Adamatinoma of tibia primary of bone tumor

adamantinoma of tibia bone tumor

2. ACKNOWLEDGMENTS 

3. First and foremost thanks to ALLAH

It is a pleasure to express my great appreciation to Prof.
Dr.
Eissa raghb Refaie ,
Professor of orthopedic surgery, Faculty
of Medicine, Al-Azhar University, for his expert guidance, kind
supervision, and for his acceptance to discuss this essay
I would like to thank Prof.
Dr.kamal abdelrahman
,
Professor of orthopedic surgery, Faculty of medicine, Al-Azhar
university, for his accurate supervision , kind
cooperation ,continuous support, and honest guidance.

4. I Would Like To Thank Prof. Dr.Mohammed
Abd Elrahmn
Professor Of Orthopedic Surgery, Faculty Of Medicine, Ain
Shams University For His Acceptance To Discuss This Essay
I Would Like To Thank Prof.Dr.Mahmoud
Seddik,
Professor
Of Orthopedic Surgery, Faculty Of Medicine, Al-azhar
University, For His Accurate Supervision , Kind
Cooperation ,Continuous Support, And Honest Guidance.
Finally ,I Would Also Like To Thank All Those Helped Me To
Finish This Work.

5. Introduction and
aim of the work

6. The aim of this study is to through lights on
pathology, radiology, clinical features, management
adamantinoma of the tibia and evidence based studies
of adamantinoma of tibia.

7. Adamantinoma OF Tibia
Adamantinoma is a primary low-grade,malignant
bone tumor that is predominantly located in the mid-
portion of the tibia..

8. Pathology of Adamantinoma OF Tibia
Epidemiology:
Adamantinoma comprises about 0.4% of all primary bone
tumors. Patients present with this tumor from 3 up to 86 years,
with a median age of 25-35 years.
Sites of involvement:
The tibia,in particular the anterior (meta-) diaphysis, is
involved in 85-90% of cases. In 10% this is combined with
one or more lesions in the ipsilateral fibula. Rare sites have
been reported, especially the ulna.

9.  Origin:
It seems to suggest that adamantinoma is
a tumor of epithelial origin. Based on
ultrastructural and immune-histochemical
studies

10. Theory Authors ,Year
1.Fetal crest Fisher,1917
2 .Basal cell lineage, Trauma Ryrie, 1932
3 .Implantation
Dockerty and Meyerding, 1942
 
4 .Unknown Baker et al, 1954
5 .Synovial cell like Lederer and Sinclair, 1954 , Naji et al 1964
 6 .Angioblastic
Changus et al, 1957, Elliot 1962, Llombart bosch and
Ortuno-pacheco, 1978,
Reed, 1982
7 .Mesenchymal Vinogradova, 1969
8 .Dermal inclusion Lichtenstein, 1977
9 .Epithelial cell
Jaffe, 1958, Saacebra et al, 1968, Rosai and
Pincus,1982, Ishida et al,1992, Hazelbag et al, 1993,
Jundt et al, 1995

11. Histologically Adamantinoma is classified into 2 distinct types: classic and
differentiated.
Features Classic Differentiated
Age More than 20 years, Adults Less than 20 years, Children
Radiology
Soft tissue or intramedullary
involvement regularly
observed
Intra cortical location indistinguishable
from OFD
Histopathology
Admixture of both epithelial and
osteofibrous component,
most commonly solid nests of
basaloid cells
OFD like pattern lacks conspicuous nests
and masses of epithelial cells. Scattered
positivity of epithelial elements for
cytokeratin
Behavior Aggressive clinical course Relatively benign

12. Macroscopy:
The appearance varies but most often the tumor is yellow
gray or grayish white and fleshy or firm in consistency
Larger tumours show intramedullary extension and cortical
breakthrough with soft tissue invasion in a minority of cases.
Macroscopically detectable cystic spaces are common, filled
with straw coloured or blood-like fluid.

