Chapter 9
Chapter 9
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Epidemiology
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Classification
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• Benign
• Intermediate (locally aggressive; rarely metastasizing)
• Malignant
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Staging
Enneking stages
Stage 1A Low-grade, intra-compartmental tumour
Stage 1B Low-grade, extra-compartmental tumour
Stage 2A High-grade, intra-compartmental tumour
Stage 2B High-grade, extra-compartmental tumour
Stage 3 Any of the above with metastases
TNM stages
Stage 1A Low-grade, small, no metastases
Stage 1B Low-grade, large, no metastases
Stage 2A Intermediate or high-grade, small, no metastases
Stage 2B Intermediate-grade, large, no metastases
Stage 3 High-grade, large, no metastases
Stage 4 Any with metastases
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Clinical presentation
• Age
• Every tumour has age predilection
• Pain
• Night pain, pain not responding to simple analgesia, persistent pain
following injury
• Pathological fracture
• Fracture from disproportionate level of injury, certain fracture
pattern
• Swelling and tenderness over the affected bone
• Limitation of joint movement
• Grown into the joint
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Investigations
• X-ray • CT
• Abnormality, location within the • Intraosseous and extraosseous
bone, amount, periphery of the extension
lesion, pathognomonic feature
• MRI
• Radionuclide scanning • Tumor spread, preoperative
• Non-specific reactive changes assessment
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Investigating the suspicious bone
lesion
• X-ray→ CT scan, MRI • Open biopsy
• Blood test, biochemical work • If needle biopsy non
diagnostic or place
• Biopsy neurovascular structure at
• Minimize contamination of risk
normal tissue
• Site included in subsequent
• Taken from representative operation
tissue
• As little as possible exposure
• Image-guided biopsy
• Boundary zone
• Microbiology and histology
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Bone tumour mimics
Infection
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• Gout
• Large gouty tophus
• Osteopetrosis
• Sclerotic bone
• Osteopoikilosis
• Multiple bone island
• Melorheostosis
• Dripping candle wax
appearance
Melorheostosis
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Principles of management of
primary tumours of bone
Principles of surgical treatment and definition of margins
• Intralesional, marginal, wide, radical
• Limb salvage vs amputation
• Reconstruction options
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Neoadjuvant and adjuvant therapies
• Chemotherapy
• Shrink the tumour and treat micrometastatic disease
• Given in combination to avoid chemo-resistance and increase the
rate of tumour necrosis
• High energy radiation
• Destroy radiosensitive tumour or adjuvant therapy or alternative to
amputation.
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Benign lesion of bone
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Enchondroma
• Treatment
• Intramedullary neoplasm, • Curettage
well-differentiated hyaline
cartilage
• Clinical manifestations
• Tubular bones of the hand
• Asymptomatic, pain,
pathological fracture
• Imaging
• Rounded-well defined,
lobulated edges and a thin
rind of reactive sclerosis
• MRI: black signal voids of
internal classification
• Isotope bone scan: hot
• Histology
• Lobules of cartilage with areas
of calcification
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Periosteal chondroma
• Cartilage neoplasm at
surface of the bone
• Clinical manifestation
• Metaphysis of long bone
(humerus)
• Pain, palpable lump
• Imaging
• Superficial erosion of the
bone cortex with occasional
scalloping
• Popcorn matrix with areas of
calcification
• Treatment
• Curettage
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Multiple chondromas and associated
conditions
• Ollier’s disease
• Multiple chondroma, hemisomic
• Bone swellings, deformity, limb length
discrepancy
• Maffucci syndrome
• Multiple chondroma, multiple cutaneous or
deep haemangioma
• Expansion of the bone with cortex thinning
• Transformation to secondary sarcoma
• Increase in size, development of symptoms
• Histology: permeative growth pattern
• X-ray: change in size with new soft tissue
mass
Maffucci syndrome
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Chondromyxoid fibroma
• Lobules of fibromyxoid and chondroid tissue
• Clinical manifestation
• Metaphysis of long bone (proximal tibia), pelvis
• Slow growing lesion with insidious, mild to moderate pain
• Imaging
• Eccentric lytic lesions parallel to long axis of the bone
• Erupts through the cortex with little or no periosteal or soft tissue
reaction
• Histology
