Tumor - RC Q's Flashcards
(92 cards)
“RC 2018 - A 65 year old male comes to see you with several months of right hip pain. His X-rays are shown below. What would be your choice with respect to management of his problem? (very close to the exact XR given) <div>a.Intramedullary nailing</div> <div>b.En-bloc resection</div> <div>c.Neoadjuvant chemotherapy followed by intramedullary nailing and localized radiation</div> <div>d.Intralesional extended curettage and bone grafting</div> <div><img></img></div>”
B
<div>RC 2018 - Regarding atypical lipoma/well-differentiated liposarcoma in the extremity, all are true except?</div>
<div>a.There is risk of metastasis</div>
<div>b.Histologically it is the same in the extremity and the retroperitoneum/abdomen</div>
<div>c.Can dedifferentiate</div>
<div>d.The risk of recurrence is up to 50% after resection</div>
“<div>A</div><ul><li><div>If they do not specify between ALT and WDL, then I would say D. if it is just ALT, I would choose A - CK</div></li></ul><ul><li><div>JAAOS 2018 Lipomatous soft tissue tumors</div></li><ul><li><div>"”Labelling a tumor without the capacity to metastasize a ““sarcoma”” is inaccurate and is why the World Health Organization now uses the term ALT for extremity-based or superficial trunk lesions”</div></li><li><div>"”ALTs have excellent prognosis with essentially 100% survival. Reported local recurrence rates vary from 8.2% - 50.61%””</div></li></ul></ul>"”Although lacking metastatic potential, WDLs are essentially incurable which differentiates them from their extremity counterparts”””
RC 2018 List 4 conditions that predispose an individual to developing a secondary osteosarcoma
Pagets<div>Post-radiation</div><div>Fibrous Dysplasia</div><div>Bone infarcts</div><div>Chronic OM</div><div><br></br></div><div><div>*They are other RF for OS - (Rb, Li Fruemeni, Rothmund) but these would be causes a <u>primary</u> OS</div></div>
<div>RC 2016 - What tumor is not typically located in the posterior spinal elements?</div>
<ol> <li>ABC</li> <li>Chordoma</li> <li>Osteoblastoma</li> <li>Osteoid Osteoma</li></ol>
B.<div><br></br></div><div>Oh oH oh Ah</div><div><br></br></div><div><ul> <li>JAAOS – Benign Tumors of the Spine</li> <ul> <li>Osteoid Osteoma = “approximately 10% occur in the vertebrae, primarily in the posterior elements”</li> <ul> <li>Most common cause of painful scoliosis in the adolescent population</li> </ul> <li>Osteoblastoma:</li> <ul> <li>Spine accounts for 28-36% of osteoblastomas</li> <li>“typically arise in the posterior elements, although extension into the vertebral body is common with larger tumors”</li> <li>Most common in lumbar spine</li> </ul> <li>ABC:</li> <ul> <li>15% of primary spine tumors</li> <li>Boriani (spine 2001)- most lesions in posterior elements, 70% thoracolumbar</li> </ul> </ul> <li>JAAOS – Chordoma of the Sacrum</li> <ul> <li>Typically found in the midline of the neuroaxis, arise from intraosseous notochordal remnants</li> <li>50% sacrococcygeal, 35% sphenooccipital, 15% mobile spine</li> </ul></ul></div>
