MALIGNANT BONE

TUMOURS (MBT)

SEPTEMBER 2012

FRANK OBENG
TTA
 OVERVIEW

Introduction
Epidemiology
Classification
Tripple Assessment

Other laboratory investigations

Treatment/ Management
Rehabilitation
 INTRODUCTION

MOST MBT ARE SECONDARIES FROM

Breast
Prostate
Lungs
Kidneys
Thyroid

PRIMARY BONE TUMOURS ARE RATHER

UNCOMMON
 BONE: COMPONENTS

CORTEX= MATRIX + CALCIUM SALTS

ZONE OF CARTILAGE AT ENDS
PERIOSTEUM AS COVER

BONE MARROW: fatty/ fatty + heamatopoitic cells

HAEMATOPEITIC CELLS produce red, white cells and
platelets
OTHER CELLS IN THE MARROW:
Reticuloendothelial cells, fibroblasts, plasma cells

ANY OF THESE CAN BECOME TUMOUROUS

 TYPES OF PRIMARY BONE TUMOURS

Classification
Cell type Benign Malignant
Bone Osteoid osteoma Osteosarcoma
Cartilage Chondroma Chondrosarcoma
Osteochondroma
Fibrous tissue Fibroma Fibrosarcoma
Bone marrow Eosinophilic Ewing's sarcoma
granuloma
Myeloma
Vascular Haemangioma Angiosarcoma
Uncertain Giant-cell tumour Malignant giant cell
 EPIDEMIOLOGY
OSTEOSARCOM ERWING’S CHONDROSAR FIBROSARCOM
A SARCOMA COMA A
The most common UNCOMMON LEAST COMMON
4 – 5 cases/ 1% of all childhood
million /yr malignancies
1000 to 1500 new
cases/yr; USA Most common
2,570 new cases in primary osseous
USA in 2005 tmr childhood/
adolescence
Dupyetren; 1806 James Erwing 1921
Virchow’s 1863
Codman 1922
Jaffe; 1959; 10% Neural origin of
5-year survival rate tmr (recent)
Non-metastatic
disease; mortality;
high 30%.
 EPIDEMIOLOGY

OSTEOSARCOM ERWING’S CHONDROSAR FIBROSARCOM

A SARCOMA COMA A
AGE: BIMODAL: AGE: 5 -15 yrs ADULTHOOD; YOUNG ADULTS
2ND. Decade and age> 25% M=F
60yrs (here M>F M:F= 1:1
associated with Whites > blacks
paget’s disease)
Anatomic Diaphysis of of About 50% arise Metaphysis or
prediliction: long bones; tibia, from pre-existing diaphysis
locations of rapid fibula, humerus, osteochondromas
growth femur, vertebrae.
Distal femur,
proximal tibia,
proximal humerus
ANATOMIC PREDILICTION
ANATOMIC PREDELICTION
 EPIDEMIOLOGY

OSTEOSARCOM ERWING’S CHONDROSAR FIBROSARCOM

A SARCOMA COMA A
Risk factors of
poor prognosis:
age> 55
Tumour stage II or
III; metastatic
tumors, proximally
located tumours
Large tumours
>8cm
Tumours with <
90% necrosis
following neo
adjuvant
chemotherapy
 EPIDEMIOLOGY

OSTEOSARCOM ERWING’S CHONDROSAR FIBROSARCOM

A SARCOMA COMA A
Cause; unknown
Associated risk
factors: trauma,
viruses; SV40, a
DNA polyoma
virus
Altered P53
tumour suppressor
gene
Drugs, chemicals
Environmental
factors
 GENERAL PRINCIPLES FOR SURGICAL
MANAGEMENT

CLINICAL DIAGNOSIS: history, examination

IMAGING STUDIES+ other labs
WELL PLANNED BIOPSY
CHEMOTHERAPY/RADIOTHERAPY
(adjuvant/neoadjuvant)
SURGERY : LIMB vrs LIFE --
AIMS AT ADEQUATE MARGINS
REHABILITATION: reconstruction,
physiotherapy,counselling, occupational therapy,
FOLLOW UP
JOURNAL CORRELATES
 HISTORY
OSTEOSARCOMA ERWING’S CHONDROSARC FIBROSARCOMA
Young male, M:F = SARCOMA OMA
1.5:1
2nd decade of life Child/ adolescent Adult , over 25yrs Young adults, M/F
Worsening Pain on Presents with a Male or female Slow growing
exertion, may awaken diaphyseal lesion equally, Locally invasive
at night followed by a tibia, fibula, femur, Slow growing hard Late metastasis to
rapidly growing humerus, tmr of the trunk, lungs
fleshly vertebrae girdles, upper Metastasize to local
mass humerus and upper LN, quite
femur characteristic
Sites: femur, tibia, Local pain, Substantial pain Has central or
humerus 80% reffered distal pain that increases with peripheral lesions
(around knee, 75%) From pelvis/hip tumour progression
Hx of minor trauma
may be a red herring
No specific systemic Wt loss, fever, Central lesions:
symptoms or signs malaise more vascular
Than peripheral one
Joint effusion, Lesions fungate and
 HISTORY

