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Bone Tumours
Osteoid osteoma & Osteoblastoma
Immature skeleton (Growth plate open) Benign osteoblastic lesions of bone
Identical histological features but differ in size, sites of origin, symptoms
Gross
Well circumscribed, roun d, hemorrhagic gritty tan tissue
HPE
Random interconne cting woven bone with prominent osteoblastic rimming
Loose vascular stroma
10-20 y/o – osteoblastoma has wider age distribution
(commonly in young adult)
Male ↑
Osteoid osteoma Osteoblastoma
Symptoms Localized pain Localized pain, dull
Worse at night No pattern
Relieved by aspirin
Sites Femur, tibia, spine Spine, femur, jaw
Size < 1cm > 2 cm
X-ray Intracortical nidus Well-circums cribed
Sclerotic rim of bone Expansile lesion
reaction Variable ossification
May be aggressive with
cortical destruction & soft
tissue extension
Mature skeleton (Growth plate closed)
Woven bone/ Yes Yes
osteoid

Osteoid osteoma Osteoblastoma

Round red nidus (left)
Cortical bone (right)

Osteoid osteoma Osteoblastoma

Anastomosing trabeculae Anastomosing trabeculae
Osteoma of woven bone of woven bone
Clinical Prominent Yes Yes
Exophytic mass from skull & paranasal sinuses osteoblastic
30-50 y/o rimming
Asymptomatic (usually) Hyaline No Sometimes
May present with sinus obstruction, extracranial extension, nerve compression cartilage (may mimic osteosarcoma)
Multiple lesions in Gardner syndrome Nerve fibers in Yes No
• Colonic polyposis reactive zone
• Pelvic fibromatosis Treatment Complete resection & Enbloc resection +/- bone
• Epidermal inclusion cysts removal of nidus in its grafting
Sites entirely +/- bone grafting
Frontal sinus, ethmoid sinus, sphenoid sinus Recurrence Rare Rare
Radiological Malignant No < 1%
Sharply demarcated mass protruding bone surface transformation
No bone destruction/ periosteal reaction
Parosteal osteoma may mimic parosteal osteosarcoma
Pathologic
Compact lamellar cortical bone
Small amount of fibrofatty stroma (CT parosteal osteosarcoma)
Prognosis & Therapy
Simple excision
Malignant transformation does not occur
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Osteosarcoma
Malignant mesenchymal tumour (cancerous cells produce bone matrix)
Bimodal age distribution
75% in age < 20 y/o
In elderly – Paget’s disease, Bone infarct, Prior irradiation
Male ↑
Arise from metaphysic (greatest bone growth)
Knee (60%), Hip (15%), Shoulder (10% ), Jaw (8%)
In age > 25 y/o – incidence of flat bones & long bones equal Osteoblastic oste osarcoma Osteoblastic oste osarcoma
Permeating pre-existing trabeculae of Finely ramifying matrix between
Pathogenesis
Rb gene mutation – hereditary retinoblastoma bone tumour cells
Abnormalities in p53, CD4, p16, INK4A, cyclin D1, MDM2
Pathologic subtypes
Intramedullary osteosarcoma
• Conventional
• Telangiectatic
• Small-cell
• Low-grade intraosseous
Surface (juxtacortical) osteosarcoma
Chondr oblastic osteosarcoma
• Parosteal
Lobules of malignant-appearing
• Periosteal cartilage
• High-grade surface Atypical spindle cells
Clinical subtypes
Osteoid production
Extraskeletal osteosarcoma
Osteosarcoma of jawbones
2° osteosarcoma – Postradiation, Paget’s disease, Fibrous dysplasia

