- Terminal neoplasms of the ovary
- Germinal tumors of the testicles
- Primary germline neoplasms of the central nervous system
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Germ cell tumors (GCT) can occur in both women and men. What are the risk factors and causes of a germline tumor? What are the types of germ cell tumors? How is their treatment going?
Germ cell tumors( germ cell tumor (GCT) are found in both women and men, and are also present in children.
Terminal neoplasms of the ovary
Terminal ovarian tumors account for approximately 20-30% of all ovarian tumors. They arise from multipotent germ cells that colonize embryonic gonads or from somatic stem cells present in the ovaries. They differentiate into mature, embryonic and extra-embryonic tissues.
They are more common in children and young women, and what's more - the younger the age at which the tumor occurs, the more likely it is to become malignant. In girls and teenagers, 70% of ovarian tumors are germinal tumors - almost 67% of them are malignant.
Fortunately, most germinal tumors are completely treatable due to their high chemo- and radiosensitivity. Interestingly, the most common germinal tumor of the ovary is cystic teratoma.
Germinal neoplasms of the ovary include:
- Germs (dysgerminoma)
- yolk sac tumor / endodermal sinus tumor
- germ cell cancer (carcinoma embryonale)
- choriocarcinoma
- teratoma:
- immature
- mature
- solid
- cystic
- single
- mixed germinal tumors
Usually, germline tumors in younger women are homogeneous in terms of histology, while older women are more likely to have mixed tumors with a worse prognosis.
It is worth mentioning here that neither the risk factors for developing germ cell tumors nor the factors protecting against the development of these tumors have been known.
Due to the very rapid growth of germinal tumors, their presence is associated with severe pain and peritoneal symptoms.
It may also be related to the tumor's twist or rupture, which is often accompaniedbleeding into the peritoneal cavity.
There is also some free fluid in the peritoneal (ascites) or pleural cavities.
Late symptoms include:
- abdominal enlargement
- flatulence
- digestive and urinary problems
- genital bleeding
Then it may appear:
- weakness
- lose weight
- wasting (cachexia)
Interestingly, germinal tumors in girls before puberty, the size of which exceeds 2 cm, should be examined histopathologically by performing a laparotomy.
In such a situation, it is also useful to test the karyotype before the surgery, because in the case of diagnosis of gonadal dysgenesis, removal of a second, dysgenetic ovary is recommended.
- Rozrodczak (dysgerminoma)
The germ cell tumor is histologically indistinguishable from the seminoma that occurs in men, with high radiance and composed of germ cells. This tumor grows rapidly, becomes large (more than 10 cm), has a pouch, and is unilateral in most cases.
Interestingly, it is the only terminal cancer of the ovary that can develop on both sides. Moreover, it is the most common malignant tumor of the ovary in pregnant women.
The treatment involves tumor excision with unilateral appendages and complementary chemotherapy.
Besides, it is necessary to take biopsy specimens from the second ovary for the intraoperative examination.
Relapses are rarely observed. They become more frequent the more advanced the tumor was originally.
As many as 95% of patients survive 5 years, regardless of the scope of the surgery.
It is worth mentioning here that the following unfavorable prognostic factors are:
- early age of the patient at the onset of the tumor (under 20)
- tumor size larger than 10 cm
- its high histological aggressiveness (anaplasia, numerous mitoses)
- Yolk sac tumor / tumor sinus endodermalis
The yolk sac tumor is a malignant epithelial tumor that originates from the primary yolk sac. It is the second most common germline tumor.
The average age of onset of this cancer is 18 years, and 1/3 of affected girls are before puberty.
This tumor grows very quickly, becomes large (approximately 15 cm) and develops unilaterally. In addition, it is solid, brittle and has a thin handbag.
Hisa distinctive feature is the release of alpha-fetoprotein, which is a specific and sensitive marker for treatment monitoring. Moreover, the serum level of patients shows an increased level of the tumor marker CA125.
The treatment involves unilateral salpingooforectomy (excision of the tumor along with the ovary and fallopian tube) with the administration of adjuvant chemotherapy.
Patients with stage I and II tumors are completely healed, and relapses are very rare.
Finally, it is worth mentioning that patients may become pregnant after one year of follow-up after the end of chemotherapy.
- Embryonic cancer (carcinoma embryonale)
Embryonic carcinoma is a very rare germ cell tumor of the ovary that grows very large (average diameter is 17 cm) and can secrete alpha-fetoprotein, chorionic gonadotrophin and estrogens.
It occurs in girls before puberty and in young women - the average age of onset is 14 years.
The symptoms, treatment, and prognosis for this cancer are similar to those of yolk sac cancer.
At diagnosis, 40% of patients have peritoneal metastases. It should be mentioned that the introduction of multi-drug chemotherapy significantly improved survival.
- Choriocarcinoma
Chorionic epithelioma is a rare, very aggressive neoplasm of the ovary. Extremely quickly metastasizes through the bloodstream to the lungs, liver, bones, central nervous system and other organs - at the time of tumor diagnosis, metastases are already clinically present.
