PAIN :- is most frequent symptom -deep constant pain,poorly localised,worse at night -initially controlled by analgesics,later requires narcoticsMASS:- may be painful/painless - rate of enlargement is important -Fluctuating mass can be cyst,ganglion or hemangioma -Family H/O masses near the joint may be indicator of Ollier’s disease orMaffucci SyndromeNEUROLOGICAL SYMPTOM:- found in few patients such as sacral tumors & withtumors located near the nerve causing compression of nerve,especially common insciatic notch ,inguinal canal & popliteal fossaUNEXPLAINED SWELLING OF THE LOWER EXTREMITY:- found in pelvic tumorswhich are painless & without a palpable mass & cause swelling due to compression ofiliac vein.Patient may present with an abnormal radiographic finding detected during evaluationof unrelated problemPatient may also come with complaint of weight loss, respiratory , gastro or genito-urinary disturbances.
Carried out in 4 phases –1st phase – involves High index of suspicion for tumors Meticulous history Thorough physical examination Routine X-rays Routine lab facilities2nd phase – is prebiopsy regional evaluation to determine size,location and type of tissue in involved in the tumor. This includes CTand MRI3rd phase – is the actual biopsy.4th phase – is undertaken if presumptive clinical & path evidence issuggestive of malignancy. Here search for metastasis is done usingCT/HRCT of lung & Tc-99 bone scan.
AGE:- most important as most tumors present in specific age group. 1st decade- usually ABC ,SBC 2nd decade-Chondroblastoma,osteosarcoma,Ewings 3rd decade- GCT 4th decade- chondrosarcoma 5th decade- Multiple myelomaSEX:- less imp than age Some tumors like GCT are more in femalesFAMILY HISTORYRACE:- little imp, Ewings rare in african descentH/O any exposure to radiation Tt or Carcinogens- bone seekingradionucleotide can cause sarcoma.Various chemlcal carcinogens- methylcholanthrene,zinc berylliumsilicate, beryllium oxide.Currently the most worrisome & controversial is Nickel which is usedin many orthopedic devices.
Evaluation of patient’s general healthTUMOR 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 tumorsCafé-au-lait spots & subcutaneous neurofibromas indicate Von Recklinghausen’sdiseaseA venous malformation on the same of body as the cartilagenous tumor is anindicator of Maffucci SyndromeREGIONAL LYMPH NODES:should be carefully palpated for signs of metastaticdiseaseAtrophy of surrounding musculature/ fasciculations should be recordedNeurological deficitsCirculation should be assessed and compared with opposite site.Joints, specially proximal and distal to the mass should be carefully examined forrange of motion, discontinuity or intra-articular masses.
HemoglobinCBCESRCRPPrastrate specific antigen, PAPElectrophoresis & urinary Bence Jones proteinAlkaline 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 tumorsPTH testSerum phosphorusIonized calcium and serum calcium
It is the most conclusive testTypes : Open Incisional Biopsy 1.Incisional 2. Excisional Closed percutaneous core Biopsy
The Enneking system for the surgical staging of bone and soft-tissuetumors is based on grade (G), site (T), and metastasis (M) and useshistologic, radiologic, and clinical criteria.GradeG0 - Benign lesionG1 - Low-grade malignant lesionG2 - High-grade malignant lesionSiteT0 - A benign tumor that is confined within a true capsule and the lesions anatomiccompartment of origin (ie, a benign intracapsular, intracompartmental lesion)T1- An aggressive benign or malignant tumor that is still confined within its anatomiccompartment (ie, an intracompartmental lesion)T2 - A lesion that has spread beyond its anatomic compartment of origin (ie, anextracompartmental lesion)MetastasisM0 - No regional or distant metastasisM1 - Regional or distant metastasis
Stage Grade Site MetastasisIA Low G-1 Intracompartmental - T1IB Low G-1 Extracompartmental - T2IIA High G-2 Intracompartmental - T1IIB High G-2 Extracompartmental - T2III Any G Any T Regional/ distant metastasis
Stage Definition Behaviour Example1 Latent Remains static or Non-ossifying heals spontaneously fibroma2 Active Progressive but Aneurysmal bone limited by natural bone cyst barriers3 Locally Aggressive Progressive growth, GCT not limited by natural barriers Barriers- Cortical bone, articular caritlage, joint capsule, fascia, tendinous origin and inertion sites of muscles
Primary tumour (T) TX: primary tumour cannot be assessed T0: no evidence of primary tumour T1: tumour 8 cm in greatest dimension T2: tumour > 8 cm in greatest dimension T3: discontinuous tumours in the primary bone siteRegional lymph nodes (N) NX: regional lymph nodes cannot be assessed N0: no regional lymph node metastasis N1: regional lymph node metastasisDistant metastasis (M) MX: distant metastasis cannot be assessed M0: no distant metastasis M1: distant metastasis M1a: lung M1b: other distant sitesG Histopathological GradingLow GradeHigh Grade
Stage T N M GIA T1 No/Nx Mo G1/2 Low GradeIB T2 No/Nx Mo G1/2 Low GradeIIA T1 No/Nx Mo G3/4 HighIIB T2 No/Nx Mo G3/4 HighIII T3 No/Nx Mo Any GIVA Any T No/Nx Any M Any GIVB Any T N1 Any M Any G Any T Any N M1b Any G
Classification (W.H.O.) Bone-forming tumours Cartilage forming tumours Giant-cell tumour Marrow tumours Vascular tumours Other connective tissue tumours Other tumours Secondary malignant tumours of bone
Bone forming tumours Benign Malignant Osteoma Osteosarcoma Osteoid osteoma or Central (Medullary) osteoblastoma Peripheral (Surface) Intermediate Parosteal Aggressive osteoblastoma Periosteal High grade surface