Tumor (2008-2019) Flashcards
1
Q
- Components of Mirel Criteria (2012)
A
- Pain
- Location
- Matrix
- Size
2
Q
- 70 year old with metastatic bone disease and pathological midshaft femur fracture. Has history consistent with thyroid mets. (2010, 2014)
- what is the best surgical management for the fracture?
- what intervention should be considered pre-op?
- what medication should be used postop for the skeletal system?
- what other treatment should be instituted at the fracture site to reduce local recurrence/expansion?
A
what is the best surgical management for the fracture?
- Cephalomedullary nail
what intervention should be considered pre-op?
- Embolization (renal and thyroid mets)
what medication should be used postop for the skeletal system?
- bisphosphonates
what other treatment should be instituted at the fracture site to reduce local recurrence/expansion?
- Radiation therapy
- Decreases likelihood of progression and to relieve pain
3
Q
- 60 yo woman with breast CA and multiple lesions. You are shown an pelvic AP xray with a large supraacetabular lesion with significant bone loss. No protrusion, acetabular sourcil was intact, but bone loss started approximately 3 mm superior to that.
- Uncemented THA
- Protrusio cup with a cemented femoral component
- PMMA and screws and cage with a cemented femoral component
- Standard cemented THA
A
ANSWER: C
2013
- 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”
4
Q
4.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?
- Metastatic breast cancer
- Myeloma
- Osteosarcoma
- Chondrosarcoma
A
ANSWER: C
2008, 2014
- Osteoblastic Metastasis:
- Suva LJ (Nature Review 2011) Bone Metastasis: mechanism and therapeutic opportunities
- Osteosclerotic metastases via secretion of endothelin-1 which decreases DKK-1 –> less Wnt Suppression
- Weldin JSES 2010:
- 208 patients with metastatic lesions of the humerus
- 64% breast, 30% kidney
- HOWEVER –> metastatic disease does not usually have a soft tissue component, therefore this is much more likely to be osteosarcoma (probably post-radiation)
5
Q
5.60 yo male with known metastatic renal ca with impending pathologic fracture through lytic lesion 5 cm distal to lesser trochanter. Best treatment?
- Short cephallomedullary nail locked distally
- Long cephalomedullary nail locked distally
- Regular IM nail locked statically
- Regular IM nail locked dynamically
A
ANSWER: B
2011, 2013
No need to diagnosis, just stabilize whole femur
6
Q
- 66 yo male with history of bladder cancer 2 yrs prior, surgery, no adjuvant therapy needed. Yesterday stumbled and hurt leg and suffered transverse fracture of midshaft femur. X-ray given with mottled edges, no obvious soft tissue or intra-diaphyseal mass. CT C/A/P, blood work all normal. Bone scan lights up at fracture site. What is next step?
- Antegrade IM nail
- Retrograde IM nail
- Biopsy
- Antegrade nail, then send reaming for biopsy, then get Prism to call CMPA for you
A
ANSWER: C
2012
Remote cancer with new lesion = biopsy
7
Q
- Older guy with metastatic cancer and 6 months to live. Aggressive Peritrochanteric lesion with LT avulsion. How do you treat?
- Palliative
- Non WB and chemo
- Non WB and rads
- Prophylactic Cephalomedullary nail
A
ANSWER: D
2012
- Argument:
- Palliative treatment would be more acceptable with shorter survival expectancy (weeks)
- Do not want to make this patient non-weightbearing at all
- Chemo in tumor types that are extremely sensitive (lymphoma) would be reasonable but still high risk area that has already had pathologic fracture
- Radiation appropriate if combined with fixation
- McCalden would do arthroplasty – makes more sense
- Somewhat difficult without more information
8
Q
- Regarding core needle biopsy, all are true except:
a. Saves a surgical procedure
b. Must resect the tract
c. A viable tumour can be accessed with image guidance
d. Biopsy should be in line with planned surgical incision
A
ANSWER: B (old answer)
2015
Argument –> don’t need to resect the tract if it is benign pathology
9
Q
- Patient comes in with hypercalcemia, positive electrophoresis and multiple lytic lesions in the pelvis. What is the next step in management?
