BONE TUMORS

BONE TUMORSDr Barun Kumar PatelAPOLLO HOSPITALS BHUBANESWAR

Qns1. discuss the principles of limb salvage surgery in malignant bone tumor. List the indications and contraindications?2.describe clinical feature , radiology ,and treatment of non ossifying fibroma ?3.discuss the anatomy of parathyroid gland, describe the clinical feature , radiological presentation of adenoma of parathyroid gland. What is hungry bone syndroma.

4. briefly describe methods to cover defects after excision of primary malignant tumors of bone .what is extracorporeal irradiated tumor bone ?5.discuss the pathology ,clinical features and management of synovial chondromatosis ?6. write shortnotes on giant cell varients , fibrous dysplacia ?

7. define giant cell tumor of bone , describe in brief clinical features ,diagnosis and management principles of GCT of upper end of tibia.8. Indication of limb salvage surgery in malignant bone tumors , describe the techniques of limb salvage in osteosarcoma ?

9.briefly describe the clinical features and pathology of ewings sarcoma . Outline the principles of treatment in a case of ewing sarcoma of upper end of humerus ?10.discuss the various methods available for treatment of giant cell tumor of proximal tibia in a 30 yr old man ?

INITIAL EVALUATIONCarried out in 4 phases 1 st phase involvesHigh index of suspicion for tumorsRoutine X-rays Routine lab facilities Meticulous history Thorough physical examination 2 nd phase -is prebiopsy regional evaluation, to determine size,location and type of tissue involved 3 rd phase is the actual biopsy. 4 th phase is undertaken if presumptive clinical & path evidence sugestive of malignancy, search for mets is done, using CT scan of lung & Tc-99 bone scan

PRESENTING SYMPTOMSPain Mass An abnormal radiographic finding detected during evaluation of unrelated problem PAIN:- is most frequent symptom -deep constant pain,poorly localised,worse at night -initially controlled by analgesics,later requires narcotics MASS:- rate of enlargement is important -Fluctuating mass can be cyst,ganglion or hemangioma -Family H/O masses near the joint may be indicator of Olliers disease or Maffucci Syndrome

ContNEUROLOGICAL SYMPTOM:- found in few patients such as sacral tumors & with tumors located near the nerve causing compression of nerve,especially common in sciatic notch ,inguinal canal & popliteal fossa

UNEXPLAINED SWELLING OF THE LOWER EXTREMITY :- found in pelvic tumors which are painless & without a palpable mass & cause swelling due to compression of iliac vein

HISTORY OF THE PATIENTAGE:- most imp information, bcoz of their presentaion in specific age group. 1 st decade- usually ABC ,SBC 2 nd decade-Chondroblastoma,osteosarcoma,Ewings 3 rd decade- GCT 4 th decade- chondrosarcoma 5 th decade- Multiple myeloma SEX:- less imp than age Some tumors like GCT are more in females

ContRACE:- little imp, Ewings rare in african descent H/O any exposure to radiation Tt or Carcinogens- bone seeking radionucleotide can cause sarcoma. Various chemlcal carcinogens- methylcholanthrene,zinc beryllium silicate, beryllium oxide.Currently the most worrisome & controversial is Nickel which is used in many orthopedic device

PHYSICAL EXAMINATIONEvaluation of patients general health TUMOR MASS should be measured & its location, shape, consistency, mobility, tenderness, local temp & change with position should be noted. SKIN & SUBCUTANEOUS TISSUE :Small dilated superficial veins overlying the mass are produced by large tumors REGIONAL LYMPH NODES: sign of metastatic diseaseAtrophy of surrounding musculature should be recorded,also neurological deficits & adequacy of circulation

LABORATORY TESTAlkaline phosphatase test: Normally, this enzyme is present in high levels when bone-forming cells are very active . High levels of alkaline phosphatase can also be an indicator of bone tumors (when the tumor creates abnormal bone tissues). PTH test: Lower-than-normal level of parathormone can be an indicator of bone cancer.Serum phosphorus: Higher than normal levels of phosphorus can be an indicator of bone cancer.Ionized calcium and serum calcium: Higher than normal levels of calcium can be an indicator of bone cancer.

