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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
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Most soft-tissue sarcomas are not associated with any known risk factors or identifiable cause. There are some exceptions:
- Studies suggest that workers who are exposed to chlorophenols in wood preservatives and phenoxy herbicides may have an increased risk of developing soft-tissue sarcomas. An unusual percentage of patients with a rare blood vessel tumor, angiosarcoma of the liver, have been exposed to vinyl chloride in their work. This substance is used in the manufacture of certain plastics, notably PVC.
- In the early 1900s, when scientists were just discovering the potential uses of radiation to treat disease, little was known about safe dosage levels and precise methods of delivery. At that time, radiation was used to treat a variety of noncancerous medical problems, including enlargement of the tonsils, adenoids, and thymus gland. Later, researchers found that high doses of radiation caused soft-tissue sarcomas in some patients. Because of this risk, radiation treatment for cancer is now planned to ensure that the maximum dosage of radiation is delivered to diseased tissue while surrounding healthy tissue is protected as much as possible.
- Kaposi's sarcoma, a rare cancer of the cells that line blood vessels in the skin and mucus membranes, is caused by Human herpesvirus 8. Kaposi's sarcoma often occurs in patients with AIDS (acquired immune deficiency syndrome). Kaposi's sarcoma, however, has different characteristics than typical soft-tissue sarcomas and is treated differently.
- In a very small fraction of cases, sarcoma may be related to a rare inherited genetic alteration of the p53 gene and is known as Li-Fraumeni syndrome. Certain other inherited diseases are associated with an increased risk of developing soft-tissue sarcomas. For example, people with neurofibromatosis type I (also called von Recklinghausen's disease, associated with alterations in the NF1 gene) are at an increased risk of developing soft-tissue sarcomas known as malignant peripheral nerve sheath tumors. Patients with inherited retinoblastoma have alterations in the RB1 gene, a tumor suppressor gene, and are likely to develop soft-tissue sarcomas as they mature into adulthood.
Soft-tissue sarcomas are relatively uncommon cancers. They account for less than 1% of all new cancer cases each year. This may be because cells in soft tissue, in contrast to tissues that more commonly give rise to malignancies, are not continuously dividing cells.
In 2006, about 9,500 new cases were diagnosed in the United States. Soft-tissue sarcomas are more commonly found in older patients (>50 years old) although in children and adolescents under age 20, certain histologies are common (rhabdomyosarcoma, synovial sarcoma).
Around 3,300 people were diagnosed with soft tissue sarcoma in the UK 2011.
Hemangiosarcoma is a rapidly growing, highly invasive variety of cancer that occurs almost exclusively in dogs, and only rarely in cats, horses, mice, or humans. It is a sarcoma arising from the lining of blood vessels; that is, blood-filled channels and spaces are commonly observed microscopically. A frequent cause of death is the rupturing of this tumor, causing the patient to rapidly bleed to death.
The term "angiosarcoma", when used without a modifier, usually refers to hemangiosarcoma. However, glomangiosarcoma (8710/3) and lymphangiosarcoma (9170/3) are distinct conditions [in humans]. Hemangiosarcomas are commonly associated with toxic exposure to thorium dioxide (Thorotrast), vinyl chloride, and arsenic.
Most heart tumors begin with myxomas, fibromas, rhabdomyomas, and hamartomas, although malignant sarcomas (such as angiosarcoma or cardiac sarcoma) have been known to occur. In a study of 12,487 autopsies performed in Hong Kong seven cardiac tumors were found, most of which were benign. According to Mayo Clinic: "At Mayo Clinic, on average only one case of heart cancer is seen each year." In a study conducted in the Hospital of the Medical University of Vienna 113 primary cardiac tumour cases were identified in a time period of 15 years with 11 being malignant. The mean survival in the latter group of patients was found to be .
Primary malignant cardiac tumors (PMCTs) are even more rare. A study using the Surveillance, Epidemiology and End-Results (SEER) Cancer Registry from 1973–2011 found 551 cases of PMCTs, with an incidence of 34 cases per million persons. The study also found that the incidence has doubled over the past four decades. The associated mortality was very high, with only 46% of patients alive after one year. Sarcomas and mesotheliomas had the worst survival, while lymphomas had better survival. When compared with extracardiac tumors, PMCTs had worse survival.
