This document summarizes information about sarcomas: 1. Sarcomas are malignant tumors that arise from connective tissues like bone, cartilage, fat, muscle, and blood vessels. They can affect many parts of the body and come in different subtypes. 2. Diagnosis involves imaging tests, biopsy of the tumor, and histological examination. Staging evaluates tumor size, spread to lymph nodes and distant organs. Prognosis depends on grade, size, and extent of disease. 3. Treatment may include surgery, radiation therapy, chemotherapy, or a combination. The goal is to remove the tumor while preserving function. Future treatments are exploring targeted therapies. Prognosis depends on grade and stage

1. SARCOMA
MODERATED BY- PRESENTED BY-
DR.HIMANSHU CHAUHAN DR.MRINALINI MATHUR
SENIOR LECTURER
DEPT. OF OMFS

2. Definition
Sarcomas Are Malignant Tumors That Arise From Skeletal And Extra Skeletal Connective
Tissues( Mesenchymal Cells).
Including:
Adipose Tissue
Bone
Cartilage
Smooth Muscle
Skeletal Muscle

3. Types Of Sarcomas
CELL OF ORIGIN SARCOMA TYPE
Adipocyte Liposarcoma
Fibrohistiocyte Malignant fibrous histiocytoma
Fibroblast Fibrosarcoma
Smooth muscle Leiomyosarcoma
Skeletal muscle Rhabdomyosarcoma
Vascular Angiosarcoma, Kaposi’s
Synovial Synovial sarcoma
Melanocyte Malignant melanoma
Unknown Ewing’s sarcoma, Epithelioid sarcoma

4. Classification(By WHO; On The Basis
Of Histology)
1.) Fibrous Tissue Tumour
- Fibrosarcoma
- Adult Fibrosarcoma
- Congenital Or Infantile Fibrosarcoma
2.) Fibrohistocytic Tumours
- Malignant Fibrous Histiocytoma
- Storiform-pleomorphic
- Myxoid
- Giant Cell
- Xanthomatous( Inflammatory)

5. 3.) Lipomatous Tumours
- Well Differentiated Liposarcoma
- Lipoma Like
. Sclerosing
. Inflammatory
- Myxoid Liposarcoma
- Round Cell( Poorly Differentiated Myxoid) Liposarcoma
- Pleomorphic Liposarcoma
- Dedifferentiated Liposarcoma
4.) Smooth Muscle Tumours
- Leiomyosarcoma
- Epithelioid Leiomyosarcoma
5.) Tumours Of Blood Vessels And Lymph Vessels
- Angiosarcoma
- Lymphangiosarcoma
- Kaposi’s Sarcoma

6. 6.) Skeletal Muscle Tumours-
- Rhabdomyosarcoma
- Embryonal Rhabdomyosarcoma
- Botryoid Rhabdomyosarcoma
- Spindle Cell Rhabdomyosarcoma
- Alveolar Rhabdomyosarcoma
- Pleomorphic Rhabdomyosarcoma
7.) Perivascular Tumours
- Malignant Haemangiopericytoma
- Malignant Glomus Tumour
8.) Synovial Tumours
- Malignant Tenosynovial Giant Cell Tumour

7. 9.) Bone And Cartilage Sarcoma
- Osteosarcoma
- Chondrosarcoma
- Giant Cell Tumor Of The Bone
- Ewing Sarcoma
10.) Neural Tumours
- Malignant Peripheral Nerve Sheath Tumour
( Malignant Shwannoma, Neurofibrosarcoma)
- Malignant Granular Cell Tumour
- Clear Cell Sarcoma
- Malignant Melanotic Schwannoma
- Neuroblastoma
- Ganglioneuroma
- Neuroepithelioma

8. 11.) Miscellaneous Tumours
- Alveolar Soft Part Sarcoma
- Epithelioid Sarcoma
- Extra skeletal Ewing Sarcoma
- Synovial Sarcoma
12.) Unclassified Tumours

9. Distribution
-1% Of All Malignant Tumours In
Adults.
-15% Of All Malignant Tumours In
Children.
-More Common In Males.
-Mostly Seen In Older Age.
- Metastasis Mostly To Lungs.