• Stellate cells on a myxoid background with cellular atypia
• Treatment
• Intralesional curettage
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Chondroblastoma
• Clinical manifestation
• Epiphysis of long bone
• Pain with occasional joint effusion or
stiffness
• Imaging
• Round or oval lytic lesion with expanded
cortex
• MRI: homogenous high signal
• Histology
• Wet sawdust with areas of chondroid
matrix, calcification and haemorrhage,
• Chicken wire calcification
• Treatment
• Simple curettage with or without bone graft
• Radiofrequency ablation
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Osteoid osteoma • Treatment
• Osteoid and woven bone • Radiofrequency ablation
surrounded by halo of reactive
bone
• Clinical manifestation
• Long bone
• Pain that is worse at night,
relieved with NSAID
• Joint stiffness and effusion
• Imaging
• Dense sclerosis with a small
rounded area of osteolysis
• Isotope bone scan: positive
• CT scan: central nidus
• Histology
• Thin, woven bone with
osteoblastic rimming, osteoclast
and dilated capillaries
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Osteoblastoma
• Osteoblast producing osteoid and woven bone
• Clinical manifestation
• Posterior arch of the spine
• Pain at the site of tumour, nerve root compression
• Imaging
• Central lytic lesion with rind of sclerosis
• Periosteal reaction
• Histology
• Large osteoblasts producing osteoid and woven bone
• No evidence of permeation
• Treatment
• Intralesional curettage
• Radiofrequency ablation
• En bloc resection
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Desmoplastic fibroma of bone • Treatment
• Spindle cell and collagen • En-bloc, curettage
• Clinical manifestation
• Mandible, pelvis, long bone
metadiaphysis
• Lesion grow slowly, pain,
pathological fracture
• Imaging
• Lytic, expansile lesion with thin
shell of new bone
• Bone scan: cold
• MRI: low signal lesion on both
T1 and T2
• Histology
• Hypocellular spindle lesion with
large amounts of collagen and
bland nuclei without atypia
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Non ossifying fibroma
• Fibrous tissue appears within
bone
• Clinical manifestation
• Metaphysis of long bone
• Aysmptomatic, pathological
fracture
• Imaging
• Oval radiolucent area
surrounded by a thin margin of
dense bone
• Adjacent to or within the cortex
• Treatment
• Unnecessary
• Curettage and bone graft
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Giant-cell tumour of bone
• Proliferation of
mononuclear cells with
scattered macrophages
• Clinical manifestation
• Pain, increasing mass,
pathological fracture
• Imaging
• Eccentric, expansile, lobulated
lytic lesion with narrow zone
of transition
• CT: estimation of cortical bone
involvement
• MRI: low signal on T1 and
intermediate or high on T2
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• Histology
• Osteoclast-like giant cells among round or spindle-shaped
mononuclear cells
• Campanacci classification
• Stage 1: completely intraosseous
• Stage 2: cortical erosion without destruction
• Stage 3: cortical destruction with a soft tissue component
• Treatment
• Extended intralesional curettage
• En-bloc resection
• Denosumab
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Haemangioma of bone Epithelioid haemangioma
• Capillary blood vessels • More locally aggressive
• Clinical manifestation • Clinical manifestation
• Vertebral bodies • Long bones, flat bones, vertebra,
• Asymptomatic, pain, tubular bones
neurologic symptoms • Pain
• Imaging • Imaging
• Radiolucent lesion with • Well-defined, lytic,expansile,
coarse trabeculae septated lesion which erodes cortex
• Histology • Histology
• Thin-walled blood-filled
vessels lined by a thin layer of • Epitheloid cells in the center
fat formed into solid sheets, periphery
contain small arteriolar-like vessels
• Treatment lined by epitheloid cells
• Curettage and stabilization
• Treatment
• Curettage, en-bloc, radiotherapy
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Simple bone cyst
• Cystic bone cavity lined by
fibrous membrane and filled
with serous or serosanguinus
fluid
• Clinical manifestation
• Metaphysis of the proximal long
bone
• Asymptomatic, mild pain or
swelling, pathological fracture
• Imaging
• Well-outline, centrally placed
lytic lesion with thinning cortex
• Fallen-leaf sign
• Treatment
• Supportive, percutaneous steroid
injection, curettage and bone
grafting
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Aneurysmal bone cyst
• Blood-filled cystic spaces • Histology
• Clinical manifestation • Blood-filled, cystic spaces
• Metaphysis of long bone, • Treatment
spine • Curettage, debriding the
• Pain and swelling, neurologic cystic wall, radiation
symptoms
• Imaging
• Subperiosteal, poorly defined
osteolytic lesion, elevating
and progressively eroding
cortex
