BY : DR.ASHWIN CHAIRPERSON: PROF.B.S.NAZEER

INTRODUCTIONy A team comprising an orthopaedic surgeon,

radiologist, pathologist, radiation oncologist, and medical oncologist is necessary to treat the spectrum of musculoskeletal tumors. y Other surgical specialists frequently are required, such as a vascular surgeon, thoracic surgeon, or plastic surgeon. y The orthopaedic surgeon must be well versed in the principles of oncological surgery, and the radiologist and pathologist should have a special interest in bone and soft-tissue tumors.

GENERAL APPROACH TO MUSCULOSKELETAL TUMOURSy An adequate history and physical examination

are the first and most important steps in evaluating a patient with a musculoskeletal tumor. y Patients may present with pain, a mass, or an abnormal radiographic finding detected during the evaluation of an unrelated problem y Patients with bone tumors most frequently present with pain.

y Patients with soft-tissue tumors rarely

complain of pain, but more often complain of a mass.y Although some tumors show a sex predilection

(e.g., female predominance with giant cell tumors), this is rarely of diagnostic significance.y Family history occasionally can be helpful, as

in cases of multiple hereditary exostosis and neurofibromatosis.

y The physical examination include

1) evaluation of the patient's general health 2) a careful examination of the part in question. y A mass should be measured, and its location, shape, consistency, mobility, tenderness, local temperature, and change with position should be noted. y Atrophy of the surrounding musculature recorded, as should neurological deficits and adequacy of circulation. y Caf au lait spots or cutaneous hemangiomas also may provide diagnostic clues.

STAGING OF TUMOURSy Benign and malignant tumors of bone and soft

tissue can be staged according to the

Enneking staging system .y BENIGN

1. Latent 2. Active 3. Aggressive

Enneking Staging (malignant)STAGEIA

GRADELOW

SITEINTRACOMPARTMENTAL

METASTASESNONE

IB

LOW

EXTRACOMPARTMENTAL

NONE

IIA

HIGH

INTRACOMPARTMENTAL

NONE

IIB

HIGH

EXTRACOMPARTMENTAL

NONE

III

ANY

ANY

REGIONAL OR DISTANT METASTASES

AJCC System for staging soft tissue sarcomasStageI

GradeLow

SizeAny

DepthAny

MetastasesNone

II

Low

5 cm

Any

None

High

>5 cm

Superficial

None

III

High

>5 cm

Deep

None

IV

Any

Any

Any

Regional or distant

AJCC System for staging bone sarcomasStageI-A I-B II-A II-B III IV-A IV-B

GradeLow Low High High Any Any Any

Size8 cm >8 cm 8 cm >8 cm Any Any Any

MetastasesNone None None None Skip metastasis Pulmonary metastases Nonpulmonary metastases

BENINGN TUMOURSSTAGE 1 ( LATENT )y lesions are intracapsular. y usually asymptomatic, and frequently

incidental findings. y Radiographic features include a well-defined margin with a thick rim of reactive bone. There is no cortical destruction or expansion. y These lesions do not require treatment because they do not compromise the strength of the bone and usually resolve spontaneously.

STAGE 2( ACTIVE )y lesions also are intracapsular. y are actively growing and can cause symptoms

or lead to pathological fracture. y They have well-defined margins on radiographs but may expand and thin the cortex. y Usually they have only a thin rim of reactive bone. y Treatment usually consists of extented curettage .