<div>RC 2015 - Regarding core needle biopsy, all are true except:</div>
<div> a. Saves a surgical procedure</div>
<div> b. Must resect the tract</div>
<div> c. A viable tumour can be accessed with image guidance</div>
<div> d. Biopsy should be in line with planned surgical incision</div>
B. if benign, dont have to resect tract.
<div>RC 2011 - List 4 long-term complications of radiation therapy for sarcoma treatment</div>
<div><ul> <li>Secondary sarcoma</li> <li>Fragility fracture</li> <li>Growth arrest</li> <li>Fibrosis/joint contracture</li> <li>Osteonecrosis</li> <li>Edema</li> <li>Poor wound healing</li></ul><div><div><b><u>Acute</u></b></div> <div>Local</div> <ul> <li>Skin irritation/erythema/desquamation</li> <li>Local edema</li> <li>Growth arrest (peds)</li> <li>Infection risk</li> <li>Wound healing (risk 35% pre-op, 17% post-op)</li> </ul> <div>Systemic</div> <ul> <li>GI upset</li> <li>Anorexia</li> <li>Fatigue</li> <li>Urinary frequency</li> </ul> <div><b><u>Chronic (RC EXAM)</u></b></div> <div>Local</div> <ul> <li>Secondary Sarcoma</li> <li>Pathologic fractures</li> <li>Fibrosis</li> <li>Osteonecrosis</li> <li>Chronic edema</li> <li>Growth arrest</li> </ul> <div>Systemic</div> <ul> <li>Chronic Edema</li> <li>Chest wall abnormalities (peds)</li> <li>Scoliosis (peds)</li></ul></div></div>
<div>RC 2017 - 3 risk factors for the development of metastatic disease in an isolated soft tissue sarcoma</div>
<ul> <li>Tumour type</li> <li>Size of tumour</li> <li>Depth of tumour / compartment involvement (deep to fascia)</li></ul>
<div>RC 2015 - List 3 components of soft tissue sarcoma staging (AJCC score)</div>
“<ul> <li>American Joint Commission on Cancer Staging of Soft-tissue Sarcoma</li> <ul> <li>Grade</li> <li>Tumor (size (5cm), depth of primary tumor (superficial vs deep to fascia) )</li> <li>Regional Lymph Nodes</li> <li>Distal Metastasis</li> </ul> </ul> <div><img></img></div>”
RC 2012 - In soft tissue sarcoma, other than metastatic disease, what are the 3 most important determinants of a worse prognosis
<ul> <li>JAAOS - Soft Tissue Sarcomas</li> <ul> <li>High histologic grade</li> <li>Deep location </li> <li>Size greater than 5cm</li> </ul> <li>Lahat G (Annal Surg Onc 2008) New perspectives for staging and prognosis in soft tissue sarcoma</li> <ul> <li>Size greater than 5cm</li> <li>Non-extremity primary site (axial skeleton)</li> <li>High histologic grade</li> <li>Positive Tumor resection margins</li> <li>Recurrent disease</li> </ul></ul>
<div>RC 2017, 2014 - Man with 20 years of tibia draining sinus after a fracture. In the last 4 months, it has increased size and amount of draining significantly. You are shown an xray with crazy amount of bone loss and picture of terrible soft tissues. What is the most likely diagnosis. </div>
<ol> <li>Squamous cell carcinoma</li> <li>Fibrous sarcoma</li> <li>Osteosarcoma</li> <li>Chronic osteomyelitis</li></ol>
A.<div><ul> <li>JAAOS 2014 - Malignant Transformation in Chronic Osteomyelitis</li> <li>Marjolin Ulcer</li> <ul> <li>Usually aggressive squamous cell carcinoma </li> <li>Other associated cancers BCC, fibrosarcoma, myeloma, angiosarcoma, rhabdomyosarcoma, fibroblastic osteosarcoma, adenocarcinoma, B cell lymphoma, plasmacytoma, MFH, reticulosarcoma, fibroblastic osteosarcoma</li> </ul></ul></div><div><br></br></div>
“RC 2017, 2013 - A mom comes to your clinic after soft tissue sarcoma surgery and is worried about having kids. What do you counsel her? <ol> <li>Children’s risk is low because its autosomal recessive</li> <li>Children’s risk is low because it’s usually a spontaneous mutation</li> <li>Children’s risk is high because it’s autosomal dominant</li> <li>Children’s risk is high because environmental exposure plays a large role</li></ol>”
B.<div><ul> <li>Li-Fraumeni - AD</li> <li>Retinoblastoma - AD</li> <li>NF - AD</li></ul></div>
RC 2015 - 35yo male with café-au-lait lesions and multiple skin nodules. Presents with absent motor/sensory function in lower extremity and deep soft tissue mass in the posterior thigh. Most likely diagnosis? <ol> <li>Neurofibroma</li> <li>Malignant nerve sheath tumor</li> <li>Schwannoma</li> <li>Lipoma</li></ol>
B.<div><br></br></div><div><div>Large, deep soft tissue mass with neurologic symptoms in setting of NF1</div></div>
<div>RC 2015 - 65 yo male with previous resection for soft tissue tumour in the buttocks with approx half of glut max gone. Skin on ischium is sensitive and thin. Vascular studies show most distal level of perfusion is 2 cm below the tibial articular surface. Where should you amputate?</div>
<div> a. Hip disarticulation</div>
<div> b. Transfemoral</div>
<div> c. Through knee</div>
<div> d. BKA</div>
A.<div><ul> <li>2cm distal skin too little for BKA or through knee</li> <ul> <li>Although ? Skin viability and level of perfusion equivalent</li> </ul> <li>Transfemoral relies on ischial weightbearing prosthesis and with the thin skin this is not a viable option either</li> <li>Therefore….hip disarticulation with anterior flap (takes care of thin skin at the same time)</li></ul></div>
<div>RC 2011 - What is the worse prognostic factor associated with soft tissue sarcoma?</div>
<ol> <li>size of lesion</li> <li>metastatic disease</li> <li>Grade</li> <li>extracompartmental disease</li></ol>
B.