• MRI: cystic features with
multiple intralesional
septations and fluid levels
• Angiography: persistence of
contrast and blush of flow
within the lesion
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Fibrous dysplasia
• Medullary fibro-osseous lesion
• Clinical manifestation
• Monostotic or polyostotic
• Craniofacial bone, femur, pelvis,
tibia
• Asymptomatic, pain, fracture,
deformity
• Imaging
• Well-circumscribed lesion with
ground-glass matrix, shepherd’s
crook deformity
• Histology
• Chinese letter formation
• Treatment
• Deformities correction
• Curettage and bone grafting
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Osteofibrous dysplasia
• Treatment
• Clinical manifestation • Indicated for severe
• Painless, palpable bone lump or progressive deformiy
progressive tibial bowing
• Imaging
• Intracortical osteolysis, soap
bubble appearance, narrow
transition zone, cortical expansion
• Histology
• Irregular fragment of woven bone
rimmed by layers of lamellar bone
• Spindle cells with collagen
production and fibrous or myxoid
matrix
• Absence of keratin-positive
epithelial cells and epithelial
cluster cells
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Langerhans cell histiocytosis • Treatment
• Clonal proliferation of • Systemic anti-inflammatory
pathological Langerhans cells
• Several names
• Clinical manifestations
• Skull, pelvis, femur, mandible
• Pain, swelling, others based on
location
• Imaging
• Lytic, well-demarcated lesion
with thick periosteal new bone
formation
• Hole within a hole, vertebral
plana
• Histology
• Langerhans cell with tennis-
racket intracytoplasmic inclusion
bodies, Birbeck granules
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Primary malignant bone tumours
Osteosarcoma • Codman’s triangle, sunburst
appearance
• High-grade, medullary
osteoid-producing tumour • MRI: medullary and extra-
spreading rapidly outwards osseous extent
through the periosteum
• Primary vs secondary
• Clinical manifestations
• Bimodal distribution
• Metaphyseal or
metadiaphyseal of long bone
• Worsening pain and swelling,
fever, warmth skin and venous
engorgement
• Imaging
• Ill-defined, permeative bone-
forming lesion causing cortical
destruction
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• Histology
• Anaplastic, pleomorphism neoplastic cells
producing primitive woven bone and osteoid and
permeative pattern
• Treatment
• Chemotherapy (doxorubicin, cisplatin, ifosfamide,
methotrexate)
• Limb salvage (wide resection and reconstruction)
• Amputation
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Variants of osteosarcoma
• Rare subtypes
• Telangiectatic, small cell osteosacoma
• Parosteal osteosarcoma
• Low grade sarcoma on the surface of long
bones
• Imaging: dense mass with broad-base
enveloping the bone
• Periosteal osteosarcoma
• Chondroblastic intermediate-grade tumour
arises from the periosteum of long bone
diaphysis
• Painful mass
• Histology: osteoid and chondroid matrix
deposition
• Secondary osteosarcoma
• Paget disease, post-radiation sarcoma
Parosteal osteosarcoma
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Chondrosarcoma
• CT: matrix calcifications and
• Aggressive malignant cartilage permeative destruction
tumours with heterogenous
clinical, radiological and • MRI: low or high grade
histological features
• Clinical manifestation
• Bone derived from enchondral
ossification
• Deep pain, gradually enlarging
mass
• Forms
• Location, primary or secondary,
predominant cell type
• Imaging
• Honeycomb or popcorn
appearance
• Endosteal scalloping of cortex
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• Histology
• Macro: lobular white hyaline
cartilage with areas of
mineralization and cystic
changes, erosion of the
cortex and soft tissue
expansion
• Micro: high cellularity, atypia,
mitoses and permeation into
host bone
• Treatment
• High grade: wide excision
margins
• Low grade: intralesional
curettage, radiofrequency
ablation
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• Secondary central chondrosarcomas
• Ollier’s disease, Maffucci syndrome
• Asymmetric limb shortening, swelling of the fingers and toes,
disturbed movements of the interphalangeal joints, pathological
fractures
• Secondary peripheral chondrosarcomas
• Solitary or multiple osteochondroma
• Cartilage cap thickening more than 1.5 cm
• Periosteal chondrosarcomas
• Periosteum of the metaphysis of femur or humerus
• Pain, swelling
• Imaging: lobular mass adjacent to cortex containing flecks of
calcification, sunray streaks, new bone formation
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• Clear-cell chondrosarcomas
• Epiphyseal location of the proximal or distal femur or humerus
• Pain and joint restriction
• Imaging: osteolytic lesion with narrow sclerotic rim and small matrix
calcification; lack of peritumoural oedema