Although reliable and comprehensive incidence statistics are nonexistent, LCLC-RP is a rare tumor, with only a few hundred cases described in the scientific literature to date. LCLC's made up about 10% of lung cancers in most historical series, equating to approximately 22,000 cases per year in the U.S. Of these LCLC cases, it is estimated that about 1% will eventually develop the rhabdoid phenotype during tumor evolution and progression. In one large series of 902 surgically resected lung cancers, only 3 cases (0.3%) were diagnosed as LCLC-RP. In another highly selected series of large-cell lung carcinoma cases, only 4 of 45 tumors (9%) were diagnosed as the rhabdoid phenotype using the 10% criterion, but another 10 (22%) had at least some rhabdoid cell formation. It appears likely, therefore, that LCLC-RP probably comprises between 0.1% and 1.0% of all lung malignancies.
Similar to nearly all variants of lung carcinoma, large cell lung carcinoma with rhabdoid phenotype appears to be highly related to tobacco smoking. It also appears to be significantly more common in males than in females.
Hemangioendothelioma is used to describe a group of vascular neoplasms that may be considered benign as well as malignant, depending on the specific group member's activity.
Hemangiosarcoma is quite common in dogs, and more so in certain breeds including German Shepherd Dogs and Golden Retrievers. It also occurs in cats, but much more rarely. Dogs with hemangiosarcoma rarely show clinical signs until the tumor has become very large and has metastasized. Typically, clinical signs are due to hypovolemia after the tumor ruptures, causing extensive bleeding. Owners of the affected dogs often discover that the dog has hemangiosarcoma only after the dog collapses.
The tumor most often appears on the spleen, right heart base, or liver, although varieties also appear on or under the skin or in other locations. It is the most common tumor of the heart, and occurs in the right atrium or right auricular appendage. Here it can cause right-sided heart failure, arrhythmias, pericardial effusion, and cardiac tamponade. Hemangiosarcoma of the spleen or liver is the most common tumor to cause hemorrhage in the abdomen. Hemorrhage secondary to splenic and hepatic tumors can also cause ventricular arrythmias. Hemangiosarcoma of the skin usually appears as a small red or bluish-black lump. It can also occur under the skin. It is suspected that in the skin, hemangiosarcoma is caused by sun exposure. Occasionally, hemangiosarcoma of the skin can be a metastasis from visceral hemangiosarcoma. Other sites the tumor may occur include bone, kidneys, the bladder, muscle, the mouth, and the central nervous system.
Giant-cell lung cancers have long been considered to be exceptionally aggressive malignancies that grow very rapidly and have a very poor prognosis.
Many small series have suggested that the prognosis of lung tumors with giant cells is worse than that of most other forms of non-small-cell lung cancer (NSCLC), including squamous cell carcinoma, and spindle cell carcinoma.
The overall five-year survival rate in GCCL varies between studies but is generally considered to be very low. The (US) Armed Forces Institute of Pathology has reported a figure of 10%, and in a study examining over 150,000 lung cancer cases, a figure of 11.8% was given. However, in the latter report the 11.8% figure was based on data that included spindle cell carcinoma, a variant which is generally considered to have a less dismal prognosis than GCCL. Therefore, the likely survival of "pure" GCCL is probably lower than the stated figure.
In the large 1995 database review by Travis and colleagues, giant-cell carcinoma has the third-worst prognosis among 18 histological forms of lung cancer. (Only small-cell carcinoma and large-cell carcinoma had shorter average survival.)
Most GCCL have already grown and invaded locally and/or regionally, and/or have already metastasized distantly, and are inoperable, at the time of diagnosis.
Angiosarcoma is a cancer of the cells that line the walls of blood vessels or lymphatic vessels. The lining of the vessel walls is called the endothelium. Cancers from the walls of blood vessels are called hemangiosarcomas, and cancers from the walls of lymphatic vessels are called lymphangiosarcomas. However, they should not be confused with cherry hemangiomas.
Most tumors of visceral blood and lymphatic vessel walls are cancerous (malignant). Because these cancers are carried by the blood flow or lymphatic flow, they can more easily metastasize to distant sites, particularly the liver and lungs.