RC 2015 - 60M has a history of a previous desmoid tumor that was excised 3 months ago. He is in your clinic and is found to have an basal skull ENT lesion (osteoma) seen on CT-Head. What is next step in management/investigation? <ol> <li>Bone scan</li> <li>CT chest</li> <li>SPEP</li> <li>Colonoscopy</li> <li>Skeletal survey</li></ol>
D.<div><ul> <li>Gardner Syndrome (The FAPPENING - i.e. Familial adenmoatous polyposis)</li> <ul> <li>Intestinal polyposis, osteomas, multiple cutaneous and subcutaneous lesions</li> <li>Dental abnormalities, abdominal desmoids</li> <li>Associated neoplasms:</li> <ul> <li>Papillary carcinoma of the thyroid</li> <li>Glioma, medulloblastoma</li> <li>Peri-ampullary carcinoma of the duodenum</li> </ul> </ul></ul></div>
RC 2010 - Which doesn’t require post op chemo <ol> <li>MFH of bone</li> <li>Synovial cell sarcoma of soft-tissue</li> <li>Rhabdomyosarcoma of soft tissue</li> <li>Angiosarcoma of soft-tissue</li></ol>
D.<div><ul> <li>AAOS Comprehensive Review:</li> <ul> <li>MFH treatment is similar to osteosarcoma</li> <li>Synovial cell sarcoma –> chemo used in younger patients sometimes</li> <li>Rhabdomyosarcoma –> wide resection and chemo (in kids)</li> <li>Angiosarcoma –> no survival benefits shown with chemo</li> </ul></ul></div>
RC 2008 - Soft tissue sarcoma, most common location of mets: <ol> <li>Lungs</li> <li>Lymph Nodes</li> <li>Bone</li> <li>Liver</li></ol>
A.
RC 2009 - What soft tissue tumor metastasizes to a place other than lungs? <ol> <li>MFH</li> <li>Myxoid liposarcoma</li> <li>Lipoma</li> <li>Leiomyosarcoma</li></ol>
2.<div><br></br></div><div>CREAMS</div><div>clear cell chondrosarc</div><div>rhabdomyosarcoma</div><div>epithelioid</div><div>angiosarc</div><div>myxoid liposarc</div><div>synovial cell</div>
<div>RC 2018 - With regards to primary tumors in the spine: </div>
<ol> <li>Osteoid osteoma is usually on the convex side of the curve </li> <li>Vertebral plana caused by langerhans cell histiocytosis can reconstitute height</li> <li>GCT in the mobile spine is usually in posterior elements </li> <li>Osteoblastoma is usually in the vertebral body</li></ol>
“B<div><ul> <li>Wheele’s: at least 50% reconstitution of vertebral height may be expected</li> </ul> <div></div> <div>A-false: typically on CONCAVE side</div> <div>C-false: GCT is in ANTERIOR</div> <div>D-false: OB is in POSTERIOR</div></div><div><br></br></div>”
<div>RC 2018 - What is associated with Osteoid osteoma of the spine?</div>
<ol> <li>Scoliosis </li> <li>Spondylolisthesis </li> <li>Spina bifida occulta </li> <li>radiculitis</li></ol>
A.<div><br></br></div><div>OO is found on CONCAVE side.</div>
“RC 2011 - Mirel’s criteria?”
