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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.
For botryoid rhabdomyosarcoma of the vagina, the most common clinical finding is vaginal bleeding but vaginal bleeding is not specific for sarcoma botryoides: other vaginal cancers are possible. They may appear as a polypoid mass, somewhat yellow in color and are friable: thus, they (possibly) may break off, leading to vaginal bleeding or infections.
Sarcoma botryoides or botryoid sarcoma or botryoid rhabdomyosarcoma is a subtype of embryonal rhabdomyosarcoma, that can be observed in the walls of hollow, mucosa lined structures such as the nasopharynx, common bile duct, urinary bladder of infants and young children or the vagina in females, typically younger than age 8. The name comes from the gross appearance of "grape bunches" ("botryoid" in Greek).
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.
The uterine sarcomas form a group of malignant tumors that arises from the smooth muscle or connective tissue of the uterus.
The internal location of the fallopian tubes makes it difficult to reach an early diagnosis. Symptoms are nonspecific and may consist of pain and vaginal discharge or bleeding. A pelvic mass may be detected on a routine gynecologic examination.
Vaginal discharge in fallopian tube carcinoma result from "intermittent hydrosalphinx" that is called as "hydrops tubae profluens".
Unusual or postmenopausal bleeding may be a sign of a malignancy including uterine sarcoma and needs to be investigated. Other signs include pelvic pain, pressure, and unusual discharge. A nonpregnant uterus that enlarges quickly is suspicious. However, none of the signs are specific. Specific screening test have not been developed; a Pap smear is a screening test for cervical cancer and not designed to detect uterine sarcoma.
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
Primary fallopian tube cancer (PFTC), often just tubal cancer, is a malignant neoplasm that originates from the fallopian tube.
Almost all women present with uterine fibroids, approximately 76% with dermal manifestations and 10-16% with renal tumors.
The uterine fibroids tend to occur at younger age and larger and more numerous than in general population. They may be distinguishable from sporadic fibroids by special histological features such as prominent nucleoli with perinucleolar halos.
The skin presentation is of asymmetrical, reddish-brown nodules or papules with a firm consistency, predominantly located on the limbs (multiple cutaneous leiomyoma), although they may occur anywhere, including the face. The lesions, which are typically painful and most often present during the third decade of life, are piloleiomyomata—a benign smooth muscle tumour arising from the arrectores pilorum muscles of the skin. These tumours may also arise in the tunica dartos of the scrotum and the mammillary muscle of the nipple (genital leiomyoma), the smooth muscle of blood vessels (angioleiomyoma) and the lung (pulmonary lymphangioleiomyomatosis). A pseudo-Darier sign may be present.
The renal cell carcinoma tends to be of the papillary (type 2) form and tends to occur more commonly in women than men with this syndrome. These cancers present earlier than is usual for renal cell carcinomas (typically in the twenties and thirties) and to be at relatively advanced stages at presentation. Tumours have rarely been reported in children. These tumours occur in ~20% of those with this mutation suggesting that other factors are involved in the pathogenesis.
Smooth muscle cells make up the involuntary muscles, which are found in most parts of the body, including the uterus, stomach and intestines, the walls of all blood vessels, and the skin. It is therefore possible for leiomyosarcomas to appear at any site in the body. They are most commonly found in the uterus, stomach, small intestine and retroperitoneum.
Uterine leiomyosarcomas come from the smooth muscle in the muscle layer of the uterus. Cutaneous leiomyosarcomas derive from the pilo-erector muscles in the skin. Gastrointestinal leiomyosarcomas might come from smooth muscle in the GI tract or, alternatively, also from a blood vessel. At most other primary sites—retroperitoneal extremity (in the abdomen, behind the intestines), truncal, abdominal organs, etc.—leiomyosarcomas appear to grow from the muscle layer of a blood vessel (the tunica media). Thus a leiomyosarcoma can have a primary site of origin anywhere in the body where there is a blood vessel.
The tumors are usually hemorrhagic and soft and microscopically marked by pleomorphism, abundant (15–30 per 10 high power fields) abnormal mitotic figures, and coagulative tumor cell necrosis. There is a wide differential diagnosis, which includes spindle cell carcinoma, spindle cell melanoma, fibrosarcoma, malignant peripheral nerve sheath tumor and even biphenotypic sinonasal sarcoma.