Angiosarcomas will show signs of hemorrhage and necrosis. Pathologically, tumor cells will show increased nuclear to cytoplasm ratio, nuclear hyperchromasia, nuclear pleomorphism and high mitotic activity.
In dogs, hemangiosarcoma is relatively common, especially in larger breeds such as golden retrievers and Labrador retrievers. In humans, hemangiosarcomas and lymphangiosarcomas of the skin are uncommon. Angiosarcoma of the liver, a rare fatal tumor, has been seen in workers intensively exposed to the gas vinyl chloride monomer (VCM) for prolonged periods while working in polyvinyl chloride (PVC) polymerization plants. It has also been associated with individuals exposed to arsenic-containing insecticides and Thorotrast.
Treatment is varied and depends on the site and extent of tumor involvement, site(s) of metastasis, and specific individual factors. Surgical resection, radiotherapy, and chemotherapy have all been used to treat these masses, although studies on survival have yet to be conducted to delineate various treatment regimens.
LCLC-RP are considered to be especially aggressive tumors with a dismal prognosis. Many published cases have shown short survival times after diagnosis. Some studies suggest that, as the proportion of rhabdoid cells in the tumor increases, the prognosis tends to worsen, although this is most pronounced when the proportion of rhabdoid cells exceeds 5%. With regard to "parent" neoplasms other than LCLC, adenocarcinomas with rhabdoid features have been reported to have worse prognoses than adenocarcinomas without rhabdoid features, although an "adenocarcinoma with rhabdoid phenotype" tumor variant has not been specifically recognized as a distinct entity under the WHO-2004 classification system.
Interestingly, there are case reports of rhabdoid carcinomas recurring after unusually long periods, which is unusual for a fast-growing, aggressive tumor type. One report described a very early stage patient whose tumor recurred 6 years after initial treatment. Although rapidly progressive, fulminant courses seem to be the rule in this entity, long-term survival has also been noted, even post-metastectomy in late stage, distant metastatic disease.
It was previously a relatively common complication of the massive lymphedema of the arm which followed removal of axillary (arm pit) lymph nodes and lymphatic channels as part of the classical Halstedian radical mastectomy, as a treatment for breast cancer. The classical radical mastectomy was abandoned in most areas of the world in the late 1960s to early 1970s, being replaced by the much more conservative modified radical mastectomy and, more recently, by segmental breast tissue excision and radiation therapy. Because of this change in clinical practice lymphedema is now a rarity following breast cancer treatment—and post-mastectomy lymphangiosarcoma is now vanishingly rare. When it occurs following mastectomy it is known as Stewart-Treves syndrome (which can be both lymphangiosarcoma and hemangiosarcoma following mastectomy). The pathogenesis of lymphangiosarcoma has not been resolved, however several vague mechanisms have been proposed. Stewart and Treves, proposed that a cancer causing agent is present in lymphedematous limbs. Schreiber "et al." proposed that local immunodeficiency as a result of lymphedema results in a "immunologically privileged site" in which the sarcoma is able to develop.
The most successful treatment for angiosarcoma is amputation of the affected limb if possible. Chemotherapy may be administered if there is metastatic disease. If there is no evidence of metastasis beyond the lymphedematous limb, adjuvant chemotherapy may be given anyway due to the possibility of micrometastatic disease. Evidence supporting the effectiveness of chemotherapy is, in many cases, unclear due to a wide variety of prognostic factors and small sample size. However, there is some evidence to suggest that drugs such as paclitaxel, doxorubicin, ifosfamide, and gemcitabine exhibit antitumor activity.
Because of its rarity, there have been no randomized clinical trials of treatment of GCCL, and all information available derives from small retrospective institutional series or multicenter metadata.
Heart cancer is an extremely rare form of cancer that is divided into primary tumors of the heart and secondary tumors of the heart.
In breast pathology, pseudoangiomatous stromal hyperplasia, commonly abbreviated PASH, is an overgrowth of myofibroblastic cells and has an appearance similar to fibroadenomatoid changes.
The diagnostic significance is currently uncertain, but it appears to be benign. There have been cases of PASH diagnosed where the tumors co-exist with breast cancer. Other cases have made screening for breast cancer difficult and in some cases impossible due to the number and density of the existing PASH tumors. These cases have resulted in the necessity of a mastectomy and double mastectomy.
