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GCNIS is seen in the following settings:
- Almost all invasive germ cell tumours of the testis in adults
- Fifty percent of patients with GCNIS developed invasive germ cell tumours within five years of initial diagnosis.
- Five percent of contralateral testes in men with a history of prior testicular germ cell tumour.
- Less than five percent of cryptorchid testes.
- Less than one percent of patients with infertility.
Not all germ cell tumors (GCTs) arise from "intratubular germ cell neoplasia". The following testicular GCTs do not arise from ITGCN:
- Spermatocytic seminoma
- Pediatric Yolk sac tumors (endodermal sinus tumour). This is currently an area of controversy as some authors dispute the absence of ITGCN in these cases.
- Teratoma (rare exceptions)
The signs and symptoms are similar to other cervical cancers and may include post-coital bleeding and/or pain during intercourse (dyspareunia). Early lesions may be completely asymptomatic.
Clear-cell adenocarcinoma is a type of adenocarcinoma that shows clear cells.
Types include:
- Clear-cell adenocarcinoma of the vagina
- Clear-cell ovarian carcinoma
- Uterine clear-cell carcinoma
- Clear-cell adenocarcinoma of the lung (which is a type of Clear-cell carcinoma of the lung)
See also:
- Clear-cell squamous cell carcinoma of the lung
Urachal cancer can exist for some years without any symptoms. The most frequent initial symptom is haematuria which occurs when the urachal tumour has penetrated the bladder wall, but mucinuria (mucin in the urine), local pain or swelling, recurrent local or urinary tract infections and umbilical discharge can (but is not always) be seen.
In the ovary, embryonal carcinoma is quite rare, amounting to approximately three percent of ovarian germ cell tumours. The median age at diagnosis is 15 years. Symptoms and signs are varied, and may include sexual precocity and abnormal (increased, reduced or absent) uterine bleeding.
There may be elevations in serum human chorionic gonadotropin (hCG) and alpha fetoprotein (AFP) levels but it would be in association with other tumors, (e.g. yolk sac tumor) because they themselves do not produce the serum markers. At surgery, there is extension of the tumour beyond the ovary in forty percent of cases. They are generally large, unilateral tumours, with a median diameter of 17 centimetres. Long-term survival has improved following the advent of chemotherapy. The gross and histologic features of this tumour are similar to that seen in the testis.
In the testis pure embryonal carcinoma is also uncommon, and accounts for approximately ten percent of testicular germ cell tumours. However, it is present as a component of almost ninety percent of mixed nonseminomatous germ cell tumours. The average age at diagnosis is 31 years, and typically presents as a testicular lump which may be painful. One fifth to two thirds of patients with tumours composed predominantly of embryonal carcinoma have metastases at diagnosis.
A malignant mixed Müllerian tumor, also known as malignant mixed mesodermal tumor, MMMT and carcinosarcoma, is a malignant neoplasm found in the uterus, the ovaries, the fallopian tubes and other parts of the body that contains both carcinomatous (epithelial tissue) and sarcomatous (connective tissue) components. It is divided into two types, homologous (in which the sarcomatous component is made of tissues found in the uterus such as endometrial, fibrous and/or smooth muscle tissues) and a heterologous type (made up of tissues not found in the uterus, such as cartilage, skeletal muscle and/or bone). MMMT account for between two and five percent of all tumors derived from the body of the uterus, and are found predominantly in postmenopausal women with an average age of 66 years. Risk factors are similar to those of adenocarcinomas and include obesity, exogenous estrogen therapies, and nulliparity. Less well-understood but potential risk factors include tamoxifen therapy and pelvic irradiation.
Adenocarcinoma (; plural adenocarcinomas or adenocarcinomata ) is a type of cancerous tumor that can occur in several parts of the body. It is defined as neoplasia of epithelial tissue that has glandular origin, glandular characteristics, or both. Adenocarcinomas are part of the larger grouping of carcinomas, but are also sometimes called by more precise terms omitting the word, where these exist. Thus invasive ductal carcinoma, the most common form of breast cancer, is adenocarcinoma but does not use the term in its name—however, esophageal adenocarcinoma does to distinguish it from the other common type of esophageal cancer, esophageal squamous cell carcinoma. Several of the most common forms of cancer are adenocarcinomas, and the various sorts of adenocarcinoma vary greatly in all their aspects, so that few useful generalizations can be made about them.
In the most specific usage (narrowest sense), the glandular origin or traits are exocrine; endocrine gland tumors, such as a VIPoma, an insulinoma, or a pheochromocytoma, are typically not referred to as adenocarcinomas but rather are often called neuroendocrine tumors. Epithelial tissue sometimes includes, but is not limited to, the surface layer of skin, glands, and a variety of other tissue that lines the cavities and organs of the body. Epithelial tissue can be derived embryologically from any of the germ layers (ectoderm, endoderm, or mesoderm). To be classified as adenocarcinoma, the cells do not necessarily need to be part of a gland, as long as they have secretory properties. Adenocarcinoma is the malignant counterpart to adenoma, which is the benign form of such tumors. Sometimes adenomas transform into adenocarcinomas, but most do not.
