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Chapter 9

This document provides information on various bone tumors. It discusses the epidemiology, classification, staging, clinical presentation, investigations, differential diagnosis, management principles, and details on specific benign and malignant bone lesions. Key points include that bone sarcomas are rare, most arise in long bones, grading systems exist, and treatment involves biopsy, imaging, surgery, with or without chemotherapy/radiation depending on tumor type and stage.

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0% found this document useful (0 votes)
92 views62 pages

Chapter 9

This document provides information on various bone tumors. It discusses the epidemiology, classification, staging, clinical presentation, investigations, differential diagnosis, management principles, and details on specific benign and malignant bone lesions. Key points include that bone sarcomas are rare, most arise in long bones, grading systems exist, and treatment involves biopsy, imaging, surgery, with or without chemotherapy/radiation depending on tumor type and stage.

Uploaded by

Francisco Gani
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PDF, TXT or read online on Scribd
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Book Reading

dr. Francisco Gilbert Timothy


dr. Adam Moeljono, Sp.OT (K) Spine
Section 1
General Orthopaedics
Chapter 9
Tumours

2
Epidemiology

• <1% of all new cancer diagnosis


• Bone sarcoma incidence 8.2 cases per million of
population, while soft tissue sarcomas incidence 45 cases
per million of population.
• >60% of primary bone tumour will arise from long bones
of lower limb, particularly around the knee

3
Classification

• Based on principle cell type


• Histological grade of soft tissue sarcomas classified
according to Federation Nationale des Centres de Lutte
Contre le Cancer (FNCLCC) system

4
• Benign
• Intermediate (locally aggressive; rarely metastasizing)
• Malignant

5
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

6
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

7
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
8
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

9
Bone tumour mimics

• Soft tissue haematoma


• History and rapid onset of
painful lump
• Myositis ossificans
• Tender swelling following an Myositis ossificans
injury
• Stress fracture
• Diagnostic minefield
• Tendon avulsion injuries
• Infection
• Painful swelling in metaphysis
of long bone

Infection
10
• Gout
• Large gouty tophus
• Osteopetrosis
• Sclerotic bone
• Osteopoikilosis
• Multiple bone island
• Melorheostosis
• Dripping candle wax
appearance

Melorheostosis

11
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

12
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.

Locally advanced disease


• Unresectable tumour and based on location, extent of
tumour, capabilities of the team and patient’s wishes
• Upper limb vs lower limb

13
Benign lesion of bone

Osteochondroma • Grow with skeletal maturity


• Aberrant subperiosteal nest • Treatment
of physeal cartilage • Excision
• Clinical manifestation
• Long bone (femur, humerus)
• Painless mass, secondary
symptoms
• Imaging
• Bony protuberance with well-
defined limits, thin outer
cortex and inner cancellous
structure
• Pedunculated vs sessile base
• MRI: cartilaginous cap

14
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
15
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

16
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
17
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

18
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

19
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
20
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

21
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

22
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

23
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

24
• 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

25
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

26
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

27
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
28
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

29
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

30
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
31
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

32
• 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

33
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
34
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

35
• 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

36
• 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

37
• 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

38
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

39
• Histology
• Primitive, undifferentiated, small, round blue cells
• FISH
• Chromosome 11 and 22 translocation→EWSR1-FL11
• Treatment
• Chemotherapy: vincristine, ifosfamide, doxorubicin, etoposide
• Surgical wide excision

40
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

41
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

42
• 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

43
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
44
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

45
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

46
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
47
Metastatic bone disease

• Original site to one or more • Imaging


sites elsewhere via blood • X-ray, MRI, bone scintigraphy, CT
vessels or lymphatics scan
• Osteosclerotic, osteolytic, mixed
• Source
• Breast, lung, prostate, renal,
thyroid, adrenal
• Clinical manifestation
• Spine, proximal femur, pelvis,
proximal humerus, femur,
scapula, distal femur, ribs
• Pain, swelling, pathological
fracture, spinal cord
compression, hypercalcaemia

48
• 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

49
Soft tissue tumours

• Radiation exposure or malignant transformation


• Clinical manifestation
• Lower limbs
• Painful or painless swelling that grows insidiously, regional
lymphadenopathy, distal neurovascular deficit, localized skin
changes
• Raised concern if >5 cm, painful, deep to fascia, increasing in size,
recurrence after previously excised
• Imaging
• Plain X-ray: exclude bone lesion and assess bone invasion
• MRI: extent of the lesion and local invasion of critical structure,
characterize lesion prior to biopsy, define margins prior to surgery
• CT scan, PET-CT

50
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

51
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
52
Fibrous tumour

Superficial lesions Desmoid-type fibromatosis


• Dupuytren, Ledderhose, • Locally aggressive
Peyronie, Garrod’s pad myofibroblastic disorder
• Myofibroblast proliferation • Clinical manifestation
• Clinical manifestation • Abdominal wall, chest wall,
head, neck, mesentery,
• Palmar and plantar appendicular skeleton
fibromatosis causing firm and
painless nodules • Pain, paraesthesia, deformity

• Treatment • Imaging
• Collagenase injection, • CT and MRI
dermofasciectomy • Treatment
• NSAID, hormone therapy,
radiotherapy, chemotherapy,
surgery

53
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
54
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
55
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

56
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

57
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

58
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

59
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

60
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

61
62

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