Ewing's Sarcoma Case Study
Ewings sarcoma
Ewing's sarcoma (ES) named after Dr. James wing in 1921; he was the first man identified the disease that uncontrollably destroys the bone or body tissue of the human body (Randall, n.d.). ES is a disseminated small round neoplasm, which commonly occurs in the bones and unusually in body tissue (Iwamoto, 2007). ES is the second leading tumor in the bone of children between the age of ten and twenty years old, and the occurrence is slightly more in male than female (Iwamoto, 2007). ES affects the femur, tibia, pelvis, humerus and thoracic wall, but it can affect any bone (Jedlicka, 2010; Miser et., 2010). The term paper provided the general outline; diagnostic imaging used, research and role of physical therapist related to ES.
ES is a significant malignant bone tumor, and the disease is originated from a red bone marrow, which occur in children and adolescent (Lemons, n.d.). The skeletal region most …show more content…
commonly affected related to ES are the long bones (diaphyses of the femur predominates, succeeding the bones in the upper and lower arm) and truncal skeleton (pelvis is the most common site followed by the shoulder blade, spinal column, ribs and collar bone) (Iwamoto, 2007).The genetic exchange between somatic chromosomes can develop the body tissue to become carcinogenic (Cecil, Goldman & Schafer, 2012). According to Goodman and Fuller (2014), ninety-five percent of ES originated from genetic imbalance called translocation between chromosomes 11 and 22. The translocation of the between chromosomes 11 and 22, which combine the EWS gene of chromosome 22 to the FL11 gene of chromosome 11 (Cecil, Goldman & Schafer, 2012); the gene EWS/FL1 act as the chief regulator (Owen, Kowalewski & Lessnick, 2008). Moreover, ES have a similar tissue tumor known as primitive neuroectodermal tumor (PNET) microscopically, and 95 % of circumstances had a similar chromosomal imbalance called translocation (Aurias, 1984; Whang, 1984 & Burchill, 2003). Eventually, ES and primitive neuroectodermal tumors are sometimes classified together called Ewing's sarcoma family of tumors (ESFT) (Iwamoto, 2007). ES spread instantaneously (Iwamoto, 2007). The first clinical manifestation of ES is a mild intermittent pain (Iwamoto, 2007). As the tumor grows, it leads to visible or tangible swelling of the involved site (Iwamoto, 2007). Other clinical symptoms of ES includes fever, anemia, extreme fatigue, loss of appetite, weight loss, high sedimentation rate, leukocytosis and increased serum level (Iwamoto, 2007). Moreover, de Alava, Lozano, Patiño, Sierrasesúmaga & Pardo-Mindán (1998) found that the EWS-FL1 combined gene often identified in the cellular components of blood tests from patients with EWS. The prognosis of ES is seventy percent survival, generally for five years with localized disease; Nonetheless, the survival rate is a 30 percent of an eight year period for patients with a malignant type of ES (Henninger, 2013).
Diagnostic imaging According to Lemons, J. M. (n.d.). , there is no particular study or useful modality or method supports the accurate diagnosis of ES, however, the outcome of the test contribute to the staging of the tumor.
Radiography
X-ray is the primary imaging modality used for suspected bone tumor. Commonly, in radiography, the clinical feature of ES, such as indistinct locally moth- eaten appearance in bone, damaged intramedullary bone, combined with periostitis (onion-skin peeling/sunburst pattern) involving the long bones (Iwamoto, 2007). The periostitis pattern attributed to patients with ES at a rate of 50%, which indicates an aggressive form of a tumor (Lemons, J. M. (n.d.). However, the infiltration of the bone marrow is not visible in radiography Magnetic resonance imaging (MRI) MRI is the leading imaging technique for assessing, provides specific information and staging the disease for suspected ES (Iwamoto, 2007).
