Musculoskeletal tumors

Musculoskeletal tumors

bone tumors 1- Primary: A -Benign is common B -Malignant- rare Important -- Either disabling or fatal. Affect young age group 2- Secondary- COMMON 3- Lesions similar to tumors 4- Soft tissue tumors.


Warning signs – bone tumors ●● Non-mechanical bone pain ( no history of trauma) , Especially around the knee in young adolescentsOsteosarcoma is serous differential diagnosis●● Concerning radiographsWhen a musculoskeletal tumors is suspected, clinicians should: ●● stop;●● think;●● investigate.

Learning objectives of musculoskeletal tumors

1- To know the symptoms and signs. 2- staging should be completed before biopsy. 3- Understand the principles of biopsy 4- Describe the principles of surgical treatment 5 - List the aims and types of surgical treatment for bone metastases.

Staging depend onaggressiveness and spread

Bone tumors staged to:1- stage I – A : low grade sarcoma, intra-compartmental. stage I – B : low grade sarcoma, extra-compartmental.2 - stage II – A : High grade sarcoma, intra-compartmental stage II – B : High grade sarcoma, extra-compartmental.3 - stage III : Sarcoma with metastases.

Bone lesion may osteolytic or osteoblastic

Metastases in distal femur
Pathological fracture of the proximal femur through metastatic breast carcinoma.

Classification most system of classification depend on tissue origin and dominant tissue

1- osteoma, osteoid osteoma – osteoblastoma – osteosarcoma.2- chondroma (inside bone; commonest in hands and feet), osteochondroma (cartilage capped; grows away from physis), chondromyxoid fibroma, chondroblastoma (in epiphyses of adolescents)) , chondrosarcoma of varying malignancy.3- fibroma, fibrosarcoma, malignant fibrous histeocytoma.4- haemangioma, haemangiosarcoma.5- giant cell tumors.6- Ewing's sarcoma


Diagnosis of bone tumorsdepend on
1- History and clinical examination: Age, sex, history, physical examination.2- Imaging:X-ray , CT, MRI, Radionuclide scanning.3 – laboratory investigation.4- biopsy.

Biopsy

A biopsy is performed only when staging investigations have been completed. Because removal of the biopsy track is important principle in the treatment of sarcomas, biopsies should be performed in, specialist centre where the definitive surgical procedure will be performed. Image-guided biopsies (usually ultrasound- or CT-guided) have a higher diagnostic accuracy because areas of radiological concern can be targeted. If image-guided biopsy is performed, close discussion between radiologist and surgeon is required to ensure an appropriate biopsy route is used

Open biopsy

Although most biopsies are performed with a needle, sometimes an open biopsy is required, which should be performed according to the following principles.●● A pneumatic tourniquet can be used.●● Use longitudinal incisions that are part of an extensile approach.●● Do not cross anatomical compartments or contaminatecritical anatomical structures (e.g. nerves or blood vessels).●● Use a biopsy track that can be excised at the time of definitivesurgery.●● Ensure specimens are sent for microbiology as well ashistopathology.●● Some specimens should be sent fresh to the laboratory forcytogenetic studies.

Differential diagnosis

1- infection. 2- Soft tissue haematoma and tendon injuries. 3- myositis ossificans. 4- Stress fractures and callus. 5 - Metabolic disorders and dysplasia. 6- Bone infarction.

Method of treatment

1- Tumor excision ( intracapsular, marginal , wide local , or radical). 2- limb sparing surgery. 3- amputation. 4- multi-agent chemotherapy. 5- radiotherapy.

Lesions similar to tumors

1- fibrous dysplasia. 2- fibrous cortical defect. 3- simple bone cyst. 4- anurysmal bone cyst. 5- eosinophilic granuloma.

Fibrous dysplasia

Eosinophilic granuloma

Haemangioma

Haemangioma Most of these tumors are symptomless and discovered accidentally during x-ray examination for another reason.

Fibrous cortical defect(non ossifying fibroma)

Common benign lesion formed of a Cavity filled by Translucent fibrous tissue. Usually discovered accidentally. X- ray show well define osteolytic lesion in cortex of long bone metaphysis surrounded by a thin margin of dense bone. No treatment or bone graft for large lesions.

Simple bone cystsolitary bone cyst or unicameral bone cyst

Appear during childhood typically in metaphysis of long bone, most commonly in proximal humerus , followed by metaphysis around knee. Either presented as pathological fractures or found incidentally. X-ray : well define central osteolytic lesion in metaphysis sometime attached to epiphyseal plate ( active cyst) . Cyst lined by thin fibrous tissue contain straw color fluid.

Treatment of simple bone cyst

Asymptomatic need no treatment. Active cyst treated by aspiration and Depomedrol injection. Aspiration and bone marrow injection is other alternative. If cyst continue to enlarged or pathological fracture occur in weight bearing bone , the curettage and bone graft is the best treatment.