RMS can occur in almost any soft-tissue site in the body; the most common primary sites are genitourinary (24%), parameningeal (16%), extremity (19%), orbit (9%), other head and neck (10%), and miscellaneous other sites (22%). RMS often presents as a mass, but signs and symptoms can vary widely depending on the site of the primary tumor. Genitourinary tumors may present with hematuria, urinary tract obstruction, and/or a scrotal or vaginal mass. Tumors that arise in the retroperitoneum and mediastinum can become quite large before producing signs and symptoms. Parameningeal tumors may present with cranial nerve dysfunction, symptoms of sinusitis, ear discharge, headaches, and facial pain. Orbital tumors often present with orbital swelling and proptosis. Extremity tumors generally present as a rapidly enlarging, firm mass in the relevant tissue. The cancer's prevalence in the head, face, and neck will often allow for earlier signs of the disease simply due to the obvious nature of tumors in these locations. Despite the varying presentation and typically aggressive nature of the disease, RMS has the potential to be diagnosed and treated early. The fourth IRSG study found that 23% of patients were diagnosed in time for a complete resection of their cancer, and 15% had resection with only minimal remnants of the diseased cells.
Leiomyosarcoma, also referred to as LMS, is a malignant (cancerous) smooth muscle tumor. A benign tumor originating from the same tissue is termed leiomyoma. It is also important to note that while it has been believed that leiomyosarcomas do not arise from leiomyomas, there are leiomyoma variants for which classification is evolving.
About 1 person in 100,000 gets diagnosed with LMS each year. Leiomyosarcoma is one of the more common types of soft-tissue sarcoma, representing 10 percent to 20 percent of new cases. (Leiomyosarcoma of the bone is more rare.) Sarcoma is rare, consisting of only 1 percent of cancer cases in adults. Leiomyosarcomas can be very unpredictable. They can remain dormant for long periods of time and recur after years. It is a resistant cancer, meaning generally not very responsive to chemotherapy or radiation. The best outcomes occur when it can be removed surgically with wide margins early, while small and still in situ.
Vaginal intraepithelial neoplasia (VAIN) is a condition that describes premalignant histological findings in the vagina characterized by dysplastic changes.
The disorder is rare and generally has no symptoms. VAIN can be detected by the presence of abnormal cells in a Papanicolaou test (Pap smear).
Like cervical intraepithelial neoplasia, VAIN comes in three stages, VAIN 1, 2, and 3. In VAIN 1, a third of the thickness of the cells in the vaginal skin are abnormal, while in VAIN 3, the full thickness is affected. VAIN 3 is also known as carcinoma in-situ, or stage 0 vaginal cancer.
Infection with certain types of the human papillomavirus ("high-risk types") may be associated with up to 80% of cases of VAIN. Vaccinating girls with HPV vaccine before initial sexual contact has been shown to reduce incidence of VAIN.
One study found that most cases of VAIN were located in the upper third of the vagina, and were multifocal. In the same study, 65 and 10% patients with VAIN also had cervical intraepithelial neoplasia and vulvar intraepithelial neoplasia, respectively.
In another study, most cases of VAIN went into remission after a single treatment, but about 5% of the cases studied progressed into a more serious condition despite treatment.
Reed’s syndrome (or familial leiomyomatosis cutis et uteri) is a rare inherited condition characterised by multiple cutaneous leiomyomas and, in women, uterine leiomyomas. It predisposes for renal cell cancer, an association denominated hereditary leiomyomatosis and renal cell cancer, and it is also associated with increased risk of uterine leiomyosarcoma. The syndrome is caused by a mutation in the fumarate hydratase gene, which leads to an accumulation of fumarate. The inheritance pattern is autosomal dominant.
Myosarcoma is a malignant muscle tumor. People with myosarcoma often wake up with the feeling as if they had a cramp during their sleep.
Leiomyosarcoma is sarcoma of smooth muscle, and rhabdomyosarcoma is sarcoma of striated muscle. However, the term myosarcoma itself still appears in the literature.
Ancillary testing for fibrosarcoma includes IHC, where vimentin is positive, cytokeratin and S100 are negative, and actin is variable.
Fibrosarcoma (fibroblastic sarcoma) is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern. It is usually found in males aged 30 to 40 . It originates in fibrous tissues of the bone and invades long or flat bones such as femur, tibia, and mandible. It also involves periosteum and overlying muscle.
Given the difficulty in diagnosing rhabdomyosarcoma, definitive classification of subsets has proven difficult. As a result, classification systems vary by institute and organization. However, rhabdomyosarcoma can be generally divided into three histological subsets:
- "Embryonal rhabdomyosarcoma" (ERMS) is the most common histological variant, comprising approximately 60–70% of childhood cases. It is most common in children 0–4 years old, with a maximum reported incidence of 4 cases per 1 million children. ERMS is characterized by spindle-shaped cells with a stromal-rich appearance, and the morphology is similar to the developing muscle cells of a 6–8 week old embryo. Tumors often present in the head and neck as well as the genitourinary tract. ERMS also has two defined subtypes, botryoid and spindle cell ERMS, and these subtypes are associated with a favorable prognosis.
- Subtypes of ERMS
- Botryoid ERMS is almost always found in mucosal lined organs including the vagina, bladder, and nasopharynx (although presentation in the nasopharynx typically affects older children). It often presents in patients <1 year old as a round, grape-like mass on the affected organ. Histologically, cells of the botryoid variant are defined by a dense tumor layer under an epithelium (cambium layer).