13. Microscopic Examination :
The histologic patterns varied within each case and from case to case.
However, the following five basic patterns were recognized: basaloid,
spindle, tubular, squamoid, and osteofibrous dysplasia-like.
Ultrastructure :
showing intra-cytoplasmic hemi-desmosomes, tonofilaments,and
microfilaments.

14. Genetics :
Review of previously reported cases reveal extra
copies of chromosomes 7, 8, 12, 19, and/or 21 in
classic and differentiated adamantinoma.

15. Prognostic factors
Risk factors for recurrence
 male sex/females at young age
pain at
 presentation
short duration of symptoms

young age (<20 years)
 lack of squamous differentiation of the tumour
intralesional or marginal surgery and extra-compartmental
growth.

16. Recurrence percentages after non-radical
surgery may rise up to 90%.

Recurrence is associated
with an increase in epithelium to stroma ratio and
more aggressive behaviour

17. Behavior :
Adamantinomas are locally aggressive tumor and
are extremely slow growing with the potential to
metastasize.Recurrence of tumor is frequent after
inadequate therapy, and the behavior of the recurrent
neoplasm resembles more and more that of a
sarcoma .

18. This low-grade, slowlygrowing malignancy
metastasizes in about 15–30 percent of cases by both
hematogenous and lymphatic routes to other parts of
the body, usually to the lungs or nearby lymph nodes;
bone and abdominal viscera make up a minority .

19. Diagnosis
of Adamantinoma

20. Diagnosis
The initial symptoms of adamantinoma are often indolent and
nonspecific and depend on location and extent of the disease.
The onset is insidious and its course shows a slow,
progressive character.
Patients usually complain of pain and swelling, often of long
duration.

21. •Approximately one-third of patients have had
symptoms for longer than 5 years.
•Pathological fracture, paraneoplastic hypercalcemia
and pulmonary metastasis may be present.

22. Differential Diagnosis

23. Relationship between adamantinoma and OFD
Features Osteofibrous dysplasia Adamantinoma
Nature Benign condition Locally aggressive
Age Less than 10 years 2 year to 86 years
Site May involve both tibia and fibula.
90% tibial involvement, In 10–15% cases, ipsilateral
fibular
involvement, Rarely pretibial soft tissue and other bones
Clinical presentation
•Pain, swelling, pseudoarthrosis, bowing,
pathological fracture may occur
•seldom progresses during childhood, and any
progression of the lesion stops after puberty
•With or without Pain, swelling, pathological fracture in
25% cases
•progressive during adult age
Predisposing history of
trauma
Absent Present
Radiology
Periosteal reaction present Intra cortical
Limited to anterior cortex
Well marginated with marginal sclerosis, ground
glass appearance
Periosteal reaction is variable
15% of cases, there is extracortical extension into soft
tissues
Single or multiple nodular lesions in one or more foci in
medulla
Sharply or poorly delineated osteolytic lesion. With
septations and peripheral sclerosis, characteristic
"soapbubble" appearance
Histopathology
Zonal phenomenon present Scattered epithelial
cells recognized on IHC
Absent Presence of epithelial cells forming small
nests/strands recognized in H&E
Recurrence Local recurrence in 25%. Tends to recur in 18–32%
Metastasis No metastasis
Metastases may occur in 15–30%, Lung and Lymph
nodes
usually involved
Regression Spontaneous regression at puberty in 33% cases Regression±

24. Relationship between adamantinoma and Ewing's sarcoma.
Recently, the least common variant of adamatinoma has been described
as Ewing's-like adamantinoma or adamantinoma- like Ewing's.
The variant is characterized by anastomosing cords of small, uniform,
round cells set in a myxoid stroma. These cells exhibit features of both
epithelial cells and neuroendocrine cells on ultrastructural examination.
Immunohistochemical studies have shown the tumor cells to contain
both epithelial and neural antigens including the Ewing's sarcoma-
related antigen .

