Radiology of Bone Tumors Part 2 Dr P Tripathi

Medical education

1. Bone Tumours Dr Prassan Tripathi (MS) Assistant professor R.S.D.K.S.G.M.C. AMBIKAPUR Part-2

2. Osteosarcoma

3. Most common malignancy of the bone is SECONDARIES. Most common primary malignancy of the bone is MULTIPLE MYELOMA. SECOND MOST COMMON PRIMARY MALIGNANCY OF THE BONE IS OSTEOSARCOMA.

4. PROBLEM STATEMENT Accounts for 20% of primary malignancies of bone. Untreated it is highly lethal With surgery alone the 5 yr survival is 20% With the combined modalities of surgery and chemotherapy the 5 yr survival rate in many centers has touched as high as 75%

5. I ncidence- higher in males M:F ratio is 3:2 Locations - All skeletal locations can be affected; THE LOCATION IS ALMOST ALWAYS METAPHYSIS Primary osteosarcomas occur at the sites of the most rapid bone growth- THE DISTAL FEMUR, THE PROXIMAL TIBIA PROXIMAL HUMERUS.

6. CLASSIFICATION (ON THE BASIS OF CLINICAL SETTING) TWO TYPES :- PRIMARY OCCURS IN 15-25 YEARS OF AGE PROPER ETIOLOGY UNKNOWN SECONDARY OCCURS AFTER 40-45 YEARS OF AGE UNCOMMON Associated with PREMALIGNANT CONDITIONS Eg : PAGETS DISEASE, MULTIPLE ENCHONDROMATOSIS, OSTEOCHONDROMAS, FIBROUS DYSPLASIA

7. CLASSIFICATION OSTEOBLASTIC-OSTEOBLASTS PREDOMINATE CHONDROID TYPE-CHONDRBLASTS PREDOMINATE FIBROBLASTIC-FIBROBLASTS PREDOMINATE OSTEOLYLIC OR TELANGIECTATIC (WORST PROGNOSIS) (on the basis of histopathology)

8. ALL OSTEOSARCOMAS ARE MALIGNANT AND METASTASIS OCCURS VIA THE HAEMATOGENOUS ROUTE –BLOOD STREAM REMEMBER SARCOMAS LYPHATIC SPREAD IS UNCOMMON ALMOST ALWAYS THE FIRST METASTASIS IS TO LUNGS

9. PATHOLOGY MALIGNANT TUMOUR OF MESENCHYMAL CELLS CHARACTERISED BY BONE FORMATION / OSTEOID FORMATION BY MALIGNANT CELLS

10. GROSS APPEARANCE GROSS APPEARANCE IS OF VIVID TYPES DEPENDING UPON THE TYPE OF OSTEOSARCOMA THE CONVENTIONAL OSTEOBLASTIC TYPE TYPICALLY IS GREYISH WHITE,HARD AND HAS A GRITTY FEELING WHEN CUT. MOST TUMOURS HAVE MIXED AREAS OF BONE FORMATION AND CYSTIC SPACES.

11. Gross specimen Resected Osteosarcoma Osteosarcoma of the distal femur shows irregular expansion of bone by a sclerotic mass with erosion of the cortex and extension into soft tissue

12. HISTOPATHOLOGY HISTOLOGICALLY CHARACTERISED BY “MALIGNANT MESENCHYMAL SPINDLE SHAPED CELLS SURROUNDING THE OSTEOID”

13. Histology of Osteosarcoma-Osteoid deposition

14. CLINICAL FEATURES PAIN – 1 ST SYMPTOM SOON FOLLOWED BY SWELLING HISTORY OF TRAUMA IS ALMOST ALWAYS PRESENT ONLY INCIDENTAL TAKES PATIENTS ATTENTION TOWARDS THE SWELLING. PATHOLOGICAL FRACTURE LOCAL INVASION-NEUROVASCULAR DAMAGE FUNGATING MASS

15. SEVERE SWELLING IN THIGH-PATIENT HAD PRESENTED WITH A PATHOLOGICAL FRACTURE

16. CUT SECTION THROUGH THE TUMOUR

17. EXAMINATION SWELLING – IN THE METAPHYSIS SKIN OVERLYING IS SHINY WITH PROMINENT VEINS ILL DEFINED MARGING MOVEMENTS RESTRICTED DUE TO SIZE OF THE TUMOUR REMEMBER THE TUMOUR NEVER CROSSED THE EPIPHYSEAL PLATE

18. INVESTIGATIONS:- 1. X-RAY-1 ST INVESTIGATION 2. MRI-GIVES INFORMATION ABOUT THE EXTENT OF TUMOUR AND SOFT TISSUE AND BONY INVOLVEMENT 3.BIOPSY-GOLD STANDARD IN DIAGNOSIS OF BONE TUMOURS/HIGHEST SENSITIVITY AND SPECIFICITY HRCT CHEST-FOR SECONDARIES/METS

19. X-RAYS IRREGULAR BONY DESTRUCTION IN THE METAPHYSIS NEWBONE FORMATION IRREGULAR PERIOSTEAL REACTION CODMANS TRIANGLE SUNRAY APPEARNCE

