Musculoskelet

al Tumors
Rizal Daulay MD, SpOT. MARS
INTRODUCTION
TUMORIGENESIS :
 SELF-SUFFICIENCY IN GROWTH SIGNALS
 INSENSITIVITY TO GROWTH-INHIBITORY SIGNALS
 EVASION OF PROGRAMMED CELL DEATH
 LIMITLESS REPLICATIVE POTENTIAL
 SUSTAINED ANGIOGENESIS
 TISSUE INVASION AND METASTASIS
Introduction & Incidence:
• Rare - 0.5% of cancer deaths.
• 40% Malignant.
• Primary & Secondary/metastatic.
• Primary in Young. (Osteosarcoma)
• Secondary in the old. (Breast, Kidney, thyroid, lung,
prostate)
• Marrow neoplasms – (hemopoietic) myeloma, leuke
mia, lymphoma etc.
STAGING SYSTEM

• Anatomic extent of the lesion

• Degree of malignancy
• Potential for development of metastatic disease
 The Staging System
Clinical Benefits

• Prognostic factors
• Surgical margin planning
• Guiding for adjunctive treatment
 Musculoskeletal Tumors
The Staging System

The Musculoskeletal Tumor Society (MSTS)

The American Joint Committee on Cancer (AJC)
Memorial Sloan-Kettering Cancer Center
The MSTS Staging System
Factors

• Grade (G)
• Site (T)
• Metastasis (M)
The MSTS Staging System
Grade

• G0 = Benign
• G1 = Low-grade Malignant
• G2 = High-grade Malignant
 The MSTS Staging System
Site

• T0 = Intracompartment (true capsule)

• T1 = Intracompartment (no true capsule)
• T2 = Extracompartment
The MSTS Staging System
Metastasis

• M0 = Absence
• M1 = Presence

Organ, Lymph node or Skip lesion

The MSTS Staging System
Benign

Stage 1
Latent Benign (G0, T0, M0)
The MSTS Staging System
Benign

Stage 2
Active Benign (G0,T0, M0)
The MSTS Staging System
Benign

Stage 3
Aggressive Benign (G0, T1-2, M 0-1)
The MSTS Staging System
Malignant

Stage IA
(G1, T1, M0)
The MSTS Staging System

Stage IB
(G1, T2, M0)
The MSTS Staging System

Stage IIA
(G2, T1, M0)
The MSTS Staging System

Stage IIB
(G2, T2, M0)
The MSTS Staging System

Stage III
(G1-2, T1-2, M1)
 Workup of bone tumors
• Primary bone tumors, unlike most other tumors, maybe difficult to dia
gnose based on histology
• The radiology and clinical features are essential components of diagn
osis.
• A good history is important and very helpful
• Important factors:
• Age
• Presentation (mass, pain, paraesthesia, trauma)
• Any known malignancy
• Sex.
Analytic approach to evaluation of the bone
neoplasm
Biopsy
• When needed biopsy, should be the last step in the diagnostic w
orkup
• Biopsy often done by open (surgical) or by FNAB (with/without
CT guidance)
Radiographic diagnosis
• First decide: “lytic” or “blastic”
• Lytic: “Hole in the bone”
• Blastic: Area that too dense or “white”
• Some lesions are a combination (mixed)
Radiologic Workup
• For most bone tumors plain x-rays offer the mos
t important information about the diagnosis.
• X-ray shows how the bone is reacting to the tumor, a
nd how the tumor is reacting the bone
• CT, MRI and bone scans mostly useful in staging
Location

