Disease Information for Ewings sarcoma (Clinical Manifestations)

ES, Ewing sarcoma, Ewing Tumor, Ewing's family of tumors, Ewing's Family of Tumours, Ewings sarcoma, Ewing's sarcoma, Ewing's sarcoma (morphologic abnormality), Ewing's Sarcoma Peripheral Primitive Neuroectodermal Tumor, Ewing's sarcoma stage unspecified, Ewings Tumor, Ewing's Tumor, Ewing's tumour, Sarcoma Ewing, Sarcoma Ewings, sarcoma Ewing's, Tumor Ewing's, Tumors of Ewing's Family, Tumors of the Ewing's Family

A type of bone cancer that usually forms in the middle (shaft) of large bones; Also called Ewing"s sarcoma/primitive neuroectodermal tumor (PNET); Primary malignant bone tumors are rare; Four types make up the majority; Malignant fibrous histiocytoma (MFH) - a pleomorphic sarcoma of storiform pattern without differentiation; Osteosarcoma - similar to malignant fibrous histiocytoma with differentiation to osteoid production; Osteosarcoma - cellular cartilaginous lesion with abundant binucleate cells, myxoid areas, and pushing borders; Ewing sarcoma - small, blue-round cell neoplasm;<br>

Genetics: Ewing sarcoma has 11/22 chromosomal translocation and EW5-FLI-1 fusion protein; Osteosarcomas shows loss of retinoblastoma and p53 suppressor genes and amplification of the genes C-myc, mdm-2, SAS, and cyclin-dependent kinase;Prevalence in USA: Rare: 5000 bone and soft tissue sarcomas per year, a practicing orthopedic surgeon may see one primary malignant tumor of bone in every five years of practice; Ewing sarcoma is less common in blacks; Age:MFH - teens and elderly; Osteogenic sarcoma - teens and early twenties; Chondrosarcoma - very young and very old; Ewing sarcoma - children, teens, and early twenties ; Male = Female; Pain with weight bearing, at rest and at night; Swelling; Bone Tenderness ; Fracture with minor trauma; Minor injury may bring attention to lesion; MFH often follows irradiation or arises in old bone infarct; <br>

Osteosarcoma has association with loss of suppressor retinoblastoma and p53 genes; Chondrosarcoma may arise in pre-existing enchondroma or exostosis; Multiple enchondromatosis (Ollier"s disease) - chondrosarcoma; Multiple hereditary exostosis - chondrosarcoma; Previous irradiation, risk factor for MFH; Previous history of bilateral retinoblastoma - osteosarcoma; Pagets disease osteosarcoma; DDX: Solitary metastatic lesion or myeloma especially in the patient over age 40; Lymphoma at any age; Benign bone tumors and benign bone tumors that look aggressive (aneurysmal bone cyst, giant cell tumor, eosinophilic granuloma); Infection (osteomyelitis); Metabolic bone disease (osteopenia, Pagets, <br>

hyperparathyroidism; Synovial diseases (pigmented villonodular synovitis; [Griffith clincal advisor 2005]<br>

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