Ewing’s sarcoma (ES), a malignant osteolytic tumor, characterized as small round cell tumors was first documented by James Ewing in the year 1921 as diffuse endothelioma of bone. Rarely, it also has extra osseous manifestations which resembles intraosseous ES. This extra osseous forms of ES was first described by Tefft in 1969.(1) Ewing’s sarcoma (ES) family of tumors include: classical ES (osseous origin), atypical ES (extra osseous), Primitive neuroectodermal tumor (PNET) and Askin tumor[2] . All these tumors have common morphology, immunophenotypic features and cytogenetics, hence included in the same family of tumors. These tumors were believed to be derived from a primitive cell. The primitive cell can be either the neural crest or mesenchymal …show more content…
Rarely, many other genes from the family members of ETS have also been identified as the fusion partners of EWS (7). According to Lin et al., 95% of cases of ES occur from EWS/FLI1 fusion gene formation as a result of transformation. Due to the difference in the locations of the EWS and FLI1 genomic breakpoints, it resulted in the existence of alternative forms of the chimeric gene. There are two most common forms, type 1 and type 2 accounting to 60% and 25% of the cases respectively. Type 1 consists of the first 7 exons of EWS joined to exons 6-9 of FLI1 and type 2 includes FLI1 exon 5 also.( 8) The prognosis of type 1 fusion is significantly better prognosis than the other fusion types as observed by Lin et al. (1999). A less active chimeric transcription factor is encoded by the type 1 fusion gene explaining the heterogeneous forms of ES at the molecular level. Particular chromosomal translocations is strongly associated with the development of PNET. The products of the fusion genes resulted from the translocations are specific to the type of tumors. In ES/PNETs, karyotype of t[11;22][q24;q12], which results from the EWS-FLII gene infusion and t[21;22][q22;q12], which results from EWS-ERG gene infusion account for 85% and 10% respectively (9-11). In reports of ES/PNETs, there are eight cases which chromosome translocation are t[11;22][q24; q12] …show more content…
The classic ES appears like that of a primitive, undifferentiated neoplasm morphologically. Histologically, there are small round blue cells in the form of monotonous sheets with hyperchromatic nuclei and scant cytoplasm (8). All the 24 cases in this review satiate these histologic criteria (Table 1). The tumor consists of extensive necrotic areas but viable tumor is usually preserved around blood vessels. Some tumor cells can also invade blood vessels. Some features are absent typically like nuclear atypia, palisading, and formation of rosettes (where the tumor cells are arranged in a circle about a central fibrillary space, indicative of neural differentiation or pseudo rosettes. The markers like P30/32MlC2 and at least two kinds of neuronal markers are the immunohistochemical criteria for the diagnosis of PNET. (12) Monoclonal antibodies like CD99, O13, HBA71, 12E7, RFB1 are also tested although none of them are actually specific for PNETs. (12) The neural markers like Neuron Specific Enolase(NSE), Chromogranin A(CgA), Synaptophysin(Syn) are usually positive but markers such as desmin, actin, S-100, insulin, glucagons, somatostatin are rarely
Many people suffer from all types of cancer like, leukemia, lymphoma, multiple myeloma, malignant mesothelioma and so much more. These types of cancers are very common and are usually found and treated as soon as possible. Despite the fact that technology and medicine has advanced in many ways, Clear cell sarcoma is still a puzzle to most doctors.
Breast genus Malignant neoplastic disease is a disease in which prison cell in the boob grow out of control. There are different variety of tit Cancer the Crab the Crab . The kind of white meat cancer depends on which cell in the titty turn into cancer. Breast cancer can begin in different piece of the chest . A titty is made up of three briny function : lobule , epithelial duct , and conjunction tissue paper paper . The lobule are the glands that green goods Milk . The canal are vacuum tube that carry milk to the nipple . The connexion tissue (which consists of fibrous and fatty tissue) surrounds and holds everything together. Most breast cancers begin in the epithelial duct or lobules. Breast cancer can banquet ing head outside the breast
The asymptomatic nature of the lesion means that the presentation is late in most patients. Local recurrence is a problem with this tumor,
Ewing’s sarcoma is a rare bone cancer.1-3 Ewing’s sarcoma is from the Ewing’s family of tumors.1-9 There are three types of Ewing tumors in bone and they include a classic Ewing’s sarcoma, a primitive neuroectodermal tumor or PNET, and Askin tumor which is a PNET of the chest wall. Ewing’s sarcoma is the second most common malignant bone tumor in children and adolescents. It accounts for 2% of all childhood cancers.1-3 Ewing’s sarcoma accounts for approximately 10-15% of all primary bone tumors.2 It is slightly more common in males than females. The ratio has been described as 2:1 and 1.5:1.1-4 It was first discovered in 1921 by James Ewing.1,5 Ewing’s sarcoma is more common in Caucasians. It was described as a “diffuse endothelioma of bone” by Ewing.4 Ewing’s sarcoma can affect any bone. The most common sites are the lower extremities and pelvis.1-4 Tumors can occur in bone or soft tissue (extraosseous). This paper will discuss the causes, the signs and symptoms, diagnosis, treatment and prognosis of the disease.