Anurysmal bone cyst

Tumor like lesion, lining membrane is thick soft tissue contain vascular cavities. It affect metaphysis of long bone and vertebra.It is expanding lesion, asymmetrical, thinning cortex.X – ray: well define, trabeculated, eccentric metaphyseal osteolytic lesion.Treated by curettage and bone graft

Osteoid osteoma

It is a benign tumor consisting of osteoid tissue and newly formed bone, it is less than 1 cm in diameter, oval or rounded and is encased by dense bone. Commonly affect the shaft of long bone in young age . The main symptom is severe pain more at night ,relieved by aspirin. X-ray: small radiolucent area surrounded by dense sclerosis. CT show lesion better, radioactive scanning show increased activity. Differential diagnosis include infection, callus of fractures, and tumors like Ewing's sarcoma. Excision of lesion cures the pain by open surgery or ablation by radiofrequency or leaser. CT-guided thermocoagulation Lesion larger than one cm named osteoblastoma.


Tumors producing cartilage●● Osteochondroma – cartilage capped; grows away from physis (joint)●● Enchondroma – inside bone; commonest in hands and feet●● Chondroblastoma – in epiphyses of adolescents●● Chondrosarcoma – of varying malignancy

Osteochondromacartilage capped exostosis

It is the most common tumor of bone. It start as cartilaginous over growth at margin of epiphyseal plate and gradually ossified with growth. It grow with growth of body. Continuous growth of mass after maturity suggest malignant changes. It affect metaphysis, may be single or multiple (diaphyseal achalasia).


Patient may complain of pain, mass, interfere with movement or found accidentally. The exostosis my be covered by tender bursa

X-ray show well define bone mass in metaphysis directed away from joint, look smaller the clinical examination because it covered by cartilage.



Treatment: When exostosis cause symptoms it should be excised, or when it become painful or larger. Malignant transformation is 1-2%

Chondroma ( enchondroma)

Benign cartilaginous tumor .it may be single or multiple (Ollier’s disease). Commonly affect tubular bone of hand and feet and other long bone. Patients may be presented as local pain or pathological fractures.X-ray show well define osteolytic lesion with spot of calcification.The lesion should be excised or curetted and replaced by bone graft.

chondroblastoma

Rare benign epiphyseal osteolytic lesion. Affect end of long bones Presented with pain. X-ray well define epiphyseal osteolytic lesion, it may cross the epiphyseal plate to metaphysis Treated by local excision and bone graft

Chondromyxoid fibroma

Rare benign lesion of metaphysis. X ray show well define osteolytic oval or rounded eccentric, metaphyseal lesion with endosteal sclerosis. Treatment: curettage and bone graft.

Giant cell tumor

Pathology: This disease characterized by presence of giant multinucleated cells which seen in large number. The tumor cell is the stromal spindle shape cell. The tumor is soft and friable occupying the cavity which extend to subchondral region. It occur in mature bone. One third of tumor is benign, one third locally invasive, and one third metastasize.

Clinical features of giant cell tumors

Patients age: usually 20-40 years. More in female. Patient presented by local pain and swelling or pathological fractures. Common site is the distal femur, proximal tibia, distal radius. X-ray show eccentric osteolytic lesion in the end of long bone ,subchondral, trabeculated (soap bubble appearance). The cortex is thin, expanded or even perforated.

Treatment of giant cell tumors

1- curettage and bone graft. 2- curettage and bone cement. 3- excision and bone grafting. 4- excision and prosthetic replacement. 5- radiotherapy for unrespectable lesion.

Osteosarcoma

Definition: primary malignant tumor arise from bone forming cells and produce malignant osteoid tissue. In addition to malignant osteoid tissue fibroblast or cartilage tissue may be predominate. It destroys the bone and form malignant osteoid and spread to surrounding tissue and metastasized to far organs.

Clinical features

Age : 10-20 years commonly but it may occur in old following irradiation and Paget’s disease. Site: metaphysis of long bone specially around knee and proximal humerus is common sites. History: history of trauma may present. Increasing pain, worse at night is the first symptom , swelling may be other symptom. Examination: the affected site swollen with ill define edge , tender, and overlying skin is hot, shiny ,with dilated veins. The ESR is raised.

: X-ray are very variable but it show combination of bone destruction and bone formation. The metaphysis show osteolytic and osteoblastic areas, the cortex is usually perforated and soft tissue shadow may be seen. There is new bone formation in form of Codman's triangle at periphery of when cortex penetration cause periostium elevation and vertical streaks of calcification in the adjacent soft tissues called sunray appearance.

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CT,MRI, RADIOISOTOPE SCANNING needed to show the extent of tumor, to stage the tumor and to plan treatment. Chest x-ray and US to look for metastasizes. Always biopsy is indicated to establish diagnoses.

