Musculoskeletal tumors Prepared by Dr Fadel Naim Orthopedic Surgeon IUG

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

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

Metastatic neoplasms Metastatic neoplasms primary origin can be in descending frequency : Prostate Breast. Lung. GIT. Kidney. Thyroid.

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.

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

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

Benign vs. Malignant

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

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 )

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?

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

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

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

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

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

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

“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.

sunburst reaction Codman’s triangle

Onion-skin

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

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

Cartilaginous Expansile Lesions Osteoblastic

Lytic Blastic

Number

Leukemia Multiple Myeloma

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

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

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

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

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.

CT guided FNA confirmed…

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

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

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

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

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

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

• 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

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.

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

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

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 ).

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

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

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.

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

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.

Histologic examination demonstrates a bony stalk covered by a cartilaginous cap

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

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

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

Maffucci’s Syndrome Multiple enchondromatosis with soft tissue hemangiomas

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

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

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.

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

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.

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.

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

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

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.

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.

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.

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

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

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

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.

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%.

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.

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.

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

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

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

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

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

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

Liposarcoma The most common malignant soft tissue tumor Adults (peak incidence 40-60 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

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