Rhabdomyosarcoma
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Rhabdomyosarcoma
Rhabdomyosarcoma (RMS) is a highly aggressive form of cancer that develops from mesenchymal cells that have failed to fully differentiate into myocytes of skeletal muscle. Cells of the tumor are identified as rhabdomyoblasts. There are four subtypes – embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma, pleomorphic rhabdomyosarcoma, and spindle cell/sclerosing rhabdomyosarcoma. Embryonal, and alveolar are the main groups, and these types are the most common soft tissue sarcomas of childhood and adolescence. The pleomorphic type is usually found in adults. It is generally considered to be a disease of childhood, as the vast majority of cases occur in those below the age of 18. It is commonly described as one of the small-blue-round-cell tumors of childhood due to its appearance on an H&E stain. Despite being relatively rare, it accounts for approximately 40% of all recorded soft tissue sarcomas. RMS can occur in any soft tissue site in the body, but is primarily found in t ...
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Embryonal Rhabdomyosarcoma
Embryonal rhabdomyosarcoma (EMRS) is a rare histological form of cancer in the connective tissue wherein the mesenchymally-derived malignant cells resemble the primitive developing skeletal muscle of the embryo. It is the most common soft tissue sarcoma occurring in children. Embryonal rhabdomyosarcoma is also known as PAX''-''fusion negative or Fusion-Negative rhabdomyosarcoma, as tumors of this subtype are unified by their lack of a PAX3-FOXO1 fusion oncogene (or other PAX fusions seen in alveolar rhabdomyosarcoma). Fusion status refers to the presence or absence of a fusion gene, which is a gene formed from joining two different genes together through DNA rearrangements. These types of tumors are classified as embryonal rhabdomyosarcoma "because of their remarkable resemblance to developing embryonic and fetal skeletal muscle." Classification Embryonal rhabdomyosarcoma is the more common of the two major sub-types of rhabdomyosarcoma. ERMS accounts for 60% to 70% of rhabdomyosar ...
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Embryonal Rhabdomyosarcoma
Embryonal rhabdomyosarcoma (EMRS) is a rare histological form of cancer in the connective tissue wherein the mesenchymally-derived malignant cells resemble the primitive developing skeletal muscle of the embryo. It is the most common soft tissue sarcoma occurring in children. Embryonal rhabdomyosarcoma is also known as PAX''-''fusion negative or Fusion-Negative rhabdomyosarcoma, as tumors of this subtype are unified by their lack of a PAX3-FOXO1 fusion oncogene (or other PAX fusions seen in alveolar rhabdomyosarcoma). Fusion status refers to the presence or absence of a fusion gene, which is a gene formed from joining two different genes together through DNA rearrangements. These types of tumors are classified as embryonal rhabdomyosarcoma "because of their remarkable resemblance to developing embryonic and fetal skeletal muscle." Classification Embryonal rhabdomyosarcoma is the more common of the two major sub-types of rhabdomyosarcoma. ERMS accounts for 60% to 70% of rhabdomyosar ...
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Rhabdomyoblast
A rhabdomyoblast is a cell type which is found in some rhabdomyosarcomas. When found histologically, a rhabdomyoblast aids the diagnosis of embryonal, alveolar, spindle cell/sclerosing, and pleomorphic rhabdomyosarcomas; however, in a tumor, expression of the rhabdomyoblast phenotype is not the only factor in diagnosing a rhabdomyosarcoma. Mesenchymal malignancies can exhibit this phenotype as well. Immunohistochemistry techniques allow for the sensitive detection of desmin, vimentin, muscle specific actin, and MyoD1. Similarly the rhabdomyoblast phenotype can be detected morphologically. Rhabdomyoblasts are early stage mesenchymal cells, having the potential to differentiate into a wide range of skeletal cells. Each stage of differentiation exhibits unique and distinguishable histological characteristics. In its initial from, stellate cells with amphiphilic cytoplasm and ovular central nuclei are observed. Commonly referred to as rhabdoid features, the maturing rhabdomyoblast will li ...