25. Radiology

26. in long bones it is found in the diaphyseal location although
metaphyseal extension of lesions or isolated involvement of
a metaphysis is seen occasionally.
 The metaphyseal involvement makes the diagnosis more
challenging because other tumors have to be considered in
the differential diagnosis.
The lesions are well-circumscribed involving anterior tibial
cortex, with septations and peripheral sclerosis. Multifocality
in the same bone is regularly observed
Radiologic features

27. Radiologic feature
 These multifocal radiolucencies surrounded by ring-shaped
densities producing the characteristic "soap-bubble"
appearance. The lesion is commonly intra-cortical, but may
destroy cortex and invade the extracortical soft tissues in
about 15% of cases.
The entire lesion may have a prominent sclerotic margin
indicative of slow growth. The periosteal reaction is variable
from minimal to prominent

28. Radiologic features
Plain Radiograph Of An
Adamantinoma In The Anterior
Tibial Diaphyseal Cortex
Plain radiograph of a classic
adamantinoma in a distal tibial shaft.
There are intercortical lucencies ,
involvement of the medullary canal

29. Computed Tomography (CT)
CT is superior to MRI in the assessment of cortical involvement,
matrix mineralization, periosteal reaction, and pathological
fractures.
CT is the technique of choice for detecting pulmonary metastases
and lymphadenopathy in suspected metastatic AD
(Adamantinoma).
CT scan shows the cortical involvement and the soft tissue
extension when it exist. However, does not show the intra-osseous
extension of the tumor.

30. Computed Tomography (CT)
CT scan of the right leg centered on the lesion 2D reconstructions
show the dense margins of the lesion, thedestruction of the
posteromedial side of the tibial cortex and the chronic periosteal
reaction on the anterolateral side of the tibial cortex.No fracture line
is visible

31. Computed Tomography (CT)
A sagittal CT scan shows the soap bubble appearance of the
lesion. The tumor was less than 1 cm away from the distal tibial
articular surface.

32. Magnetic Resonance Imaging (MRI)
The following characteristics were evaluated Tumor localization,
cortical involvement and tumor margins, Bone marrow
involvement and tumor margins, Soft-tissue extension .
MRI has proved to be very useful in the workup and staging of
AD. AD generally shows a homogeneous, intermediate signal on
T1-weighted images. On T2-weighted images, the signal intensity
of an AD lesion is always high, whether homogeneous or
heterogeneous

33. Magnetic Resonance Imaging (MRI)
sagittal spin-echo T1-weighted image obtained after contrast
medium injection shows intense and homogeneous enhancement

34. Magnetic Resonance Imaging (MRI)
20-year-old woman with adamantinoma of tibia. Gadolinium-
enhanced sagittal T1-weighted images withfat-selective
presaturation show lesion consisting of multiple small nodules
within anterior cortical bone of diaphysis (arrow). Separate focus
is seen in proximal epiphysis (arrowhead)

35. Bone Scan of Tibial Adamantinoma
Use of nuclear medicine to study adamantinomas is a relatively
new undertaking.
Increased blood flow in the region of the tumor, increased blood
pooling, and increased accumulation of technetium-99m
methylene diphosphate in the area of the tumor.

36. 99mTc-MDP whole body bone and 99mTc-MIBI whole body
imagings are useful in the diagnostic management of patients
with suspected adamantinoma.
After surgery, 99mTc-MIBI whole body imaging also can be
used to exclude residual tumor and to discriminate between
tumor recurrence and postsurgical tissue change.

37. Bone Scan of Tibial Adamantinoma
Bone scanning showed an increased uptake in the tibial shaft

38. Bone Scan of Tibial Adamantinoma
Bone Scan

39. Staging

40. Staging
There are two major systems for staging tumors, the American
Joint Committee on Cancer (AJCC) system and the Enneking
staging system.
The stage of a tumor is one of the most important factors
determining a person's outlook (prognosis) and in choosing
treatment.