“<ul> <li>Pain</li> <li>Location</li> <li>Matrix</li> <li>Size</li> <li><img></img></li></ul>”
<div>RC 2014, 2010 - 70 year old with metastatic bone disease and pathological midshaft femur fracture. Has history consistent with thyroid mets.</div>
<ul> <li>what is the best surgical management for the fracture?</li></ul>
<ul> <li>what intervention should be considered pre-op?</li></ul>
<ul> <li>what medication should be used postop for the skeletal system?</li></ul>
<ul> <li>what other treatment should be instituted at the fracture site to reduce local recurrence/expansion?</li><li><br></br></li><ul> </ul> </ul>
<ul><li>what is the best surgical management for the fracture?</li> <ul> <li>Cephalomedullary nail</li> </ul> </ul>
<div></div>
<ul> <li>what intervention should be considered pre-op?</li> <ul> <li>Embolization (renal and thyroid mets)</li> </ul> </ul>
<div></div>
<ul> <li>what medication should be used postop for the skeletal system?</li> <ul> <li>bisphosphonates</li> </ul> </ul>
<div></div>
<ul> <li>what other treatment should be instituted at the fracture site to reduce local recurrence/expansion?</li> <ul> <li>Radiation therapy</li> <ul> <li>Decreases likelihood of progression and to relieve pain</li> </ul> </ul></ul>
<div>RC 2014, 2010 - An 80yo female with a history of breast cancer treated with mastectomy, lymph node dissection, and radiation with no recurrence 20 years ago presents with a humeral lesion which is sclerotic, and associated with an ossified soft tissue mass. Most likely diagnosis is?</div>
<ol> <li>Metastatic breast cancer</li> <li>Myeloma</li> <li>Osteosarcoma</li> <li>Chondrosarcoma</li></ol>
- likely post-radiation<div><ul> <li>Osteoblastic Metastasis:</li> <ul> <li>Suva LJ (Nature Review 2011) Bone Metastasis: mechanism and therapeutic opportunities</li> <ul> <li>Osteosclerotic metastases via secretion of endothelin-1 which decreases DKK-1 –> less Wnt Suppression</li> </ul> <li>Weldin JSES 2010:</li> <ul> <li>208 patients with metastatic lesions of the humerus</li> <li>64% breast, 30% kidney</li> </ul> </ul> <li>HOWEVER –> metastatic disease does not usually have a soft tissue component, therefore this is much more likely to be osteosarcoma (probably post-radiation)</li></ul></div>
<div>RC 2013, 2011 - 60 yo male with known metastatic renal ca with impending pathologic fracture through lytic lesion 5 cm distal to lesser trochanter. Best treatment?</div>
<ol> <li>Short cephallomedullary nail locked distally</li> <li>Long cephalomedullary nail locked distally</li> <li>Regular IM nail locked statically</li> <li>Regular IM nail locked dynamically</li></ol>
2.<div><br></br></div><div><br></br></div>
- Antegrade IM nail
- Retrograde IM nail
- Biopsy
- Antegrade nail, then send reaming for biopsy, then get Prism to call CMPA for you
- Palliative
- Non WB and chemo
- Non WB and rads
- Prophylactic Cephalomedullary nail
- Fix the pelvis
- Bone scan
- Skeletal survey
- Drink martini
- Scoliosis
- Radiculitis
- Spondylolisthesis
- Spina Bifida Occulta
- Radiofrequency ablation
- En bloc resection
- Curettage and bone grafting
- Resection and fusion
- Currettage and bone grafting
- Radiofrequeny ablation
- Wide resection
- ?chemo or rads
- Chemo and radiation
- Surgical excision and chemo
- Surgical excision and radiation
- Surgical excision
- Tumor Factors
- Location - axial skeleton
- Large > 8 cm
- Poor response to chemo <90%
- Positive margins
- Secondary sarcoma
- Grade: conventional vs surface
- Stage: Advanced stage, Metastatic disease
- Systemic Markers: Increased ALP/LDH, expression of P-glycoprotein
- Traction, neoadjuvant chemo, wide resection, adjuvant chemotherapy
- Traction, neoadjuvant chemo, wide resection, adjuvant chemotherapy and radiation
- Wide resection and proximal femoral replacing prosthesis
- Immediate hip disarticulation and then chemo
- Radiation
- Paget’s
- Li Fraumeni
- Hand-Schuller-Christian
- Li Fraumeni Syndrome (p53)
- Retinoblastoma (Rb)
- Rothmund Thomson Syndrome:
- AR, RECQL4 gene
- neoadjuvent chemo, wide resection, post-op chemo
- pre-op radiation, wide resection, post-op radiation
- wide resection
- debulking
- Synovial Sarcoma
- Osteosarcoma
- Ewing Sarcoma
- Liposarcoma
- Retinoblastoma (Rb-1) and osteosarcoma
- NF-1 and malignant central nervous system tumors
- EXT-1 and EXT-2 and multiple osteochondromas
- Chromosomal translocation and Ewing family of tumors
- Chordoma
- Osteosarcoma
- MPNST
- Metastatic Disease
- Chordoma
- most common primary malignant spinal tumor in adults
- 50% occur in the sacrum and coccyx
- motor deficits rare because most lesions at S1 or distal
- bowel and bladder changes are common
- CT = will show midline bone destruction and soft tissue mass
- calcifications often present within the soft tissue lesion
- Chordomas often encroach on the spinal canal, and they may cause compression of the spinal cord, cauda equina, or nerve roots.
