Presentation is loading. Please wait.

Presentation is loading. Please wait.

Rare tumors (< 2%) More in young ages

Similar presentations


Presentation on theme: "Rare tumors (< 2%) More in young ages"— Presentation transcript:

1 Rare tumors (< 2%) More in young ages
Bone tumors Rare tumors (< 2%) More in young ages

2 Outline Introduction (signs, symptoms, investigation) Benign tumor
Malignant tumor Metastatic bone tumor

3 Classification Origin Primary
Secondary (95%): breast, lung, prostate, kidney and thyroid cell type: Bone Osteoma, osteosarcoma Cartilage Chondroma, Chondrosarcoma Marrow Hemangioma, angiosarcoma Fibrous tissueFibroma, fibrosarcoma Tumor type: Benign: Osteoma, osteochondroma Malignant:: Osteosarcoma, chondrosarcoma

4 Symptoms and Signs Asymptomatic and discovered accidentally.
General: fatigue, fever, wt. loss Mass can be felt at the tumor site. Pain: May worsen at night and awakes patient, caused by Tumor compression on surrounding tissue Hemorrhage in the tumor Pathological fractures Swelling Local tenderness Warmth Pathological fracture: may be the first sign.

5 Malignant vs. Benign Tumors
Rapid growth Warmth Tenderness Ill defined edges All are suggestive of malignancy.

6 Investigations History and examination
Imaging (x-ray, CT, MRI & radionuclide scanning) Biopsy Labs Ca & P: Greater than normal levels may indicate bone metastasis. PTH: Lower than normal levels may indicate bone metastasis. ALP isoenzyme: Higher than normal ALP levels may indicate Paget's disease, osteoblastic bone cancers, osteomalacia and rickets. LDH: High values indicate poor prognosis

7 Plain x-ray Most useful Could see: Lump Cysts Bone destruction
Cortical thickening & periosteal reaction Important to notice: Where How many Cystic or not Margins Destruction

8 Periosteal reaction Periosteal hypertrophy which develops in response to periosteal irritation. They are a non-specific sign. They have many causes: Infections Tumors (both benign & malignant) Benign tumors are thick & smooth or completely absent Malignant tumors are thinner & irregular (wavy). Healing fractures Chronic stress

9 Periosteal reactions seen in the distal tibia and fibula.

10 Periosteal reaction at the distal radius with irregular edges: malignant.

11 Osteosarcoma of the distal femur: Codman’s triangle can be seen: periosteum is being “lifted off”.

12 Midshaft periosteal reaction with smooth + thick edges: this is a benign osteoma.

13 Periosteal reaction of tibia healing from a fracture showing a smooth and thick.

14 CT and MRI Asses the extent of the tumor Relation to surrounding structures Radionuclide scanning: Helpful in revealing site of a small tumor Skip lesions Silent secondary deposits

15 Multiple hot spots seen: lung cancer which has metastasized to vertebrae.

16 Biopsy Allows us to reach a diagnosis.
Allows us to plan for treatment. Methods: Open biopsy: surgical procedure done under GA. Zonal biopsy: open biopsy from transition zone. Excisional biopsy: excision of the entire tumor. Large–bore needle biopsy: aspirating cells. Complications: Hemorrhage Wound break down Infection Pathological fractures

17 Benign tumors

18 Benign bone tumors Non ossifying fibroma
Osteochondroma (the Commonest) Osteoid osteoma Enchondroma Giant cell tumor of bone Osteoblastoma

19 Most bone tumors are benign
Most often are asymptomatic Unlikely to spread Usually found in the biggest bones (femur, tibia & humerus)  Some types are more common in specific places (near the growth plates) Appearance and location of mass on radiographs are keys to diagnosis.

20 Osteochondroma (exostosis)
Commonest benign tumor of bone More in male <25y. Solitary Mature bone with cartilaginous cap. Common sites are the fast growing sites of long bones (lower end of femur and upper end of tibia), crest of ileum & shoulder. Flattened (sessile) or stalk-like (exostosis) Most likely caused by Congenital defect or trauma of the perichondrium, which results in the herniation of a fragment of the epiphyseal growth plate through the periosteal bone cuff. Osteochondromas Small risk of malignant transformation(<1%)

21 Symptoms: A hard, immobile, detectable mass that is painless Lower-than-normal-height for age Muscle soreness One leg or arm may be longer than other Pressure or irritation with exercise X-Ray: Exostosis: well defined bony projection Calcified cartilage in large lesions Treatment: excision if becoming bigger and more painful.