In the 1960s, the incidence 5 years after a radical mastectomy varied from 0.07% to 0.45%.
Today, it occurs in 0.03% of patients surviving 10 or more years after radical mastectomy.
The management of PASH is controversial. Excision may be indicated in enlarging masses or lesions with atypical features.
Intravascular papillary endothelial hyperplasia (also known as "Masson's hemangio-endotheliome vegetant intravasculaire," "Masson's lesion," "Masson's pseudoangiosarcoma," "Masson's tumor," and "Papillary endothelial hyperplasia") is a rare, benign tumor. It may mimic an angiosarcoma, with lesions that are red or purplish 5-mm to 5-cm papules and deep nodules on the head, neck, or upper extremities.
Early detection is key. Untreated patients usually live 5 to 8 months after diagnosis.
Ultraviolet radiation from sun exposure is the primary environmental cause of skin cancer. Other risk factors that play a role include:
- Smoking tobacco
- HPV infections increase the risk of squamous-cell skin cancer.
- Some genetic syndromes including congenital melanocytic nevi syndrome which is characterized by the presence of nevi (birthmarks or moles) of varying size which are either present at birth, or appear within 6 months of birth. Nevi larger than 20 mm (3/4") in size are at higher risk for becoming cancerous.
- Chronic non-healing wounds. These are called Marjolin's ulcers based on their appearance, and can develop into squamous-cell skin cancer.
- Ionizing radiation such as X-rays, environmental carcinogens, artificial UV radiation (e.g. tanning beds), aging, and light skin color. It is believed that tanning beds are the cause of hundreds of thousands of basal and squamous-cell skin cancer. The World Health Organization now places people who use artificial tanning beds in its highest risk category for skin cancer. Alcohol consumption, specifically excessive drinking increase the risk of sunburns.
- The use of many immunosuppressive medications increases the risk of skin cancer. Cyclosporin A, a calcineurin inhibitor for example increases the risk approximately 200 times, and azathioprine about 60 times.
Sunscreen is effective and thus recommended to prevent melanoma and squamous-cell carcinoma. There is little evidence that it is effective in preventing basal-cell carcinoma. Other advice to reduce rates of skin cancer includes avoiding sunburning, wearing protective clothing, sunglasses and hats, and attempting to avoid sun exposure or periods of peak exposure. The U.S. Preventive Services Task Force recommends that people between 9 and 25 years of age be advised to avoid ultraviolet light.
The risk of developing skin cancer can be reduced through a number of measures including decreasing indoor tanning and mid day sun exposure, increasing the use of sunscreen, and avoiding the use of tobacco products.
There is insufficient evidence either for or against screening for skin cancers. Vitamin supplements and antioxidant supplements have not been found to have an effect in prevention. Evidence for a benefit from dietary measures is tentative.
Zinc oxide and titanium oxide are often used in sun screen to provide broad protection from UVA and UVB ranges.
Eating certain foods may decrease the risk of sunburns but this is much less than the protection provided by sunscreen.
The true incidence of cavernous hemangiomas is difficult to estimate because they are frequently misdiagnosed as other venous malformations. Cavernous hemangiomas of the brain and spinal cord (cerebral cavernous hemangiomas (malformations) (CCM)), can appear at all ages but usually occur in the third to fourth decade of a person's life with no sexual preference. In fact, CCM is present in 0.5% of the population. However, approximately 40% of those with malformations have symptoms. Asymptomatic individuals are usually individuals that developed the malformation sporadically, while symptomatic individuals usually have inherited the genetic mutation. The majority of diagnoses of CCM are in adults; however, 25% of cases of CCM are children. Approximately 5% of adults have liver hemangiomas in the United States, but most are asymptomatic. Liver hemangiomas usually occur between the ages of 30-50 and more commonly in women. Cases of infantile liver cavernomas are extremely rare. Cavernous hemangioma of the eye is more prevalent in women than men and between the ages of 20-40.
The number of new cases of hepatocellular carcinoma per year in India in males is about 4.1 and for females 1.2 per 100,000. It typically occurs between 40 and 70 years of age.
Liver cancer is the eighteenth most common cancer in the UK (around 4,300 people were diagnosed with liver cancer in the UK in 2011), and it is the twelfth most common cause of cancer death (around 4,500 people died of the disease in 2012).