- Chordoma classically appears as an osteolytic lesion centered in the midline and in association with a large soft-tissue mass
- Wide resection and chemo
- Wide Resection, chemo, and radiation
- Wide resection and radiation
- Wide resection
- Urinary dysfunction
- Impotence
- Pain
- Mass
- CT Chest
- Bone Scan
- Bone Marrow Biopsy - iliac crest
- Lab Studies - LDH
- Cytogenic/Molecular studies of EWS translocation- FISH
- Local Staging - X-ray whole bone, MRI
- S100
- CDA1
- CD99
- Cytokeratin
- Exact etiology is unknown but it commonly demonstrates reproducible staining of CD99 and translocations of the EWS gene.
- JAAOS 2010 - Ewing Sarcoma Family of Tumors
- CD99 expressed in 95%
- S-100 can be expressed
- LDH
- ALP
- Nuclear Ploidy Testing
- Cytogenetics
- Older age
- Multiple injections
- Single cystic component
- Small lesion
- Proximal humerus
- Uniloculated
- Methylprednisolone and marrow injection have equivalent outcomes
- Cross sectional area of the cyst determines the fracture risk
- 15-20% likelihood of spontaneous resolution following fracture in the upper extremity
- Malignant transformation is more likely to occur after skeletal maturity
- Autologous bone marrow injection has markedly improved outcomes compared to steroid injection
- ABC is rarely associated with formation of a neocortex
- UBC rarely expands the cortex beyond the width of the physis
- Recurrence is less likely with open physes
- UBCs appear as lytic, expansile lesions within the emdullary cavity of a long bone. The cortex is thinned but typically is not compromised
- ABC is a lytic, intramedulary bone lesion, with eccentric expansion and a transverse diameter that is wider than the epiphyseal plate
- Prognosis is better with age > age 10 than under
- Mixed evidence for ABM vs steroid injections, definitely not marked improvement
- Large size
- multiple septations
- radiographically active lesion
- calcaneal UBC
- Indicators that they will do better with steroids: (unknown source)
- Age > 8
- Small lesion
- Proximal humerus
- Uniloculated
- Leg Length Discrepancy (limb length discrepancy?)
- Varus Angulation
- Physeal Arrest
- Malignant Transformation
- Unknown etiology, possibly trauma related, possibly venous obstruction
- PATH: singular fluid filled cavity, occasionally giant cells can be present
- Ddx
- ABC
- FD
- enchondroma
- Complications: Varus malunion, osteonecrosis of femoral head, growth arrest, growth disturbance
- MRI show fluid fluid levels
- Heal better with addition of an adjuvant such at cryotherapy
- Most regress spontaneously with age
- Associated with about 20% recurrence after treatment.
- Amputate (O’Neill style)
- Open biopsy
- Reduce and immobilize
- CRPP
- Not an inherited condition
- If associated with hemangiomas and viscera, can be life threatening
- Associated with angular deformities
- Prognosis of a patient that develops a secondary chondrosarcoma is worse than a patient who develops it from a solitary enchondroma.