Angiosarcomas are malignant tumors that can develop from the endothelium of blood vessels (hemangiosarcoma) or lymphatics (lymphangiosarcoma). Lymphangiosarcomas are thought to be secondary malignant tumors arising from large, protracted lymphedema. Despite their name, they originate in vascular endothelial cells rather than the lymphatic vessels themselves. They may initially appear as “blue-red or purple skin lesions” that can manifest either a macular or papular shape. Multiple lesions are common and may form subcutaneous nodules. The clinical prognosis of patients with angiosarcomas in general, and lymphangiosarcomas in particular, remains poor, with less than 10 percent of patients surviving longer than 5 years after the initial diagnosis per one study. While many modalities of treatment including wide resection, chemotherapy, and radiation therapy have been attempted, currently only early amputation of the affected limb seems to give the best chance of relative long-term survival. Amputation after local recurrence, however, has been shown to be ineffective in preventing pulmonary metastases and death.
Ewing’s Sarcoma is a disease of the bones, that is mainly diagnosed in children and adolescents. In order to confirm the Ewing’s, a biopsy must be performed on the individual. Different factors including; age, gender, location, and stage of tumor must all be determined to allow to accurate course of treatment. Many times, the location of the tumor will result in secondary organ disfunction.
Medulloblastoma, also called cerebellar primitive neuroectodermal tumor (PNET), is a fast growing embryonal tumors[A] located in the cerebellum caused by a glial cell (1)(4). The seven types of medulloblastoma are, classic,desmoplastic, nodular,, large-cell,(anaplastic), neuroblastic, glial differentiation, medullomyoblastoma, and melanotic. According to American Brain Tumor Association, medulloblastoma is relatively rare, and only accounts for two percent of all primary brain tumors(1).However, medulloblastoma is common in children making up to twenty percent of childhood brain tumors(2), while it is less common in adults(1). It is also more common in men than in women(3). (Thesis)
Giant cell tumor is an osteolytic, generally benign neoplasm, accounts for 5% of primary skeletal tumors[5]. According to SEER(National Cancer Institute’s Surveillance, Epidemiology and End Results) database, the estimated annual incidence of GCT in the United States was 1.6 per 10,000,000 persons per year with a highest incident age of 20 to 45 years old[6,7]. While studies in the United States reported an incident accounts for 5%-7% of all primary bone tumors, incidence reported in Asian population was higher than 10% while that of Chinese population was reported to be 20% of all biopsy-analyzed primary bone tumors[2,4]. Furthermore, differ from reported in the Caucasian population shown a female predominance, Asian patients demonstrated
Normally, your body reproduces new cells as you need them and replaces the old cells that die (Lindstrom, 2011). Sometimes, this process goes wrong where new cells are produced uncontrollably even when you don’t need them, and old cells don’t die when they should (Lindstrom, 2011). These abnormal growth of the cell tissues can form tumors which can be benign or malignant (Lindstrom, 2011). A benign tumor is not a cancerous tumor which cannot spread throughout the body like those cancerous ones (Usmani, 2015). Malignant tumors are cancerous tumors that have the ability to multiply uncontrollably and spread to different locations in the body (Lindstrom, 2011). Abnormal cells that form a malignant tumor are very unstable. They travel via blood
American Cancer Society (2014) states that osteosarcoma is a rare cancer that starts primarily in the bone. According to the American Cancer Society (2014), annually, about 800 people are newly diagnosed with osteosarcoma in the United States of America. Among the newly diagnosed cases, 400 are children and teenagers. Osteosarcoma is usually observed in children, adolescents and adults ages 10 to30. Of the three mentioned, teenagers are mostly affected, nonetheless osteosarcoma can happen at any stage. Osteosarcoma usually begins in the legs, close to the end of the long bones primarily targeting the knee. Another commonplace for osteosarcoma to be present is near the
A common definition of bone cancer is cancer in the bones that has originated or spread from another place to the bone.”Cancer can begin in any type of bone tissue” (Cancer,gov). There are three most common forms of bone cancer Osteosarcoma, Chondrosarcoma, and Ewing Sarcoma Family of Tumors (EFSTs). Osteosarcoma arises from osteoid in the bone and mostly occurs in the knee and upper arm. It also occurs for people ages from 10 to 19 but can be with an adult at the age of 40. Chondrosarcoma begins in the cartilaginous tissue and most often in the pelvis, upper leg and shoulder. Also in cause of finding a cancer cell it can be classified as osteosarcoma.Found in adults over the age of 40. Ewing Sarcoma Family of Tumors are most commonly found in the bone is sometimes found in soft tissue like muscles, blood vessels, or fat. Often found in children under the age 19. Specialist thinks that according to the article, “EFSTs arise from elements of primitive nerve tissue in the bone or soft tissue” (Cancer,gov). EFSTs are found normally in backbone, pelvis, arms, and legs.