22 Well defined bony projection

23 Solitary osteochondroma.
Lateral radiograph of a sessile osteochondroma of the distal femur

24

25 Non-ossifying fibroma
Fibrous tissue within bone ossiffy in time Asymptomatic, incidental findings Metaphysis of long bones Occasionally multiple lesion Developmental defect, seen in children As bone grows, defect becomes less obvious May enlarge and cause pathological fracture No treatment, unless there is pathological fracture

26 Well marginated radiolucent lesion
Distinct multilocular appearance

27 Osteoid osteoma Neoplastic proliferation of osteoid and fibrous tissue
More in male < 30 year more in vertebra or long bones Less in mandible or other craniofacial bones. Small in size (<1.5 cm) Oval or rounded shape Pain (severe) worse at night ,not relieved by rest but relieved by aspirin. Types: Cortical, cancellous, subperiosteal. Treatment: Complete removal after careful localization by CT.

28 X-Ray Nidus: tiny radiolucent area
Diaphysis  surrounded by dense bone and thickend cortex Metaphysis  less cortical thickening

29 Male 23 years old History of increasing pain in the knee & relieved by aspirin.

30 Plain radiograph in a 25-year-old male with cortical osteoid osteoma
Plain radiograph in a 25-year-old male with cortical osteoid osteoma. shows a radiolucent nidus surrounded by fusiform cortical thickening

31 a 25-year-old male with cortical osteoid osteoma.
Radionuclide bone scan shows focal intense activity at tumor site.

32 6y old child who presented with left hip pain.
Well-defined area of sclerosis surrounded by a ring of radiolucency in the left femoral neck. Note the absence of periosteal reaction that suggests intramedullary or cancellous osteoid osteoma.

33 Transaxial CT scan through the proximal shaft of the right femur in a 17-year-old boy.
Osteoid osteoma adjacent to the endosteal margin of the cortex. Central sclerotic focus in the radiolucent nidus

34 Osteoid osteoma of transverse process of vertebra

35 Primary Secondary (metastasis)
Malignant tumors Primary Secondary (metastasis)

36 Uncommon cancer Most commonly affects the long bones 20 % of pediatric bone tumors are malignant. 66% of adult bone tumors are malignant, most commonly mets. The most common type of bone cancer in adults is metastatic cancer from other organs

37 Primary bone cancer Risk factors: Radiotherapy & chemotherapy
Paget's disease Family Hx (hereditary retinoblastoma) Signs & symptoms Fever, Night sweats, Fatigue & Unintended weight loss Bone pain that often is nocturnal Swelling & tenderness near the affected area Pathological fractures

38 Osteosarcoma Most common primary bone malignancy
Incidence: 2.8 per million M >F Age years (the 8th most common childhood cancer) Prognosis Aggressive tumor Metastasis to the lung 5-year survival Without mets is 70% With mets is 25%

39 Where Mainly affects metaphysis of long bones More in: Knee
Distal femur Upper tibia Humerus (prox.end) Maxilla

40 Clinical features Pain: Swelling Redness Hotness Tenderness
Dull aching Progressive Constant Worse at night Swelling Redness Hotness Tenderness Pathological fracture

41 DDX Stress fracture Ewing's sarcoma Osteomyelitis Osteochondroma
Osteoblastoma Bone cysts Chondroblastoma Chondrosarcoma Giant cell tumor

42 Diagnosis: Radiological studies: X-Ray CT-scan Bone scan & MRI Bone biopsy, the only definitive method to determine whether a tumor is malignant or benign. Treatment: Surgical resection Preoperative & postoperative chemotherapy

43 X-ray findings Lesion Cortical destruction
Extension to the marrow or soft tissue Codman’s triangle a term used to describe the triangular area of new subperiosteal bone that is created when a lesion, often a tumour, raises the periosteum away from the bone. Sunburst Effect Osteosarcomas can be Predominantly osteolytic Predominantly osteoblastic Mixture

44 Sunburst Appearance

45 Clinical appearance of a teenager who presented with osteosarcoma of the proximal humerus
Swelling throughout the deltoid region Disuse atrophy of the pectoral muscule

46

47

48 White arrow: codman triangle
Black arrow: soft tissue mass

49 Patholigical fracture

50

51

52

53

54 Ewing sarcoma A malignant round-cell tumor.
Rare disease (incidence 0.6 per million 2nd most common bone malignancy in pediatrics. M>F Age years Usually the lesions are diaphyseal Mets (30%), most commonly in the lungs & other bones & less commonly in the bone marrow.