Treatment:

in developed countries the 5 years survival more than 50% by the use of chemotherapy ,early diagnosis, proper staging and proper surgery. prognosis in our patient is bad. The most certain way of eradicating osteosarcoma is by radical amputation. Chemotherapy used to control metastases and to limit resection to degree that limited resection and prosthetic replacement can be used (limb sparing surgery). Chemotherapy is started soon after operation and continued for at least one year. Inaccessible tumors are treated by radiotherapy combined with chemotherapy.


Ewing’s sarcoma Definition: it is rare primary malignant tumor of vascular endothelial tissue of bone marrow (neuroectodermal origin).it give rise florid periosteal reaction (onion peel ) and early metastases Age: commonly 10-20 years. Site: usually diaphysis of long bone. Clinical features: pain and swelling are the chief presenting symptoms. Fever and features of osteomyelitis may seen. The swelling is ill-define, warm and tender. ESR is elevated.



Ewing’s tumor – histology There is a monotonous pattern of small round cells clustered around blood vessels.


X-RAY: show ill-define osteolytic lesion with overlying periosteal reaction in form of layers (onion peel). Radioisotope scanning show active area. Treatment: chemotherapy together with amputation or radiotherapy used. prognosis in our patient is bad.

chondrosarcoma

Definition: primary malignant tumor of cartilage forming cells. It may be primary and arise from remnant of cartilage in the bone. It may be secondary and arise from previous lesion like cartilage capped Osteochondroma or chondroma. Metastases are usually late.


Age : usually more than 40 years. Clinical features: pain , swelling . swelling and pain over previous cartilage capped Osteochondroma or chondroma or pathological fractures are the common presenting feature.

Imaging : X-RAY: ill-define medullary osteolytic lesion with flecks of calcifications or large exostsis with ill-define margin with flecks of calcifications in cartilage cap. CT, MRI, Radioisotope scanning used to establish extent of lesion and to plan treatment. Treatment: wide local resection applied when possible, amputation , radiotherapy and chemotherapy are other alternative.

Adamantinoma

Rare tumor of shaft of tibia. Affect young adult, presented as pain and swelling. The lesion formed of epithelial like cell in dense stromal cell. X-ray show multilocular osteolytic lesion of tibial cortex. It is low grade malignant tumor rarely metastasized. Treated by excision with adequate margin of normal bone.

Fibrosarcoma and malignant fibrous histeocytoma.

Both rare These tumor affect adults and old patients, Pain and pathological fractures are common presentation. X-ray show ill define osteolytic lesion in bone. Treatments by surgical resection and chemotherapy

Multiple myloma

Malignant disorder of B cell (plasma cell) of lymphoproliferative tissue of marrow, stimulate osteoclastic activity result in osteoporosis and multiple small osteolytic lesion. Presented usually by pain and pathological fractures

Secondary metastases

The commonest bone tumors is secondary metastases. It had bad prognosis. It arise from breast, prostate, lung, kidney ,thyroid and gastrointestinal tract. Some time no primary tumor can be found even after meticulous search. Metastases usually appear in central skeleton (bone contain red marrow) like vertebra, pelvis, ribs, and proximal end of femur and humerus.


Carcinomas usually spread to bone by blood : the spine is the third most common site for metastases, after the lung and liver. Although most patients with metastatic cancer will have bone metastases in the spine before they die, only 10% are symptomatic.Tumor cells metastasize to the spine via Batson’s venous plexus. These retroperitoneal veins have no valves and allow retrograde embolic spread to the spine and proximal longbones (

Most metastases are osteolytic by replacing bone by their gradual expansion and by stimulating active bone resorption. Rarely metastases are osteoblastic( prostate ca.). Clinical features: common age 50-70 years. Common symptom is local pain, pathological fracture or local pressure may occur. The primary tumor might be obvious. Detailed clinical examination including neck, breast, axillae, abdomen, chest, genitalia and (PR&PV) are essential.

Investigations : x-ray of chest and urogental tract. CBP, ESR, protein electrophoresis, serum alkaline and acid phosphatases. Radioisotope scanning may reveal increased activity of other unsuspected lesion. X-RAY: Commonly there were ill-define moth eaten osteolytic lesion. Sometime there are osteoblastic deposits.

Treatment

The treatment are mainly palliative. Quality of life improved by careful management. Pathological fractures , large osteolytic lesion or neurological compression need surgical fixation with bone cement and decompression. Radiotherapy chemotherapy, hormone therapy and analgesia ere used also. Prophylactic internal fixation my used.

Synovial sarcoma

Malignant tumor of joint or tendon sheath synovial tissue in adult and adolescent. Patients usually complain of painful swelling in large joints or related to tendon sheath. X-ray show soft tissue swelling with extensive calcification ( snowstorm) , MRI is useful. Biopsy done to confirm diagnosis. Treated by wide excision in early cases, amputation in advance lesion with chemotherapy and radiotherapy.