10. Incidence

11. GRADING OF SARCOMAS ( FRENCH
FEDERATION OF CANCER CENTRES SYSTEM FOR SARCOMA GRADING)
PARAMETER CRITERION
A.) TUMOR DIFFERENTIATION
Score 1 Sarcoma Closely Resembling Normal
Adult Mesenchymal Tissue( Ex- Well
Differentiated Liposarcoma)
Score 2 Sarcomas For Which Histologic Typing
Is Certain ( Ex- Myxoid Liposarcoma)
Score 3 Embryonal And Undifferentiated
Sarcomas( Ex- Rhabdomyosarcoma)

12. B.) MITOSIS COUNT
Score 1 0-9/10 HPF
Score 2 10-19/10 HPF
Score 3 ≥20/10 HPF
C.) TUMOR NECROSIS(MICROSCOPIC)
Score 0 No Necrosis
Score 1 ≤50% Tumor Necrosis
Score 2 >50% Tumor Necrosis
Final Score= Score A+ Score B+ Score C
Grade 1 Sarcoma= Score 2-3
Grade 2 Sarcoma= Score 4-5
Grade 3 Sarcoma= Score 6-8

13. STAGING OF SARCOMAS( AMERICAN JOINT
COMMITTEE ON CANCER)
PRIMARY TUMOR(T)
TX Primary Tumor Cannot Be Assessed
T0 No Evidence Of Primary Tumor
T1 Tumor 5cm Or Less In Greatest Dimension
Two Types-
T1a : Superficial Tumor
T1b : Deep Tumor
T2 Tumor More Than 5cm In Greatest Dimension
Two Types-
T2a : Superficial Tumor
T2b : Deep Tumor

14. REGIONAL LYMPH NODES (N)
NX Regional Lymph Nodes Cannot Be Assessed
N0 No Regional Lymph Node Metastasis
N1 Regional Lymph Node Metastasis
DISTANT METASTASIS (M)
M0 No Distant Metastasis
M1 Distant Metastasis
GRADE (G)
GX Grade Cannot Be Assessed
G1 Grade 1
G2 Grade 2
G3 Grade 3

15. PRIMARY TUMOR REGIONAL LYMPH NODES METASTASIS GRADE
Stage IA T1a N0 M0 G1,GX
T1b N0 M0 G1,GX
Stage IB T2a N0 M0 G1,GX
T2b N0 M0 G1,GX
Stage IIA T1a N0 M0 G2,G3
T1b N0 M0 G2,G3
Stage IIB T2a N0 M0 G2
T2b N0 M0 G2
Stage III T2a,T2b N0 M0 G3
Any T N1 M0 Any G
Stage IV Any T Any N M1 Any G

16. ETIOLOGY
► Genetic Factors ► Viruses
Neurofibromatosis Human Herpes Virus 8
Li- Fraumeni Syndrome Epstein-Barr Virus
Familial Retinoblastoma
Gardner Syndrome
► Radiation Exposure ► Trauma
► Chemicals ► Immunological Factors
Thorotrast
Polyvinyl Chloride
Arsenic
Alkylating Agents

17. DIAGNOSIS
1- Clinical History
Age
Sex
Presence Of Genetic Syndromes
Duration And Nature Of Symptoms
2- Physical Examination-
Pain
Paraesthesia And Paresis
Tumour Size
Tumor Location
Fixity/ Mobility
Lymph Nodes Examination

18. 3- Radiographic Imaging Of Soft Tissue Tumours
A.) Radiography
Primary Liposarcoma Of Bone:
Anteroposterior And Lateral
Radiographs Showing An
Aggressive Lytic Lesion

19. B.) Computed Tomography
Primary Liver Sarcoma:
Axial CT Scan Showing A
Multiloculated Cystic Mass

20. C.) Magnetic Resonance Imaging
Schwannoma Of The Popliteal Region:
Left- T1 Weighted Image, Lesion Has A
Homogenously Low Signal Intensity
Right- T2 Weighted Image, The Periphery Of
The Lesion Displays High Signal Intensity But
The Center Area Shows A Low Signal Intensity
Myxofibrosarcoma Of Tibia:
Left- T1 Weighted Image, Lesion Has A
Homogenously Low Signal Intensity
Right- T2 Weighted Image, Lesion Has A
Homogenously High Signal Intensity