- Pain is the most common presenting symptom
- Often times localized pain is the only finding on examination
- The lesion is usually located in the metaphysis
- Treatment consists of curettage and bone grafting
- JAAOS - Cartilage Tumors
- Histologic grading - grade 1 vs Grade 2/3
- Staging/Metastasis
- Location - Axial vs Peripheral Skeleton vs Hand
- Andreou D (Acta Orthop 2011)
- Increased size > 100cc
- Older age (>40)
- High grade tumor
- Sjoerd PFT (Sarcoma 2015)
- Dedifferentiated subtype
- Pathologic fracture
- Local recurrence
- Orthobullets:
- Increased telomerase activity
- MFH
- Osteosarcoma
- Chondrosarcoma
- Fibrosarcoma
- Olliers
- Mafuccis
- MHE
- McCune Albright syndrome
- Ollier's Disease --> multiple enchondromatosis --> chondrosarcoma
- Mafucci's Disease --> multiple enchondromatosis --> chondrosarcoma
- MHE --> osteochondroma --> chondrosarcoma
- McCune Albright --> fibrous dysplasia --> fibrosarcoma, osteosarcoma, MFH
- Fibrous Dysplasia:
- Benign medullary proliferation of fibrous tissue and immature bone (woven bone)
- RC EXAM answer: 'development bone malformation'
- Monostotic (80%)
- Polyostotic:
- Monomelic - unilateral lesions, one bone of one extremity
- Polymelic - wide spread involvement
- Metabolic Syndromes: (higher likelihood of fracture)
- Hyperthyroidism
- Hypophosphatemia
- Most common entity causing oncogenic osteomalacia (renal phosphate wasting due to fibroblast growth factor 23)
- Acromegaly
- Hyperprolactinemia
- Cushing's Disease
- Non-endocrine anomalies of brain, thymus, heart, bone marrow, liver, spleen and GI tract
- Syndromes:
- McCune Albright - triad of fibrous dysplasia, café au lait, endocrine anomalies
- Triad only present 4% of the time
- GNAS mutation
- Café au lait spots are ""coast of Maine"" type
- 30-50% have polyostotic fibrous dysplasia
- Precocious puberty
- Autonomous ovarian cysts
- 50% moderate to severe scoliosis
- Not always a result of spinal FD
- Mazabraud - soft tissue myxoma (intramuscular) overlying
- No reports of malignant transformation of myxomas
- Myxomas develop later than fibrous dysplasia
- Myxoma symptoms from mass effect
- Cherubism:
- Fibrous dysplasia with symmetric involvement of both mandible and maxilla
- Can distort orbit and deviate eyes
- Presents in second decade, lesions stable once skeletally mature
- MFH
- Osteosarcoma
- Ewing Sarcoma
- Chondrosarcoma
- OKU states that CS Grade 1 can be treated with intralesional curettage and bone grafting
- Grade 1 lesions look relatively benign on Xray - as in our image.
- Grade 2 ""demonstrate a much more aggressive behavior pattern. These tumors often breach the cortex (Enneking stage 1B) and cause sub- stantial bony destruction""
- Subsequent clinical series demonstrated that intralesional curettage was safe, effective, and yielded a functional result superior to that of wide bony resection.10-14 Although local re- currences after this more limited treatment have occurred and necessitated either revision curettage or wide resection, adverse oncologic outcomes (metastasis or death by disease) have rarely occurred, and recurrence with dedifferentiation has not been common. Intralesional treatment has therefore become more common than bony resection for this disease.
- Epiphyseal
- Eccentric
- Multi-loculated
- Less than 1cm
- wide resection
- wide resection and chemo
- wide resection and radiation
- observe
- Osteofibrous dysplasia
- Fibrous dysplasia
- Enchondroma
- CN
- McCune Albright - Café-au-lait spots, precocious puberty, endocrine abnormalities
- Mazabraud - Soft tissue myxomas
- Endocrine - hyperthyroidism, hypophosphatemia, acromegaly, Cushing's disease, Hyperprolactinemia
- Cherubism - eye deviation and blindness
- It is a developmental malformation of bone
- Monostotic is often symptomatic
- Polyostotic is more common
- Xray and MRI are necessary for diagnosis
- 80% are monostotic
- Inability of bone to produce mature lamellar bone, tissue is arrested in woven bone stage
- Results in a mass of immature trabeculae in dysplastic fibrous tissue with slow turnover and mineralization --> low mechanical strength
- Bone graft using allograft
- Bone graft using autograft
- Bone graft using fibular strut grafts
- Maintain normal bone mechanics with implant support
- Pleomorphic undifferentiated sarcoma
- Chondrosarcoma
- Osteosarcoma
- Fibrosarcoma
- Cortical allograft
- Cancellous allograft
- Cortical autograft
- Cancellous autograft
- Bone scan
- CT chest
- SPEP
- Colonoscopy
- Skeletal survey
- Gardner Syndrome
- Intestinal polyposis, osteomas, multiple cutaneous and subcutaneous lesions
- Dental abnormalities, abdominal desmoids
- Associated neoplasms:
- Papillary carcinoma of the thyroid
- Glioma, medulloblastoma
- Peri-ampullary carcinoma of the duodenum
- Extensive intralesional curettage is the standard of care
- They do not often extend to subchondral bone