- Fix the pelvis
- Bone scan
- Skeletal survey
- Drink martini
A
ANSWER: C
2012
10
Q
- List 5 steps to systemically stage an Ewing’s sarcoma. (2012, 2014)
A
- CT Chest
- Bone Scan
- Bone Marrow Biopsy
- Lab Studies - LDH
- Molecular studies - FISH
- Local Staging - X-ray whole bone, MRI
11
Q
- What are 5 prognostic factors for osteosarcoma (2013)
A
JAAOS 2009 - Osteosarcoma
- Metastatic disease
- Tumor grade (conventional vs surface)
- Primary tumor located in axial skeleton
- Large tumor volume
- Increased ALP/LDH
- Poor response to chemo (<90% necrosis)
- Secondary sarcoma
12
Q
- List 4 extra-osseous features of fibrous dysplasia. (2011, 2013)
A
JAAOS 2004 - Fibrous Dysplasia
McCune Albright - Café-au-lait spots, precocious puberty
Mazabraud - Soft tissue myxomas
Metabolic Syndromes - hyperthyroidism, hypophosphatemia, acromegaly, Cushing’s disease, Hyperprolactinemia
Cherubism - eye deviation and blindness
13
Q
- Shown a photo of fibrous dysplasia. Told that a new lytic lesion developed. (2016)
- List ONE benign cause
- List ONE malignant cause
A
- List ONE benign cause - ABC
- Diercks RL (JBJS Br 1986) ABC in association with fibrous dysplasia
- Mintz MC (Radiol 1987) ABC arising in fibrous dysplasia during pregnancy
- List ONE malignant cause - Sarcoma (osteosarcoma > fibrosarcoma > chondrosarcoma)
- JAAOS 2004 - Fibrous Dysplasia
14
Q
- A 23 y.o. male has 4 month history of increasing pain in proximal phalanx of middle finger. Not shown the x-ray, but told that there is a lytic lesion that the radiologist thinks is benign. List 7 possible diagnoses (2009, 2010)
A
JAAOS 2016 - Enchondroma of the Hand
- Infections and Tumor-like Conditions
- Pyogenic osteomyelitis
- Cystic tuberculosis
- Coccidiodomycosis
- ABC
- UBC
- Intra-osseous Epidermal Inclusion cyst
- Benign Tumor:
- Enchondroma
- GCT
- Chondroblastoma
- Malignant Tumor:
- Chondrosarcoma
- Ewing Sarcoma
- Lymphoma
- Osteogenic osteosarcoma
- Acrometastasis
15
Q
- List 3 predictors for successful treatment of UBC of the proximal humerus with methylprednisone (2010)
A
- Older age
- Single cystic component
- Proximity to the physis
- Multiple injections
- Although most UBCs resolve by the time the patient reaches skeletal maturity, patient age and the location of the cyst within the physis are predictors of successful treatment that may aid clinicians in counseling patients and their families about the expected clinical course. For some authors, the only predictor of successful treatment is the age of the patient; patients older than 10 years heal at a higher rate (90%) than do younger patients (60%), no matter what treatment regimen is used.32 Alternatively, a lesion located ,2 cm from the physis may be a risk factor for recurrence.45 The risk of recurrence, however, may be related to the treatment modality used rather than the location of the lesion. Surgeons are likely to be less aggressive when the cyst is closer to the physis to avoid the potential for physeal damage.
16
Q
- Chondrosarcoma - list 3 factors for prognosis before planning definitive treatment.(2015)
A
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
17
Q
- A patient with an osteoid osteoma of the C5 lamina is seen in clinic. She has been refractory to non-op treatment. What is the next step?
- Radiofrequency ablation
- En bloc resection
- Curettage and bone grafting
- Resection and fusion
A
ANSWER – A (if compression or spinal deformity = en bloc resection)
- Tough question. Old group spoke to Lewkonia, he says that with new ablation tips, the risk to dura/roots is minimal unless there is significant compression by the lesion, which in this case there isn’t.
