Detailed presentation on osteoblastic osteosarcoma

1. OSTEOBLASTIC
OSTEOSARCOMA
DR. FAIZA AHMED KHAN
PGR MEDICINE (ROTATIONAL)

2. Overview
• Definition
• Epidemiology
• Pathogenesis
• Skeletal distribution
• Clinical presentation
• Classification
• Work up
• Investigations
• Treatment
• Prognosis

3. What is osteosarcoma ?
• Highly malignant tumor arising from
primitive mesenchymal bone-forming cells.
• Histologic hallmark is production of
malignant osteoid.
• Most common histological form of primary
bone cancer
• 2nd most common primary malignant bone
tumor after Multiple myeloma.

4. • Any bone can be involved but the Most
common sites are – distal femur, proximal
tibia, proximal humerus
• • Most common metastasis to the lungs
through blood stream
• INCIDENCE:
• More prevalent in males than female.
• involves any age but highest occurrence in
adolescence i.e. 12-25yrs.

5. Skeletal distribution
• Distal femur
• Proximal tibia
• Proximal humerus
• (sites of rapid bone growth)
• others
Metaphyseal(89%)>diaphyseal(10%)>epiphys
eal(1%)

6. Etiology
• Rapid bone growth – adolescence growth spurt in the
metaphyseal area near the growth plate.
• Radiation exposure – mostly causes secondary forms.
• Genetic predisposition :-
• hereditary form of retinoblastoma( RB gene mutation)
• Li-Fraumeni syndrome (p53 gene mutation)
• Rothmund – Thomson syndrome ( autosomal
recessive)
• Paget's disease of bone – mostly secondary forms

7. Classification
PRIMARY or SECONDARY :-
PRIMARY OSTEOSARCOMAS (15 – 25 yrs)
• Conventional /classic osteosarcoma (high
grade, intra medullary)
• Low-grade intramedullary osteosarcoma
• Paraosteal osteosarcoma
• Periosteal osteosarcoma
• High-grade surface osteosarcoma
• Telangiectatic osteosarcoma
• Small cell osteosarcoma.

8. SECONDARY OSTEOSARCOMAS
• Osteosarcoma occurring at the site of another
disease process
• more common in >50 years of age
most commonly a/w premalignant condition like
• Paget disease
• Previous radiation treatment
• endochondromatosis
• Fibrous dysplasia
• Osteochondromas
• Osteogenesis imperfecta

9. HISTOPATHOLOGICAL VARIANT
• 1. Conventional type: osteoblastic,
chondroblastic & fibroblastic OS
• 2. Telangiectatic or osteolytic type OS
• 3. Small cell OS
• 4. Low grade central OS
• 5. Periosteal OS
• 6. Paraosteal OS
• 7. Secondary OS
• 8. High grade surface OS
• 9. Extra skeletaL

10. Gross pathology
• Osteoblastic tumor – grayish white hard &
gritty feeling when cut.
• Chondroid type – opalescent & bluish grey.
• Fibroblastic – typical fish flesh sarcomatous
appearance.
• Telangiectatic – areas of tumor necrosis &
blood filled spaces.

11. Clinical Presentation
• Pain– progressive pain particularly with activity
• Swelling - Palpable mass in the region of
metaphysis. - skin over the swelling shiny with
prominent veins. - swelling may be warm &
tender
• Decreased range of motion of the involved joint.
• Lymphadenopathy – unusual focal & regional
lymph node involvement
• Respiratory finding – late stage with lung
metastasis
• Fever & night sweats are rare

12. Investigations:
• Radiological examination
-plain X-rays
- MRI
- CT scan/PET SCAN
-bone scan
• Laboratory studies – serum alkaline
phosphatase. SERUM LDH
• Biopsy – core biopsy, FNAC
• Angiogram

14. Plain X-ray
• Irregular destruction in
metaphysis.
• New bone formation.
• Periosteal reaction.
• Sunray appearance or hair on end
tumor grows into the overlying soft tissue.
• Codman’s triangle:area off sub peri
bone formation seen at the
area of tumor -host cortex junction.