55 Most common areas: Pelvis Femur Humerus Ribs Clavicle

56 Clinical feature: Pyrexia Pain: Constant Increase with movement Limping Swelling, warm, tender & red Radiological studies: X-Ray Lytic medullary lesion Onion skin appearance CT-scan Bone scan & MRI

57 White arrow: onion skin apperance
Red circle: sunburst periosteal reaction Blue circle: osteolytic lesion

58 Periosteal reaction Osteolytic lesion

59

60 Periosteal reaction Osteolytic lesion

61 Periosteal reaction Osteolytic lesion

62 Treatment: Local radiotherapy combined with systemic chemotherapy In young children amputation may be necessary due to severe compromise of bone growth. Prognosis, 5-year survival 50% with the 1st approach 75% with the 2nd approach

63 Metastatic bone tumor

64 Most common malignant lesion of the bone.
The most common sites of spread of cancers are lung, liver & skeleton. Carcinomas are much more likely to metastasize to bone than sarcomas Typically multifocal BUT renal and thyroid carcinomas produce only a solitary lesion. Common sites for metastasis: Vertebrae, pelvis, proximal parts of the femur, ribs, proximal part of the humerus, and the skull. Batson venous plexus >> more mets to the axial skeleton Hands & feet are rare 50% of them originate from lung neoplasms. Mets: Direct extension Retrograde venous flow Seeding with tumor emboli via the blood circulation

65 Destructive expanded osteolytic lesion in the metacarpal of the thumb in a 55-year-old man with lung carcinoma.

66 Mets (adults) Osteoblastic behaviour Osteolytic behaviour Prostate
Stomach Bladder Breast Osteolytic behaviour Lung Kidney Colon Thyroid Breast

67 Typical x-ray appearance of osteolytic bone metastases
Typical x-ray appearance of osteolytic bone metastases. This plain pelvic x-ray film of a 75-year-old patient with breast carcinoma shows multiple osteolytic bone lesions. =>decrease in bone density.

68 Typical x-ray appearance of osteoblastic bone metastases
Typical x-ray appearance of osteoblastic bone metastases. This plain pelvic x-ray film of a patient with prostate cancer shows multiple osteoblastic metastases to the pelvis and lumbar (L4) and sacral (S1) vertebral bodies.=>increase in bone density

69 Mets (kids) Neuroblastoma Wilm’s tumor Osteosarcoma Ewing’s sarcoma
Rhabdomyosarcoma

70 Presentation Pain which results in reduced mobility.
Bone weakness which predispose to pathologic fractures. Palpable masses (large bony lesions). Neurologic impairment due to spinal epidural compression. Anemia (decreased red blood cell production) is a common blood abnormality in these patients. Past history of primary malignant tumor symptoms, BUT others did not complain of anything before.

71 A radiograph of a destructive pathological fracture on the left hip, in a man with metastatic renal cell cancer. The patient underwent replacement of the upper femur due to extensive destruction of the bone around the hip.

72 Approach History & physical examination Radiological studies
Plain X-ray MRI CT scan Bone scan (Technetium-99m) Laboratory studies Biopsy

73 Radiological studies X-ray: destruction of bone and/or lucent Lesions of Bone Bone scan: most cost-effective and available whole-body screening test for the assessment of bone metastases. CT Useful in evaluating suspicious bone scintiscan findings Useful in guiding needle biopsy, particularly in vertebral lesions. MRI Help in detecting metastatic lesions before changes in bone metabolism Helpful in determining the extent of local disease in planning surgery or radiation therapy.

74 Hot spots: Increased osteoclastic activity

75

76 X-ray RadioIsotope Pt. presented with pain in the right upper thigh
X-ray showing METS in upper 1/3 of the femur Radioisotope scan revealed many deposits in other parts of the skeleton.

77 Treatment Divided into: Systemic therapy Local therapy Chemotherapy
Hormone therapy Immunotherapy Local therapy Radiation therapy Surgery

78 Treatment Radiation therapy combined with chemotherapeutic or hormonal agents, is the most common treatment modality. Early use of radiation and bisphosphonates (zoledronic acid, pamidronate) slows bone destruction. Some tumors are more likely to heal after radiation therapy: Blastic lesions of prostate and breast Lytic destructive lesions of lung and renal cell Surgery is indicated in fractures or large metastatic mass.

79 A woman with advanced metastatic breast cancer to bone
Pain in both her right and left hips A special partial hip replacement was necessary on the right because the hip joint was involved. On the left, a special nail could be used to strengthen the femur bone below the hip.

80 Thank You Good Luck


Download ppt "Rare tumors (< 2%) More in young ages"

Similar presentations


Ads by Google