STAGE 3( AGGRESSIVE )y lesions are extracapsular. y Their aggressive nature is apparent clinically

and radiographically. They usually have broken through the reactive bone and possibly the cortex. y MRI may show a soft-tissue mass, and metastases may be present in 5% of patients with these lesions. y Treatment consists of extended curettage and marginal or even wide resection, and local recurrences are common.

y A biopsy should be planned as carefully as the

BIOPSY

definitive procedure. Biopsy should be done only after clinical, laboratory, and radiographic examinations are complete. y provide more information leading to a more accurate pathological diagnosis, and avoids artifacts on imaging studies. y Placement of the biopsy incision is a crucial decision because the biopsy track needs to be excised en bloc with the tumor. y If a tourniquet is used, the limb may be elevated before inflation but should not be exsanguinated by compression.

y Transverse incisions should be avoided . y Soft-tissue extension of a bone lesion should be y

y

y

y

sampled, if possible. hole made in the bone, should be round or oval to minimize stress concentration and prevent a subsequent fracture. The periphery of a lesion usually contains the most viable tissue and is the best tissue on which to base a diagnosis. If a tourniquet has been used, it should be deflated and meticulous hemostasis ensured before closure, because a hematoma would be contaminated with tumor cells. A biopsy can be done by fine needle aspiration, core needle biopsy, or an open incisional procedure

ADJUVANT THERAPYy In includes : y 1) RADIATION THERAPY y 2) CHEMOTHERAPY

RADIATION THERAPY Radiation causes cell death by inducing the formation of intracellular free radicals that subsequently cause DNA damage. The goal of radiation treatment is to deliver the highest possible dose of radiation to the tumor cells, while minimizing toxicity to normal tissues.

y After a single treatment of 200 cGy, all cells in

the most sensitive phase of the cell cycle are killed. y Most primary bone malignancies are relatively radioresistant. y Exceptions are the marrow cell tumors, including multiple myeloma, lymphoma, and Ewing sarcoma, which are each exquisitely sensitive. y Radiation therapy is associated with significant acute and long-term complications.

BRACHYTHERAPYy Radiation can be delivered by brachytherapy y y

y y

(from the Greek, brachys, meaning close ). By this method, hollow catheters are implanted in the tumor bed at the time of resection. These catheters exit through the skin. Postoperative radiographic evaluation and computer calculations determine the optimal loading of the catheters with radioisotopes. This technique allows for high doses to be delivered to the target tissues. Currently using this technique more frequently with good results

Brachytherapy catheters

CHEMOTHERAPYy 80% of patients with apparently localized

osteosarcoma actually have undetectable metastases, or micrometastases, on presentation. With the use of modern chemotherapy protocols, the current 5-year survival rate for osteosarcoma is approximately 70%. y Adjuvant chemotherapy refers to chemotherapy administered postoperatively to treat presumed micrometastases. y Neoadjuvant chemotherapy refers to chemotherapy administered before surgical resection of the primary tumor.

y Alkylating Agents

Mustards : Ifosfamide Cyclophoshamide Platinum Compounds : Cisplatin Carboplatin Antimetabolites: Methotrexate Topoisomerase

Interactive Agentsy Antitumor Antibiotics :Doxorubicin

Dactinomycin y Epipodophyllotoxins :Etoposide Antimicrotubule Agents Vinca Alkaloids (Vincristine)

BENIGN BONE TUMOURS1) BONE FORMING TUMOURS

Osteoma Osteoid osteoma Bone island 2) CARTILAGE LESIONS Chondroma Osteochondroma 3) FIBROUS LESIONS Nonossifying fibroma Cortical desmoid Fibrous histiocytoma Fibrous dysplasia Osteofibrous dysplasia Desmoplastic fibroma

3) CYSTIC LESIONS Unicameral bone cyst Aneurysmal bone cyst Intraosseous ganglion cyst Epidermoid cyst 4) FATTY TUMOURS Lipoma 5) VASCULAR TUMOURS Hemangioma 6) NON NEOPLASTIC CONDITIONS Paget disease Brown tumor of hyperparathyroidism

OSTEOID OSTEOMAy Osteoid osteoma is a benign neoplasm most

often seen in young men. y Found in the second or third decades of life. y predilection for the lower extremity, with half the cases involving the femur or tibia. y The tumor may be found in cortical or cancellous bone. y Multicentric lesions have been reported. y No malignant change has ever been documented. y The typical patient with an osteoid osteoma has pain that is worse at night

y Imaging studies usually are diagnostic. y The lesion consists of a small (