20. IRREGULAR BONY DESTRUCTION WITH NEW BONE FORMATION

21. CODMANS TRIANGLE-SUBPERIOSTEAL NEW BONE FORMATION

22. Sunburst appearnce

23. BLOOD INVESTIGATIONS SERUM ALKALINE PHOSPHATASE LEVELS:- BONE FORMING TUMOUR -HIGH LEVELS OF SERUM ALKALINE PHOSPHATASE LEVELS PROGNOSTIC IMPORTANCE /NOT DIAGNOSTIC LEVELS FALL AFTER AMPUTATION LEVELS RISE BACK IN RELAPSE/STUMP CARCINOMA

24. TREATMENT DESPITE ALL TREATMENT –VERY POOR PROGNOSIS VERY LOW 5 YEAR SURVIVAL RATE

25. BEST TREATMENT IS RADICAL AMPUTATION-AMPUTATION AT A LEVEL ONE JOINT ABOVE THE LESION EG ABOVE KNEE AMPUTATION FOR A TUMOUR OF TIBIA HOWEVER WITH CHEMOTHERAPY SOME RECOMMEND A SAFE MARGIN OF 10 CM FOR RESECTION. RADIOTHERAPY HAS ABSOLUTELY NO ROLE AS THE TUMOUR IS RADIORESISTANT.IS RESERVED FOR PATIENTS REFUSING SURGERY OR TUMOUR AT INACCESSIBLE SITES

26. CURRENT PROTOCOL IS : NEO ADJUVANT CHEMOTHERAPY FOLLOWED BY AMPUTATION/WIDE RESECTION FOLLOWED BY ADJUVANT CHEMOTHERAPY

27. CHEMOTHERAPY USES METHOTREXATE WITH CITROVORUM FACTOR(FOLINIC ACID) CISPLATIN ENDOXAN

28. SECONDARIES ARE TREATED BY CHEMOTHERAPY SOLITARY LUNG METASTASIS CAN BE RESECTED OUT.

29. SECONDARY OSTEOSARCOMA OCCURS IN PREMALIGNANT STATES:- PAGETS DISEASE DIAPHYSIAL ACHLASIA OSTEOCHONDROMA FIBROUS DYSPLASIA MULTIPLE ENCHONDROMATOSIS POST IRRADIATION

30. EWING SARCOMA

31. UNDIFFERENTIATED PRIMITIVE NEUROECTODERMAL TUMOUR (P-NET FAMILY) DIAPHYSEAL TUMOUR-***ALMOST ALWAYS OCCURS IN DIAPHYSIS

32. Highly malignant tumour Found in young age 5-15 years of age More commonly male population is affected

33. PATHOLOGY MOST COMMONLY OCCURS IN DIAPHYSIS OF FEMUR FOLLOWED BY FIBULA AND THEN ILEUM AND THEN TIBIA. IN ONE THIRD CASES IT MAY ALSO OCCUR IN FLAT BONES USUALLY PELVIS AND THE CALCANEUM

34. COMMON CYTOGENIC ABNORMALITY:- ****CYTOGENIC TRANSLOCATION OF CHROMOSOMES 11 AND 22 t(11:22) seen in 85-90 % of cases. Followed by t(21:22) followed by t(7:22)

35. PATHOLOGY Tumour characteristically involves a large area almost all of the Medullary Cavity. The tumour is grey white, soft and thin almost like pus. The bone expanded and periosteum elevated with the new bone formation occuring in layers giving rise to the onion peel appearance which is the characteristic radiological feature . The tumour ruptures from the cortex and extends into the soft tissues.

36. histopathology Histopathology is characteristic Consists of sheets of uniform lymphocyte like round cells. Sometimes these cells surround a central clear area forming a pseudo-rosette. *** round neural cells containing glycogen and reticulin which are PAS positive and diastase digestible.

37. Histological picture of ewing sarcoma

38. Clinical features This is one malignancy which is associated with systemic symptoms**** such as weight loss ,fever,raised ESR and anemia. Lab abnormalities like leukocytosis,raised LDH and ESR may be present. And others signs and symptoms are like with other tumours PAIN SWELLING TENDERNESS PATHOLOGICAL FRACTURE RESTRICTED JOINT MOVEMENTS NEUROVASCULAR COMPRESSION

39. INVESTIGATIONS 1. X ray 2. MRI 3.Biopsy 4.HRCT chest for metastasis

40. Extremely poor prognosis *** Bone to Bone metastasis is seen Most common metastsis is to the lung Micrometastasis is almost always there at the time of diagnosis.

41. Radiological finding mottled rarefaction of the bone with permeation of the overlying cortex indicating rapid bone destruction Soft tissue mass Lamellated periosteal reaction with new bone formation known as onion peel appearance

42. Ewing sarcoma of the radius

43. TREATMENT The tumour is highly radiosensitive and chemosensitive It is said it melts like snow on radiotherapy However it will always recur. Chemotherapy includes Vincristine,Adriamycin and Cyclophosphamide.

44. Treatment Neoadjuvant chemotherapy Followed by Amputation Followed by adjuvant chemotherapy/radiotherapy.

45. THANK YOU!

46. STAGING OF BONE TUMOURS