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Sarcoma Botryoides
Sarcoma botryoides or botryoid sarcoma 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). Presentation 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. Histology Under the microscope one can see rhabdomyoblasts that may contain cross-striations. Tumor cells are crowded in a distinct layer beneath the vaginal epithelium ( cambium layer). Spindle-shaped tumor cells that are desmin positive. Trea ...
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Pleomorphic Rhabdomyosarcoma
Pleomorphism is a term used in histology and cytopathology to describe variability in the size, shape and staining of cells and/or their nuclei. Several key determinants of cell and nuclear size, like ploidy and the regulation of cellular metabolism, are commonly disrupted in tumors. Therefore, cellular and nuclear pleomorphism is one of the earliest hallmarks of cancer progression and a feature characteristic of malignant neoplasms and dysplasia. Certain benign cell types may also exhibit pleomorphism, e.g. neuroendocrine cells, Arias-Stella reaction. A rare type of rhabdomyosarcoma that is found in adults is known as pleomorphic rhabdomyosarcoma. Despite the prevalence of pleomorphism in human pathology, its role in disease progression is unclear. In epithelial tissue, pleomorphism in cellular size can induce packing defects and disperse aberrant cells. But the consequence of atypical cell and nuclear morphology in other tissues is unknown. See also *Anaplasia *Cell growt ...
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Pleomorphic Rhabdomyosarcoma
Pleomorphism is a term used in histology and cytopathology to describe variability in the size, shape and staining of cells and/or their nuclei. Several key determinants of cell and nuclear size, like ploidy and the regulation of cellular metabolism, are commonly disrupted in tumors. Therefore, cellular and nuclear pleomorphism is one of the earliest hallmarks of cancer progression and a feature characteristic of malignant neoplasms and dysplasia. Certain benign cell types may also exhibit pleomorphism, e.g. neuroendocrine cells, Arias-Stella reaction. A rare type of rhabdomyosarcoma that is found in adults is known as pleomorphic rhabdomyosarcoma. Despite the prevalence of pleomorphism in human pathology, its role in disease progression is unclear. In epithelial tissue, pleomorphism in cellular size can induce packing defects and disperse aberrant cells. But the consequence of atypical cell and nuclear morphology in other tissues is unknown. See also *Anaplasia *Cell growt ...
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Soft Tissue Sarcoma
A soft-tissue sarcoma (STS) is a malignant tumour, a type of cancer, that develops in soft tissue. A soft tissue sarcoma is often a painless mass that grows slowly over months or years. They may be superficial or deep-seated. Any such unexplained mass must be diagnosed by biopsy. Treatment may include, surgery, radiotherapy, chemotherapy, and targeted drug therapy. The other type of sarcoma is a bone sarcoma. There are many types. The World Health Organization lists more than fifty subtypes. Types ''An earlier version of this article was taken from the US National Cancer Center's Cancer Information Service.'' Signs and symptoms In their early stages, soft-tissue sarcomas usually do not cause symptoms. Because soft tissue is relatively elastic, tumors can grow rather large, pushing aside normal tissue, before they are felt or cause any problems. The first noticeable symptom is usually a painless lump or swelling. As the tumor grows, it may cause other symptoms, such as ...
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Alveolar Rhabdomyosarcoma
Alveolus (; pl. alveoli, adj. alveolar) is a general anatomical term for a concave cavity or pit. Uses in anatomy and zoology * Pulmonary alveolus, an air sac in the lungs ** Alveolar cell or pneumocyte ** Alveolar duct ** Alveolar macrophage * Mammary alveolus, a milk sac in the mammary glands * Alveolar gland * Dental alveolus, also known as "tooth socket", a socket in the jaw that holds the roots of teeth ** Alveolar ridge, the jaw structure that contains the dental alveoli ** Alveolar canals ** Alveolar process * Arteries: ** Superior alveolar artery (other) *** Anterior superior alveolar arteries *** Posterior superior alveolar artery ** Inferior alveolar artery * Nerves: ** Anterior superior alveolar nerve ** Middle superior alveolar nerve ** Inferior alveolar nerve Uses in botany, microbiology and related disciplines * Surface cavities or pits, such as on the stem of Myrmecodia species * Pits on honeycombed surfaces such as receptacles of many angiosperms * Pits o ...