Conventional Osteosarcoma
Clinicals
80% - 90% of all osteosarcomas
Pain of short duration, soft tissue swelling
Pathological fracture uncommon
Elevated alkaline phosphatase – relatively non-specific Telangiectatic osteosarcoma Telangiectatic osteosarcoma
Radiologic Spaces containing blood separated by Spaces containing blood separated by
Permeative lesions, metaphyseal centered septa septa
Periosteal reaction, Codman triangle Cells appear malignant Cells appear malignant
Soft tissue extension Nuclear pleomorphism Nuclear pleomorphism
Mineralization – from purely lytic to mixed to densly sclerotic
Gross
Intramedullary fleshy tumour
Destruction of cortex & soft tissue involvement
HPE
↑ grade sarcomatous cells
Abnormal mitosis & necrosis
Osteoid (deposition by malignant cells)
Stroma – osteoblastic, chondroblastic, fibroblastic Central low-grade osteosarcoma
Sclerosing osteoblastic variant – minimal pleomorphism (che ck permeation & Extension into soft-tissue posteriorly
lamellar bone entrapment)
Osteoclastic-type giant cells (randomly distributed)
Treatment
Multidrug preoperation chemotherapy
Surgical resection – limb salvage, amputation
Adverse prognosis
< 95% necrosis after pre-op chemotherapy
• Grade 1 – no necrosis
Low-grade osteosarcoma (↑ power)
• Grade 2 – focal necrosis Low-grade osteosarcoma Stroma is hypocellular
o A - >50% viable tumour (<50% necrosis) Invading surrounding skeletal muscle Slight atypia of spindle cells
o B - <40% viable tumour (>50% necrosis)
• Grade 3 – rare foci of viable tumour, <5% (>95% necrosis)
• Grade 4 – no viable tumour
Advanced stage
Proximal anatomical location

Parosteal osteosarcoma Parosteal osteosarcoma (↑ power)

Heavily mineralized mass attached to Spindle cells – minimal atypia
posterior aspect of distal femoral Bone arise directly from spindle cells
metaphysic

Conventional oste osarcoma Conventional oste osarcoma

(Distal femoral metaphysis) Epiphyseal plate acts as relative
Lesion is very dense barrier Periosteal osteosarcoma Periosteal osteosarcoma
New bone formation in soft tissues (Proximal tibia) Lobules of cartilage with peripheral
Sunburst pattern Lesion lightly mineralised on surface spindling
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Osteochondroma Enchondroma
Benign bony projection from b one surface & remains in continuity with bone of Benign hyaline cartilage neoplasm
origin – exostosis Arise within medullary canal of bone
Solitary Solitary
Multiple – familial – osteochondromatosis Multiple – enchondromatosis (rare)
10-20 y/o 10-30 y/o
Male ↑ Sites
Asymptomatic Hands, Feet, Proximal humerus, Distal femur
Clinical Clinical
Localized pain, Distal nerve impingement, Limitation of movement Soft tissue swelling, Pain, Pathologic fracture
Sites Radiology
Distal femur, Proximal humerus, Proximal tibia Well-marginated at metaphyseal/ diaphyseal region (large bone) with punctuate
Radiology pattern
Pedunculated/ sessile bony projection Expansile, lytic lesion (small bone)
Cartilage cap – lobulated & variably calcified Cortical erosion & thinning
Remodeling defe cts of bone in osteochondromatosis Pathology
Pathologic Lobules of bland -appearing hyaline cartilage
Medullary bone with marrow fat Partially surrounded by lamellar bone
Cartilaginous cap < 1cm Treatment
Treatment Simple curettage
Complete surgical resection
Local recurrence rare
Malignant transformation
Solitary – 1-2%
Multiple – 5-25%

Enchondroma (Proximal Tibia)

Uniform mineralization
Osteochondroma (Femur ) Lesion is circums cribed
Medullary cavity of lesion is continous Does not invade cortex
with medullary cavity of bone
Cortices are similarly continuous

Endochondroma (Low power)(Fibula) Endochondroma (High-

Sharp cortical boundary without power)(Femur)
evidence of permeation Tumor is hypocellular
Nuclei are small
Osteochondroma Osteochondroma
Sessile osteochondroma of distal Cartilage cap mature into underlying
femur cancellous bone
Smooth, pale, blue cartilage cap Orderly arrangement of cartilage cells
Chondr osarcomatous change in Osteochondroma

Clinical & Radiology

Localized pain
Recent growth in adult patient
Irregular thickened cartilage cap with lucent areas

Pathology
Periosteal chondroma
Cartilage cap > 2cm Periosteal chondroma Hypocellular
Invasive nodules of cartilage Lesion on surfa ce of bone Large, Atypical chondrocytes
Myxoid stromal change Well circumscribed, small
Necrosis
Loss of single file arrangement at ossification zone
Hypercellularity & atypia

