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Uterine sarcoma are rare, out of all malignancies of the uterine body only about 4% will be uterine sarcomas. Generally, the cause of the lesion is not known, however patients with a history of pelvic radiation are at higher risk. Most tumors occur after menopause.
Women who take long-term tamoxifen are at higher risk.
Leiomyoma is the most common benign mesenchymal tumor of esophagus and second most common benign tumor of the small bowel (with gastrointestinal stromal tumor as most common). Although leiomyoma is the most common benign esophageal tumor, malignant carcinoma is still 50 times more likely. Approximately 50% of cases are found in the jejunum, followed by the ileum in 31% of cases. Almost one half of all lesions are less than 5 centimeters.
Mesenchymal neoplasms of the gallbladder are rare and in particular leiomyomas of the gallbladder have been rarely reported, all of them in patients with immune system disorders. Although, recently, a case was reported in absence of associated immunodeficiency at Monash Hospital in Melbourne Australia in a healthy 39-year-old woman with no symptoms.
Diets high in fruits and vegetables tend to lower the risk of developing fibroids. Fruits, especially citrus, have a greater protective benefit than vegetables. Normal dietary levels of vitamin D is shown to reduce the risk of developing fibroids. No protective benefit has been found with the consumption of folate, whole grains, soy products, or fiber. No association between the consumption of fat, eggs, dairy products has been shown to increase the risk of fibroids.
A 2006 review stated that RS often leads renal cancer between ages 30-50. Renal cancer kills about 1 in 3 people, but 5-year survival rates improved between 1974-1976 and 1995-2000, from 52% to 64%.
Benign metastasizing leiomyoma is a rare condition characterized by the growth of uterine leiomyoma in the other regions especially the lungs.
Other relatively rare conditions have been reported in association with this disease. It is not yet known if these associations are fortuitous or manifestations of the condition itself.
Cerebral cavernomas and massive, macronodular adrenocortical disease have also been reported in association with this syndrome. A case of cutis verticis gyrata, disseminated collagenoma and Charcot-Marie-Tooth disease in association with a mutation in the fumarate hydratase gene has also been reported. Two cases of ovarian mucinous cystadenoma have also been reported with this mutation.
Intravenous leiomyomatosis is a rare condition seen exclusively in women in which leiomyomata, benign smooth muscle tumors, are found in veins. The masses are benign-appearing but can spread throughout the venous system leaving the uterus and even cause death when growing into the heart from the IVC. While the possibility that these arose de novo from the smooth muscle in the blood vessel wall was considered, chromosomal analysis suggests a uterine origin. Intravenous leiomyomata are usually but not always associated with uterine fibroids, and tend to recur.
This condition is related to benign metastasizing leiomyoma, in which the masses appear in more distant locations such as the lung and lymph nodes.
Some risk factors associated with the development of uterine fibroids are modifiable.
Fibroids are more common in obese women. Fibroids are dependent on estrogen and progesterone to grow and therefore relevant only during the reproductive years.
Inflammatory fibroid polyp, abbreviated IFP, is a benign abnormal growth of tissue projecting into the lumen of the gastrointestinal tract.
Industrialized nations, with the exception of Japan, have high rates of epithelial ovarian cancer, which may be due to diet in those countries. Caucasian are at a 30–40% higher risk for ovarian cancer when compared to Black and Hispanic people, likely due to socioeconomic factors; white women tend to have fewer children and different rates of gynecologic surgeries that affect risk for ovarian cancer.
Cohort studies have found a correlation between dairy consumption and ovarian cancer, but case-control studies do not show this correlation. There is mixed evidence regarding the effect of red meat and processed meat in ovarian cancer.
Tentative evidence suggests that talc, pesticides, and herbicides increase the risk of ovarian cancer. The American Cancer Society notes that as of now, no study has been able to accurately link any single chemical in the environment, or in the human diet, directly to mutations that cause ovarian cancer.
Alcohol consumption does not appear to be related to ovarian cancer. Other factors that have been investigated, such as smoking, low levels of vitamin D in the blood, presence of inclusion ovarian cysts, and infection with human papilloma virus (the cause of some cases of cervical cancer), have been disproven as risk factors for ovarian cancer. The carcinogenicity of perineal talc is controversial, because it can act as an irritant if it travels through the reproductive tract to the ovaries. Case-control studies have shown that use of perineal talc does increase the risk of ovarian cancer, but using talc more often does not create a greater risk. Use of talc elsewhere on the body is unrelated to ovarian cancer. Sitting regularly for prolonged periods is associated with higher mortality from epithelial ovarian cancer. The risk is not negated by regular exercise, though it is lowered.