Sarcoma is a cancerous disease which develops from the soft tissues and or bone of the human body. Given this, sarcomas have been divided into two general groups: soft tissue sarcoma (STS) and osteosarcoma. STS may develop from soft tissues such as muscle, fat, nerves, blood vessels, or deep skin tissues. Sarcomas may spread throughout the body or may stay in one place. Furthermore, being affected by a cancerous disease causes proliferation of the connective tissue and or organs causing lumps to be formed. Given the status of the lumps they may be identified as various types. STS is a very rare cancer with approximation of about 1% among adults and 15% among the children. There has been no identification of definite causes but risk factors
If bone cancer is suspected, x-rays can be taken which may show key characteristics of a specific type of cancer. Osteosarcoma can often be identified by its classic “sunburst” pattern in radiographs. What makes up the pattern is a soft tissue mass accompanied by horizontal bony spicules extending out through the mass (Eisenberg and Johnson, 2012, p. 124). Chondrosarcoma can be seen on radiographs as endosteal scalloping with destruction of the compact layer of bone, and often contains a splotchy or shapeless calcification. Ewing’s sarcoma is
Bone cancer is a rare form of cancer that starts in a person’s bone structure. Bone cancer can develop in any bone of the person, but is commonly seen in the longer bones in the person’s upper and lower extremities. There are several types of bone malignancies that are known, some of which affect children while others primarily impact adults (Mayo Clinic, 2015, p. 1). Among the more prevalent types of bone malignancies include the following (a) Osteosarcoma, this type of malignancy begins in the osteoid tissue of the bone, occurring mainly in the region of the upper arm and the knees; (b) Chondrosarcoma, mainly begins in the cartilage of the bone, and; (c) The Ewing Sarcoma Family of Tumors (ESFTs), occurs in the bone, but also impacts the soft tissue. This type is commonly discovered in the spine and the pelvis region as well as the arms and lower extremities of the person (National Cancer Institute, 2008, p. 1).
CASE DISCUSSION:Desmoplastic small round cell tumour (DSRCT) belongs to the family of ‘‘small round blue cell tumours’’, which include DSRCT,Wilms’ tumour, rhabdomyosarcoma, , primitive neuroectodermal tumour, ,neuroblastoma, Ewing sarcoma ,small cell osteosarcoma, poorly differentiated synovial cell sarcoma and lymphomas which are characterised by sheets of small cells round nuclei. The primary tumour typically arises in the peritoneal cavity of the pelvis, although DSRCT arising from brain, lung, pleura, stomach, pancreas, ovary, paranasal sinuses and scrotum has been described.[5]It is a very aggressive neoplasm with mean age of survival of 23 months.[6] CT frequently shows multiple bulky, lobulated, heterogeneous,and peritoneal soft-tissue masses with a predilection for spread to distant organs without obvious primary organinvolvement. On histology these tumors show sharply demarcated,angulated, cohesive nests of uniform tumor cells with round to oval nuclei, inconspicuous nucleoli, and scant cytoplasm; surrounded by abundant fibrous stroma. DSRCT immunohistochemistry shows