San-Julian, Aquerreta, Benito & Cañadell (1999) reported in one study, which showed that MRI has 90.3 percent of the accuracy with a positive predictive value and negative predictive value in staging patients with possible ES. MRI provides a clear view of the extent and localization of the bone tumors, such as the severity of bone tumor and the connection of the impairment of the blood vessels and nerves (Iwamoto, 2007). Furthermore, MRI able to identify or assess the expansion of soft tissue or cell growth, which extend beyond the area indicated by plain x-ray film (Iwamoto, 2007). According to Lemons (n.d.), MRI and CT scan are problematic in differentiating ES and other similar in nature of a disease. However, MRI able to identify an ES lesion in patients of a particular age group in comparison to other kinds of cancer, such as osteosarcoma, fibrosarcoma and other malignant tumors (Lemons
(n.d.)
Computerized tomography (CT) CT able to detect or delineate the accurate size and the expansion of the tumor and the relationship adjacent to the affected musculoskeletal site related to ES (San-Julian, Aquerreta, Benito & Cañadell, 1999; Zhang, Xie, Mo & Li, 2007). Furthermore, CT is also important in evaluating a patient with metastasis in the pulmonary system (Iwamoto, 2007). There is about fifty percent chance of metastasis in the lungs of patients with ES (Bellan, 2012). However, CT scan may have uncertainties in differentiating ES from other tumors of the bone, such as osteomyelitis; and the false positive outcome may take place (Lemons, n.d.).
Ultrasonography (US) US is not commonly used for specifying or classifying the initial diagnosis in ES (Lemons, n.d.). Nevertheless, US can assist to direct a biopsy of a disease and assist in ruling out malignancy in the liver (Lemons, n.d.).
Nuclear imaging
Bone scintigraphy- Technetium-99-m whole body radionucleotide bone scan also assists in assessing skeletal metastasis (Iwamoto, 2007). Full body bone scans can support an information about the primary tumor of ES and delineate intestinal damage (Lemons, n.d.). Moreover, bone scintigraphy can be utilized in localizing and staging the outlying malignancy (Lemons, n.d.). However, lesions in ES and other benign bone lesions causing for false positive findings in the bone scan(Lemons, n.d.).
Fluorine-18fluorodeoxyglucose-positiron emission tomography
(FGD-PET)
FGD-PET has a highers spatial resolution and has an increased sensitivity of identifying skeletal cell growths and treatment responses. FGD-PET used as a guide of the viability of the tumor, and it may assist that are not visible on the standard bone scan (Lemons, n.d.). Yet, FGD-PET management for ES remains to be determined (Iwamoto, 2007).
Angiography Angiography is not a standard means of diagnosing of suspected ES, but the vascularity of tumor during chemotherapy can be assessed with magnetic resonance angiography (Lemons, n.d.).
Physical therapy implications: As a clinician, thorough physical therapy evaluation and cautious with treatment when providing a patient with any bone tumors including ES. Patients with complain of severe pain and other symptoms related to the bone tumor without relief from pain medication and physical therapy management are signs of suspected bone or soft tissue tumor and further referral to a medical practitioner is necessary (Miville, L. (n.d.). Furthermore, patient education is important for patients with bone tumor such as the precaution of limiting of bearing weight on the affected limb with tumor in preventing complications, as well as guidelines for stretching, strengthening and aerobic conditioning exercise pre-operatively and post-operatively (Miville, L. (n.d.)
Conclusion
ES is a bone tumor that commonly occurs children with a low survival rate. MRI remain an ideal method of assessing the extent and stage of a lesion of suspected ES in comparison to radiography. Although, CT scan have a similar process of evaluating the expansion of the disease related to ES. However, MRI able to distinguish ES to a particular age group. Furthermore, due to the similarity in nature of the description of bone tumor in ES and other types of diseases with abnormal cellular growths, it 's hard to identify the difference. Therefore, to specifically detect and provide the lesion related to ES is by physical examination (signs and symptoms, medical history and others), clinical test such as laboratory values. Clinical symptoms of ES includes pain, swelling, fever, anemia, highs sedimentation rate, leukocytosis and increase serum level (Iwamoto, 2007). The EWS-FL1 combined gene often identified in the cellular components of blood tests from patients with EWS related to ES (de Alava et al.,1998).
