BONE TUMORS IN CHILDREN: OSTEOSARCOMA & EWING SARCOMA

Neoplastic disease can occur from any tissue involved in bone growth. In children Primary bone cancer Metastatic bone cancer Accounts to 85% of all primary bone tumors

TYPES: PRIMARY BONE CANCER

Osteosarcoma Chondrosarcoma Ewing Sarcoma

INCIDENCE
Primary bone cancer is rare Accounts to <1 % of all cancers Osteosarcoma & ewing sarcoma occurs between ages 10 and 19 [pk 15-19 yrs]: ADOLESCENT Male : Female 2:1 Chondrosarcoma occurs mainly in older adults (over age 40).

CAUSES: BONE CANCER

Inherited genetic mutations Radiation Trauma

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 Some retinoblastomas, osteosarcomas, and soft tissue sarcomas may share a common etiology R R Weichselbaum M Beckett and A Diamond University of Chicago, Department of Radiation and Cellular Oncology, IL 60637. Free Powerpoint Templates

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 DNA and RNA were extracted from primary human osteosarcomas and soft tissue sarcomas obtained from patients without retinoblastoma and were analyzed by hybridization with a cDNA probe for RB mRNA; absence or alterations of the RB gene are associated with development of retinoblastoma. Most of the osteosarcomas or soft tissue sarcomas examined by us did not express detectable levels of RB mRNA, whereas normal cells and epithelial tumor cells did. One osteosarcoma expressed a 2.4-kilobase transcript in addition to a normal 4.7-kilobase species. Our data suggest that transcriptional inactivation or post-transcriptional down-regulation of the RB gene may be important in the etiology of some osteosarcomas and soft tissueTemplates sarcomas as well as Free Powerpoint Page 7 retinoblastomas.

Etiology of Osteosarcoma Fuchs, Bruno MD*,**; Pritchard, Douglas J. MD* Several chemical agents such as beryllium, viruses such as FBJ, subsequently found to contain the sarconcogene, and radiation were shown to be potent inducers of osteosarcoma. Paget's disease, electrical burn, or trauma all are thought to be other factors that may contribute to the pathogenesis.

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 More recently, patients with hereditary diseases such as Rothmund-Thomson syndrome, Bloom syndrome, and LiFraumeni syndrome were found to have an increased risk of having osteosarcoma develop.

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 During the past few years, the molecular analysis brought a wealth of new information with numerous genes that were associated with osteosarcoma and its clinical disease progression. They can be categorized into self-sufficiency in growth signals, insensitivity to growth inhibitory signals, evasion of apoptosis, limitless replicative potential, sustained angiogenesis, and tissue evasion and metastasis. Although the understanding of these processes in osteosarcoma still is incomplete, it may have the potential to significantly affect the patient care in the future.

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SYMPTOMS
Bone fracture, especially fracture from slight injury (trauma) Bone pain Occasionally a mass and swelling can be

felt at the tumor site

Some benign tumors have no symptoms
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DIAGNOSTIC MEASURES
Patients personal and family, medical history. Physical examination and Laboratory and other diagnostic tests These tests may include the following X-rays A bone scan A CT or CAT

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 MRI PET scan An angiogram Biopsy Blood tests e.g : alkaline phosphatase.

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TREATMENT

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Surgery Chemotherapy Radiation therapy Cryosurgery

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GOAL: to provide the adolescent with a functional extremity. May involve amputation of the affected limp or limb salvage procedure Resection surgery is performed unless there is extensive involvement of soft tissue or neuromuscular muscle The primary bone tumor is removed & the diseased bone is replaced with an internal prosthesis.
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Involves metaphyseal ends of long bones like femur,humerus, tibia ,pelvis, jaw, phalanges. It may also occur in other locations

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 The metaphysis is the wider portion of a long bone adjacent to the epiphyseal plate- are responsible

for growth in the length of the bone. Has rich blood supply It is this part of the bone that grows during childhood; as it

grows, it ossifies near the diaphysis and the epiphyses.

PATHOPHYSIOLOGY
Etiological factors Tumor from bone producing mesenchymal cell Tumor extends beyond the bone & into a soft tissue mass that encircle the bone Tumor cells may spread into the blood stream & produce metastasis

CLINICAL MANIFESTATIONS
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Pain at the site of tumor Localised swelling and warmth Limitation of movements, limp Tenderness

Erythema
Metastasis
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DIAGNOSIS
Suspected : unexplained bone pain in association with a palpable mass. X-rays CT scan - the magnitude of the medullary involvement CT scan of chest - metastases

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TREATMENT
Optimal Treatment Surgery Radical surgical resection Amputation Chemotherapy: in combination or singly before and after surgery -high dose methotrexate with citrovorum rescue, adriamycin, actinomycin D, cyclophosphamide, cisplatin etc

PROGNOSIS

50-60% survival

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EWING SARCOMA/ PRIMITIVE NEURO ECTODERMAL TUMOR

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Ewing tumor characteristically occur in childhood and adolescents [4-20 yrs] Involving either long bones of extremities (most often femur) or flat bones of head and trunk- femur, tibia, ulna, humerus, vertebrae, pelvis, scapula, ribs, skull
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MANIFESTATIONS
Painful swelling with constitutional symptoms like fever and malaise

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DIAGNOSIS
X ray showing mottled, lytic, onion skin pattern must be confirmed by surgical bone biopsy showing round cell tumor.

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-CT scan to define the magnitude of the tumor

-Chest X ray -CT scan and radio nuclide bone scan -Bone marrow biopsy
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TREATMENT OF CHOICE: High dose irradiation of the primary tumor site & combination of chemotherapy employing vincristine, cyclophosphamide, dactinomycin and doxorubicin.

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PROGNOSIS
With metastasis , survival is 5-15%. Without metastasis it is around 60%.