- Spindle cell rhabdomyosarcoma comprises about 3% of all RMS cases. This subtype is very similar to that of leiomyosarcoma (cancer of the smooth muscle tissue), and it has a fascicular, spindled, and leiomyomatous growth pattern with notable rhabdomyoblastic differentiation . It occurs most commonly in the paratesticular region, and the prognosis for this particular form of RMS is excellent with a reported 5 year survival rate of 95%.
- "Alveolar rhabdomyosarcoma" (ARMS) is the second most common type. ARMS comprises approximately 20–25% of RMS-related tumors, and it is equally distributed among all age groups with an incidence of about 1 case per 1 million people ages 0 to 19. For this reason, it is the most common form of RMS observed in young adults and teenagers, who are less prone to the embryonal variant. This type of RMS is characterized by densely-packed, round cells that arrange around spaces similar in shape to pulmonary alveoli, although variants have been discovered without these characteristic alveolar spacings. ARMS tends to form more often in the extremities, trunk, and peritoneum. It is also typically more aggressive than ERMS.
- "Anaplastic (undifferentiated) rhabdomyosarcoma", also known as "pleomorphic rhabdomyosarcoma", is the final variant of RMS recognized in most classification systems. Anaplastic rhabdomyosarcoma is defined by the presence of anaplastic cells with large, lobate hyperchromatic nuclei and multipolar mitotic figures. These tumors display high heterogeneity and extremely poor differentiation. The anaplastic cells may be diffuse or localized, with the diffuse variation correlating to a worse prognosis. It occurs most often in adults, rarely in children, and is often discovered in the extremities. Due to the lack of discernible separation among cancers of this type, clinicians will often label undiagnosed sarcomas with little to no discernible features as anaplastic RMS. It is the most aggressive type of RMS, and will often require intensive treatment.
There is also an extremely rare subtype of RMS that has been described as "sclerosing rhabdomyosarcoma" by "Folpe, et al", but it is not a currently recognized subtype by the NCI or WHO. This subtype has characteristic histology involving hyaline sclerosis and pseudovascular development. Its origins are unclear, but some studies have pointed to an association with embryonal RMS.
Multiple classification systems have been proposed for guiding management and treatment, and the most recent and widely used classification system is the "International Classification of Rhabdomyosarcoma" or ICR. It was created by the IRSG in 1995 after their series of four multi-institutional trials aimed at studying the presentation, histology, epidemiology, and treatment of RMS (IRSG I–IV). The ICR system is based on prognostic indicators identified in IRSG I–IV. Pleomorphic rhabdomyosarcoma usually occurs in adults rather than children, and is therefor not included in this system. Sclerosing rhabdomyosarcoma is also not included in this system due to its rare presentation and weak classification schema.
Clear-cell adenocarcinoma (CCA) of the vagina (or cervix) is a rare Adenocarcinoma (cancer) often linked to diethylstilbestrol (DES), a drug which was prescribed in the mistaken belief that it prevented miscarriage and ensured a healthy pregnancy.
Invasive hydatidiform mole, also known as invasive mole and chorioadenoma destruens is a type of neoplasia that grows into the muscular wall of the uterus. It is formed after conception (fertilization of an egg by a sperm). It may spread to other parts of the body, such as the vagina, vulva, and lung.
Smooth muscle tumor of uncertain malignant potential, abbreviated STUMP, is an uncommon tumor of the uterine smooth muscle that may behave like a benign tumor or a cancerous tumor.
This tumor should not be confused with the prostatic stromal tumor of uncertain malignant potential which may be abbreviated the same way ("STUMP").
The Bell criteria were developed to help categorize them and differentiate them from their main differential diagnoses, leiomyosarcoma and uterine leiomyoma.
Clear cells are rich in glycogen, which accounts for their histology.
Smooth muscle tumours show a smooth muscle differentiation. There are two main types of smooth muscle tumour: the benign leiomyoma and the malignant leiomyosarcoma.
Some women with uterine fibroids do not have symptoms. Abdominal pain, anemia and increased bleeding can indicate the presence of fibroids. There may also be pain during intercourse, depending on the location of the fibroid. During pregnancy, they may also be the cause of miscarriage, bleeding, premature labor, or interference with the position of the fetus. A uterine fibroid can cause rectal pressure. The abdomen can grow larger mimicking the appearance of pregnancy. Some large fibroids can extend out through the cervix and vagina.
While fibroids are common, they are not a typical cause for infertility, accounting for about 3% of reasons why a woman may not be able to have a child. The majority of women with uterine fibroids will have normal pregnancy outcomes. In cases of intercurrent uterine fibroids in infertility, a fibroid is typically located in a submucosal position and it is thought that this location may interfere with the function of the lining and the ability of the embryo to implant.