21. D.) Ultrasound
Malignant Fibrous Histiocytoma:
Ultrasound Reveals A Hypoechoic Mass

22. E.) Positron Emission Tomography
Sarcoma Of Right Anterior Thigh With
Central Necrosis

23. 4- Biopsy
Types-
A.) Incisional Biopsy B.) Excisional Biopsy

24. C.) Trephine Core Biopsy D.) Fine Needle Aspiration

25. 5- Histology
A.) Embryonal B.) Histiocytic C.) Osteosarcoma
Rhabdomyosarcoma Sarcoma

26. 6- Immunohistochemistry
A. ) Tumor Cells
Showing Vimentin
Positivity
B.) Tumor Cells
Showing Cytokeratin
C.) Tumor Cells
Showing Epithelial
Membrane Antigen
Positivity

27. Complementary Diagnostic Tools
7- Ultrastructural Analysis
- Use Of Electron Microscopy.
- For Example: Neuroendocrine Differentiation In Extra skeletal Myxoid Chondrosarcoma
Was Confirmed Through Detection Of Neurosecretory Granules By Electron Microscopy.
8- Cytogenetics And Molecular Genetics
- Fluorescence In Situ Hybridization(FISH): Used For Chromosomal Analysis.
-It Relies On The Use Of Fluorochrome Labelled Complementary DNA( cDNA) Probes
That Hybridize To Specific DNA Sequences For Direct Cell Visualization.
- Reverse Transcriptase-polymerase Chain Reaction( RT-PCR): Used For Detecting
Abnormal Recombinant mRNA Transcripts. Used In Place Of PCR Because Recombinant
mRNAs Have More Structural Stability.

28. Differential Diagnosis Of Sarcoma
In Oral Cavity
1- Fibrosarcoma
C/F- Fleshy Mass With Ulceration In The Soft Tissue Region.
R/ F- Saucer Shaped Erosion Of The Bone.
H/ F- Proliferation Of Fibroblasts And Formation Of Collagen And Reticular Fibres. Mitotic
Figures Are Prominent In Small Group Of Poorly Differentiated Tumours.

29. 2- Ewing’s Sarcoma
C/F- Rapidly growing swelling with intermittent pain.
R/F - Onion skin appearance
H/F- Sheets of small round cells with very little stroma, although a few connective tissue septa
can be seen.

30. 3- Kaposi’s Sarcoma
C/F- Patch Stage: Pink, Red Or Purple Macule.
Plaque Stage: Large, Raised, Violaceous Plaque.
Nodular Stage: Multiple Nodular Lesion.
H/F-Vascular Channels Lined By Spindle Type Cells.

31. 4- Liposarcoma
C/F- Soft, Yellow Colour Slow Growing, Firm Sometimes Resilient Mass Mostly On The
Cheeks Followed By Tongue.
H/F- Consists Of Fat Cells And Lipoblast In Varying Degrees Of Differentiation

32. 5- Multiple Myeloma
C/F- Oral Amyloidosis With Bleeding Tendency.
R/ F- Punched Out Lesion In The Skull.
H/F- Chromatin Clumping In ‘Cartwheel’ Pattern.
- Bence Jones Proteins In Urine.

33. 6- Leukaemia
C/F- Petechiae, Ecchymosis, Ulceration On The Oral Mucous Membrane. Gingival
Hypertrophy. Lymph Nodes Maybe Enlarged.
L/F- Wbc Levels Increased.
H/F- Hypercellular Bone Marrow With Replacement Of Normal Marrow Elements By
Leukaemic Blast Cells In Varying Degree. Peripheral Blood Smear Shows Mild Anaemia.

