2. ο
ο A case of a Patient
ο Clinical Presentation
ο Standard work up
ο Principles of management
ο Prognostic factors
ο Follow up
ο Case Critics
Outlines of presentation
3. ο
ο A 50 years old male and camera man patient come
from AA presented with falling down accident of 3
days duration from standing position
ο 3 months prior to his accident he has left leg pain
and swelling .
ο Otherwise he has no medical illness ,and trauma hx
ο He has no hx of irradiation
ο He has no cough
ο He is refered from menilik hospital
MRN 549475
4. ο
ο GA ;acutely sick looking in pain
ο VS BP 140/70,PR 114
ο Msk;posterior long leg gutter and 4x5cm swelling inside
gutter
ο ASST:left tibia pathologic fracture
ο PLAN :was to do left leg x ray,CXR,MRI of left leg,OFT
ο ON 28/4/2011
ο On 10/5/2011
ο Incisional biopsy and external fixation was done
P/E
ON 28/4/2011
5. ο
ο Hx :same
ο ECOG1
ο V/S: stable
ο HEENT:pink conjuctiva and NIS
ο P/E :there is 5x4cm hard immobile mas on left distal
tibia
In our OPD
11. ο
ο ASST:High grade conventional osteosarcoma
ο Plan :Neoajuvant cisplatin and adriamycine
ο Mid cycle evaluation for surgery and then ajuvant
cisplatin and adriamycin
Cont..
13. ο
ο Most patients present with localized pain in the
affected bone.
ο Pain is usually of several months duration and may
wax and wane.
ο There may be associated soft tissue swelling or a
palpable mass.
ο Some patients present with pathologic fracture.
ο Osteosarcoma has a predilection for involvement of
the metaphysis of long bones.
Clinical Presentation
14. ο
ο The most common site of involvement is the knee
(distal femur or proximal tibia), followed by the
proximal humerus, mid and proximal femur, and
then other bones.
ο Although most patients have micrometastatic
disease at the time of presentation, only 10% to 20%
of patients present with clinical evident macro
metastases.
ο The lung is the most common site of metastatic
involvement, followed by bone.
Cont..
24. ο
ο The mainstay of surgical management is the
complete en bloc resection of tumor.
ο The extent of surgery have dramatically evolved
over time, with an emphasis on more conservative,
limb-sparing resections with maintenance of
function rather than amputation.
ο Neoadjuvant chemotherapy has played an important
role in this evolution
Surgery
25. ο
ο For extremity lesions, limb preservation is preferred
and can be accomplished in the majority of cases.
ο Retrospective studies have shown equivalent results
of limb-sparing surgery and amputation as long as
adequate margins can be achieved
cont.,β¦
26. ο
ο 1
JBJS Classics
227 patients
From26 institutions
Limbs paring
Procedure
73disarticulati
on of the hip
39
above-the
knee
amputation
115
LR
9
7.8%
SUR
67
58%
Met
65
56%
LR
0
0%
SUR
21
53%
MET
21
53%
LR
8
10%
SUR
40
54.7%
MET
43
58.9
%
PEPTS
local
recurrence
Metastasis
survival
after a median length of follow-up of 5.5years
27. ο
ο The Kaplan-Meier estimates of the percentage of
patients who survived and the percentage of patients
without recurrent disease showed no difference
among the three surgical groups (Mantel-Cox test
statistic: p = 0.8)
ο It appears that, compared with above-the-knee
amputation or disarticulation of the hip, the use of a
limb salvage procedure for osteosarcoma of the
distal end of the femur did not shorten the disease-
free interval or compromise long-term survival.
Cont.β¦
28. ο
ο However, not all patients are candidates for more
conservative surgery .
ο Axial tumors, although much less common, pose a
particular challenge because achieving complete
surgical resection can be difficult.
Contβ¦
29. ο
ο Pelvic tumors typically require a hemipelvectomy
for en bloc resection.
ο Some patients can undergo resection of the
hemipelvis with preservation of the extremity .
ο This has a better functional outcome compared to an
external hemipelvectomy, also referred to as a
hindquarter amputation
Cont..
32. ο
ο compromise between amputation and limb salvage
ο most commonly used for osteosarcomas of the distal
femur in skeletally immature patients
ο It is a procedure where the neurovascular structures
and distal aspect of the limb (leg) are retained
Rotationplasty
33. ο
ο For functional purposes, the distal segment is turned
180 degrees so that the ankle joint functions as a knee
joint,
ο thus converting an above-knee to a below-knee
amputation in order for prosthetic use to be
maximized
Contβ¦
36. ο
ο Contraindications to limb-sparing surgery include;
ο nerve or vascular encasement,
ο presence of large, biopsy-related hematoma,
ο And pathologic fracture.
ο Reconstructive options include use of allografts,
endoprostheses, and occasionally rotationplasty
Cont..
37. ο
ο In the absence of chemotherapy, 80% to 90% of
patients will subsequently develop distant
metastases.