• Mesenchymal chondrosarcomas
• Highly malignant and poorly differentiated chondrosacromas
• Wide variety of sites
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Ewing sarcoma
• Mesenchymal stem cells in
the bone marrow
• Clinical manifestations
• Diaphysis of long bones, flat
bones
• Pain, swelling, low-grade fever
• Imaging
• Aggressive, permeative,
poorly defined osteolytic
lesion with cortical
destruction
• Onion skin appearance
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• Histology
• Primitive, undifferentiated, small, round blue cells
• FISH
• Chromosome 11 and 22 translocation→EWSR1-FL11
• Treatment
• Chemotherapy: vincristine, ifosfamide, doxorubicin, etoposide
• Surgical wide excision
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Spindle cell sarcomas of the bone
• Collagen producing malignancy
• Clinical manifestation
• Long bones of the lower limb
• Pain, swelling, restriction, pathological fracture
• Imaging
• Poorly defined, lytic lesion without osteoid formation or punctate
calcification and very little periosteal reaction
• Treatment
• Chemotherapy
• Wide excision
• Limb salvage
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Multiple myeloma
• Malignant proliferation of neoplastic plasma cells within the
bone marrow
• Clinical manifestation
• Vertebrae, pelvis, femur
• Bone pain, spinal cord compression, diffuse osteoporosis,
hypercalcaemia, pathological fracture
• Diagnosis
• Plasma or urine electrophoresis
• Bone marrow biopsy
• Evidence of end organ or bone damage
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• Imaging
• Skeletal survey: x-ray of spine, skull, pelvis, ribs/sternum,
humerus/femur: punched out lesion, pepper pot appearance
• CT: fractures, osteolytic lesions, staging
• MRI: extent of marrow infiltration, focal masses
• Treatment
• Alkylating chemotherapy, steroid, immunomodulatory agents
• Autologous stem cell transplantation
• Surgical decompression and stabilization
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Primary lymphoma of bone
• Clinical manifestation
• Metadiaphysis of long bone
• Pain, palpable mass,
pathological fracture
• Imaging
• Permeative, osteolytic lesions
eroding through the cortex
with soft tissue mass, without
a periosteal reaction
• Histology
• Small, round cell tumour of
reticuloendothelial system
• Treatment
• Radiotherapy, chemotherapy,
surgery
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Chordoma
• Primitive embryonic • Treatment
notochordal remnants • Surgical wide resection
along the spinal column • Proton beam and carbon-ion
therapies
• Clinical manifestation
• Sacrum, occiput, vertebra
• Dull pain worse with sitting,
cauda equina, cranial nerve
palsies
• Imaging
• Ill-defined osteolysis of the
sacrum with tumoural
calcifications
• MRI: anatomical level,
relation with rectum and
nerve roots
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Adamantinoma
• Clinical manifestation
• Tibial diaphysis
• Pretibial pain, swelling,
pathological fracture
• Imaging
• Expansion and lobulation
of a lucent cystic lesion
with anterior cortical
disruption
• Treatment
• Complete excision
• Reconstruction
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Malignant vascular tumours of bone
• Haemangioma
• Incidental, metaphysis of bone
• Imaging: well-demarcated
• Locally aggressive epithelioid haemangioma
• Pain at lower limb and spine
• Imaging: well-defined lytic lesions that can erode the cortex
• Treatment: intralesional curettage
• Epithelioid haemangioendothelioma
• Pain and swelling at lower limbs, spine, pelvis
• Imaging: expansile, lytic, invasive tumour eroding the cortex
• Treatment: wide resection
• High-grade angiosarcomas of bone
• Significant pain and swelling at femur or pelvis
• Imaging: aggressive, osteolytic lesion with cortical destruction and
soft tissue mass
• Treatment: wide surgical excision, radiation, chemotherapy
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Metastatic bone disease
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• Biopsy
• No known primary cancer
• Long disease-free interval following previous malignancy
• More than one previous invasive cancer
• Treatment
• Hormonal, biphosphonate, chemotherapy, biologically targeted
therapy, radiotherapy
• Surgery
• Mirels’ score for fracture risk assessment
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Soft tissue tumours
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Lipomatous tumour
Lipoma
• Clinical manifestation
• Superficial vs intramuscular
• Solitary, soft, painless, mobile and slow-growing lesion
• Imaging
• X-ray: radiolucent swelling
• MRI: encapsulated, homogenous, high-signal in T1
• Bone scintigraphy, angiography, contrast: no uptake
• Histology
• Lobulated mature adipocyte with empty cytoplasm
• Treatment