Ewing's sarcoma (ES) named after Dr. James wing in 1921; he was the first man identified the disease that uncontrollably destroys the bone or body tissue of the human body (Randall, n.d.). ES is a disseminated small round neoplasm, which commonly occurs in the bones and unusually in body tissue (Iwamoto, 2007). ES is the second leading tumor in the bone of children between the age of ten and twenty years old, and the occurrence is slightly more in male than female (Iwamoto, 2007). ES affects the femur, tibia, pelvis, humerus and thoracic wall, but it can affect any bone (Jedlicka, 2010; Miser et., 2010). The term paper provided the general outline; diagnostic imaging used, research and role of physical therapist related to ES.
ES is a significant malignant bone tumor, and the disease is originated from a red bone marrow, which occur in children and adolescent (Lemons, n.d.). The skeletal region most …show more content…
commonly affected related to ES are the long bones (diaphyses of the femur predominates, succeeding the bones in the upper and lower arm) and truncal skeleton (pelvis is the most common site followed by the shoulder blade, spinal column, ribs and collar bone) (Iwamoto, 2007).The genetic exchange between somatic chromosomes can develop the body tissue to become carcinogenic (Cecil, Goldman & Schafer, 2012). According to Goodman and Fuller (2014), ninety-five percent of ES originated from genetic imbalance called translocation between chromosomes 11 and 22. The translocation of the between chromosomes 11 and 22, which combine the EWS gene of chromosome 22 to the FL11 gene of chromosome 11 (Cecil, Goldman & Schafer, 2012); the gene EWS/FL1 act as the chief regulator (Owen, Kowalewski & Lessnick, 2008). Moreover, ES have a similar tissue tumor known as primitive neuroectodermal tumor (PNET) microscopically, and 95 % of circumstances had a similar chromosomal imbalance called translocation (Aurias, 1984; Whang, 1984 & Burchill, 2003). Eventually, ES and primitive neuroectodermal tumors are sometimes classified together called Ewing's sarcoma family of tumors (ESFT) (Iwamoto, 2007). ES spread instantaneously (Iwamoto, 2007). The first clinical manifestation of ES is a mild intermittent pain (Iwamoto, 2007). As the tumor grows, it leads to visible or tangible swelling of the involved site (Iwamoto, 2007). Other clinical symptoms of ES includes fever, anemia, extreme fatigue, loss of appetite, weight loss, high sedimentation rate, leukocytosis and increased serum level (Iwamoto, 2007). Moreover, de Alava, Lozano, Patiño, Sierrasesúmaga & Pardo-Mindán (1998) found that the EWS-FL1 combined gene often identified in the cellular components of blood tests from patients with EWS. The prognosis of ES is seventy percent survival, generally for five years with localized disease; Nonetheless, the survival rate is a 30 percent of an eight year period for patients with a malignant type of ES (Henninger, 2013).
Diagnostic imaging According to Lemons, J. M. (n.d.). , there is no particular study or useful modality or method supports the accurate diagnosis of ES, however, the outcome of the test contribute to the staging of the tumor.
Radiography
X-ray is the primary imaging modality used for suspected bone tumor. Commonly, in radiography, the clinical feature of ES, such as indistinct locally moth- eaten appearance in bone, damaged intramedullary bone, combined with periostitis (onion-skin peeling/sunburst pattern) involving the long bones (Iwamoto, 2007). The periostitis pattern attributed to patients with ES at a rate of 50%, which indicates an aggressive form of a tumor (Lemons, J. M. (n.d.). However, the infiltration of the bone marrow is not visible in radiography Magnetic resonance imaging (MRI) MRI is the leading imaging technique for assessing, provides specific information and staging the disease for suspected ES (Iwamoto, 2007).