Increased age (up to the 70s) is a risk factor for epithelial ovarian cancer because more mutations in cells can accumulate and eventually cause cancer. Those over 80 are at slightly lower risk.
Smoking tobacco is associated with a higher risk of mucinous ovarian cancer; after smoking cessation, the risk eventually returns to normal.A diet high in animal fats may be associated with ovarian cancer, but the connection is unclear. Diet seems to play a very small role, if any, in ovarian cancer risk.
Higher levels of C-reactive protein are associated with a higher risk of developing ovarian cancer.
To remove the tumor from the body, a myomectomy or hysterectomy is often required.
Surgery, with as wide a margin of removal as possible, has generally been the most effective and preferred way to attack LMS. If surgical margins are narrow or not clear of tumor, however, or in some situations where tumor cells were left behind, chemotherapy or radiation has been shown to give a clear survival benefit. While LMS tends to be resistant to radiation and chemotherapy, each case is different and results can vary widely.
LMS of uterine origin do frequently, but not always respond to hormonal treatments.
IFPs consist of spindle cells that are concentrically arranged around blood vessels and have inflammation, especially eosinophils.
They may have leiomyoma/schwannoma-like areas with nuclear palisading.
They typically stain with CD34 and vimentin, and, generally, do not stain with CD117 and S100.
The endoscopic differential diagnosis includes other benign, pre-malignant and malignant gastrointestinal polyps.
Therapy is based on staging and patient condition and utilizes one or more of the following approaches.
Surgery is the mainstay of therapy if feasible involving total abdominal hysterectomy with bilateral salpingo-oophorectomy. Other approaches include radiation therapy, chemotherapy, and hormonal therapy.
Prognosis is relatively poor.
Mucinous cystadenoma is a benign cystic tumor lined by a mucinous epithelium. It is a type of cystic adenoma (cystadenoma).
Mucinous cystadenoma may arise in a number of locations; however, mucinous cystadenoma at different locations are not generally considered to be related to one another.
The overall incidence is 0.5 to 1 cases per 100,000 people per year. It is slightly more common in women than men (male:female ratio = 9:11). The median age at presentation is typically about 50 years with a range of 20–25 years.
They are of two types.
- The leiomyoma occurs in the skin or gut but the common form is the uterine fibroid.
- Rhabdomyomas are rare tumors of muscles, they occur in childhood and often become malignant.
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.
Mucinous cystadenomas make up 15-20% of all ovarian tumors. They often become very large and can extend up into the abdomen.
These tumors are usually evaluated using ultrasound, CT scan, or MRI. Findings on imaging studies are nonspecific. These ovarian tumors are usually multi-septated, cystic masses with thin walls. They also contain varying amounts of solid tissue which consists of proliferating stromal tissue, papillae, or malignant tumor cells.
Benign mucinous cystadenomas compose 80% of mucinous ovarian tumors and 20-25% of benign ovarian tumors overall. The peak incidence occurs between 30-50 years of age. Benign tumors are bilateral in 5-10% of cases.
Pseudomyxoma peritonei (PMP) is a clinical condition caused by cancerous cells (mucinous adenocarcinoma) that produce abundant mucin or gelatinous ascites. The tumors cause fibrosis of tissues and impede digestion or organ function, and if left untreated, the tumors and mucin they produce will fill the abdominal cavity. This will result in compression of organs and will destroy the function of colon, small intestine, stomach, or other organs. Prognosis with treatment in many cases is optimistic, but the disease is lethal if untreated, with death by cachexia, bowel obstruction, or other types of complications.
This disease is most commonly caused by an appendiceal primary cancer (cancer of the appendix); mucinous tumors of the ovary have also been implicated, although in most cases ovarian involvement is favored to be a metastasis from an appendiceal or other gastrointestinal source. Disease is typically classified as low- or high-grade (with signet ring cells). When disease presents with low-grade histologic features the cancer rarely spreads through the lymphatic system or through the bloodstream.
Angioleiomyoma (vascular leiomyoma, angiomyoma) of the skin is thought to arise from vascular smooth muscle, and is generally acquired.
Birt-Hogg-Dubé Syndrome patients, families, and caregivers are encouraged to join the NIH Rare Lung Diseases Consortium Contact Registry. This is a privacy protected site that provides up-to-date information for individuals interested in the latest scientific news, trials, and treatments related to rare lung diseases.
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.