This tumor is usually part of mixed germinal tumors and produces chorionic gonadotrophin. Its presence in the blood makes it easier to diagnose the disease and monitor the response to treatment.
The treatment uses chemotherapy regimens as in pregnancy-related chorionic cancer. The prognosis for this neoplasm is unfavorable - most patients die shortly after the initiation of treatment.
- Teratoma
Teratomas constitute only 1% of all malignant neoplasms of the ovary, and their structure includes cells and tissues that originate from more than one germ layer.
Interestingly, these are cancers that usually occur unilaterally. They can be divided into mature teratomas, immature teratomas, malignant teratomas and monocellular teratomas.
Due to the fact that these tumors are less sensitive to chemotherapy, it is very importantcomplete removal of all neoplastic lesions.
It is known that in patients who underwent an incomplete resection, the 5-year survival rate is only 50%. Usually, relapse is seen within 2 years of tumor removal.
Mature teratomas are benign lesions and are among the most common ovarian cancers. Foci of malignant transformation can be found in them very rarely.
The most common form of mature teratoma is the dermal cyst, which occurs bilaterally in approximately 8-15% of cases.
Immature teratomas make up 20% of all embryonic tumors of the ovary and consist of elements that resemble embryonic tissues. Moreover, it often happens that the elements of immature teratoma constitute a mixed component of germinal tumors.
Immature teratomas usually occur unilaterally in girls and young women.
In the treatment of girls and teenagers, excision of the appendix and all tumor foci are used. However, in women who have completed reproduction, it is advisable to perform a hysterectomy (excision of the uterus) with appendages.
The prognosis depends largely on the proportion between mature and immature tissues.
Malignant transformation teratomas occur in women usually over 40 years of age. Their presence is suspected when the histopathological examination of the cyst wall reveals the presence of solid nodular lumps, necrosis and hemorrhages.
The most common monolithic teratomas include ovarian goitre (struma ovarii) and carcinoid carcinoid.
Sometimes there are tumors composed of these two elements.
The ovarian goiter is a tumor that resembles the structure of a mature thyroid and in which there may be lesions typical of the thyroid gland, e.g. inflammation or nodular hyperplasia of the thyroid gland.
A carcinoid in the ovary can be either a metastatic tumor, a primary tumor that is a component of a mature teratoma or a primary ovarian carcinoid - then it is usually benign.
- Mixed germ tumors
Mixed germline tumors consist of at least the two above-described neoplasms.
The most common combination is that of a cancer of the yolk sac with a germ cell carcinoma. The treatment involves tumor excision and chemotherapy.
The prognosis for mixed germinal tumors is usually good.
Germinal tumors of the testicles
Testicular germinal tumors are neoplasms arising from the cells of the sperm-forming epithelium of the seminal tubules (epitheliumreproductive).
These cells are multi-potential - this means that they can differentiate into mature, embryonic and extra-embryonic tissues.
They constitute about 90-95% of testicular tumors and can be divided into seminomas - they constitute about 1/3 of germinal tumors and non-seminomatous tumors - they constitute about 1/2 of the germinal tumors.
Testicular germinal tumors are very malignant, metastasize early and occur in young men.
Interestingly, between the ages of 15 and 34, these tumors are the most common cancers in men.
Their peak occurrence falls on the 30th year of life.
The highest incidence is observed in Denmark, Switzerland and the United States, and the lowest in Japan.
Germinal tumors of the testicles are divided as follows (division according to the World He alth Organization WHO):
1. Precursor lesion - intra-tubular germ cell neoplasia
2. Histologically homogeneous tumors
a) seminoma
b) semoma (seminoma spermatocyticum)
c) carcinoma embryonale
d) neoplasm of the yolk sac (yolk sac tumor)
e) choriocarcinoma
f) teratoma
- mature
- immature
- with transformation malicious
3. Histologically heterogeneous tumors - these are mixed forms of tumors from the reproductive epithelium
Interestingly, about 40% of germinal tumors are composed of one histological type, and the rest are a mixture of different histological types.
The latter most often consist of teratoma, germ cell carcinoma, chorionic epithelioma and yolk sac tumor.
Embryonic cancers and teratomas occur in young people (between 20 and 30 years of age), seminomas in slightly older people (after 40 years of age), and spermatocytic seminomas even later (after 50 years of age).
Most likely testicular germline neoplasms arise through the neoplastic transformation of the cells of the sperm-forming epithelium of the seminal tubules. Then the so-called intracubular neoplasia of reproductive cells.
The most important risk factors are
- genetic factors
- testicular dysgenesis
- cryptorchidism
- previously diagnosed testicular tumor
Patients see a doctor when they have a painless tumor, because of pain and swelling of the testicle, or because of symptoms related to metastasis.
Metastases are most often found in retroperitoneal peraortic and mediastinal lymph nodes, but also in the lungs, liver and central nervous system.
CoInterestingly, the histological picture of the metastases may differ from the primary site.
Such a phenomenon can often be observed in the case of chemotherapy of mixed germinal tumors. It happens that metastases include a part of the tumor that is resistant to treatment.
