1. Musculoskeletal tumors
Prepared by
Dr Fadel Naim
Orthopedic Surgeon
IUG

2. Early detection, proper techniques of diagnosis and treatment can dramatically improve the chances of achieving functional limb salvage.

3. Classification of Bone tumors
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. Metastatic neoplasms
Metastatic neoplasms primary origin can be in descending frequency :
Prostate
Breast.
Lung.
GIT.
Kidney.
Thyroid.

5. Symptoms and Signs It may be asymptomatic and discovered accidentally.
Pain: may worsen at night and awakes patient, caused by tumor compression on surrounding tissue, hemorrhage in the tumor, pathological fractures also cause pain
Swelling.
Increase in size :
Bleeding, malignant change
Local tenderness.

6. Pathological fracture:
Warmth
Pathological fracture:
may be the first sign.
With any diaphyseal fracture in the elderly, malignancy should be ruled out .
General: fatigue, fever, wt. loss
A mass can be felt at the tumor site.
Loss of function
Neurovascular involvement
Deformity

7. Malignant vs. Benign Tumors
“Rapid growth, warmth, tenderness, and ill defined edges are suggestive of malignancy.”

8. Benign vs. Malignant

9. investigations History and examination Imaging Biopsy Labs
Calcium: Greater than normal levels may indicate metastasis.
Serum phosphorus: 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

10. Plain x-ray Most useful Could see: Important to notice: A lump
Bone destruction
Cortical thickening+ periosteal reaction
Cysts
Important to notice:
site
properties
Number (monostotic or polyostotic)
Effect and reaction (aggressive / non aggressive )

11. Four questions of Enneking
Where is the lesion?
What is the lesion doing to the bone ?
What is the bone doing (endosteally and periosteally) ?
What is in the lesion?

12. Four questions of Enneking
1. Where is the lesion?
Medullary centric
Medullary eccentric
Cortical
Juxtacortical
Diaphyseal
Metaphyseal
Epiphyseal

15. Four questions of Enneking
2. What is the lesion doing to the bone ?
Usually causes 30-40% bone destruction before detection
destructive and ill-defined
circumscribed and localized

16. Patterns of Bone Destruction
Geographic
Moth-eaten
Permeative
Less malignant
More malignant

17. Benign lesions with aggressive features
Giant cell tumour
Chondroblastoma
Aneurysmal bone cyst
Unicameral bone cysts
Aggressive GCT

18. Four questions of Enneking
3. What is the bone doing (endosteally and periosteally ) ?
Clue to the biologic activity
Smooth periosteum, benign process
Onion-skin ( ewing’s sarcoma )
Sunburst reaction ( osteosarcoma )
Codman’s triangle

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

20. “Onion skin” layers of reactive bone formation
Codman triangle
a triangle of reactive bone formation found at the proximal and distal extremes of the elevated periosteum.
“Onion skin” layers of reactive bone formation
represent a more competent response; successive waves of tumor expansion create layers of reactive bone formation.

23. sunburst reaction Codman’s triangle

24. Onion-skin

25. Periosteal Reactions Solid Sunburst onion-peel Codman’s triangle
Less malignant
More malignant

27. Four questions of Enneking
4.What is in the lesion?
Neo-bone formation
Osteoid forming process
Stippled calcification
Cartilaginous

28. Cartilaginous
Expansile Lesions
Osteoblastic

29. Lytic
Blastic

30. Number

31. Leukemia
Multiple Myeloma

32. Radionuclide scanning:
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

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

34. Angiography Diagnostic : Therapeutic :
Feeding vessels identified as well as the tumor proximity to major vessels
Therapeutic :
Embolisation of vascular tumors prior to surgery , Intra-arterial chemotherapy

35. Biopsy of Bone Tumors Longitudinal incision.
Should know probable diagnosis and stage of tumor before biopsy
Performed by the surgeon who will perform the definitive surgery
do not biopsy what you are not equipped to treat.
Meticulous haemostasis to avoid tracking haematomas
Send samples for microbiological analysis
Longitudinal incision.
sharp dissection through the muscle.
Uninvolved compartments should not be exposed
Windows in bone should be oval and small

36. Biopsy A biopsy is performed after appropriate staging studies
Alternatives include
Fine needle aspiration (FNA)
A simple procedure that can be done using local anesthesia.
Most helpful in diagnosing soft tissue tumors and bony tumors with homogenous cell types
Obtains the least amount of material.
Core needle biopsy
May be aided by ultrasonography, fluoroscopy, computed tomography (CT), and magnetic resonance imaging (MRI).
The surgeon obtains a core of tissue that measures 10 mm by 2 mm.
open biopsy or incisional, biopsy
A surgical procedure that provides the largest amount and the best sampling of tissue for pathologic identification.