OSTEOSARCOM ERWING’S CHONDROSAR FIBROSARCOM

A SARCOMA COMA A
Death from
haematologic
metastatic disease
usually due to
pulmonary failure
due to flourid lung
mets. leading to
Pulmonary
heamorrhage
Pneumothorax
Superior vena cava
obstruction
 HISTORY

OSTEOSARCOM ERWING’S CHONDROSAR FIBROSARCOM

A SARCOMA COMA A
Sites of metastasis
Lungs
Other bones >
same bone(skip
lesions)
rarely; to
pericardium,
pleura, kidneys,
adrenal glands
Lymph nodes
brain
 EXAMINATION
OSTEOSARCOM ERWING’S CHONDROSAR FIBROSARCOM
A SARCOMA COMA A
Warm, fleshy, Highly
tender, vascular vascularised mass
swelling hyperaemic
with engorged, Pathological Fungating lesions,
tourtous veins fracture easily bleed
encoursing
subcutaneously
Stretched glossy Pathological
skin fracture common
Joint may be
involved
Limited joint High LDH, CRP,
movements due to leucocytosis
huge tumour
Pathological
fractures
EXAMINATION FINDIDINGS
 GENETICS
OSTEOSARCOM ERWING’S CHONDROSARC FIBROSARCOMA
A SARCOMA OMA
SYNDROMES:
Li Fraumeni
syndrome,
Werner’s syndrome
Rothman-
Thompson
syndrome
Famililies with
multiple
osteosarcomas:
Hereditary
retinoblastoma
(RB)
RB and P53 tumor
suppressor gene
CDK4, cyclin D1, C-
myc, fos, Her2/neu
 PATHOLOGY
OSTEOSARCOMA ERWING’S CHONDROSARC FIBROSARCOMA
SARCOMA OMA
Usually primary
May be secondary; Grayish white Skin fungation
arising from tumour common with life
preexisting macroscopically threatening
osteochondroma hemorrhage.
Vascular tumour with A sheet of small
heamorrhagic and round cells like
necrotic areas neuroblastoma
Arise from primitive Arise from
pluripotent cells osteoblasts,
chondroblasts and
May contain elements Haematogenous Fibroblasts.
of malignant bone, spread to lungs Has no tumour
cartilage, fibrous and bone osteoid
tissue; well or poorly
differentiated
Pleomorphic spindle NB:
shaped cells and fibrosarcoma+osteid
PATHOLOGY
 IMAGING
OSTEOSARCOM ERWING’S CHONDROSARC FIBROSARCOMA
A SARCOMA OMA
X-RAYS X-RAYS
Shortfall: does not Onion peel Transluscent areas Radioluscent lesion
show extent of soft appearance on x- of cartilage and in the bone with soft
tissue and rays represents stapled calcification tissue expansion
intramedulary tissue Layers of new Bone
involvement laid under the
elevated periosteum
FINDINGS: CXR: bone or
Metaphyseal lesion, pulmonary mets
aggressive lesion
Destroyed
trabecular pattern of
bone
Lifted cortex,
intense periosteal
bone formation
Codman’s triangle
Radiodense,
OSTEOSARCOMA ERWING’S CHONDROSARC FIBROSARCOMA
SARCOMA IMAGING OMA
MRI+/_ Gadalonium/ MRI help
CT scans are better establish the
extent of
disease.