• Location within the bone is also as a factor, sin

ce certain tumors prefer the diaphysis, metaph
ysis, or epiphysis
• The metaphyseal location is the least helpful, since i
t has a rich blood supply, all etiologies have a predil
ection for metaphysis.
• Central, eccentric, cortical or bone surface.
Tumor Location
• Most primary malignant bone tumors in young p
eople arise in areas of rapid growth such as the
distal femur, proximal tibia, prox. humerus.
• Some primary tumors have a predilection for cer
tain locations.
• Most metastatic lesions occur in regions that con
tain haematopoetic marrow: axial skeleton, proxi
mal extremities
Remember
• Primary bone tumors are much less common than metastatic tu
mor.
• A “hole in the bone” is most often due to metastatic disease.
Tumor Aggressiveness
• How the bone reacts to the tumor provides impo
rtant clues to the behavior of the tumor such as
the rate of growth.
- Pattern of bone destruction and pattern of bon
e
response to the lesion gives a sense of the rate
of
growth.
- Extension through the cortex/ associated soft
tissue mass.
Types of Bone Destruction
( Lytic Lesions)
• The tumor margin (margin of the hole in the bone) can
be sharp or fuzzy / ill – defined.
• Sharp is least aggressive (especially if sclerotic rim).Fuz
zy is more aggressive,and no clear border between tum
or and normal bone is the most aggressive.
• Terminology: geographic = focal and well circumscribed
(the least aggressive,especially if sharp and sclerotic m
argin).
• Motheaten / permeative = multiple holes ( the most ag
gressive).
Work up of possible primary bone tumo
rs
• Diagnosis should include a thorough radiologic w
ork up in conjunction with a multidisciplinary tea
m that will be providing the definitive care.
• Diagnosis for many bone tumors is made by radi
ographic features alone.
- Pathologic findings may be misleading !
• When reguired,a biopsy should be the last,not th
e first step in the diagnostic process.
WORK UP
• Next a whole body bone scan should be obtained.This is often th
e best way to look for other bone lesions.Skeletal survey is some
times best for purely lytic / lucent lesions ( e.g...myeloma someti
mes).
• If there are multiple lesions the diagnosis will almost always be
metastatic disease.
REMEMBER
• Radiographic and clinical work up should be complete before any
biopsy.
• Plain films are often the best test to evaluate primary bone tumo
rs – sometimes better than pathology !.
WHO Classification
Benign Bone Tumors

• Bone-Forming Tumors
• Cartilage-Forming Tumors
• Giant Cell Tumor
• Benign Vascular Tumors
• Tumor-Like Conditions
WHO Classification
Benign Bone Tumors

Bone-Forming Tumors
A. Osteoma
B. Osteoid Osteoma
C. Osteoblastoma
WHO Classification
Benign Bone Tumors

Cartilage-Forming Tumors
A. Chondroma
B. Osteochondroma
C. Chondroblastoma
D. Chondromyxoid Fibroma
CHONDROMA (ENCHONDROMA)
• Benign, Any age
• Single or multiple sites
• Often involves small bones of hands and feet.
• Well demarcated, mature cartilage.
CHONDROMA (ENCHONDROMA)
• Hereditary – multiple enchondromatosis. Usually over one sid
e of the body. (Ollier’s disease).
• Maffucci's syndrome - multiple bone chondromas and heman
giomas of soft tissue
• Increased risk for chondrosarcoma
Ollier’s Disease: Multiple
enchondroma.
OSTEOCHONDROMA(TOSIS)
• Hereditary (multiple) or sporadic (single)
• mushroom-shaped bony projections
• Lateral aspects of cartilage joints.
• Chondrosarcoma – in hereditary type.
Osteochondroma:
Osteochondroma
Osteochondroma
WHO Classification
Benign Bone Tumors

Benign Vascular Tumors

Hemangioma
Lymphangioma
Glomus Tumor
WHO Classification
Benign Bone Tumors

Other Benign Connective Tissue Tum

ors
Desmoplastic Fibroma
Fibrous Histiocytoma
Lipoma
Neulilemoma
Neurofibroma
WHO Classification
Benign Bone Tumors

Tumor-Like Conditions
Solitary Bone Cyst
Aneurysmal Bone Cyst
Metaphyseal Fibrous Defect
Eosinophilic Granuloma
Fibrous Dysplasia
WHO Classification
Benign Bone Tumors

Tumor-Like Conditions
Osteofibrous Dysplasia
Myositis Ossificans
Brown Tumor of Hyperparathyroidism
Intraosseous Epidermoid Cyst
Giant Cell (Reparative) Granuloma
Malignant Bone Tumors

• Osteosarcoma
• Chondrosarcoma
• Malignant Fibrous Histiocytoma
• Adamantinoma
• Chordoma
OSTEOSARCOMA