34. 7- Osteosarcoma
C/F- Rapidly Growing Swelling Of The Jaw.
R/F- PDL Space Widening, Sunray Appearance, Codman’s Triangle.
L/F- Serum Alkaline Phosphatase Level Is Increased.
H/F- Neoplastic Osteoblasts Are Spindle Shaped Or Polyhedral.

35. 8-Non-hodgkin’s Lymphoma
C/F- Bluish Color Mass Of The Palate With Multiple Lymph Node Involvement.
R/F- Expansion Of Bone With Radiolucency.
L/F- Blood Count Shows Hypersplenism Or Hemolytic Anemia , Reduced WBC, RBC And
Haemoglobin Count.

36. 9- Hodgkin’s Lymphoma
C/F- Discrete Enlargement Of Lymph Nodes , The Lymph Nodes Are Rubbery In
Consistency.
R/F- Foci Of Radiolucency In Jaw.
L/F- Normocytic Normochromic Anemia And Raised ESR.
H/F- Reed Sternberg Cells Are Present, Presence Of Malignant Lymphoid Cells.

37. 10- Chondrosarcoma
C/F- Facial Asymmetry , Swelling Present On The Jaw With Associated Pain And
Tenderness. Paresthesia In Later Stages.
R/F- Moth Eaten Radiolucent Areas With Ill Defined Borders, Flecks Of Radio-opacities
Present.
H/F- Presence Of Hyaline Cartilage.

38. 11- Malignant Fibrous Histiocytoma
C/F- Fast Expanding, Exophytic, Fleshy, Ulcerated Mass. Pain, Hemorrhage, Paresthesia Of
Surrounding Structures.
R/F- Large, Multilocular Radiolucent Area With Severe Expansion Of Cortical Plates.
H/F- Spindle Cells Arranged In Cartwheel Or Storiform Pattern.

39. 12-Angiosarcoma
C/F- Rapidly Growing Lesion That Tends To Ulcerate, Margins Ill-defined, Surface Firm.
R/F- Ill- Defined Destruction Of Bone.
H/F- Irregular Vascular Channels Lined By Endothelial Cells That Are Often Pleomorphic.

40. 13- Leiomyosarcoma
C/F- Rare In Oral Cavity. Lesion Appears As A Painful Swelling. Ulcerations Of Mucosal
Surfaces Present.
H/F- Neoplastic Proliferation Of Spindle-shaped Malignant Smooth Muscle Cells.

41. 14- Neurofibrosarcoma
C/F- Rapidly Growing Mass Present On Lip, Palate And Gingiva. Pain And Paresthesia Present.
R/F- Diffuse Radiolucent Lesion.
H/F- Clumps Of Spindle Shaped Cells Arranged In Streams And Cords With Randomly
Distributed Nuclei.

42. MANAGEMENT
1- SURGERY
:Surgical Resection Remains The Corner Stone Of Therapy For Localized Primary Soft Tissue
Sarcoma.
:Historically, Amputation Was The Primary Treatment For Patients With Extremity STS.
:However, There Has Been A Marked Decline In The Rate Of Amputation As The Primary
Therapy For Extremity STS. The Criteria For Patient Selection For Amputation Include: -
A-.Radiologically Defined Major Vascular, Bony, Or Nerve Involvement Such That A “Limb
Sparing” Primary Tumor Resection Will Result In Critical Loss Of Function Or Tissue
Viability.
B-.Localized Nonmetastatic Disease (Amputation Is Usually Not Considered For Patients With
Established Metastatic Disease).

44. 2- RADIATION
-External Beam Radiation Treatment: Is The Most Commonly Used Radiation Treatment
Technique For Patients With Soft Tissue Sarcomas(Both High Grade And Low Grade)
-Treatment Is Usually Administered On An Outpatient Basis In Daily Fractions Of 1.8 To 2.0
Gy (Monday To Friday) To Total Doses Of 50 Gy (Preop Dose; 5-week Duration) Or 60 To
66 Gy
(Postop Dose; 6½ Weeks).
-Brachytherapy: High Dose Rate >12Gy/h
Medium Dose Rate 2-12 Gy/h
Low Dose Rate < 2Gy/h
-For 4-6 Days
-Only For High Grade Sarcoma
-Treatment On An Inpatient Basis

45. 3- CHEMOTHERAPY
Doxorubicin 50 mg/m²/cycle
Dacarbazine 800 mg/m²/cycle
Ifosfamide 6 g/m²/cycle
Every 2 Weeks With Mesna And Filgrastim Support Following
Surgical Resection Of The Primary Sarcoma.