ο Chemotherapy thus plays an important role for all
patients with intermediate- and high-grade tumors
ο Eilber et al. reported on 59 patients with
nonmetastatic osteosarcoma
ο randomized to surgery followed by observation
versus adjuvant chemotherapy
chemotherapy
38. ο
ο
Eilber et al.
To determine the role of
chemotherapy
59 patients of conventional
high grade
osteosarcoma
32 received
MAP,and
BCD
.27 No chemo
DFS
55%
OS
80%
DFS
20%
OS
48%
(P < .01).
PEPTS
OS,DFS
At a median follow-up of 2 years
39. ο
ο At a median follow-up of 2 years, there was a
statistically significant improvement in both disease-
free and overall survival in those who received
adjuvant chemotherapy.
42. ο
ο Link et al. reported similar results in a group of 36
patients with nonmetastatic, high-grade
osteosarcoma randomized to observation versus
adjuvant chemotherapy after primary surgery.
ο Disease-free survival at 2 years was 66% with
chemotherapy and 17% with observation (P < .001).
Cont..
43. ο
ο
Link et al
36 patients
To see effect of ajuvant
multiagent chemo
18
Received
chemo
18 No
chemo
RFS at 2y
66%
RFS at 2 year
17%
P<0.001
At a median follow-up of 2
years
PEPTS
RFS
46. ο
ο The concept of neoadjuvant chemotherapy arose in
conjunction with evolving surgical techniques
striving for limb-preserving procedures and
improved functional outcomes.
ο This led to a randomized clinical trial by the
PediatricOncology Group (POG8651).
neoadjuvant
chemotherapy
47. ο
ο Advantages of neoadjuvant chemotherapy: -
οΌ regression of the primary tumor, making a successful
limb salvage operation easier.
οΌ may decrease the spread of tumor cells at the time of
surgery
οΌ Effectively treating micrometastases at the earliest
time possible.
οΌ It avoid tumor progression, which may occur during
any delay before surgery.
Contβ¦
50. ο
ο The optimal choice of chemotherapy and
administration schedule remains a subject of active
research
The optimal choice of
chemotherapy
51. ο
ο phase 3 randomised, controlled trial.
ο Consenting patients with newly diagnosed,
resectable, high-grade osteosarcoma aged 40 years
or younger were eligible for randomisation
EURAMOS-1
52. ο
ο
EURAMOS-1
2260 patients
325 sites in 17 countries
to see IE effect in poor
responders
1642
Good
response
neoajuvant
MAP
618
Poor to MAP
310 to
receive
MAP
308 to
receive
MAPIE
Median
follow-up
was 62.1
months
EFS
55%
EFS
53%P = .69
PEPTS
OSDFS
55. ο
ο Final results were unfortunately negative with
neither ifosfamide and etoposide nor interferon
showing improved event free survival.
ο For the poor response group, at 62 months median
follow-up, 3- year event-free survival was 55% for
MAP and 53% MAP + IE (P = .69).
ο For the good response group, at 44 months median
follow-up, 3-year event-free survival was 74% for
MAP and 77% MAP with interferon (P = .214
Cont..
57. ο
ο
EOI
407 patients with operable,
non-metastatic
osteosarcoma
PEPTS
OS,PFS
16
patients
excluded
199 patients
Receive 18
week of AC
192 patients
Receive PREOP MAV
,POSTOP,BCD,MAP
OS
65%
PFS
47%
OS
65%
PFS
47%
at median of 3 years
60. ο
ο
Chemotherapy in older
patients
GAETANOBACCI.ET AL
53 patients
aged between 40
and 60 years
Cisp
+ad
m
29
patients
Received
surg,neoa
juvant
chemo
24
patients
Received
surgery
alone
PEPTS
EFS,OSa good
histologic
response
received the
same two
drugs
poor response
ifosfamide
and etoposide
were added to
cisplatin and
adriamycin.
63. ο
ο Operative resection is the only potentially curative
treatment for patients with thoracic metastases from
osteogenic sarcoma.
ο Following resection, adjuvant chemotherapy can
improve disease-free survival and decrease the
burden of metastatic pulmonary disease.
ο Resection of pulmonary metastases should always be
part of a combined modality approach
Role of metastatectomy
64. ο
ο Surgical margins; Negative surgical margins (defined as
at least 1 cm in bone with 2β5 cm recommended)
ο Therefore, limb salvage is recommended when adequate
surgical margins can be achieved
ο tumor responsiveness are directly associated with local
recurrence:
ο In patients with marginal resections and with tumor
necrosis less than 90% after preoperative chemotherapy,
local recurrence has been reported as high as 30%
Prognostic factores
65. ο
ο Stage at diagnose
ο tumor volume
ο old age,
ο sex,
ο and possibly p-glycoprotein expression
Cont.β¦
68. ο
ο Good documentation
ο Proper work up with available set up except
ALP,LDH
ο EXTERNAL fixation ??
ο Proper treatment planning
Case critics