Seminomas, unlike non-seminomas, are very sensitive to radiotherapy and give patients a better prognosis. Very often, seminoma remains confined to the testicle for a long time and then metastasizes to the lymph nodes, while non-seminoma tumors also metastasize through blood vessels.
Germinal tumors produce biomarkers. They are polypeptide hormones and enzymes that are secreted into the bloodstream, such as, for example, AFP, hCG and LDH. They are used both to diagnose these tumors and monitor the response to therapy. The concentration of these markers in the blood serum decreases after treatment and increases with local recurrence or the presence of metastasis.
Treatment and prognosis depend on the clinical stage of the disease and the histological type of the tumor. Usually, surgical treatment of the tumor (excision) is used, followed by chemotherapy or radiotherapy.
- Nasieniak (seminoma)
Seminoma is the most common germinal tumor of the testicle and accounts for approximately 50% of all such tumors. It usually occurs around the age of 40 and is extremely rare before puberty.
Patients see a doctor with a painless testicular tumor and sometimes with gynecomastia, which is associated with elevated levels of hCG (chorionic gonadotropin).
Cancer remains confined to the testicle for a long time, therefore only about 30% of patients have metastases at diagnosis.
In the treatment of this cancer, orchidectomy (testicle excision) and radiotherapy are used, to which he is very sensitive.
Interestingly, an ovarian tumor with the same histological structure is called a germ cell (dysgerminoma).
- Semenoma spermatocyticum
Spermatocytic seminoma is a cancer with a good prognosis that usually occurs after the age of 65.
It grows slowly and hardly ever recovers. Moreover, it is not related to cryptorchidism and is not included in histologically heterogeneous tumors.
Only an orchidectomy is used to treat it. It is not advisable to use radiotherapy or chemotherapy.
- Embryonic cancer (carcinoma embryonale)
Embryonic cancer is a fairly rare malignant neoplasm that is very often found as a component of mixed tumors.
It most often occurs between the ages of 20 and 35 anddoes not occur before puberty.
Patients visit their doctor with a testicular tumor and sometimes with gynecomastia. The treatment involves orchiectomy and chemotherapy.
- Yolk sac tumor / tumor sinus endodermalis
The yolk neoplasm is a malignant neoplasm whose cells differentiate into the structures of the yolk sac.
Interestingly, it is the most common malignant tumor of the testicles in newborns and children - it can appear from birth to 8 years of age.
Pure yolk sac neoplasm is typical for children, and for adults - as a component of non-seminoma germinal tumors.
About 80-90% of children see a doctor with a painless testicular tumor, and the 5-year survival rate is around 91%.
Characteristic of this neoplasm is the increased level of AFP in the blood serum.
- Choriocarcinoma
Chorionic epithelioma is an extremely malignant tumor of the testicle, which differentiates into extra-embryonic tissues such as cytotrophoblast and syncytiotrophoblast. It has a much worse prognosis compared to other germinal tumors.
It usually occurs between the ages of 20 and 40, but never before puberty. Usually it is a component of mixed germinal tumors.
It is worth mentioning here that it can also be present in the uterus, ovary and retroperitoneal space.
The testicle usually remains unchanged, sometimes there may be a palpable lump, and sometimes the tumor is only visible on ultrasound.
Patients usually present with symptoms related to the presence of metastases in the central nervous system, lungs or liver.
Laboratory tests show a very high level of hCG in the blood serum - it may contribute to the development of gynecomastia.
Chemotherapy is used in treatment.
- Teratoma
Teratomas are germline tumors that are made up of cells and tissues derived from more than one germ layer. Depending on the histological structure, they can be both benign and malignant.
In neonates and children, they are the second most common testicular germline tumors and are usually histologically homogeneous - the mean age of the tumor is around 20 months.
In adults, however, they are a component of histologically heterogeneous germinal tumors. Moreover, they may indicate the presence of congenital disordersdevelopment.
Mature teratoma consists of cells and tissues of ecto-, meso-, and endodermal origin, such as smooth muscle strands, bronchial epithelium, intestinal epithelium, nerve trunks, cartilage, teeth, glial tissue, and many others that lie within the connective tissue.
In children, it is a benign neoplasm with a good prognosis.
In adults, prognosis is uncertain, mainly due to the possible presence of immature or malignant lesions.
Immature teratoma consists of immature tissues such as embryonic glandular tubes, blastema or neuroepithelium.
On the other hand, an occasional skin cyst appears as a cyst filled with yellowish greasy masses, often with hair.
Primary germline neoplasms of the central nervous system
Germ neoplasms outside the ovary and testicle may also be present, but rarely, in the central nervous system. Their occurrence is typical for children and teenagers.
They are located along the midline - most often around the III chamber, pineal gland and suprasellar area.
These include:
- germplasm (germinoma)
- embryo cancer (carcinoma embryonale)
- yolk sac tumor
- choriocarcinoma
- teratomas - mature, immature and with malignant transformation
In the diagnosis of these tumors, imaging tests (computed tomography, magnetic resonance imaging) and the examination of the cerebrospinal fluid for the presence of alpha-fetoprotein, chorionic gonadotropin and placental alkaline phosphatase (PLAP) are used.