ContOTHER TESTS Hemoglobin CBC ESR CRP Glucose tolerance test PSA,PAP Electrophoresis & urinary Bence Jones protein

INVESTIGATIONSX-RAY CT SCAN MRITECHNETIUM BONE SCAN-This type of scan uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone. PET- Positron Emission Tomography uses radioactive glucose to locate cancer. This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera

BIOPSYThe biopsy is the most conclusive as it confirms if the tumor is malignant or benign, the type (primary or secondary ), and stage According to the tumor size and type and purpose (to remove entire tumor or only a small tissue sample), biopsies can be : needle biopsy , incisional biopsy , excisional biopsy1. Needle biopsy: a small hole is made in the affected bone and a tissue sample from the tumor is removed. There are two types of needle biopsies: Fine needle aspiration : During this procedure, the tissue sample is removed with a thin needle attached to a syringe Core needle aspiration : a small cylinder of tissue sample is removed from the tumor with a rotating knife like device. 2. Incisional biopsy : During this procedure, the surgeon cuts into the tumor and removes a tissue sample.3.excisional biopsy: entire lesion is cut

Principles of biopsy Biopsy should be done after clinical, laboratory, and radiographic examinations are complete biopsy track should be considered contaminated with tumor cells , biopsy track needs to be excised en bloc with the tumorTransverse incisions avoided because they are difficult to excise with the specimen

deep incision through a single muscle compartment not to contaminating an intermuscular planeMajor neurovascular structures be avoided Soft tissue extension of bone lesion should be sampled because leading edge contains most viable tumor for making diagnosisIf tourniquet used,limb elevated before inflation but not to be exsanguinated by compression to prevent squeezing the tumors cells into the systemic circulation

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ContIf a hole is made in bone, should be round or oval to minimize stress concentration and prevent a subsequent fracture, which could preclude limb salvage surgery The hole be plugged with methacrylate to limit hematoma formation sample more than just the pseudocapsule surrounding the lesion

Contfrozen section should be sent intraoperatively to ensure that diagnostic tissue has been obtained If drain used, it should exit in line with incision so that the drain track can be easily excised en bloc with tumorwound should be closed tightly in layers

Cont

classification Revised WHO Classification Schajowicz(1994)

STAGINGStaging of benign bone tumours as described by Ennekingstages of benign tumors designated by Arabic numbers, and malignant tumors by Roman numerals stage 1, latent; stage 2, active; and stage 3, aggressive

Stage 1 -lesions are intracapsular, usually asymptomatic, and frequently incidental findingsRadiographic features well-defined margin with thick rim of reactive bone. no cortical destruction or expansion. not require treatment not compromise the strength of the bone resolve spontaneously

Stage 2- intracapsular actively growing cause symptoms or pathological fractureRadiographs-well-defined margins expand and thin cortex. thin rim of reactive bone Treatment -extended curettage

Stage 3 extracapsular broken through reactive bone and cortexMRI shows soft tissue mass, and metastases may present in 5% of patientsTreatment -extended curettage marginal or wide resection, local recurrences are common.

The staging system for malignant tumors adopted by the Musculoskeletal Tumor Society, and originally developed by Enneking is based on the histological grade, the local extent, and the presence or absence of metastasisBone sarcomas are broadly divided as follows: Stage I All low-grade sarcomas. Stage II Histologically high-grade lesions. Stage III Sarcomas which have metastasized

stage I-lesions are well-differentiated, have few mitoses, and exhibit moderate cytological atypiarisk for metastases is low (5 cm in greatest dimension), depth (superficial or deep to the fascia), and presence of metastases

PRINCIPLES OF SURGERY AMPUTATION VERSUS LIMB SALVAGEissues to be considered whenever contemplating limb salvage instead of an amputation 1. Would survival be affected by the treatment choice? 2. How do the short-term and long-term morbidity compare? 3. How would the function of a salvaged limb compare with that of a prosthesis? 4. Are there any psychosocial consequences?

involvement of neurovascular structures, displaced pathological fracture, complications secondary poorly performed biopsy preclude limb salvage procedures choice between limb salvage and amputation made on basis of expectations and desires of patient and family multimodal treatment including surgery and chemotherapy, improved long-term survival for patients.

patients with a local recurrence despite wide margins represent aggressive or chemotherapy-resistant disease , has poor outcomes regardless of surgical procedure most important aspect of surgical procedure is attainment of a wide margin regardless of achieved by amputation or local resection

Amputation often requires nonstandard flaps for closure or bone graft augmentation for a more functional residual limb Complications - infection, wound dehiscence, chronically painful limb, phantom limb pain, and bone overgrowth requiring revision surgery