MRI:
Detects extent of local
lesion before
chemotherapy
Helps determine
optimal time for surgery
Extent of lesion within
bone marrow
Axial CT scans help
establish extent of soft
tissue involvement and
relation to
neurovascular bundle
Angiograms: if vascular
reconstruction needed.
OSTEOSARCOMA
OSTEOSARCOMA
OSTEOSARCOMA
OSTEOSARCOMA
ERWING’S SARCOMA (HE and CD99)
CHONDROSARCOMA
 Lab investigations

 The only blood tests with prognostic significance are lactic

dehydrogenase (LDH) and alkaline phosphatase (ALP). Patients
with an elevated ALP at diagnosis are more likely to have
pulmonary metastases. In patients without metastases, those with
an elevated LDH are less likely to do well than are those with a
normal LDH.
 Important laboratory studies include the following: LDH
 ALP (prognostic significance)
 Complete blood cell (CBC) count, including differential
 Platelet count
 Liver function tests: Aspartate aminotransferase (AST), alanine
aminotransferase (ALT), bilirubin, and albumin
 Electrolyte levels: Sodium, potassium, chloride, bicarbonate,
calcium, magnesium, phosphorus
 Renal function tests: blood urea nitrogen (BUN), creatinine
 Urinalysis
 PRINCIPLES OF BIOPSY TAKING
PREPARATION: clinical assessment FBC, GXM,
imaging, markers
As much as possible, biopsy must be taken by a
muskuloskeletal surgeon or MS oncologist
Incision must be placed such that the later definitve
surgery can take it out wholly
Access the tumour through the shortest distance
possible; go through as few compartments as
possible
As much as possible, the surgeon at biopsy must be
part of the definitive surgery.
Drains must be place along the incisions and not
through a new/different track.
 AJCC Staging System
T Stages of Bone Cancer
 T0: No evidence of the tumor
T1: Tumor is 8 cm (around 3 inches) or less
T2: Tumor is larger than 8 cm
T3: Tumor has "skipped" to another site or sites on the same bone

N Stages of Bone Cancer

 N0: No spread to regional (nearby) lymph nodes
N1: The cancer has spread to nearby lymph nodes

M Stages of Bone Cancer

 M0: No distant metastasis
M1: Distant metastasis (spread of the cancer to tissues or organs far away from the original
bone tumor)
 M1a: The cancer has spread only to the lung
M1b: The cancer has spread to other sites

Grades of Bone Cancer

 G1-G2: Low grade
G3-G4: High grade
 Surgical Staging System for Musculoskeletal
Sarcomas

Stage Grade Site

IA Low (G1) Intracompartmental (T1)
IB Low (G1) Extracompartmental (T2)
IIA High (G2) Intracompartmental (T1)
IIB High (G2) Extracompartmental (T2)
III Any (G) Regional or Distant Metastasis
Any (T)

(Adapted with permission from Enneking WF, Spanier SS, Goodman MA: A system for the
surgical staging of musculoskeletal sarcomas. Clin Orthop Relat Res 1980;153:106-120.)
 DIFFERENTIAL DIAGNOSIS

OSTEOMYELITIS

HEALING FRACTURE

SYPHILITIC PERIOSITIS

 OTHER BONE TUMOURS, malignant or benign.

 TREATMENT
OSTEOSARCOMA ERWING’S CHONDROSARC FIBROSARCOMA
SARCOMA OMA
Chemotherapy plus Supervoltage Radioresistant Radioresistant
surgery within 2 wks of radiation
each other
Radiotherapy is needed Chemotherapy Amenable to surgery Surgery, amputation
if there is still tumour and surgery Amputations: 50% Has a high scar
at surgical margins/ cure rate recurrence rate
paliative treatment
CHEMOTHERAPY; Adjuvant Recurrence in scar Role of
directed at chemotherapy common. chemotherapy
micrometasis has improved undetermined.
Without chemo: 5-year Prognosis form
survival =20% 5% to 50%
With chemotherapy
protocols, survival is
now 70%
AMPUTATIONS (follows principles)
AMPUTATIONS ( rotationoplasty)
AMPUTATIONS (for quarter)
 TREATMENT
OSTEOSARCOMA ERWING’S CHONDROSARC FIBROSARCOMA
SARCOMA OMA
Doxorubicin, cisplatin
(IV/IA), Methotrexate,
ifosphomide
SURGERY: complete
en-bloc resection with
tumour free margins
(2-5 yrs) : fascia as
margins is better than
muscle or fat.
AMPUTATION:
preferably, joint above
shld be taken out.
Offers better survival
compared with to limb
salvage
LIMB SALVAGE:
better psychological
benefits; but high
 PROGNOSIS