• Excision
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Liposarcoma
• Clinical manifestation
• Deep in thigh, groin, calf, popliteal
fossa and buttocks
• Pain, nerve compression, oedema
• Imaging
• MRI: heterogenous and non
specific appearance of high and
low signal regions on T2, cortical
bone erosion with modest
periosteal reaction
• Bone scintigraphy, angiography:
dense neo-angiogenesis
• Low grade vs high grade
• Treatment
• Wide excision, radical excision
• Radiotherapy and chemotherapy
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Fibrous tumour
• Treatment • Imaging
• Collagenase injection, • CT and MRI
dermofasciectomy • Treatment
• NSAID, hormone therapy,
radiotherapy, chemotherapy,
surgery
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Dermatofibrosarcoma Fibrosarcomas Myxofibrosarcomas
protuberans • Clinical manifestation • Clinical
• Clinical • Thigh, trunk, arm, manifestation
manifestation forearm • Lower limbs,
• Slow enlarging mass upper limbs,
• Trunk, groin, with or without pain
proximal girdles
extremities • Imaging • Subcutaneous>
• Slow growing, • MRI: homogenous, myofascial
low signal tumour that
nodular lesion,
may erode into the • Imaging
cutaneous bone
erythematous • Painful enlarging
plaques • Histology masses with
• Herringbone pattern infiltrative
• Treatment margins
• Wide sugical • Treatment
• Wide • Treatment
excision
excision+adjuvant • Wide excision
radiotherapy, and radiotherapy
chemotherapy
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Synovial tumours
Pigmented villonodular
synovitis (PVNS)
• Synovial joints and tendon
sheaths
• Clinical manifestation
• Articular and extraarticular
• Swelling and/or pain
• Imaging
• Blooming on gradient echo
images
• Treatment
• Surgical excision
• Arthroplasty
• Adjunctive therapies
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Synovial sarcomas
• Do not rise from synovium though histological appearance
may resemble synovium
• Clinical manifestation
• Adjacent to tendons, joint capsule, fascia, muscles
• Pain
• Imaging
• X-ray: multiple small calcifications in tumour peripher
• MRI: heterogeneous, high signal lesion with septations and fluid-
fluid levels
• FISH
• Specific chromosomal translocation t(X:18),(p11:q11)
• Treatment
• Wide excision and radiotherapy, chemotherapy
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Vascular and smooth muscle
tumours
Haemangioma Glomus tumour
• Benign vascular tumour • Mesenchymal perivascular
• Clinical manifestation tumour
• Capillary: reddish patch on • Clinical manifestation
the skin, congenital nevus • Nail beds of fingers or toes
• Cavernous: superficial or • Small, peasized blue nodules
deep causing recurrent pain
• Imaging • Imaging
• Calcified phleboliths in the • Erosion of the underlying
cavernous lesion phalanx bone
• Treatment • Treatment
• Local excision, embolization • Surgical excision
of feeding vessels
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Angiosarcoma Leiomyosarcoma
• Clinical manifestation • Spindle cells originating
• Thigh, calf, arm, trunk from smooth muscle cells
• Enlarging mass in deep • Clinical manifestation
muscles
• Vena cava, iliac, femoral vein:
• Imaging occlusion and limb swelling
• MRI: bunch of grapes • Retroperitoneal: abdominal
appearance mass
• Treatment • Pain, weight loss, nausea,
vomiting
• Wide excision
• Imaging
• Angiograph or Duplex
ultrasound: highly
vascularized lesion
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Nerve sheath tumour
Neuroma Schwannoma
• Overgrowth of fibrous • Benign tumour of nerve
tissue and randomly sheath
sprouting nerve fibrils • Clinical manifestation
following injury to a nerve • Peripheral nerve, spinal nerve
• Clinical manifestation roots
• Tenderness, positive Tinel’s • Pain or paresthesia, small
sign swelling
• Treatment • Imaging
• Epineural sleeve freed and • MRI: target sign
sealed with a synthetic or
buried into muscle or bone
• Treatment
• Careful dissection
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Neurofibroma Malignant peripheral nerve-
• Peripheral nerve sheath sheath tumours (MPNSTs)
• Clinical manifestation • Peripheral nerve or
• Painless nodule neurofibroma
• Imaging • Clinical manifestation
• Erosion of vertebral pedicle • Major nerve of thigh, brachial
or enlargement of the IV plexus, pelvis
foramen • Rapidly enlarging mass with
• MRI: target sign neurological symptoms
• Imaging
• FDG-PET imaging
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Muscle tumours
Rhabdomyosarcoma
• Malignant striated muscle cells
• Clinical manifestations
• Rapid infiltrative growth that bound to bone
• Imaging
• Bone erosion with minimal periosteal reaction
• Treatment
• Neoadjuvant chemotherapy, wide excision including regional lymph
nodes
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