Ollier disease
Cartilaginous masses involve both
ends of bone in each femur
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Chondr oblastoma Chondr omyxoid Fibroma
Rare Rarest (among cartilage tumours)
10-20 y/o 10-30 y/o
Majority arises around knee Sites
Pelvis & rib in older patients Metaphysis of long bone
Predilection for epiphyses & apophyses Symptoms
Symptoms Localized, Dull aching pain
Painful, Joint effusion, Restricted joint mobility Radiology
Radiology Eccentric geographic lucency with rim of sclerosis
Well-defined geographic lucen cy Expand overlying cortex
Spotty calcification Pathology
Pathology Nodules of poorly formed hyaline cartilage, myxoid tissue
Sheets of polyhedral chondroblasts Separated by fibrous septae
Surrounded by scanty hyaline matrix Greatest cellularity at periphery of nodules
Frequent mitosis & necrosis Foci of calcification
Scattered osteoclast-type giant cells Scattered osteoclastic-type giant cells
Hemorrhagic cystic degeneration Treatment
Treatment Simple curettage
Surgical resection or curettage Prognosis
Prognosis Good
Distant metastasis rare, usually distal pathological fracture or repeated Does not transform to malignancy even if recur
curettage

Chondr oblastoma (Distal femur)

Lesion extends to articular cartilage
Well circumscribed Chondr omyxoid Fibroma
Sclerotic rim Well-circums cribed lytic defect of
proximal tibia

Chondr oblastoma Chondr oblastoma

Eosinophilic chondroid matrix Mononuclear cells
Giant cells Cytoplasmic borders Chondr omyxoid Fibroma Chondr omyxoid Fibroma
Mononuclear cells Calcification deposits between cells Irregularly shaped hypocellular center Lobules contain tumour cells
Surrounded by cellular spindle cell Small nuclei, eosinophilic cytoplasmic
stroma extensions within myxoid background
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Chondr osarcoma
Malignant neoplasm of hyaline cartilage
Primary
Secondary
Enchondroma, Osteochondroma, Ch ondroblastoma, Fibrous dysplasia, Paget
disease
40-60 y/o
Male ↑
Sites
Pelvis, Proximal femur, Proximal humerus
Symptoms
Pain at site of involvement Grade 2 Chondrosarcoma (↑ power) Myxoid Chondrosarcoma
Radiology Necrosis (right) (Medium power)
Bone expansion with cortical erosion Nuclei are crowded & Myxoid substance occupied by thin
Variably calcified with poor magination hyperchromatic cords or strands of cells radiating
Periosteal reaction (20%) toward periphery
Soft tissue extension (50%) Spokes of a wheel
Pathology
Malignant hyaline cartilage
Grade 1-3
S100 protein +ve
Variants
Periosteal
Clear cell
Mesenchymal
Dedifferentiated
Treatment
Wide surgical resection Clear cell chondr osarcoma
Prognosis Round nu clei
Clear cell chondr osarcoma
↑ grade associated with local recurrences & distant metastasis Clear cytoplasm
(Proximal hu merus)
10% develop dedifferentiated chondrosarcoma Well-defined boundaries
Lesion is heavily mineralized
Extends to end of bone
Well-circums cribed

Grade 1 Chondrosarcoma Grade 1 Chondrosarcoma

(Femoral shaft) Mesenchymal chondrosarcoma
Erosion/ marked thickened cortices With cartilage
Small blue cells

Chondr osarcoma (Acetabulum)
Myxoid quality of matrix
Focal cystic change
Chondr osarcoma
(arising in osteochondroma )
Lesion has central necrosis
Cystic change
Dedifferentiated Chondrosarcoma
(Proximal fe mur)
Dedifferentiated Chondrosarcoma Associated with pathologic fracture
(Proximal hu merus) Medullary portion – chondrosarcoma
Mineralization is consistent with Soft-tissue mass medially – fleshy
cartilage, aggressive-looking area appearance of high-grade sarcoma
located medially

Grade 1 Chondrosarcoma Grade 1 Chondrosarcoma (↑ power)

Permeating pre-existing trabecular Nucleated cells
bone, filling marrow spaces Moderate atypia