OSTEOSARCOMA
Formerly 80% mortality in 2 years

55% - 80% survival in 5 years

Worst prognosis seen with proximal axial skeletal

lesions
 REHABILITATION
MULTIDISCIPLINARY:
Patient himself/herself
Surgeon
Physiotherapist
Orthotist
Clinincal psychologist,
Nurse,
Occupational therapist
Human resource manager
Lawyer/social workers/ social welfare officers
Spouse
Relatives
 PAGET’S DISEASE OF THE BONE

Usually associated with osteosarcoma in an older

population
Persistent pain in a patient >60yrs
Associated with swelling and usual pathological
fractures
Usual sites: diaphysis of bones or pelvic and
shoulder girdle
Multiple metastasis portend poor prognosis
PAGET’S DISEASE OF THE BONE
 MULTIPLE MYELOMA
(plasmacytoma)

Malignant; multifocal: single lesion in only 20%

initially

Age: adults>40yrs; M:F = 2:1

SITES: skull, vertebrae, pelvis, ribs, proximal humerus,

femur

Marrow is progressively replaced by tumour cells.

 MULTIPLE MYELOMA
(plasmacytoma

CLINICAL FEATURES:
Increasing pain and tenderness of the sites of
predeliction
Increasing thirst, fever , malaise, weight loss, fatigue
Girdle/referred pain from spinal deposits

COMPLICATIONS:
Kyphosis and cord compression == paraplegia
Nerve root compression with claudication
Pathological fractures
Pancytopenia; from marrow replacement
 MULTIPLE MYELOMA
(plasmacytoma

INVESTIGATIONS
X-rays: lead beaten apperance- numerous areas of
punched out rarefaction without cortical expansion
Serum proteins: increased gamma A or B globulin;
reverses the A/G ratio – monoclonal gammopathy on
electrophoresis
Bence –Jones proteinuria; 24 hr urine collection
Heamatogram (FBC): reduced RBC, WBC, platelets;
immature myelocytes
Bone marrow aspirate establishes diagnosis.
 MULTIPLE MYELOMA
(plasmacytoma

TREATMENT:
Vincristine: 0.4mg/msq and doxorubicin 9mg/msq
given on days 1 to 4. then
Methylprednisolone 1g/msq and verapamil 10 mg on
days 1 – 5
Mephalan 140mg/msq is given at height of response
to treatment with autologous bone marrow
transplantation
Interferon improves survival
Tumour responds to radiotherapy for some time.
Hoffman RD, Saltzman CL, Buckwalter JA: Outcome of lower
extremity malignancy survivors treated with
transfemoral amputation. Arch Phys Med Rehabil 2002;
83:177-182.
Hoffman RD, Saltzman CL, Buckwalter JA: Outcome of
lower extremity malignancy survivors treated with
transfemoral amputation. Arch Phys Med Rehabil 2002;
83:177-182.
The authors examined the outcomes of surviving patients
who underwent transfemoral amputation as part of the
treatment follow-in terms of employment, education level,
income, marital and home status, incidence of self-reported
health problems,
incidence of self-reported depression, and alcohol and
tobacco use. The long term cost of external prosthesis use
was noted
 Zeegen EN, Aponte-Tinao LA, Hornicek FJ, Gebhardt
MC, Mankin HJ: Survivorship analysis of 141 modular
metallic endoprostheses at early follow-up. Clin Orthop
Relat Res 2004;420:239-250.

 In this study, the survival rate of endoprosthetic devices was 88% at 3

years and 76% at 5 years.

 Based on location, the survival rate at 3 years was 100% for the
proximal humerus and proximal femur; 87% for modular knees and
53% for total femoral implants.

 Multivariate analysis showed that only location and infection were

independent risk factors for prosthetic failure

 Loosening and infection and dislocation were independently predictive

of a fair or poor clinical score.
 Mankin HJ, Hornicek FJ, Rosenberg AE, Harmon DC,
Gebhardt MC: Survival data for 648 patients with osteosarcoma treated at
one institution. Clin Orthop Relat
Res 2004;429:286-291.

 The authors of this study at one institution present the

results of an analysis to assess the factors that influenced the
survival of 648 patients with osteosarcoma centrally located
in the bone.

 The overall survival rate for the patients in the study was
68% at an average follow up of 6 ± 4 years.

 Death occurred at a mean of 3 ± 3 years. Anatomic location,

size of the tumor, and percentage of tumor cells killed after
neoadjuvant chemotherapy all had an effect on outcome.
 LOCAL CONTENT

LATE PRESENTATION

REFUSAL OF AMPUTATION
akpe!
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