• Common primary cancer of bone

• Young adults - 10 and 25 years
• Rare in later age – Secondary to previous irradiation
or Paget’s disease
• genetic (retinoblastoma gene)
• Metaphysis of a long bone (Knee)
• Tenderness / pain / Mass.
OSTEOSARCOMA
• Malignant mesenchymal cells that produce Irregu
lar lace like osteoid matrix.
• May or may not be calcified.
• pre-operative chemotherapy with surgical resecti
on.
• The five-year survival ~ 60%
Osteosarcoma – gross
Osteosarcoma – X-ray
 CHONDROSARCOMA
• Next common to Osteosarcoma.
• Older adults 30 to 60 years.
• Location - axial skeleton (pelvis & pectoral girdles, ribs & spine)
• Aggressive, erodes & invades soft tissue,
• Metastases to lungs, liver, kidney & brain.
CHRONDROSARCOMA
• Malignant cartilage with anaplastic chondrocytes in spac
es with focal enchondral ossification and calcification
• Resistant to chemo  Surgical resection
• Grade I tumors have 5-year survival rates of 90%, while
high grade tumors have poor prognosis.
• Clear cell chondrosarcoma is a histologic variant that is
associated with a better prognosis.
Chondrosarcoma
OSTEOSARCOMA CHRONDROSARCOMA

• 10-25 years of age • >40 years of age

• affects long bones • affects axial skeleton
• sensitive to chemoth • not sensitive to che
erapy motherapy
Malignant Bone Tumors
Marrow Cell Tumors

• Adults
Lymphoma
MM
Plasmacytoma
• Children
Histiocytoma
Ewing’s Sarcoma
Malignant Bone Tumors
Metastatic Bone Disease

Breast
Prostate
Lung
Kidney
Thyroid
Metastatic tumors:
Osteoblastic Metastasis: Pr
ostate
Osteolytic Metastasis: Breast
ca
Osteolytic Metastasis: Brea
st ca
Soft Tissue Tumors

• Fibrous Tumors
• Fibrohistiocytic Tumors
• Adipose Tumors
• Muscle Tumors
• Blood Vessels Tumors
• Lymph Tumors
Soft Tissue Tumors

• Synovial Tissue Tumors

• Mesothelial Tissue Tumors
• Peripheral Nerve Tumors
• Autonomic Ganglion Tumors
• Cartilage and Bone Forming Tumors
• Pluripotential Mesenchyme Tumors
• Undifferentiated Tumors
Principles of Treatment

• Psychologic
• Selecting forms of Tx. consider life expectancy (Px)
• Benign  surgical
• Malignant  surgical ablation/eradication with or withou
t Rx & adjuvant chemotx. Consider limb salvage proced
ure
• Radiotx , (Ewing, Retic. Cell Sa.)
• Adjuvant syst. Chemotx. (OsteoSa)
• ImmunoTx ?
Benign Bone Tumors
Usual Treatment

• Stage 1
Observation or Simple Curettage
• Stage 2
Extended Curettage
Benign Bone Tumors
Usual Treatment

• Stage 3
Extended Curettage, Excision-Curet
tage or Marginal or Wide Excision
Intralesional Curettage

• The Principle Treatment of Most B

enign Bone Tumors
• Curettes
• Modest-sized Bone Windows
• Bone Graft / Bone Cement
• + Adjuvant Agent
Intralesional Curettage
Simple Curettage

• Stage 1
• Large Window
• Curette and High-Speed Blu
r
Intralesional Curettage
Extended Curettage

• Stage 2 and 3
• Large Window
• Curette and High-Speed Blur
• Adjuvant Agents: Phenol, Liqui
d Nitrogen
Intralesional Curettage
Excision Curettage

• Stage 3
• En Bloc Resection
• Curettage of the Inner Portio
n
• Need Reconstruction
Surgical Margins
Intralesional

• Curettage
• Benign Bone Tumors; Stage 1 an
d2
• Extended Curettage
• Benign Bone Tumors; Stage 3
Surgical Margins
Marginal

• Noninfiltrating Benign Soft Tissue Tu

mors
• Benign Bone Tumors Stage 3 and A
djuvant Agent
Surgical Margins
Marginal

• Recurrent Benign Bone Tumors; Sta

ge 2 and 3
• Selected Low and High-Grade Sarco
mas with Successful Neoadjuvant Ch
emotherapy and Radiation Therapy
Surgical Margins
Wide

• Recurrent Stage 3 Benign Bone Tum

ors
Surgical Margins
Radical

• Recurrent Sarcomas
• Displaced Pathological Fractures of B
one Sarcomas
• Sarcomas That Cannot be Adequatel
y Imaged
Tumor Resection
Techniques

• Intra-articular
• Extra-articular
Filling Materials

• Cancellous Autograft
• Cancellous Allograft
• Bone Substitutes
• Polymethylmethacrylate