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Anaplasia
Anaplasia (from grc, ἀνά ''ana'', "backward" + πλάσις ''plasis'', "formation") is a condition of cells with poor cellular differentiation, losing the morphological characteristics of mature cells and their orientation with respect to each other and to endothelial cells. The term also refers to a group of morphological changes in a cell (nuclear pleomorphism, altered nuclear-cytoplasmic ratio, presence of nucleoli, high proliferation index) that point to a possible malignant transformation. Such loss of structural differentiation is especially seen in most, but not all, malignant neoplasms. Sometimes, the term also includes an increased capacity for multiplication. Lack of differentiation is considered a hallmark of aggressive malignancies (for example, it differentiates leiomyosarcomas from leiomyomas). The term ''anaplasia'' literally means "to form backward". It implies dedifferentiation, or loss of structural and functional differentiation of normal cells. It is n ...
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Contrast CT
Contrast CT, or contrast enhanced computed tomography (CECT), is X-ray computed tomography (CT) using radiocontrast. Radiocontrasts for X-ray CT are generally iodine-based types. This is useful to highlight structures such as blood vessels that otherwise would be difficult to delineate from their surroundings. Using contrast material can also help to obtain functional information about tissues. Often, images are taken both with and without radiocontrast. CT images are called ''precontrast'' or ''native-phase'' images before any radiocontrast has been administrated, and ''postcontrast'' after radiocontrast administration. Bolus tracking Bolus tracking is a technique to optimize timing of the imaging. A small bolus of radio-opaque contrast media is injected into a patient via a peripheral intravenous cannula. Depending on the vessel being imaged, the volume of contrast is tracked using a region of interest (abbreviated "R.O.I.") at a certain level and then followed by the CT ...
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Smooth Muscle Tissue
Smooth muscle is an involuntary non-striated muscle, so-called because it has no sarcomeres and therefore no striations (''bands'' or ''stripes''). It is divided into two subgroups, single-unit and multiunit smooth muscle. Within single-unit muscle, the whole bundle or sheet of smooth muscle cells contracts as a syncytium. Smooth muscle is found in the walls of hollow organs, including the stomach, intestines, bladder and uterus; in the walls of passageways, such as blood, and lymph vessels, and in the tracts of the respiratory, urinary, and reproductive systems. In the eyes, the ciliary muscles, a type of smooth muscle, dilate and contract the iris and alter the shape of the lens. In the skin, smooth muscle cells such as those of the arrector pili cause hair to stand erect in response to cold temperature or fear. Structure Gross anatomy Smooth muscle is grouped into two types: single-unit smooth muscle, also known as visceral smooth muscle, and multiunit smooth muscle. M ...
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Five-year Survival Rate
The five-year survival rate is a type of survival rate for estimating the prognosis of a particular disease, normally calculated from the point of diagnosis. Lead time bias from earlier diagnosis can affect interpretation of the five-year survival rate. There are absolute and relative survival rates, but the latter are more useful and commonly used. Relative and absolute rates Five-year relative survival rates are more commonly cited in cancer statistics. Five-year absolute survival rates may sometimes also be cited. * Five-year ''absolute'' survival rates describe the percentage of patients alive five years after the disease is diagnosed. * Five-year ''relative'' survival rates describe the percentage of patients with a disease alive five years after the disease is diagnosed, divided by the percentage of the general population of corresponding sex and age alive after five years. Typically, cancer five-year relative survival rates are well below 100%, reflecting excess mortality ...
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