- Irresectable lesions can be treated with radiation, denosumab
- Treatment with radiation in irresetable lesions carries an increased risk of malignant transformation
- The lesion can be quite large and encompass the metaphysis and epiphysis up to the subchondral bone
- Radiotherapy has been used and is efficacious, but early reports of malignant transfor- mation of radiated giant cell tumors have caused radio- therapy to be a last-resort treatment
- Denosumab, a fully human monoclonal antibody and a RANKL inhibitor, has enjoyed great success in the management of bone metastatic disease and in a short time radically changed the options for unresect- able GCTB, or disease where resection would leave sig- nificant functional deficit
- Osteosarcoma
- Chondrosarcoma
- GCT
- Chondroblastoma
- Present in patients 20-40 (<10% in patients >65 years)
- Occurs in metaphyseal and epiphyseal regions
- Neocortex formation in response to the treat of the advancing neoplasm
- Lytic lesion on XR (chondroblastomas have mottled or fuzzy appearance, often w/ calcifications)
- Closely related to chondroblastoma
- epiphyseally-based lesions
- age around 20 years I most common
- occur in the ends of long bones
- Occurs in the same age group as ABC
- Occurs after too much left handed masturbation while right hand is in a cast (a la Sheehan)
- Treated with resection and joint reconstruction
- Can often cause cortical thinning and invade surrounding soft tissues
- ABC common < 20 years, GCT common 20-40 years
- ABC usually treated with intra-lesional curettage and grafting
- D worded a bit awkward as tumor doesn't usually invade, but can be locally aggressive
- ""neocortex"" is being described
- 2015
- While GCT not generally ""osteogenic"" there is histologic evidence it is ""osteoblastic""
- Can occur in patients < age 20, but most commonly in age 20-40
- Dumb Question
- JAAOS 2013 - GCT of Bone
- GCT of bone typically presents in persons aged 20-40 years
- It is rare in adolescents and children
- <10% of cases are seen in patients aged > 75
- In most cases GCT occurs in metaphyseal and epiphyseal regions
- They appear to expand the bone and may appear to expand the bone and elevate the periosteum, resulting in a thin periosteal shell
- GCT of bone is a mesenchymal tumor in which the cell of origin is the fibroblastic-osteoblastic mononuclear cell that produces types I and II collagen.
- Treated by en-block resection
- Can erode through cortex, and invade into soft tissues
- Have no potential to metastasis to the chest
- ?
- Prefer intra-lesional Curretage but can certainly treat with en-block resection
- Adjacent soft tissues are invaded, a thin rim of bone is usually seen around the mass
- GCT of bone has been reported to metastasize in 2% of cases
- Can immediately weight bear
- Easier detection of recurrence
- Decreases local recurrence
- Exothermic reaction can be helpful
- Algawahmed H (Sarcoma 2010) High speed burring with and without the use of surgical adjuvants in the intralesional management of GCT of Bone
- Systematic review
- No difference between high speed burr and additional PMMA
- Blackley HR (JBJS 1999) Treatment of GCT of long bones with curettage and bone grafting
- May cause 2-3mm of thermal necrosis in cancellous bone
- Additional advantages of the use of cement include low cost, ease of use, lack of donor site morbidity, elimination of the risk of transmission of disease, immediate structural stability, potential for earlier detection of recurrence
- OKU 3
- PMMA is advantageous because it provides not only a thermal adjuvant, but also a structural fill to the cavity that ideally allows for earlier weight bearing (Figures 9 and 10).
- An additional benefit of PMMA compared with bone grafting is the improved identification of recurrences. The remodeling associated with bone graft integration can lead to difficulties in image interpretation when looking for local recurrences
- Monostotic form has high local recurrence rates
- Can present as monostotic, polyostotic and multisystem disseminated form
- Etiology is not well understood
- Often have spontaneous resolution following simple needle biopsy
- For patients with monostotic disease, the 10 year survival rate is 100% with low rates of recurrence
- Often has spontaneous resolution even without simple needle biopsy
- Can present at monostotic (EG), polyostotitic as well as dissemitated form (Hand-Schuller-Christian disease or Letterer-Siwe disease)
- The disease remains no less strange than when it first presented – mysterious etiology
- easy to diagnosis on radiograph
- can have onion skinning on radiograph
- can present as a radiolucent lytic lesion
- may be cold on Tc99 bone scan
- Surgical hip dislocation and synovectomy
- Chemotherapy and resection
- Radiation and resection
- Total hip arthroplasty
