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The uterine sarcomas form a group of malignant tumors that arises from the smooth muscle or connective tissue of the uterus.
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.
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.
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.
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".
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.
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.
Primary fallopian tube cancer (PFTC), often just tubal cancer, is a malignant neoplasm that originates from the fallopian tube.
Patients usually note a deep seated mass in their soft tissue. Only when the tumor is very large do symptoms of pain or functional disturbances occur.
Retroperitoneal tumors may present themselves with signs of weight loss and emaciation and abdominal pain. These tumors may also compress the kidney or ureter leading to kidney failure.
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.
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.
The terms uterine cancer and womb cancer may refer to any of several different types of cancer which occur in the uterus, namely:
- Endometrial cancer:
- Cervical cancer arises from the transformation zone of the cervix, the lower portion of the uterus and connects to the upper aspect of the vagina.
- Uterine sarcomas: sarcomas of the myometrium, or muscular layer of the uterus, are most commonly leiomyosarcomas.
- Gestational trophoblastic disease relates to neoplastic processes originating from tissue of a pregnancy that often is located in the uterus.
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.
Liposarcoma is a cancer that arises in fat cells in deep soft tissue, such as that inside the thigh or in the retroperitoneum. Liposarcoma is a rare type of cancer that bears a resemblance to fat cells when examined under a microscope.
They are typically large bulky tumors, and tend to have multiple smaller satellites that extend beyond the main confines of the tumor.
Liposarcomas, like all sarcomas, are rare.
A myxoid liposarcoma is a malignant adipose tissue neoplasm of myxoid appearance histologically.
Myxoid liposarcomas are the second most common type of liposarcoma, representing 30–40% of all liposarcomas in the limbs; occurring most commonly in the legs, particularly the thigh, followed by the buttocks, retroperitoneum, trunk, ankle, proximal limb girdle, head and neck, and wrist. They occur in the intermuscular fascial planes or deep-seated areas. They present as a large, slow-growing, painless mass.
They are associated with a fusion between DDIT3 or "CHOP" (at 12q13.1-q13.2) and FUS or "TLS" (at 16p11.2) or EWS (at 22q12.2).
The specific translocation of FUS-DDIT3 is t(12;16)(q13;p11).
Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare subtype of renal cell carcinoma (RCC), that is included in the 2004 WHO classification of RCC. MTSCC is a rare neoplasm and is considered as a low-grade entity. It may be a variant of papillary RCC. This tumor occurs throughout life (age range 17–82 years) and is more frequent in females.
A hemangiopericytoma (HPC) is a type of soft tissue sarcoma that originates in the pericytes in the walls of capillaries. When inside the nervous system, although not strictly a meningioma tumor, it is a meningeal tumor with a special aggressive behavior. It was first characterized in 1942.
MTSCC has a gross appearance close to papillary RCC. Microscopically, it has three histologic components: mucin, tumor cells forming tubules, and spindle cells. It is characterized by the proliferation of cuboidal and spindle cells arranged in tubular or sheet-like arrays, typically with a mucinous or myxoid background.
Uterine fibroids, also known as uterine leiomyomas or fibroids, are benign smooth muscle tumors of the uterus. Most women have no symptoms while others may have painful or heavy periods. If large enough, they may push on the bladder causing a frequent need to urinate. They may also cause pain during sex or lower back pain. A woman can have one uterine fibroid or many. Occasionally, fibroids may make it difficult to become pregnant, although this is uncommon.
The exact cause of uterine fibroids is unclear. However, fibroids run in families and appear to be partly determined by hormone levels. Risk factors include obesity and eating red meat. Diagnosis can be performed by pelvic examination or medical imaging.
Treatment is typically not needed if there are no symptoms. NSAIDs, such as ibuprofen, may help with pain and bleeding while paracetamol (acetaminophen) may help with pain. Iron supplements may be needed in those with heavy periods. Medications of the gonadotropin releasing hormone agonist class may decrease the size of the fibroids but are expensive and associated with side effects. If greater symptoms are present, surgery to remove the fibroid or uterus may help. Uterine artery embolization may also help. Cancerous versions of fibroids are very rare and are known as leiomyosarcomas. They do not appear to develop from benign fibroids.
About 20% to 80% of women develop fibroids by the age of 50. In 2013, it was estimated that 171 million women were affected. They are typically found during the middle and later reproductive years. After menopause, they usually decrease in size. In the United States, uterine fibroids are a common reason for surgical removal of the uterus.
Uterine cancer, also known as womb cancer, is any type of cancer that emerges from the tissue of the uterus. It can refer to several types of cancer, with cervical cancer (arising from the lower portion of the uterus) being the most common type worldwide and the second most common cancer in women in developing countries. Endometrial cancer (or cancer of the inner lining of the uterus) is the second most common type, and fourth most common cancer in women from developed countries.
Risk factors depend on specific type, but obesity, older age, and human papillomavirus infection add the greatest risk of developing uterine cancer. Early on, there may be no symptoms, but irregular vaginal bleeding, pelvic pain or fullness may develop. If caught early, most types of uterine cancer can be cured using surgical or medical methods. When the cancer has extended beyond the uterine tissue, more advanced treatments including combinations of chemotherapy, radiation therapy, or surgery may be required.
Hemangiopericytoma located in the cerebral cavity is an aggressive tumor of the Mesenchyme with oval nuclei with scant cytoplasm. "There is dense intercellular reticulin staining. Tumor cells can be fibroblastic, myxoid, or pericytic. These tumors, in contrast to meningiomas, do not stain with epithelial membrane antigen. They have a grade 2 or 3 biological behavior, and need to be distinguished from benign meningiomas because of their high rate of recurrence (68.2%) and metastases (Maier et al. 1992; Kleihues et al. 1993 )."
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.
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.
Patients present with a slow-growing, painless, solitary mass, usually of the subcutaneous tissues. It is much less frequently noted in the intramuscular tissue. It is not uncommon for symptoms to be present for years.
Benign neoplasm with "BROWN FAT" is noted.