41. Staging
The American Joint Committee on Cancer (AJCC) The AJCC
staging system for bone cancers is based on 4 key pieces of
information:
T describes the size of the main (primary) tumor
N describes the extent of spread to nearby (regional) lymph node
M indicates whether the cancer has metastasized(spread) to other
organs of the body
G stands for the gradeof the tumor, which describes how the cells
from biopsy samples look under a microscope.

42. Staging
Enneking Staging System:
•In 1980, Enneking Et Al Described A System For Staging
Bone Sarcomas. Staging With The Enneking System Is Based
On Three Criteria.
•The First Criterion Is That Of The Extent Of The Tumor
•The Second Criterion Is That Of Metastasis
•The Third Criterion Is That Of The Grade Of The Tumor.

43. Staging
According to this classification there are six stages:
1- Stage IA (G1, T1, Mo): low grade intracompartmental without
metastases.
2- Stage IB (G1, T2, Mo): low grade extracompartmental without
metastases.
3- Stage IIA (G2, T1, Mo): high grade intracompartmental
without metastases.

44. 4- Stage IIB (G2, T2, Mo): high grade extracompartmental without
metastases.
5- Stage IIIA (G1or G2, T1, and M1): low or high grade intra-
compartmental with metastases
6- Stage IIIB (G1or G2, T2, M1): low or high grade extra-
compartmental with metastases
Staging

45. Treatment

46. TREATMENT
Once the diagnosis of Adamantinoma has been confirmed, an
individual treatment plan is made for each patient .
Current treatment of adamantinoma, including en bloc tumor
resection with wide operative margins with limb reconstruction and
limb salvage, provides lower rates of local recurrence than has been
previously reported .
The limb reconstruction can be performed with distraction
osteogenesis, allografts, vascularised fibular autografts (preferred) and
metallic segmental replacement

47. Treatment
The trend towards limbpreserving surgery is largely related to evolving
and improving experience of surgeons with other tumors and patient
preference for limb-preservation.
Quereshi et al reported in a review of 70 patients, en bloc tumor
resection with wide margins and limb salvage was shown to have 10
year survival rate of 87.2%
Reconstruction with tibial allograft is also an option. However, tibial
allograft reconstruction may be accompanied by high rates of nonunion
(24%) and fracture (23%).

48. Treatment
Rates of local recurrence vary from 18% to 32% ,while rates of
metastasis are reported to be 15% to 30%.
Metastatic spread to lymph nodes is a late finding in the course
of the disease. Mortality rates in the literature have varied from
13% to 18%.
Unfortunately neither radiation therapy nor chemotherapy has
been proven effective in the treatment of this insidious tumor

49. Treatment
A) Gross appearance of the tumor resected en bloc is shown. (B) A
contralateral vascularized osteocutaneous fibular autograft is shown
before and (C) after inlay into tibial allograft. (D) The final
appearance of the graft at the end of the procedure is shown

50. Treatment
A) Antero-posterior and (B) lateral plain radiographs taken 6
months postoperatively showthe tibial allograft inlaid with
contralateral fibular auto-graft secured with locking plates
proximally and distally

51. Anteroposterior radiograph made after en blocresection of an
adamantinoma followed by reconstruction with use of a
vascularized graft from the contralateral fibula. Note that an
external fixator was used to stabilize this construct.Fig. 26-B:
Antero-posterior radiograph made after removal of the external
fixator. Note the union at the proximal and distal graft sites.
Treatment

52. Summary

53. A low grade, malignant biphasic tumor characterized by a
variety of morphological patterns, most commonly epithelial cells,
surrounded by a relatively bland spindle-cell osteo-fibrous
component.
The histologic patterns varied within each case and from case to
case. However, the following five basic patterns were recognized:
basaloid, spindle, tubular, squamoid, and osteofibrous dysplasia-
like

54. •The initial symptoms of adamantinoma are often indolent
and nonspecific and depend on location and extent of the
disease.
•Current treatment of adamantinoma, including en bloc
tumor resection with wide operative margins with limb
reconstruction and limb salvage, provides lower rates of
local recurrence