38. CT guided FNA confirmed…

39. MANAGEMENT PRINCIPLES
AIMS:
safe margins,
minimum recurrence,
minimum morbidity

40. Surgical Margins : 1. Intra-lesional
through the tumour , leaves macroscopic tumour ,,not therapeutic .
100% recurrence
For benign lesions or for debulking

41. 2. Marginal Through pseudo-capsule of tumour / reactive zone
controls non-invasive benign tumours
recurrence of malignant tumours = 25-50%

42. 3. Wide around reactive zone, leaving a cuff of normal tissue
skip lesions left
recurrence of malignant tumours = < 10%

43. 4. Radical removal of entire compartment or compartments
distant metastases left

44. 5. Amputation
should be thought of as a form of reconstruction where surgical control of the tumor precludes useful function.

45. • Criteria for limb salvage:
1. local control of the lesion must be at least equal to amputation ( no skip lesions )
2. the saved limb must be functional

46. Limb Salvage versus Amputation
The surgeon must determine whether a limb salvage operation or an amputation will provide the best outcome in terms of postoperative function and local control.
Amputation usually provides better postoperative function and reduced risk of recurrence for tumors located in the distal leg
Limb salvage surgery should be considered for treatment of tumors in the more proximal aspect of an extremity, because an amputation at this level would be very disabling.

48. Rotationplasty is a new solution to disfiguring surgical resections of lower limb sarcomas:

49. Benign Bone Tumors Osteoid Osteoma Osteoblastoma Osteochondroma
Enchondroma
Nonossifying Fibroma
Fibrous Dysplasia
Langerhans Cell Histiocytosis
Giant Cell Tumor of Bone
Solitary Bone Cyst
Aneurysmal Bone Cyst

50. Osteoid Osteoma a unique and relatively common benign bone tumor
The typical age at presentation is 5 to 25 years, with peak incidence in the second decade.
M:F is 3:1
Radiographs show a radiolucent zone (the nidus) surrounded by a halo of increased density ( a sclerotic zone ).

52. Pain associated with an osteoid osteoma is frequently worse at night and often is dramatically relieved by nonsteroidal anti-inflammatory drugs (NSAIDs(

53. Treatment options include
Observation
surgical excision
CT-guided radiofrequency ablation (RFA)

54. Osteoblastoma
an uncommon benign tumor with histologic and radiographic similarities to osteoid osteoma
occurs in a slightly older population
frequently involves the posterior elements of the vertebrae
usually does not cause night pain
Patients do not report the striking relief of pain with NSAIDs
Radiographs show a radiolucent zone and a thin margin of reactive bone. Treatment is en bloc marginal excision.

56. The most common benign bony tumor
Osteochondroma
The most common benign bony tumor
Most are located at the metaphyseal portion of long bones, particularly the distal femur and proximal tibia.
May be solitary and idiopathic or multiple and genetic (called hereditary multiple exostosis).
Solitary osteochondroma has a male-to-female ratio of 2:1

57. Patients are generally in the 2ed decade
Seek medical advice because of a bony mass.
Activity-related pain occurs because the mass irritates surrounding muscles.
Compression of an adjacent nerve causes radicular pain or paresthesia.

58. Histologic examination demonstrates a bony stalk covered by a cartilaginous cap

59. May undergo malignant transformation, usually to a chondrosarcoma
May undergo malignant transformation, usually to a chondrosarcoma. (1% to 2% )
Occurs primarily in patients with hereditary multiple exostosis during the adult years.
Signs suggestive of malignant transformation include pain and continued growth of the osteochondroma after skeletal maturity

60. Treatment for a symptomatic osteochondroma is marginal excision that includes the cartilaginous cap and the overlying perichondrium.
Recurrence is uncommon.

61. Enchondroma Relatively common benign tumor that may occur at any age
More than half of cases involve the small bones of the hands and feet.
Other common sites are the metaphysis of the distal femur and the proximal humerus.
Many enchondromas are asymptomatic and are discovered by chance on radiographs taken for other reasons

62. Maffucci’s Syndrome
Multiple enchondromatosis with soft tissue hemangiomas

63. Ollier’s Disease
Multiple enchondromatosis, especially involving the hands and feet.
One extremity is affected more than the other

64. Asymptomatic solitary enchondroma may be treated with observation.
Large lesions in the hand that are expanding the cortex or that have caused a pathologic fracture can be treated by curettage with or without bone grafting

65. Nonossifying Fibroma
very common
causes no symptoms and rarely requires treatment
discovered incidentally during radiography for an unrelated injury
typically occur in the metaphysis of the femur or tibia.

66. Radiographs show a radiolucent lesion that is eccentric (abutting one cortex) and surrounded by a rim of sclerotic, reactive bone

67. Giant Cell Tumor of Bone
A distinct, benign, but locally aggressive lesion of unknown origin
Occurs in young adults after closure of the growth plate.
A slight female predominance
Common locations include the distal femur, proximal tibia, and distal radius.

68. Radiographic features include a lytic, eccentric lesion that involves the epiphysis and metaphyseal region of bone.
The cortex is frequently thin and often expanded.
The subchondral bone may be eroded.

70. Typical presenting symptoms include
localized pain
Swelling
reactive joint effusion.
Giant cell tumors are unpredictable
Occasionally, malignant transformation occurs.

