Ovarian mixed germ cell-sex cord stromal tumor
Introduction
Introduction of ovarian mixed germ cells-sex stromal tumors Ovarian mixed germ cell-sex stromal tumor is a very rare benign tumor that is different from the usual common gonadal tumor. First described by Masson (1921) and named in 1923 as an egg yolk tube epithelial (epithelimapflugeriens). Hughesdon and Kumarasamg (1970) proposed a mixed germ cell tumor (mixedgermcelltumor), Mostofi Sobin (1977), Sermant et al (1970) proposed atypical gonoblastoma (atypicalgonadoblastoma), and in 1972 Telerman first renamed the tumor to mixed reproduction. Cell-sex stromal tumors are distinguished from gonadal tumors. basic knowledge The proportion of illness: 0.1%-0.3% Susceptible people: women Mode of infection: non-infectious Complications: Germ cell tumor Ovarian yolk sac tumor
Cause
Ovarian mixed germ cell-sex stromal tumor
Causes:
Ovarian mixed germ cell-sex stromal tumor is considered to be a benign tumor derived from yolk tube. Some scholars have proposed: 1. Occurrence in primordial germ cells and luminal epithelial cells. Ovarian mixed germ cells - Sexual cord stromal tumors by germ cells And the composition of the stromal cells of the sex cord, derived from the yolk sac, the primordial germ cells have the potential of multi-directional differentiation, forming a teratoma component when differentiated into the embryo, and forming endodermal sinus tumor and choriocarcinoma components by in vitro differentiation into the embryo. Another type of supporting cells and granulosa cells belong to the sex cord interstitial component, which is derived from the cavity component, but some scholars have proposed to derive from the middle kidney tube. 2. Separate germ cells occur Ovarian mixed germ cells - Sexual stroma tumors can be derived from germ cells alone, differentiate into embryos and embryos in vitro, and can also differentiate and move the precursors of behavioral cord interstitial components.
Pathogenesis:
1. General morphology Most tumors have a complete capsule with a smooth surface, round, oval, lobulated or irregular nodules. The diameter of the tumor is 2.5-30 cm (average 12 cm) and the weight is 100-1050 g (average 540 g). The tumors are all unilateral. The cut surface is gray or light yellow, solid fish-like, crisp and accompanied by cysts of different sizes, containing transparent or milky white liquid, smooth wall, occasionally necrotic foci, and generally lack of calcification.
2. Microscopic examination of ovarian mixed germ cells - Sexual cord stromal tumors consist of a mixture of germ cells and sex cord mesenchymal cells. Talerman (1987) summarized the arrangement into the following three types:
(1) The tumor cells are long and narrow cord-like branches or trabecules, partially extending into a wide column shape, which is clustered by large and round germ cells, surrounded by dense and ede connective tissues of varying degrees. The structure is filled with stromal cells and single or piles of germ cells, and some regions show a small, round eosinophilic transparent Call-Exner-like body structure, which is similar to the histological morphology of gonadal tumors, but lacks calcified balls and Degraded (Figure 1, 2).
2. The tumor cells are tubular but no cavity, surrounded by a thin connective tissue network, and some regions are unclear. The tumor cells are composed of a small cluster or large round or oval cells, which surround the solid tumor nest. Connective tissue (Figure 3).
3. Single or aggregated germ cells are dispersed in a rich sex cord mesenchymal cell component. Although the ratio of the two cellular components varies, the sex cord stromal cells predominate (Fig. 4).
The above three types are often mixed, but often one of them is dominant. Some areas of the tumor cells are dominated by germ cells, and some areas are dominated by sex stromal cells.
Intratumoral germ cells resemble dysplasia, occasionally bipolar or multifilamental division, and sexual interstitial components tend to evolve into precursors that support cells or granulosa cells, lining the lumen wall or surrounding individual or colony The cells form a wide column or mass. The tumor often lacks interstitial hyaline degeneration, calcification and degeneration. However, testicular stromal cells or luteinized stromal cells can be seen in the interstitial, residual ovarian tissue can be seen next to the tumor, and tumors in adolescent patients. Inside, it contains primordial follicles and growing follicles. If ovulation is followed, the remaining corpus luteum and white body are seen.
Prevention
Ovarian mixed germ cell-sex stromal tumor prevention
Regular inspection, early detection, diagnosis, treatment. Follow-up after treatment to prevent malignant transformation. Ovarian mixed germ cell-sex stromal tumors are particularly important because they have tumors that can be considered as malignant potential, and follow-up after treatment to prevent malignant transformation.