Well differentiated adenocarcinomas tend to resemble the glandular tissue that they are derived from, while poorly differentiated adenocarcinomas may not. By staining the cells from a biopsy, a pathologist can determine whether the tumor is an adenocarcinoma or some other type of cancer. Adenocarcinomas can arise in many tissues of the body owing to the ubiquitous nature of glands within the body, and, more fundamentally, to the potency of epithelial cells. While each gland may not be secreting the same substance, as long as there is an exocrine function to the cell, it is considered glandular and its malignant form is therefore named adenocarcinoma.
Villoglandular adenocarcinoma of the cervix, also villoglandular papillary adenocarcinoma, papillary villoglandular adenocarcinoma and well-differentiated villoglandular adenocarcinoma, abbreviated VGA, is a rare type of cervical cancer that, in relation to other cervical cancers, is typically found in younger women and has a better prognosis.
A similar lesion, "villoglandular adenocarcinoma of the endometrium", may arise from the inner lining of the uterus, the endometrium.
Estrogens are produced by "functioning" tumours, and the clinical presentation depends on the patient's age and sex.
- Female
- If the patient is postmenopausal, she usually presents with abnormal uterine bleeding, and in some cases hemoperitoneum.
- If the patient is of reproductive age, she would present with menometrorrhagia. However, in some cases she may stop ovulating altogether.
- If the patient has not undergone puberty, early onset of puberty may be seen.
- these tumors tend to have late recurrencies ( even after 30 years )
Most vaginal cancers do not cause signs or symptoms early on. When vaginal cancer does cause symptoms, they may include:
- Vaginal discharge or abnormal bleeding.
- Unusally heavy flow of blood
- Bleeding after menopause
- Bleeding between periods; or any other
- Bleeding that is longer than normal for you
- Blood in the stool or urine
- Frequent or urgent need to urinate
- Feeling constipated
- pain during sexual intercourse
- a lump or growth in the vagina that can be felt
Enlarged pelvic lymph nodes can sometimes be palpated.
Adenomatoid tumor is a benign mesothelial tumor, which arises from the lining of organs. It generally presents in the genital tract, in regions such as the testis and epididymis. It is the second most common extratesticular scrotal mass, after lipoma, and accounts for 30% of these masses. It also has been found in the pancreas.
In the female, it has been found in the body of the uterus and the fallopian tube.
Despite their name, germ cell tumors occur both within and outside the ovary and testis.
- head
- inside the cranium — pineal and suprasellar locations are most commonly reported
- inside the mouth — a fairly common location for teratoma
- neck
- mediastinum — account for 1% to 5% of all germ cell neoplasms
- pelvis, particularly sacrococcygeal teratoma
- ovary
- testis
In females, germ cell tumors account for 30% of ovarian tumors, but only 1 to 3% of ovarian cancers in North America. In younger women germ cell tumors are more common, thus in patients under the age of 21, 60% of ovarian tumors are of the germ cell type, and up to one-third are malignant. In males, germ cell tumors of the testis occur typically after puberty and are malignant (testicular cancer). In neonates, infants, and children younger than 4 years, the majority of germ cell tumors are sacrococcygeal teratomas.
Males with Klinefelter syndrome have a 50 times greater risk of germ cell tumors (GSTs). In these persons, GSTs usually contain nonseminomatous elements, present at an earlier age, and seldom are gonadal in location.
Urachal cancer is a very rare type of cancer arising from the urachus or its remnants. The disease might arise from metaplasic glandular epithelium or embryonic epithelial remnants originating from the cloaca region.
It occurs in roughly about one person per 1 million people per year varying on the geographical region. Men are affected slightly more often than women mostly in the 5th decade of life but the disease can occur in also in other age groups.
It can involve the urinary bladder, but is not bladder cancer in the usual sense. Urachal cancer can occur at any site along the urachal tract.
Urachal cancer was mentioned by Hue and Jacquin in 1863 followed by an elaborate work by T. Cullen in 1916 about diseases of the umbilicus, while C. Begg further characterized urachal cancer in the 1930s. Detailed diagnostic and staging schemes were proposed by Sheldon et al in 1984, which remain widely used today.
Spermatocytic seminoma is a rare tumour, making up only one to two percent of all testicular germ cell tumours. Men presenting with this tumour are generally 50 to 60 years old, and its occurrence is rare in men under 30 years old. Most present with slow, painless testicular enlargement, which may involve both testes.
Spermatocytic seminoma is a neoplasm of the testis ("i.e." a tumour of the testis), and classified as a germ cell tumour.