71. Treatment options include
curettage
bone grafting
filling of the defect with cement.
Recurrent lesions or lesions that have extended into the joint require wide- margin excision and reconstruction

72. Solitary Bone Cyst A relatively common pediatric bony lesion M:F = 2:1
Common locations include
proximal humerus
proximal femur.
Patients typically experience pain after a pathologic fracture occurs.

74. The “falling-leaf” sign
Active cysts
abut the growth plate
occur more often in patients younger than 10 years
associated with a higher recurrence rate.
Latent cysts,
seen in older children
separated from the physis by a margin of normal bone.
Thicker bony cortex indicates progressive ossification and healing.
The “falling-leaf” sign
a fragment of the eggshell-thin cortex that was displaced into the cavity after a pathologic fracture.

75. Treatment of active lesions or lesions at risk of fracture includes
injection therapy with
Steroids
autologous bone marrow
another substance with the goal of promoting bone formation and healing.
Lesions of the proximal femur are at greater risk for a disabling fracture.
Flexible intramedullary nails or other fixation devices may be used as internal struts.
The perforation of a cyst by these devices stimulates the healing process.

76. Aneurysmal Bone Cyst (ABC)
Is a reactive process
Rapidly expansile lesion with multiple blood filled cystic cavities.
Age 5-20
Rapid onset of pain

77. Osteosarcoma represents only 0.2% of all malignancies
The most common primary malignant bone tumor (excluding multiple myeloma)
Usually develops during adolescence and affects males more often than females

78. May arise anywhere 70% of these lesions develop in the metaphysis of
distal femur
proximal tibia
proximal humerus

79. Standard treatment now includes
Before effective chemotherapy was developed, approximately 80% of patients died of metastases, even after prompt amputation
Standard treatment now includes
Biopsy confirmation of diagnosis
staging studies
preoperative chemotherapy for 11 weeks
surgical resection
postoperative chemotherapy for several more weeks.

80. Improved reconstructive techniques have increased the number of limb-sparing operations to approximately 80%.
With current treatment, the 5-year disease-free survival rate for persons with stage II osteosarcoma is approximately 70%.

81. Chondrosarcoma less common and grows more slowly than osteosarcoma
Primary chondrosarcomas
located centrally within the medullary canal
more common among males
a peak incidence at between 30 and 60 years of age.
Frequent sites include the proximal femur, pelvis, proximal humerus, and scapula.
Secondary chondrosarcomas
typically associated with a previous osteochondroma
located on the periphery of bone, but they may occur in an extraosseous soft tissue location.

84. Low-grade chondrosarcomas may be difficult to differentiate from benign osteochondromas or enchondromas.
Radiographs show a permeative radiolucent lesion with areas of calcification that may be diffuse (“salt and pepper” pattern) or more discrete (“popcorn” pattern).
The incidence of distant metastases is related to the degree of malignancy.

85. Chondrosarcoma is treated by surgical resection.
Radiation therapy is not helpful
Chemotherapy is reserved for patients with metastases

86. Ewing Sarcoma
The second most common malignant bone tumor, after osteosarcoma
The age range is 5 to 25 years, with peak incidence at between 10 and 20 years
more common among males.
The most common site is the femur, followed by the tibia, humerus, pelvis, and fibula

87. Pain is the most common presenting symptom.
In advanced cases, systemic symptoms such as fever and malaise may be noted
the condition may mimic osteomyelitis

88. Radiographs show a permeative pattern of destruction in the diaphysis or the metaphyseal portion of bone.
Thinning and erosion of the cortex is common and a Codman triangle, or “onion skin” periosteal reaction, may be seen

90. After appropriate staging and biopsy have been performed
the patient is treated with inductive chemotherapy.
The tumor then is further evaluated for treatment with wide-margin surgical resection or irradiation.
The survival rate is approximately 70% for patients without metastases

91. Lipoma Very common benign tumor
Subcutaneous tissue of the trunk and limbs
in the middle-aged and elderly
Soft, slowly growing mass
Lipoma variants:
Angiolipoma: thin-walled small blood vessels occupy significant portion of the lesion
Spindle-cell lipoma: mixture of mature adipocytes, short bundles of collagen and small uniform spindle cells

92. Liposarcoma The most common malignant soft tissue tumor
Adults (peak incidence years)
Site: lower limb and retroperitoneal space
Subtypes:
Well-differentiated lipoma-like liposarcoma (atypical lipomatous tumor): closely resemble lipoma, but a few spindle or stellate cells with hyperchromatic atypical nuclei, high reccurence rate, no meastases
Myxoid liposarcoma: small undifferentiated mesenchymal cells ad lipoblast set in a myxoid stroma, plexiform „chicken-wire“ network of capillaries, good prognosis, rare metastases
Pleomorphic liposarcoma: mixed cell population (spindle cells, tumor giant cells and small number of lipoblasts), poor prognosis, frequent metastases

93. Leiomyoma Leiomyosarcoma
Skin, subcutaneous tissue, uterus, gastrointestinal tract
Leiomyosarcoma
Mesentery, retroperitoneal space, wall of large veins, skin, subcutaneous tissue, deep soft tissues of limbs
Signs of malignancy:
large size, high mitotic rate, areas of necrosis, marked cellular pleomorphism