San-Julian, Aquerreta, Benito & Cañadell (1999) reported in one study, which showed that MRI has 90.3 percent of the accuracy with a positive predictive value and negative predictive value in staging patients with possible ES. MRI provides a clear view of the extent and localization of the bone tumors, such as the severity of bone tumor and the connection of the impairment of the blood vessels and nerves (Iwamoto, 2007). Furthermore, MRI able to identify or assess the expansion of soft tissue or cell growth, which extend beyond the area indicated by plain x-ray film (Iwamoto, 2007). According to Lemons (n.d.), MRI and CT scan are problematic in differentiating ES and other similar in nature of a disease. However, MRI able to identify an ES lesion in patients of a particular age group in comparison to other kinds of cancer, such as osteosarcoma, fibrosarcoma and other malignant tumors (Lemons
(n.d.)
Computerized tomography (CT) CT able to detect or delineate the accurate size and the expansion of the tumor and the relationship adjacent to the affected musculoskeletal site related to ES (San-Julian, Aquerreta, Benito & Cañadell, 1999; Zhang, Xie, Mo & Li, 2007). Furthermore, CT is also important in evaluating a patient with metastasis in the pulmonary system (Iwamoto, 2007). There is about fifty percent chance of metastasis in the lungs of patients with ES (Bellan, 2012). However, CT scan may have uncertainties in differentiating ES from other tumors of the bone, such as osteomyelitis; and the false positive outcome may take place (Lemons, n.d.).
Ultrasonography (US) US is not commonly used for specifying or classifying the initial diagnosis in ES (Lemons, n.d.). Nevertheless, US can assist to direct a biopsy of a disease and assist in ruling out malignancy in the liver (Lemons, n.d.).
Nuclear imaging
Bone scintigraphy- Technetium-99-m whole body radionucleotide bone scan also assists in assessing skeletal metastasis (Iwamoto, 2007). Full body bone scans can support an information about the primary tumor of ES and delineate intestinal damage (Lemons, n.d.). Moreover, bone scintigraphy can be utilized in localizing and staging the outlying malignancy (Lemons, n.d.). However, lesions in ES and other benign bone lesions causing for false positive findings in the bone scan(Lemons, n.d.).
Fluorine-18fluorodeoxyglucose-positiron emission tomography
(FGD-PET)
FGD-PET has a highers spatial resolution and has an increased sensitivity of identifying skeletal cell growths and treatment responses. FGD-PET used as a guide of the viability of the tumor, and it may assist that are not visible on the standard bone scan (Lemons, n.d.). Yet, FGD-PET management for ES remains to be determined (Iwamoto, 2007).
Angiography Angiography is not a standard means of diagnosing of suspected ES, but the vascularity of tumor during chemotherapy can be assessed with magnetic resonance angiography (Lemons, n.d.).
Physical therapy implications: As a clinician, thorough physical therapy evaluation and cautious with treatment when providing a patient with any bone tumors including ES. Patients with complain of severe pain and other symptoms related to the bone tumor without relief from pain medication and physical therapy management are signs of suspected bone or soft tissue tumor and further referral to a medical practitioner is necessary (Miville, L. (n.d.). Furthermore, patient education is important for patients with bone tumor such as the precaution of limiting of bearing weight on the affected limb with tumor in preventing complications, as well as guidelines for stretching, strengthening and aerobic conditioning exercise pre-operatively and post-operatively (Miville, L. (n.d.)
Conclusion
ES is a bone tumor that commonly occurs children with a low survival rate. MRI remain an ideal method of assessing the extent and stage of a lesion of suspected ES in comparison to radiography. Although, CT scan have a similar process of evaluating the expansion of the disease related to ES. However, MRI able to distinguish ES to a particular age group. Furthermore, due to the similarity in nature of the description of bone tumor in ES and other types of diseases with abnormal cellular growths, it 's hard to identify the difference. Therefore, to specifically detect and provide the lesion related to ES is by physical examination (signs and symptoms, medical history and others), clinical test such as laboratory values. Clinical symptoms of ES includes pain, swelling, fever, anemia, highs sedimentation rate, leukocytosis and increase serum level (Iwamoto, 2007). The EWS-FL1 combined gene often identified in the cellular components of blood tests from patients with EWS related to ES (de Alava et al.,1998).