The name of the tumour comes from the similarity (under the microscope) between the small cells of the tumour and secondary spermatocytes.
Signet ring cell carcinoma (SRCC) is a rare form of highly malignant adenocarcinoma that produces mucin. It is an epithelial malignancy characterized by the histologic appearance of signet ring cells.
Primary SRCC tumors are most often found in the glandular cells of the stomach (SRCC originates in the stomach in 90 percent of patients), and less frequently in the breast, gallbladder, urinary bladder, and pancreas. SRCCs do not normally form in the lungs, though a few incidences have been reported.
Among colorectal cancers, the prevalence of SRCC is less than one percent. Though incidence and mortality of gastric cancer has declined in many countries over the past 50 years, there has been an increase in occurrences of gastric SRCC-type cancers.
SRCC tumors grow in characteristic sheets, which makes diagnosis using standard imaging techniques, like CT and PET scans, less effective.
Pain is the most common symptom, followed by either sensorineural or conductive hearing loss, tinnitus or drainage (discharge). A mass lesion may be present, but it is often slow growing.
Signs and symptoms of pseudomyxoma peritonei may include abdominal or pelvic pain and/or bloating, distension, digestive disorders, weight changes, increased girth, and infertility.
Vaginal cancer is any type of cancer that forms in the tissues of the vagina. Primary vaginal cancer is rare in the general population of women and is usually a squamous-cell carcinoma. Metastases are more common. Vaginal cancer occurs more often in women over age 50, but can occur at any age, even in infancy. It often can be cured if found and treated in early stages. Surgery alone or surgery combined with pelvic radiation is typically used to treat vaginal cancer.
This tumor only affects the outer 1/3 to 1/2 of the external auditory canal as a primary site. If this area is not involved, the diagnosis should be questioned. The most common tumor type is ceruminous adenoid cystic carcinoma and ceruminous adenocarcinoma, NOS.
Granulosa cell tumours (or granulosa-theca cell tumours or folliculoma) are tumours that arise from granulosa cells. These tumours are part of the sex cord-gonadal stromal tumour or non-epithelial group of tumours. Although granulosa cells normally occur only in the ovary, granulosa cell tumours occur in both ovaries and testicles (see Ovarian cancer and Testicular cancer). These tumours should be considered malignant and treated in the same way as other malignant tumours of ovary. The ovarian disease has two forms, juvenile and adult, both characterized by indolent growth, and therefore has high recovery rates.
The staging system for these tumours is the same as for epithelial tumours and most present as stage I. The peak age at which they occur is 50–55 years, but they may occur at any age.
Juvenile granulosa cell tumour is a similar but distinct rare tumour. It too occurs in both the ovary and testis. In the testis it is extremely rare, and has not been reported to be malignant. Although this tumour usually occurs in children (hence its name), it has been reported in adults.
Mixed germ cell tumors occur in many forms. Among these, a common form is teratoma with endodermal sinus tumor.
Teratocarcinoma refers to a germ cell tumor that is a mixture of teratoma with embryonal carcinoma, or with choriocarcinoma, or with both. This kind of mixed germ cell tumor may be known simply as a teratoma with elements of embryonal carcinoma or choriocarcinoma, or simply by ignoring the teratoma component and referring only to its malignant component: embryonal carcinoma and/or choriocarcinoma. They can present in the anterior mediastinum.
There is debate over the naming of MMMT; the term carcinosarcoma was formerly used to describe lesions with homologous tumors, and "malignant mixed Müllerian tumor" or "mixed mesodermal tumor" was used to describe heterologous tumors. While "carcinosarcoma" now considered standard, "malignant mixed Müllerian tumor" has a lengthy history within gynecological literature and is expected to continue to be used. The naming issue to a certain extent reflects histological characteristics and development of the tumors, in which the different types of tissues are believed to either develop separately and join into a single mass (the "collision" theory), that an adenocarcinoma stimulates the stroma to create a tumor (the "composition" theory), or that the tumor is the result of a stem cell that differentiates into different cell types (the "combination" theory). "Collision" tumors are normally easily recognized and not considered true MMMTs; the "combination" theory is most widely held, and is due to evidence that the tumors develop from a single line of cells, developing in a fashion similar to the fundus of the uterus from the Müllerian duct - first from a stem cell into a population of cells, that then differentiates into epithelial and stromal components.
There is evidence that some tumors are better explained by the composition theory, due to the aggressive nature of the epithelial cells involved which tend to metastasize much more readily than the sarcomal component. The behavior of MMMT overall is more related to the type and grade of the epithelium than the sarcoma, which suggests the sarcomal portion is an atypical "bystander" than primary driver of the tumor. Despite this, when purely endometrial tumors are compared to MMMTs, the MMMT tumor tends to have a worse prognosis.