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CNSLF Bone Path 1&2 (v) - Bone tumours 1 (Malignant (Primary (Myeloma,…
CNSLF Bone Path 1&2 (v) - Bone tumours 1
Benign
more common in children + YAs
Malignant
Primary
accurate dx depends on clinical, radiologic + pathologic exam of lesion
many characteristically involve certain part of bone
more common in older individuals
Myeloma
primary malignant prolif of plasma cells in BM
solitary plasmacytoma or multiple myeloma
secondary (mets)
carc (breast, prostate, bronchogenic, RCC) > sarc
osteolytic - direct erosion of bone, stim of osteoclasts by cks
osteosclerotic (osteoblastic) e.g. prostatic carc
comps
pain
pathologic fracture
BM replacement - pancytopenia
hypercalcaemia (ca released into bloodstream)
N + cord compression
most common in adults = MM + mets
Bone forming
benign
osteoma
often craniofacial
probably hamartomatous or reactive growth + not a true neoplasm
Garner syndrome (FAP subtype) a/w GI polyps + multiple osteomas
osteoid-osteoma
young age (2nd decade)
M:F = 3:1
painful due to presence of high intra-lesional PG levels - relieved by aspirin
small (<2cm)
in cortex of femur or tibia - diaphysis
nidus (focus) consists of vasc spaces surround by sclerotic bone
tx = complete resection of nidus
osteoblastoma
benign but locally aggressive - a/w local recurrence
young age
most common in vertebral column
over 2cm
min or no sclerotic reaction
pain not relieved by aspirin
Tx = curettage (use of a curette to remove tissue by scraping or scooping)/resection
malignant type = osteosarcoma
bimodal age distribution: <20, 2nd peak in elderly
end of long bones (classically around knee)
most arise in metaphysis of a long bone
can be sporadic, genetic (deletions + mutations in cell cycle regulators incl Rb, P16 + P53) or secondary to radiation or Paget's
tenderness +/or pain of affected region with/without palpable mass
fractures
distant mets (lung)
spread: within medullary cavity, through periosteum, across epiphyseal plate or haematogenous
Codman's triangle = triangular area of new subperiosteal bone growth that is created when a lesion (often a tumour) raises the periosteum away from the bone
Sunday speculation: type of periosteal reaction secondary to aggressive periostitis, occurs when a lesion grows too fast + periosteum doesn't have time to lay down a new layer - instead Sharpey's fibres stretch out perpendicular to bone (also occurs in other aggressive bony tumours e.g. Ewing sarcoma or mets
morphologically composed of malignant mesenchymal cells that produce osteoid often in an irregular lace like pattern
tx: neoadjuvant (pre-op chemo), then surgical resection
poor prognosis - early mets
disease free 5yr survival = 60%
Cart forming
benign
osteochondroma
most common benign tumour
tumour of bone with an overlying cart cap
< 20y/os
multiple (osteochrondromatosis - hereditary) or single (sporadic)
mushroom-shaped bony projections from lat aspects of long bones
arise from metaphysis
chondrosarcomas can occasionally arise in osteochondromas (malignant transformation - higher risk with osteochrondromatosis)
(en)chondroma
benign tumours of cart
any age (20-50yrs)
arise in medulla of small bones of hands + feet
single
multiple sites
Ollier's disease (usually 1 side of body)
Maffucci's syndrome (haemangiomas of soft tissue - increased risk for chrondrosarcoma)
well-circumscribed mature hyaline cart
chondroblastoma
chondromyxoid fibroma
malignant type = chondrosarcoma
2nd most common malignant tumour of bone
1/2 as frequent as osteosarcoma
de novo or from previous benign tumour
middle age > 40
axial skeleton (pelvis, shoulder girdles, ribs, spine)
morphologically composed of lobules of cart with chondrocytes in lacunae + focal ossification + calcification
can be quite aggressive + erode though cortical bone to involve soft tissues
not sensitive to chemotherapeutic agents + tx is usually restricted to surgical resection
poor prognosis - 5yr survival = 40%