Malignant bone tumors nPazourek L., Ondrůšek Š., Rozkydal Z. Osteosarcoma Malignant osteoid Epidemiology n3 new cases /1 milion/ year n2. decade nMetaphysis of long bones n 1/2 in knee region n distal femur n proximal tibia n proximal humerus n Classification nPrimary –Central nHigh-grade –Conventional high-grade (80 – 90%) nOsteoblastic nChondroblastic nFibroblastic –Telangiectatic nLow-grade n Peripheral High-grade Low-Grade Parosteal Periostal n Secondary n - in Paget´s disease of bone - post radiation Symptoms npain –during night, in rest nswelling npathological fracture nmetastases in the time of diagnosis n in 10-25 % of patients Diagnostics nX-ray nCT / MRI nScintigraphy nChest X- ray or spiral CT nUltrasonography nBiopsy – excisional, needle NguenThiThijTrang91boč DoleželDavid75boč DoleželDavid75ap WirthPatrik98ap Conventional osteosarcoma YanarsanovDzhabrail95ap HubálkováDana80 OndrováMichaela87ap JurášZdeněk85ap Conventional ostesarcoma VajdíkovíMarie60ap VajdíkovíMarie60boč Parosteal osteosarcoma OSA nMissed case nWrong prognosis JurášZdeněk85ap JurášZdeněk85boč Oncologic reflex Therapy nneodjuvant chemotherapy nsurgery – radical resection / amputation nadjuvant chemotherapy nMetastasectomy in lungs n nChemotherapy: (EURAMOS protocol) –metotrexat, doxorubicin, adriamycin, cisplatina, ifosfamid, etoposid. nIn low-grade OSA – only surgical treatment n nOSA is a radioresistant tumor n Prognostic factors nMetastases nSize of the tumor nAxial localisation nRadicality of surgery nResponse to chemotherapy n Prognosis – 5 years survival n n70% - conventional high-grade OSA without MTS and with good respone to chemotherapy (to 10 % of vital tumor cells) n n n90% - u low-grade OSA after radical surgery n n Chondrosarcoma Epidemiology n10% of primary malignant bone tumors nAge: –primary: 40 – 60 years –secondary: 25 – 45 years nLocalisation- n pelvis, proximal femur, proximal humerus Etiology nSecondary –Multiple enchondromas (M.Ollier, – Maffucci sy –Exostosis disease – cartilage over 2 cm –Chondroblastoma, chondromyxoid fibroma … BřeskýJan81RTG Calcifications IlkováIvan88RTG Chondrosarcoma HrabalFrantišek42RTG1 HrabalFrantišek42CT1 JeřábekJosef74RTG Chondrosa- Fabík 1 Chondrosarcoma GojováMilada44RTGboč GojováMilada44RTGap Chondrosarcoma KrejsaJoef43RTGap KrejsaJoef43RTGboč Koukalová Ludmila44RTGboč Koukalová Ludmila44RTGap Chondrosarcoma Therapy nRadical resection – wide resection, amputation nMetastasectomy in lungs n nChemoresistant tumor n nRadioresistant tumor Prognosis nPrognostic factors: –Radicality of surgery –Size –Histological grading nIn intralesional surgery – 90% risk of local recurrence and lung metastases n nPrognosis: –Conventional low-grade 90% 10 years –Conventional high-grade 20-40% 10 years –Dediferenciated sarcoma 15% 5 years Ewing sarcoma family Group of high grade malignant round cells bone tumors with neuroectodermal differentiation and specific translocation. • Ewing sarcoma • PNET (periferal neuroectodermal tumor • Askin tumor of the chest wall • Neuroblastoma in adults Epidemiology nOne new case /1 mil./ 1 year n n5-25 years nIn metaphysis of long bones with extension into diaphysis and n in flat bones (pelvis, scapulla) Symptoms npain nswelling nFever, redness, nLeucocytosis, ESR elev. nBiopsy- + identification of specific gene translocation t(11,22)q(24,12) JedličkaPetr00 OujezdskýFilip83ap Ewing sarcoma VilémJakub88ap MRIinic3 nik19862 nik19864 Ewing sarcoma DudaPavel86ap DudaPavel86boč Ewing sarcoma KacarTomáš92ap KacarTomáš92boč Ewing sarcoma KnoflíčekRadek62 KnoflíčekRadek62MRIfront KnoflíčekRadek62MRIcor Ewing sarcoma Therapy nChemo and radio sensitive tumor nNeoadjuvant chemotherapy nLocal therapy: –Radiotherapy –Wide resection –Radiotherapy and wide resection nAdjuvant chemotherapy nIn risk patients: transplantation of bone marow nMetastasectomy in lungs Prognosis nResponse to chemotherapy (systemic disease) n5-years survival in 60 % of patients n nWorse prognosis: –metastases –Size over 100cm3 –Surgery not possible –Axial localisation –Local recurrence –Some genetic variants Malignant fibrous histiocytoma in bone nIn 5. decade nIn long bones – femur, tibia nX- ray osteolytic lesion + cortical erosions, soft tissue mass n nTherapy: neoadjuvant chemotherapy + wide resection or amputation + adjuvant chemotherapy nIt is a radioresistant tumor nSurvival 35 % 5 years MFH kosti Krasický KomárekBoleslav33MFH1 KomárekBoleslav33MFH2 Adamantinoma nVery rare n90 % in tibia nTherapy: radical resection nRadioresistant tumor nPrognosis – unclear n n Tu- Adamant Tu- Adamant Chordoma nAxial localisation nOsteolytic lesion nTh- radical surgery or radiotherapy nPrognosis- bad NovákIvan54chordomRTG2 NovákIvan54chordomMRI1 NovákIvan54chordomMRI2 Malignant vascular tumors nHemangioendotelioma nHemangiopericytoma n nAngiosarcoma n nOsteolytic lesions nTherapy: wide resection or amputation nChemotherapy in high grade n nRadiotherapy in non oper. cases x Primary malignant soft tissue tumors - sarcomas nMFH nSynovialo-Sa nLipo-Sa nLeiomyo-sa nFibro-sa nMalignant schwannoma n Primary soft tissue sarcomas nChondrosarcoma of soft tissue n nLymphoma n nMalignant mesenchymal sarcoma MFH pánev MR Malignant fibrous histiocytoma MFH MR MFH stehna MR MFH AP MFH Therapy nNeoadjuvant chemotherapy in G III nRadical resection nAdjuvant chemotherapy nRadiotherapy in inoperable tumors n Synovialo-Sa n Synovialo-Sa MR AP synovialo-sa pánev MR Liposarcoma n lipo-Sa angio lipo-sa CT Liposarcoma n lipo-sa MR lipo-SA MR řez Leiomyo-Sa n leiomyo-Sa Fibro-Sa n fibro-Sa Malignant schwanoma n mal mal Extraskeletal chondrosarcoma n OSA rtg OSA MR rekons Lymphoma n lymfom CT lymfon AG Hemoblastosis in skeleton nPrimary bone tumors –Multiple myeloma (plasmocytoma) –Solitary plasmocytom (myelom) –Primary bone lymfoma – nSecondary lesions –Hodgkin lymfoma –Non-Hodgkin lymfoma –Leukemia Therapy- chemotherapy and radiotherapy in hematooncology Multiple myeloma nMost frequent bone tumor n5 – 6. decade nSymptoms: –pain –Pathological fracture –weaknes –letargy –infections –Renal failure –Headache KuželaMiroslav46RTGhumL n Myelom Otrubová CaisbergerPavel51Myelom1 Solitary plasmocytoma nRare nOsteolytic lesion nResection with replacement + chemotherapy nPrognosis- better than in multiple myeloma n Lymfom Kychlerová KrásenskáMarie35CTpánev Primary bone lymphoma Skeletal metastases Carcinoma with MTS into the skeleton nBreast n nProstate n nLung n nKidney n nThyreoidal gland Localisation nAxial skeleton, pelvis, ribs, proximal femur and humerus X-ray nOsteolysis, osteosclerosis, periostal reaction n DosedlováJ-L1 DosedlováJ-L2 HaynováMarie35RTGpánev MadronJosef49RTGpánev PlšekOldřich41RTGfemurL Potěšilová Christina61RTGhumL LodnerGabriel43RTGhumerusP Diagnostics nHistory n nRadiological findings n nScintigraphy n nOncoscreening n nBiopsy n Complications nPathological fracture n nHypercalcemia n nSpinal cord lesion n nAnemia n SvobodováHelena42RTGhumL RosínováJitka65RTGpánev Rosinová4x Mirel´s score- risk of pathological fx Points 1 2 3 Localisation Upper extremity Lower extremity Trochanteric region Pain Mild Moderate Severe Type Osteoplastic Mixed Osteolytic Size <1/3 of diameter 1/3 – 2/3 diameter >2/3 diameter ≤ 7 points Risk 4% Preventive OS not indicated 8 points Risk 15% OS ?? ≥ 9 points Risk 33% and more OS is indicated Gostijová2x Goszijová1x Therapy nSystemic therapy of carcinoma –Chemotherapy –Hormonal therapy –Imunotherapie, biological therapy … nTherapy of bone metastases –Bisphosphonates –Radioterapy –Surgery: radical, paliative –Conservative treatment –Others - RFA, embolisation … nPaliative management n Surgery of bone metastases nRadical surgery – solitary MTS n good prognosis n nSimple surgery with mobilisation – n multiple metastases n worse prognosis • Types of surgery in MTS Removal of tumor operation No Intramedullary nailing Curretage Cementoplasty + osteosynthesis resection Total replacement Intercalary spacer Amputace No Spinal surgery Instrumentation + fusion