15. MRI :
• (BEST IMAGING MODALITY) used to know the
soft tissue extent and extent of lesion in bone
• Essential for operative planning
• Detect skip metastasis and to evaluate
anatomic relationship with surrounding
structures

16. • Ct scan /pet scan
• Angiogram : Determine vascularity of the
tumour ,Detect vascular displacement and
Relationship of vessels to the tumour
• Bone scan : To look for skeletal metastases or
multi focal disease
• Thallium scan - Monitor effects of
chemotherapy & Detect local recurrence of
tumor

17. laboratory studies
• Full blood count, ESR, CRP.
• LDH (elevated level is associated with poor
prognosis)
• ALP (highly osteogenic)
• Electrolyte levels
• Liver function tests
• Renal function tests
• Urinalysis

18. Biopsy to confirm the diagnosis.
• Types :
1. Fine needle aspiration
2. Core needle biopsy
3. Open incisional biopsy

19. Prognostic Factors
• Age
• Extent of the disease (Pts with pulmonary, non pulmonary
(bone) or skip metastasis have poor prognosis
• Grade of the tumor (High grade tumor have poor
prognosis)
• Size of the primary lesion (Large size tumors have worse
prognosis then small size tumors)
• Skeletal location (proximal tumors do worse than distal
tumors)
• Secondary osteosarcoma: Poor prognosis
• Histologic response to chemotherapy
• Type of surgery and surgical margins

26. Systemic therapy

27. RADIOTHERAPY

28. RADIOTHERAPY:
Treatment of primary tumor:
• Consider RT for Positive margins (R1) or gross
residual(R2) or unresectable disease
• Postoperative RT:55gy With 9 to 13 Gy boost
to microscopic or gross disease(total dose to
high risk sites 64-68Gy)
• Unresectable disease; 60 to 70Gy (Total dose
will depend on normal tissue tolerance )

29. For metastatic disease:
• Consider use of sm153 EDTMP
• Consider use of SRS, especially For
oligometastasis.

30. CASE
DISCUSSION

31. • A 17 years old male r/o karachi with nkcm
presented to us in oncology opd on 20th january
2021 with c/o:
left ankle swelling and pain : 4 months
Diagnosed case of conventional intramedullary
osteoblastic osteosarcoma on biopsy of left ankle

32. MRI TIBIA (17-12-21)
• Intramedullary neoplastic lesion involving Distal
tibia, fibula and talus with ant. Lateral and
posterior muscles involvement. Abnormal
signals identified along superior margins of
calcaneum raising the possibility of its
involvement
• Size is 5.8×5.6×5.0 cm
• Associated with surrounding periosteal reaction
and soft tissue Component With joint effusion.

33. 3 PHASE SKELETAL SCINTIGRAPHY
(8-1-2021)
• Active bone pathology involving Distal end of
left tibia, left fibula and talus Bone
• Focal increased tracer uptake Is seen ovr L2
Vertebra suspicious for mets.

34. CT scan Chest (16-01-2021)
• Pulmonary calcified metastaSis in both lungs
with possible bony deposits

35. Management
• Cisplatin/ doxorubicin × 3cycles (23-03-21)
• Bka (29-04-21)
• Cisplatin /Doxorubicin × 2 cycles (29-06-21)

36. HISTOPATHOLOGY REPORT
• Microscopic viable foci of Residual osteosarcoma
(comprising of 10% Of viable tumor )
• Marked tumor Necrosis (90%) With fibroblastic
replacement Consistent with chemotherapy
response
• Margins:10.5 cm away From skin resection
margin, 18.5 from proximal tibial resection
margin and 20.5 from proximal fibular resection
margin

37. CT SCAN CHEST (12-4-21)
• 6 to 8 nodules in lung parenchyma B/L
Suggesting lung mets
• 4mm in lateral segment of right middle lobe
• 5mm in apical segment of left Lower lobe
• Redemonstration of Small lytic lesion
involving L2 vertebrae On Rt. Side :- stable

38. CT scan Chest (5-8-2021)
• Redemonstration of Interval stable multiple
calcified subpleural and intraparenchymal
pulmonary Nodules likely metastatic.

39. • LOST OF FOLLOWUP AND Patient came BACK
AFTER 1 YEAR IN JULY 2022
• CT SCAN SHOWED disease progression with
Increase in pleuropulmonary mets
• Planned to start chemotherapy with
etoposide/ ifosFamide

40. • During 1st cycle of chemotherapy on 20th july
2022 he developed reaction to drug with
ALOC, DYSPNEA and chest pain
• Cxr showed massive pleural effusion, was
referred to dow hospital where he got
treated and chest tube intubation was done
• Now he presented to us for further
management plan

41. • DISCUSS ABOUT FURTHER TREATMENT
OPTIONS???