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Benign and malignant Bone tumors

Benign and malignant Bone tumors. Bone tumors . Majority of Bone tumors are due to metastasis from other sites Most tumors have a predilection for a particular site in the body Causes: Idiopathic, bone infarcts (rare), chronic osteomyelitis , Paget disease, radiation, etc.

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Benign and malignant Bone tumors

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  1. Benign and malignant Bone tumors

  2. Bone tumors • Majority of Bone tumors are due to metastasis from other sites • Most tumors have a predilection for a particular site in the body • Causes: Idiopathic, bone infarcts (rare), chronic osteomyelitis, Paget disease, radiation, etc.

  3. Benign Bone tumors

  4. Osteoma • Benign growths that involve the skull and facial bones • They may present singly or in multiples • Gardner syndrome is associated with facial osteomas ,multiple colonic adenomas and dermoid cysts • They may clinically present with a mass lesion that extends into the nasal cavity • There is no malignant association

  5. Osteoidosteoma • Growth of this tumor involves the diaphysis of long bone • The lesions are usually less than 2cm. • There is cortical involvement • Signs/Symptoms: • Pain, characteristically more intense at night, relieved by NSAIDs and eliminated by excision • Vertebral lesions may cause scoliosis • Treatment: local excision

  6. OsteoidOsteoma • Randomly oriented interconnected trabeculae of woven bone surrounded by a rim of osteoblas

  7. Osteoblastoma • Similar to osteioidosteoma ; however it is larger than osteoidosteoma (>2cm) • Has a predilection for the vertebral column • Note: this tumor is not relieved by NSAIDs • Signs/Symptoms: • Pain • Gait disturbances • Treatment: local excision

  8. Osteochondroma • These are relatively common slow growing, benign bone tumors • Probably not a true neoplasm, but rather a tumor produced by growth of aberrant foci of cartilage on the surface of bone • Autosomal dominant disorder of multiple herediatryexostosiswith risk of malignant transformation to chondrosarcoma

  9. Enchondroma • These are benign tumors of hyaline cartilage that arise in the medulla (within the bone) • These are typically solitary lesions that arise in the metaphysealregion of the long bones • Enchondroma is the most common tumor of the bones of the hand

  10. Enchondroma • 2 syndromes characterized by multiple enchondromas: • Ollier’s disease • Multiple enchondromas, usually unilateral • Maffucci’s syndrome • Multiple enchondromas associated with soft tissue hemangiomas , ovarian carcinoma and brain gliomas • Both disorders have 25% risk of malignant transformation to chondrosarcoma;

  11. Enchondroma • Signs/Symptoms: • Usually asymptomatic lesions; pain with pathologic fracture • X-Rays shows O Ring - sign

  12. Enchondroma-O ring sign

  13. Giant cell tumor • This is also known as osteoclastoma • It is relatively benign and uncommon; however, it is very aggressive • A majority of these tumors arise from the epiphyses of long bones • It is an expanding lytic lesion surrounded by a thin rim of bone

  14. Morphology: Giant osteoclasts

  15. “Soap Bubble Appearance”

  16. Giant cell tumor • They are usually present around the knee • The tumors are large and red-brown with frequent cystic degeneration • Signs/Symptoms: • Pain, loss of mobility, fracture • Treatment: curettage – surgery

  17. Fibrous Dysplasia • McCune Albright syndrome • Jaffe syndrome • Monostotic • Ground glass radiographic appearance

  18. Fibrous Dysplasia • Benign tumor • Localized developmental arrest; • All of the components of normal bone are present, but they do not differentiate into their mature structures. • Three clinical patterns: • Involvement of a single bone (monostotic); • Involvement of multiple bones (polyostotic); • Mccune-Albright syndrome -Polyostoticdisease, associated with café-au-lait skin pigmentations and endocrine abnormalities, especially precocious puberty.

  19. Fibrous Dysplasia • Somatic gain-of-function mutation occurring during embryogenesis in the GNASgene • The mutation leads to-the production of a hyperactive guanyl nucleotide binding protein, encoded by the GNAS gene, that drives abnormal growth

  20. Fibrous Dysplasia • Morphology: Well circumscribed intramedullary lesions that sometimes distort the bone. • Bone lesions are usually unilateral.skin lesions are seen on the same sides and affects ;The neck,back ,chest, shoulder and pelvis. • Micro :Curvilinear trabeculae of wooven bone that characteristically resembles Chinese letters. Osteoblastic components are mild and there may be cellular proliferation of fibroblasts.

  21. Chinese letters

  22. Malignant Bone tumors

  23. Bone tumors

  24. Chondrosarcoma • These tumors can produce neoplastic cartilage • The tumor is made of atypical chondrocytes and chondroblasts with multiple nuclei • Pelvis, Shoulder and ribs • Signs/Symptoms: • Local swelling and pain in the enlarging mass • Treatment : wide local excision ; if aggressive chemotherapy is added

  25. Anaplasticchondrocytes

  26. Osteosarcoma • This is also known as osteogenic sarcoma • This is the most common primary malignant tumor of bone • These tumors mainly arise from the metaphysesof the long bones, especially around the knee • Genetic mutations of the RB gene, p53, cyclin dependant cyclases .

  27. Commonest location is around the knee joint.

  28. Osteosarcoma • Malignant cells, produce osteoid • Most tumors arise de novo, though others arise in the setting of: • Paget’s disease • Previous radiation • Previous chemo (especially alkylating agents) • Chronic osteomyelitis

  29. Osteosarcoma • The tumor frequently breaks through the cortex and lifts the periosteum, resulting in reactive periosteal bone formation • Secondary sarcoma occurs in elderly patients, especially associated with Paget disease, radiation,and chronic osteomyelitis these are highly aggressive and unresponsive to treatment

  30. Osteosarcoma • Bilateral soft tissue masses, periosteal and cortical infiltration.

  31. Osteosarcoma • Signs/Symptoms: • Pain and swelling in the enlarging mass • Pathologic fracture may be the first presenting symptom • X-Ray:A triangular shadow on x-ray between the cortex and raised periosteum is known asCodman’s triangle • Treatment: chemotherapy and surgery

  32. Osteosarcoma • Mitotic figures lace like pattern of neoplastic bone.

  33. Codman’s Triangle

  34. Ewing’s sarcoma • Ewing sarcoma and primitive neuroectodermal tumors (PNETs) are primary malignant small round-cell tumors of bone and soft tissue • M>F,age 10-15 years. • Diaphysis of long bones • These two tumors only differ in the degree of differentiation Ewing’s sarcoma is undifferentiated and PNET is differentiated

  35. Ewing’s sarcoma • Genetics plays a role patients with Ewing tumor several transocations.t (11;22) (q24;q12) is one of the most frequent. • M/E: the tumor is composed of sheets of uniform small, round cells ;Arrangement of tumour cell around a central fibrillary space is known as Homer Wright pseudorosettes

  36. Ewing’s sarcoma • Signs/Symptoms: • May simulate osteomyelitis as patients often present with pain, fever and leukocytosis • Xrays may show periosteal reaction  “onion skin” appearance • Treatment: chemotherapy , surgery, and radiation

  37. Small blue cells

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