Complication
Ovarian mixed germ cell-sexual stromal tumor complications Complications Germ cell ovarian yolk sac tumor
There are 4 cases of squamous cell tumors in this tumor, which are malignant mixed germ cell tumors, which occur after puberty, and their ages are 16, 22, 25 and 31 years old respectively. One 16-year-old patient with yolk sac tumor (endoblast) Sinus tumors, 3 cases invaded the adjacent membrane, 1 case invaded the broad ligament, retroperitoneal and para-aortic lymph nodes.
Symptom
Ovarian mixed germ cells - Sexual stromal tumor symptoms Common symptoms Vaginal irregular bleeding Abdominal pain Early breast development Lower abdominal mass precocious
The main symptoms are irregular vaginal bleeding, abdominal pain and lower abdomen masses. There are 4 cases of endocrine disorders, all of which are young girls under 10 years old. There are pseudopregnant women with puberty precocious puberty, such as mammary gland development, pubic hair growth and vaginal bleeding. The patient only had testicular enlargement, and no abnormalities such as gonadal development, external genital and body structure were found.
Examine
Ovarian mixed germ cell-sex stromal tumor examination
Endocrine examination
In the case of pseudo-pregnant women with precocious puberty, urinary estrogen can be detected, vaginal cell smears have estrogen effects, estrogen in the urine can be converted to normal after tumor resection, symptoms can disappear, a 10-year-old girl (Talerman) , 1972) Postoperative follow-up found that normal body development and normal menstruation began at 15 years of age.
2. Genetic examination
Most patients were tested for karyotype, the gender was female, the sex chromosome group analysis was 46, XX karyotype, and 2 adult women gave birth to normal fetus in full term. All patients had normal ovary, internal and external. There are no abnormalities in the reproductive organs and postures, but it has been reported in the literature that a 5-month-old female newborn found a 22-monomer change in its chromosome.
3. Electron microscopy: There are two kinds of cells in the tumor under electron microscope:
The main spindle cells (sex-derived cells) are aggregated, or loosely dispersed between the extracellular collagen fiber bundles or on the periphery of the collagen fiber bundle, and the substrate is surrounded by a plurality of layers outside the single cell or group of cells, but no The concentric pattern of gonadal cells, spindle cells are often connected by desmosomes, the nucleus is irregular, there are dense nucleoplasms and small nucleoli around the nucleus, cytoplasm has monosaccharides or polysaccharides, a few mitochondria and short segments The rough endoplasmic reticulum, the medium cytoskeletal system is more obvious, the cytoskeleton is composed of 6-8nm microfilaments, and the microfilaments are often dense around the cells, so it is related to cell membrane thickening.
4. The germ cells are in the shape of spindle cells, which are not connected to the spindle cells and have less cytoplasmic bridges. The cytoplasmic bridge is continuous with the cytoplasm between the local cell membrane and the adjacent cells, and the cytoplasmic bridge is thickened. Germ cells have regular elliptical nuclei with fine and uniform nucleoplasms, prominent and loosely arranged nucleoli, abundant cytoplasm (Fig. 5), and a small number of organelles, including monosaccharides, polysaccharides and mitochondria. Found glycogen, part of the tumor's germ cells.
Diagnosis
Diagnosis and differentiation of ovarian mixed germ cell-sex stromal tumor
diagnosis
According to the clinical manifestations, physical signs and the above experimental examinations can make a diagnosis.
Differential diagnosis
1. Gonadoma Although both the tumor and the gonadal tumor are composed of the same two components, the different points of the tumor are arranged differently from the gonadal cell tumor, lacking concentric circles of calcified spheres and tumor nests. The eosinophilic cellulose is deposited at the basement membrane. The tumor is larger than the gonadal tumor. It is clinically non-gonadal dysplasia, the reproductive organs are also normal, and the chromatin is positive. The karyotype of the female patient is 46, XX.
2. Asexual cell tumor If the tumor is dominated by germ cells, it can be similar to asexual cell tumor, but this tumor has sex cord interstitial components, so it can be distinguished.
3. Sexual stromal tumor If the tumor is dominated by the sex cord interstitial component, it is similar to granulosa cell tumor when it is derived from granulosa cells. It seems to support cells when it is derived from supporting cell tumor, but it lacks germ cell components. Can be identified with this tumor.
4. Amphoblastic blastoma Amphotericoma has a Call-Exner body as a representative of granulosa cells, and a supporting cell composed of tubules as a representative of supporting cells. However, seroblastoma lacks germ cell components, so it can be combined with this. Tumor identification.
