal hemorrhages and fields of necrosis. At tekoma, the capsule is usually absent: a solid structure is usually visible in the section, a fabric with a yellowish tinge up to an intense yellow color. Foci of hemorrhage, cysts are not typical. In most cases, tecoms are unilateral and rarely malignant. Diameter ranges from 5 to 10 cm.

On echograms, feminizing tumors are visualized in the form of a one-sided formation of a rounded shape with a predominantly echopositive internal structure and echo-negative inclusions, often multiple. The diameter of the tumor is 10-12 cm.

The tumor may have cystic variants and in such cases resembles ovarian cystadenoma. Sound conductivity of tumors is common. A comparison of anamnestic data, an echographic picture with a visualized pathology of the endometrium (especially in the postmenopausal age) helps to establish the correct diagnosis.

With CDC, multiple vascularization zones are visualized both in the tumor itself and on its periphery. The internal structures of the formation look like a mottled mosaic with a predominance of venous blood flow. In spectral Doppler mode, blood flow in ovarian tumors has a low systolic rate and low resistance (IR below 0.4).

Diagnostic accuracy for ultrasound with CDC is 91.3%, while using CT and MRI 84.9 and 89.9%.

Feminizing tumors can be benign (80%) and malignant. Malignancy is determined by metastases and relapses. Metastases occur mainly in the serous cover of the abdominal cavity organs, on the parietal peritoneum and in the omentum. Granulosa cell tumor is most often malignant, tecom is extremely rare. Some descriptions of malignant techomas refer to observations of fibrosarcomas or diffuse granulosa cell tumors of the ovary.

Treatment of Tumors of the Stroma of the Genital Cord (Hormone-active)

Treatment of feminizing tumors is only surgical. Volume and access (laparotomy – laparoscopy) depend on the patient’s age, education size, condition of the other ovary and para-aortic lymph nodes, concomitant genital and extragenital pathology.

During the operation, an urgent histological examination is done, an abdominal cavity is revised, another ovary is carefully examined, with an increase in it, a biopsy is indicated.

In girls with a benign granulosa cell tumor, only the affected ovary is removed, in patients of the reproductive period, removal of the uterine appendages of the affected side is indicated. In perimenopausal age, supravaginal amputation of the uterus with appendages or extirpation of the uterus with appendages is performed (depending on changes in the endometrium). Small tumors can be removed by surgical laparoscopy.

In a malignant tumor (according to the results of an urgent histological conclusion), hysterectomy with appendages on both sides and removal of the omentum are indicated.

Ovarian fibroma occupies a special place among fibroma-tech and develops from connective tissue. This is essentially a hormone-inactive tecom. The structure of the tumor is represented by interwoven bundles of spindle-shaped cells producing collagen. Ovarian fibroma is a relatively rare benign tumor. Fibromas make up from 2.5 to 4% of all tumors, are found at any age, often aged 40-60 years, the size of the tumor is from 3 to 15 cm. Until puberty, ovarian fibroma does not occur. Patients have an unfavorable premorbid background with frequent violations of menstrual and generative functions. Perhaps these disorders are due to the same etiological factor that caused the tumor.

Ovarian fibroma is often combined with uterine myoma. Fibroma and cyst in the same ovary are not excluded. When combined with other diseases, the clinical picture is determined by the combination of their symptoms.

Ovarian fibroma is often detected accidentally during surgery. Fibroma growth is slow, but with dystrophic changes, the tumor can grow rapidly.

Ovarian fibroma does not produce steroid hormones, but in 10% of cases it may be accompanied by Meigs syndrome (ascites in combination with hydrothorax and anemia). The development of these processes is associated with the release of edematous fluid from the tumor tissue and its entry from the abdominal cavity into the pleural cavity through the hatches of the diaphragm. In the section, fibroma tissue is usually dense, white, fibrous, sometimes with zones of edema and cystic degeneration, calcification is possible, sometimes diffuse. The tumor is localized in one ovary in the form of a clearly defined node.

With an increase in mitotic activity, the tumor is classified as borderline with a low malignant potential.

Ovarian fibroma is diagnosed based on the clinical course of the disease and data from a two-handed vaginal-abdominal examination. The tumor has to be differentiated with a subserous myomatous node on the pedicle, as well as with tumors of a different structure. A gynecological examination on the side or behind the uterus determines a volumetric formation with a diameter of 5-15 cm, round or ovoid in shape, of a dense, almost rocky consistency, with a flat surface, mobile, painless. Ovarian fibroma is often accompanied by ascites, so it is sometimes mistaken for a malignant neoplasm.

Diagnosis helps ultrasound with CDK. On the echograms, a rounded or oval formation with clear even contours is visible. The internal structure is predominantly homogeneous echopositive. medium or low echogenicity. Echo-negative inclusions that indicate degenerative changes are sometimes detected. Directly behind the tumor, pronounced sound absorption is determined. With CDC, vessels in fibromas are not visualized, the tumor is avascular.

The sensitivity and specificity of MRI and CT in the diagnosis of ovarian fibroma are equivalent to ultrasound.

When laparoscopy of ovarian fibroma is round or ovoid, with a smoothed surface topography and scanty vascularization. The capsule is usually whitish, vessels are determined only in the area of ​​the fallopian tube. A whitish-pink hue of the capsule color is also possible. The consistency of the tumor is dense.

Fibroma treatment is operational. The volume and access of the surgical intervention depend on the size of the tumor, the age of the patient, and associated gynecological and extragenital diseases. As a rule, uterine appendages of the affected side are removed in the absence of indications for removal of the uterus in patients of reproductive age. For a small tumor, laparoscopic access is used.

The forecast is favorable.