47. FUTURE TREATMENTS
- Use Of The Peroxisome Proliferator Activated Receptor- Gamma Ligand Troglitazone To
Induce Terminal Adipocytic Differentiation In Liposarcoma.
- Antisense FLI/EWS And Anti-mic2(cd99) for Ewing sarcoma/PNET.
-Tyrosine Kinase Inhibitors(STI-576) for c-Kit Proto-Oncogene Product Expressing
Tumours And Angiogenesis Inhibitors.

48. PROGNOSTIC FACTORS
-Increased Risk 0f Local Recurrence
-Age >50 Years
- Positive Surgical Margins
- Fibrosarcoma
- Malignant Peripheral Nerve Sheath Tumor
- Increased Risk Of Distant Metastasis
- Size > 5cm
- High Grade
- Deep Location
- Recurrent Disease
- Leiomyosarcoma

49. CASE HISTORY-
CC- A 72 Year Old Male Patient With A History Of BFH Reported To The Department Of Oral
Medicine & Radiology With A Chief Complaint Of Swelling And Pain Over The Middle Third
Region Of The Face Since 1 Month.

50. HOPI- Pain Was Severe, Continuous, Localized In Nature, Associated With Swelling And
Had Gradually Increased In Size Over A Period Of 1 Month. A History Of Trauma Was
Elicited In The Middle Third Of Face Initially Around 18 Years Back (In Year 1982)
Leading To The Loss Of Vision Of Right Eye And Fracture Of The Nose. Subsequently,
The Soft Tissue Growth Started Over The Upper Lip And Labial Mucosa. Patient Was
Operated For The Same In The Year 1996 And The Histopathological Report Revealed
Spindle Shaped Cells Arranged In Storiform Pattern And Ovoid Histiocytic Cells. Cellular
Atypia Was Absent, With No Evidence Of Necrosis Or Hemorrhage, Suggestive Of Fibrous
Histiocytoma. The Soft Tissue Growth Recurred In The Year 2001 And Was Surgically
Excised And The Histopathological Report Was Confirmatory For Benign Fibrous
Histiocytoma With No Cellular Atypia. The Patient Visited A Local Dental Practitioner For
Relief Of Pain. An Incision And Drainage Of The Swelling Along With The Extraction Of
Tooth No. 22 Was Done, Followed By A Course Of Analgesics And Antibiotics. There Was
No Significant Relief And The Swelling Persisted, But The Pain Had Reduced.

51. M/H- The Patient Was Diabetic (Type II Diabetes Mellitus) Since The Last 10 Years And Was
On Oral Medication Which Consisted Of Tab. Metformin 500mg 12 Hourly. The Patient Had
Discontinued The Medication Since 6 Months Due To Negligence.
E/O- INITIALLY 20 DAYS LATER

52. I/O-
INITIALLY 20 DAYS LATER

53. RADIOGRAPHS-
HISTOLOGY -

54. TREATMENT-
A wide surgical excision of the soft tissue mass with a segment of the maxillary bone
extending from tooth no. 12 to 23 was done followed by stent placement.

55. REFERENCES
1.- Enzinger And Weiss’s “ Soft Tissue Tumors”.
2.-Elizabeth Montgomery And Alan D. Aaron “Clinical Pathology Of Soft- Tissue Tumors”.
3.- Louis B. Harrison, Roy B. Sessions, Waun Ki Hong “ Head And Neck Cancer: A
Multidisciplinary Approach”.
4.- Malcolm R. Alison “ The Cancer Handbook”.
5.- Robert E. Marx, Diane Stern “ Oral And Maxillofacial Pathology: A Rationale For
Diagnosis And Treatment.”

56. THANK
YOU