- OKU: In the hip, open treatment may require a dual anterior-posterior approach or dislocation of the femoral head, which can lead to osteonecrosis. An arthroscopic approach therefore may be preferable
- Arthroscopy, however:
- Technically demanding
- Can't access extra-capsular disease
- Risk of iatrogenic nerve or labral injury
- Uncemented THA
- Protrusio cup with a cemented femoral component
- PMMA and screws and cage with a cemented femoral component
- Standard cemented THA
- Type I lesion (dome is deficient, but medial wall ok):
- Biermann JS (JBJS 2009) Metastatic bone disease: diagnosis, evaluation and treatment
- ""Reconstruction of peri-acetabular defects requires a stable construct that distributes weight from the lower extremity to the remaining pelvis and spine. This may be accomplished with use of a standard reconstruction cup, an antiprotrusio cage, PMMA +/- screw augmentation as described by Harrington""
- ""Lesions that do no breach the joint may be treated withs crews or pins and PMMA, whereas a larger defect is better treated with an antiprotrusio cage that bypasses the lytic zone and rests on solid bone""
- Resection arthroplasty
- Saddle prosthesis
- Allograft/prosthetic composite
- Custom metallic devices
- Iliofemoral arthrodesis
- Harrington rods and flange cup
- Cage-cup
- Triflange
- Allograft/prosthetic composite
- Metal augments + highly porous multihole cup
- Achilles
- FHL
- Tib Ant
- Tib Post
- Medial gastroc flap has more excursion than lateral
- Soleus flap good for mid tibia coverage
- Peroneal flap good for prox tibia coverage
- Flexor hallucis flap good for heel coverage
- MRI
- While body bone scan
- CT chest, abd, pelvis
- Serum electrophoresis
- Osteoid osteoma is usually on the convex side of the curve
- Vertebral plana caused by langerhans cell histiocytosis can reconstitute height
- GCT in the mobile spine is usually in posterior elements
- Osteoblastoma is usually in the vertebral body
- Wheele's: at least 50% reconstitution of vertebral height may be expected
- Scoliosis
- Radiculitis
- Spondylolisthesis
- Spina Bifida Occulta
- Some potential for metastasis
- MDM2 gene amplification is rare
- Can dedifferentiate
- 10% risk of recurrence with resection
- JAAOS 2018
- ALTs and WDLs may best be conceptualized as precan- cerous, and the risk of malignant transformation depends on the anatomic location and lesional duration.4
- The risk of malignant dedifferentiation ranges from zero to 5%.4,5,15,18-21
- Although lacking metastatic potential, WDLs are essentially incurable
- C is TRUE - The risk of malignant dedifferentiation is higher with WDLs compared with ALTs, approximately 10% to 20%.4,13,14,26
- The reported local recurrence rates vary from 8.2% to 50.61%, with the largest series of 151 patients demonstrating a 10% risk.18-21
- Recurrence of 30% after extended intralesional curretage
- Most common in diaphysis of femur
- Can move to adjacent bone along the ligaments
- Cement decreases rate of recurrence
- Metaphysis and epiphysis
- Eccentric
- Although the medullary margins are well-defined, they are usually not sclerotic; in some cases, they may appear moth-eaten.
- Can cross to adjacent bones across ligaments!
- Recurrence of intralesional curettage approaches 20% without adjuvant therapy.
- PMMA does NOT lower the risk of recurrence.
- Use of PMMA allows for earlier WBAT
- Use of PMMA allows to see recurrence easier
- Exothermic reaction may be helpful with hemostasis.
- Bone cement decreases recurrence to 17%
- Metastatic to lung in 2-5%
- Hand lesions have greater chance of metastasis
- In a histopathologic study, Oliveira et al reported that gene rearrangements localized to t(16;17) in 36 of 52 primary ABCs (69%) in which the ubiquitin-specific protease 6 oncogene was placed under the regulatory influence of the highly active cadherin-11 promoter.
- Others have shown that upregulation of USP6 may induce matrix metalloproteinase production by activation of nuclear factor κ-light-chain-enhancer of activated B cells, which ultimately leads to tumorgenesis.
- In patients with ABC, age of onset ranges from 1 to 59 years, with the greatest prevalence between ages 12 and 13.
- Males are affected more than females. ABC being one of the most common malignancies (along with osteoid osteoma and osteoblastoma) that affect the posterior elements of the spine.
- Mass may elevate the periosteum, but it typically remains contained by a thin shell of cortex. Lesions in the epiphysis should raise suspicion of secondary changes caused by a different neoplastic process!
- Typically, ABCs are eccentric but may also be central or subperiosteal/surface based
- Fluid-filled levels: is highly suggestive of an ABC but is not pathognomonic because it is also a radiographic characteristic of
- Telangiectatic osteosarcoma
- GCT
- Secondary ABC
- Fracture through a simple cyst
- Chondroblastoma
- Recurrence was associated with young age and open growth plates.
