15.11.2018 What is new in orthopaedic tumor surgery? Marko Bergovec, Andreas Leithner Department of Orhtopaedics and Trauma Medical University of Graz, Austria 1 15.11.2018 Two stories • A story about the incidental finding • A scary story with a happy end The story about the incidental finding • 12-year old football player • Small trauma during sport • Still, let’s do X-RAY 2 15.11.2018 The story about the incidental finding • Panic !!! • A child has a tumor !!! • All what a patient and parents hear is: – bone tumor = death – amputation – will he ever do sport again? • Still – what now? 3 15.11.2018 A scary story with a happy end • 12-year old football player • Small trauma during sport • 3 weeks pain not related to physical activity • “It is nothing, just keep it cool, take a rest” 4 15.11.2018 A scary story with a happy end • A time goes by • After two weeks of rest, pain increases... • OK, let’s do X-RAY 5 15.11.2018 TUMORS benign vs malign bone vs soft tissue primary vs metastasis SARCOMA • Austria – cca 200 patients / year – 1% of all malignant tumors • (breast carcinoma = 5500 / year) 6 15.11.2018 Distribution of the bone tumoris according to patients’ age and sex 250 200 150 100 broj bolesnika broj 50 M Ž 0 5 10 15 20 25 30 35 40 45 50 55 60 65 70 75 80 80+ dob 7 15.11.2018 WHO Histologic Classification of Bone Tumors Bone-forming tumors Benign: Osteoma; Osteoid osteoma; Osteoblastoma Intermediate; Aggressive (malignant) osteoblastoma Malignant: Conventional central osteosarcoma; Telangiectatic osteosarcoma; Intraosseous well differentiated (low grade) osteosarcoma, ; Round-cell osteosarcoma; Parosteal (juxtacortical) osteosarcoma; Periosteal osteosarcoma; High grade surface osteosarcoma; Cartilage-forming tumors Benign: Enchondroma; Periosteal (juxtacortical) chondroma; Solitary osteochondroma; Multiple hereditary osteochondromas; Chondroblastoma (epiphyseal chondroblastoma); Chondromyxoid fibroma Malignant: Chondrosarcoma; Juxtacortical (periosteal) chondrosarcoma; Mesenchymal chondrosarcoma; Dedifferentiated chondrosarcoma; Clear cell chondrosarcoma; Malignant chondroblastoma Giant cell tumor (osteoclastoma) Marrow tumors (round cell tumors) Ewing sarcoma; Primitive neuroectodermal tumor of bone (PNET); Malignant lymphoma of bone; Myeloma Vascular tumors Benign: Hemangioma; Lymphangioma; Glomus tumor (glomangioma) Intermediate or indeterminate: Hemangioendothelioma; Hemangiopericytoma Malignant: Angiosarcoma; Malignant hemangiopericytoma Other connective tissue tumors Benign: Benign fibrous histiocytoma; Lipoma Intermediate: Desmoplastic fibroma Malignant: Fibrosarcoma ; Malignant fibrous histiocytoma ; Liposarcoma ; Malignant mesenchymoma ; Leiomyosarcoma ; Undifferentiated sarcoma Other tumors Chordoma ; Adamantinoma of long bones ; Neurilemoma ; Neurofibroma ; Other tumors Unclassified tumors Tumor-like lesions Solitary bone cyst (simple or unicameral bone cyst) ; Aneurysmal bone cyst ; Juxta-articular bone cyst (intraosseous ganglion) ; Metaphyseal fibrous defect (nonossifying fibroma) ; Eosinophilic granuloma (histiocytosis X, Langerhans cell granulomatosis) ; Fibrous dysplasia ; Osteofibrous dysplasia; Myositis ossificans (heterotopic ossification) ; Brown tumor of hyperparathyroidism ; Intraosseous epidermoid cyst ; Giant cell (reparative) granuloma 8 15.11.2018 9 15.11.2018 Conference on bone sarcomas in the 1920s: “If you do not operate - they die; if you do operate - they die just the same. Gentlemen, this meeting should be concluded with prayers.” 10 15.11.2018 Survival nowadays: 75-85% Arndt C, N Engl J Med 1999 Osteosarcoma before and after chemotherapy 11 15.11.2018 12 15.11.2018 Problems in tumor diagnosis • rarity prevents most orthopaedic surgeons from gaining sufficient experience (even with all available diagnostic and imaging possibilities) • despite their rarity, there is a wide spectrum of “tumor” lesions with overlapping morphologic features (infection, developmental disorders trauma, sport injuries and overuse injuries...) • diagnosis of malignant bone tumors often has dramatic consequences in terms of surgical and adjuvant treatment 13 15.11.2018 Problems in tumor diagnosis • even more..... time from the first complain to diagnosis: – 9 weeks for osteosarcoma – 4 months for Ewing sarcoma – 5 months for chondrosarcoma – months and months for soft tissue sarcomas • HOW TO DETECT IT SOONER? Oculo-brachial reflex 14 15.11.2018 WHOOPS - about 30% malignant tumors treated not adequately - “did not know it is sarcoma” - “sarcoma? really?” - “whoops! now what?” 15 15.11.2018 16 15.11.2018 17 15.11.2018 So ..... ..... let‘s do a biopsy ! 18 15.11.2018 DIAGNOSTIC PROCEDURES LAB. FINDINGS HISTORY CLINICAL FINDINGS HISTOLOGY (PHD) X-RAY BONE ULTRA- D I A G N O S I S SCAN SOUND MRI ANGIOGRAPHY CT ARTHROGRAPHY ? 