Dedifferentiated Chondrosarcoma
Chondrosarcoma (left) is juxtaposed
with high-grade malignant fibrous
histiocytoma (right)
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Fibrous Cortical Defect Giant Cell Tumour
Common in children > 2 y/o Osteoclastoma
Developmental defect (rather than neoplasm) Benign but locally aggressive
Small site (about 0.5 cm) Giant cells have a monocyte-macrop hage lineage
May grow to 5-6cm in adolescence = non ossifying fibroma 20-50 y/o
Sites Adult – involve both epiphysis & metaphysic
Metaphysis of distal femur Adolescent – Confined to metaphysic
Proximal femur Sites
50% are bilateral or multiple Distal femur
Radiology Proximal tibia
Eccenteric lobaulated radiolucency Radiology
Sclerotic margin Purely lytic & eccentric with thin shell of reactive bone
Pathology Erode into subchondral bone plate
Cellular lesions of fibroblasts & histiocytes Pathology
Fibroblasts form storiform pattern Large, red brown & cystic degeneration
Histiocytes – multinucleated giant cells/ foam cells Uniform oval monon uclear cells with indistinct cytoplasm
Prognosis Osteoclastic giant cells
Spontaneous resolutaion Treatment & Prognosis
Nonossifying fibroma – pathological fracture Biologic behaviour unpredictable
Curettage is associated with 40-60% recurrence rate
Metastatic lesions have same morphology as 1°
Sarcomatous change is rare

Metaphyseal Fibrous Defect

Spindle cells
Storiform pattern
Metaphyseal Fibrous Defect Scattered benign giant cells Giant Cell Tumour Giant Cell Tumour
(Distal tibia) (Proximal hu merus) (Distal femur)
Sclerotic margin
Lesion is lytic Lesion extends to end of bone
Scalloped appearance
Appears aggressive

Fibrous Dysplasia
Localized developmental arrest
Monostotic fibrous dysplasia (70%)
• Adolescent
• Ribs, Femur, Tibia, Jawbones, Calvaria, Humerus
• Doesn’t evolve into polyostotic form
Polyostotic fibrous dysplasia without endocrine dysfunction (27%)
• Femur, Skull, Tibia, Humerus, Ribs, Fibula, Radius, Ulna, Mandible,
Vertebrae
• If Shoulder & pelvic girdle – crippling & fracture
McCune-Albright syndrome (3%)
• Mutation in G-protein – excessive production of cAMP Giant Cell Tumour Giant Cell Tumour
• Sexual precocity, hyperthyroidism, pituitary adenoma, primary Nuclei of giant cells similar to Nuclei of giant cells similar to
adrenal hyperplasia mononu clear cells mononu clear cells
• Cafe au lait skin pigmentation
Radiology
Ground glass appearance
Well-defined margins
Pathology
Curvilinear trabeculae of woven bone
Moderate cellular fibroblastic proliferation
Nodules of hyaline cartilage (20%)
Cystic degeneration, hemorrhage, foamy cells
Treatment & Prognosis
Conservative surgery for fracture
Polyostotic type may undergone malignant transformation if irradiated

Fibrous dysplasia (Proximal tibia) Fibrous dysplasia

Lesion well demarcated Spindle cell proliferation
Sclerotic rim Metaplastic bone formation
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Ewing Sarcoma Metastatic Tumour
1° malignant small round cell tumours of bone Most common skeletal malignancy
2nd most common in children after osteosarcoma Pathways of spread
10-15 y/o Direct extension
Sites Lymphatic or hematogenous dissemination
Diaphyses of long bone (fe mur) Intraspinal seeding
Flat bones of pelvis Origin (75%)
Clinical Prostate, Breast, Kidney, Lung (adult)
Painful enlarging masses, warm, swollen Neuroblastoma, Wilm tumour, Osteosarcoma, Ewing & RMS (children)
Fever, ↑ ESR, anemia, leukocytosis Typically multifocal (but those from kidney & thyroid are solitary lesions)
Radiology Sites
Destructive lytic tumour with permeative margins, extending into soft tissue Axial sekelton (vertebra, pelvis, rib, skull, sternum)
Marked periosteal reaction – layers of reactive bone (onion-skin appearance ) Proximal femur
Proximal humerus
Type of reactions
Majority induces mixed lytic & blastic reaction
Lytic reaction by carcinoma of kidney, lung, GIT, melanoma
Blastic reaction by carcinoma of prostate

Ewing Tumour
Ewing Tumour
Destructive lesion with periosteal new (Femur)(Pathologic fracture)
Tumour is soft, fleshy
bone formation
Onion skin appearance

Ewing Tumour (↑ power view)

(large cell tumour)
Ewing Tumour (↑ power) Nuclei are more pleomorphic than
Uniform round n uclei classic Ewing tumour
Indistinct cytoplasmic borders