Limb salvage is associated with greater perioperative and long-term morbidityComplications- greater risks of infection, wound dehiscence, flap necrosis, blood loss, and dvt long term complications like periprosthetic fractures, prosthetic loosening or dislocation, nonunion of the graft-host junction, allograft fracture, leg-length discrepancy, and late infectionmore likely to need multiple future operations for treatment of complications including amputation

regard to function, location of tumor is most important issueResection of upper extremity lesion with limb salvage, even sacrificing one or two major nerves, provides better function than amputation and prosthetic fittingresection of a proximal femoral or pelvic lesion with local reconstruction provides better function than after hip disarticulation or hemipelvectomy

Around ankle and foot, large sarcomas treated with amputation and prosthetic fitting osteosarcoma around the knee treated with wide resection with prosthetic knee replacement or transfemoral amputation osteoarticular allograft reconstruction, allograft arthrodesis, and rotationplasty are less prefered

patients with amputations had difficulty walking on steep, rough, or slippery surfaces but active and least worried about damaging limb Patients with arthrodesis performed most of physical work but had difficulty with sitting, especially in back seats of cars, theatersPatients with arthroplasty led more sedentary lives , protective of limb but had less difficulty with activities of daily living

probability of limb survival after resection depends on type of reconstruction and location of tumor(most imp issue). proximal reconstructions outlasting distal reconstructions. ( inverse of the prognosis for patient survival, with distal sarcomas better prognosis than proximal ones.)

No study has shown significant difference between amputation and limb salvage with regard to psychological outcome or quality of life in long-term survivors of sarcoma. patient ultimately make the final decision in light of long-term goals and lifestyle decisions.

MARGINS In oncological surgical procedure, the surgical margin must be appropriately defined orthopaedic oncology, surgical margin described by one of four : intralesional, marginal, wide, or radical

intralesional margin- plane of surgical dissection is within the tumor appropriate for symptomatic benign lesions when the surgical alternative would be to sacrifice important anatomical structures , or as a palliative procedure in case of metastatic disease

marginal margin -plane of dissection passes through pseudocapsule(surrounding reactive tissue referred as pseudocapsule) treat most benign lesions and some low-grade malignancies. marginal resection leaves microscopic disease leading to local recurrence in high grade malignanciesmarginal resection preferable if alternative is more mutilating procedure

Cont..Wide margins -plane of dissection is in normal tissue no specific distance , entire tumor remains completely surrounded by rim of normal tissue quality of margin is more important than the quantity (thickness) of the margin wide margins are goal of most procedures for high-grade malignancies

Radical margins -all compartments containing tumor removed en bloc previously the procedures of choice for most high-grade neoplasms amputations defined further by any of the four margins

CURETTAGEMany benign bone tumors treated by curettage curettage is associated with higher rate of local recurrence than resection, but allows a better functional result PRINCIPLES OF CURETTAGEdone by first making large cortical window over lesion at least as large as the lesion itself

Next, cavity is enlarged to normal host bone in each direction with a power burr Finally, cavity and wound TO be copiously irrigated to remove any debris and tumor cells Extended curettage - use of adjuvants, such as liquid nitrogen, phenol, polymethyl methacrylate, or thermal cautery to extend destruction of tumor cells

filling the cavity-through autogenous bone graft, allograft, demineralized bone matrix, artificial bone graft substitutes, or bone cement Autogenous bone graft must be harvested using different set of instruments to prevent contamination of the donor site

Autogenous bone graft provides most rapid and most reliable healing rate as it is osteogenic, osteoinductive, and osteoconductive But is associated with morbidity harvest site, may not available in sufficient quantity to fill a large cavity cancellous allograft (only osteoinductive)incorporated easily, available in large quantities and not involve further operative morbidity

demineralized bone matrix used as filling agent after curettage of benign bone tumors is osteoconductive and osteoinductiveArtificial bone graft substitutes ( calcium sulfate, calcium phosphate) are osteoconductive, easy to use, and readily available used alone or in combination with autogenous bone graft, bone marrow aspirates, or demineralized bone matrix

bone cement is also used as a filling agenthas the advantage of providing immediate stability, makes rehabilitation easier and lessens risk of pathological fracture another advantage of bone cement is detection of local recurrence recurrent tumor recognized as an expanding lucency adjacent to bone cement