19 15.11.2018 Biopsy • do not do it if you do not have idea which tumor could it be • biopsy sample to small no diagnosis • sample inadequate no diagnosis • wrong biopsy approach definitive operation and biopsy tract excision difficult / not possible / amputation necessary • local haematoma after biopsy patient’s life in danger Biopsy • simple only from technical point of view • planing biopsy approach difficult • should be the last diagnostic procedure • biopsy = diagnosis (96%) • culture every biopsy / biopsy every culture 20 15.11.2018 Biopsy (and tumor surgery in general) • avoid: – oculo-brachial reflex – whoops – approach If not sure, let tumor-center handle it. There are rules – follow them! 21 15.11.2018 Before we start with the surgery... Treatment consequences • Tumor removal – considered mostly positive – (although when it is not - overtreatment) • Operation is always a trauma for the a patient (and a family) • Various localisations various patients’ expectations – Hand: 100% function “must have” – Knee: flexion • QoL - support and inform – QoL is difficult to measure – we know which factors are positive influences Treatment algorithm 1. Diagnostic procedures 2. Biopsy 3. Oncological procedures (chemotherapy, RTX...) 4. Surgery 5. Oncological procedures (chemotherapy, RTX...) 22 15.11.2018 Surgical options • wide range of possibilities with a really fun instruments and power tools • ranking from “do nothing” to “amputate” • sometimes influenced by “non-strictly- medical” factors: – surgical experience – availability of bone bank / special implants – availability of support (other tumor- team members) – economical opportunities... So, let’s do some surgery • The optimal management of a patient with a tumor is by a multidisciplinary team experienced in the diagnosis, oncologic and surgical management of orthopaedic pathology. • Individual approach! – (is there another way in medicine?) 23 15.11.2018 “Just resect” tumor type • most benign tumors • soft tissue sarcoma – postoperative RTX • postoperative function related to tumor extent Marginal tumor removal • resection of the tumor at the border / capsule • benign soft tissue tumors 24 15.11.2018 J.E., 53yo, ♀ Lipoma P.M., 63yo, ♀ Schwanomma 25 15.11.2018 26 15.11.2018 G.G., 1952 27 15.11.2018 28 15.11.2018 K.R., 15yo, ♀ Ewing 29 15.11.2018 Bone tumor removal • opening the tumor (capsule) • subtotal resection • enchondroma Bone defect filling • autograft • allograft • bone supstitute 30 15.11.2018 The first transplant... • bone allograft • a long time ago... • even before that... • at the very beginning... The first bone transplant Bible, Genesys .... So the LORD God caused the man to fall into a deep sleep; and while he was sleeping, he took one of the man’s ribs and then closed up the place with flesh. Then the LORD God made a woman from the rib he had taken out of the man, and he brought her to the man. .... 31 15.11.2018 K.M., 29yo, ♂ Enchondroma P.S. ♀ 31yo, Enchondroma 5y postop 32 15.11.2018 B.F. ♂ 9 god FIBROMA NON-OSSIFICANS spongioplasty 13 months after the operation F.Ž. ♂ 67 y.o. Secondary bone tumor 12 cm 33 15.11.2018 12 months after the operation 34 15.11.2018 35 15.11.2018 OSA 36 15.11.2018 Dj.M., 51yo, ♂ GCT Endoprosthetic reconstruction • tumor endoprosthesis • mega endoprosthesis 37 15.11.2018 38 15.11.2018 39 15.11.2018 40 15.11.2018 41 15.11.2018 42 15.11.2018 Just for fun… • there are (at least) two things you can not do: – sneeze with your eyes open – kiss your elbow 43 15.11.2018 ELBOW • why do we need elbow? bring food to the mouth (110°) 44 15.11.2018 wipe ass (20°) BAC, 28y, ♂ osteosarcoma +2M 45 15.11.2018 MJ, 53y, ♀ Chondrosarcoma 46 15.11.2018 47 15.11.2018 48 15.11.2018 Case VA, ♂, 9y Femur fracture (fall from 1m height) No other comorbidities 49 15.11.2018 50 15.11.2018 51 15.11.2018 TM, 32y, ♀ MPNST 52 15.11.2018 HIGH-TECH • intraoperative RTX 53 15.11.2018 HIGH-TECH • intraoperative CT / MR HIGH-TECH • intraoperative navi + CT/MR 54 15.11.2018 Wide tumor resection • malignant bone (and soft tissue!) tumors • local agressive tumors (GCT) • no compromise in wide resection / tumor removal • priorities: 1. save life 2. save limb 3. preserve function 55 15.11.2018 Complications • “oncological” – local: tumor recurrence / rest – systemic: metastases – other • “orthopaedic" – mechanical failure: fracture (implant fracture, periprosthetic fracture), non-union, loosening – infection – soft tissue failure B.K.* ♀ 7 years Age: 7 EWING SARCOMA follow-up: 77 months Jan 1998 resection length: 22 cm * informed consent 56 15.11.2018 B.K. ♀ 7 years EWING SARCOMA follow-up: 77 months 22 cm (70%) 9 cm 6 cm (30%) B.K. ♀ 10 g. EWING SARCOMA praćenje: 41 mj Dob: 10 1. 2001 Subluksacija EP 3,5 god. iza resekcije i