- Argument for B vs C:
- Don’t really need to bone graft a lamina, also don’t need to fuse it
- Parsons - no ablation in the spine
- Random other consideration … if pediatric patient <15 years, then fusion reasonable because of high risk of progressive post-lami kyphosis
- Vanderschueren GM (Spine 2009) RFA of spinal osteoid osteoma
- Success in 79% of patients after one treatment, 96% after multiple treatments, 43% had persistent scoliosis
- In our opinion, lesions abutting the dura can be safely treated with RFA with a 5-mm noncooled tip, because of the heat sink effects of the spinal fluid and venous plexus. The first successful RFA of such an osteoid osteoma (abutting the dura) was reported in 2000 by Dupuy et al. Two-thirds (16 of 24) of our patients had lesions <10 mm away from vital neural structures (Table 1). In only 1 patient RFA failed (Table 1), because the lesion in this patient caused nerve root compression, and, in retrospect, this patient should probably not have been considered for RFA. The 5-mm noncooled tip we used allows precise destruction of the target tissue. Other authors used a cooled tip to treat spinal osteoid osteomas. Although no complications occurred, a cooled tip is not recommended because of the risk of neural injury, related to the larger but also unpredictable size of the treatment zone.
- We conclude that CT-guided RFA of spinal osteoid osteoma with a 5-mm non-cooled electrode tip is an effective treatment for spinal osteoid osteoma, and can be safely performed close to the dura or exiting nerve root. It should be the treatment of choice in lesions > 2 mm away from the nerve root, and surgery should be re- served for lesions adjacent (< 2 mm) to the nerve root causing nerve root compression. In addition, RFA is easily repeatable after unsuccessful treatment.
- JAAOS Review: Benign Tumors of the Spine
- Proximity of the lesion to neural structures may preclude the use of RFA, and use of these techniques may risk thermal damage to adjacent neural structures.
- En bloc excision is recommended in patients with osteoid osteoma with associated fixed spinal deformity, with neurologic compression, or if RFA is deemed unsafe due to the anatomic location or has been previously unsuccessful.
18
Q
- Patient with pain to his lateral lower leg that is worse at night and was initially relieved with NSAIDs. CT shows a well organized sclerotic border with a central nidus. He has ongoing pain no longer relieved with NSAIDs. What would you do next?
- Currettage and bone grafting
- Radiofrequeny ablation
- Wide resection
- ?chemo or rads
A
ANSWER: B
2016
JAAOS 2011 - Osteoid Osteomas and Osteoblastomas
Radiofrequency ablation for everything except vertebral column, close to peripheral nerves, fingers, toes and carpal bones
19
Q
- A 25yo female with (a few months?) of leg pain twists and feels a crack in her hip. X-rays (described, not given) show a sclerotic lesion with an ossified soft tissue mass. MRI shows a small hematoma. Biopsy showed osteoid. What is the most appropriate treatment?
- 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
A
ANSWER: A
2014
- Osteosarcoma doesn’t respond to radiation so B wrong
- C - need addition of chemotherapy
- A vs D
- Bacci G (Acta Orthop Scand 2003)Nonmetastatic osteosarcoma of the extremity with pathologic fracture at presentation: local and systemic control by amputation or limb salvage after preoperative chemotherapy.
- ….We conclude that with neoadjuvant chemotherapy, osteosarcoma patients presenting with a pathologic fracture can be surgically treated like those with no fracture, and that limb salvage procedures do not increase the risk of local recurrence or death of these patients.
- Pathological fractures in primary bone sarcomas. (Injury 2008)
- Pathological fractures in patients with primary bone sarcomas should not be considered an absolute indication for amputation. Initial fracture management should include cast immobilization or external fixation avoiding tumour-cell dissemination. The extent of fracture displacement and the type of fracture stabilisation may affect the outcome of patients with primary bone sarcomas presenting with pathological fractures. Patients with high-grade primary bone sarcomas should be treated by neo-adjuvant chemotherapy, and limb-salvage surgery. Pathological fractures in chemo
20
Q
- Concerning a giant cell tumour of the forearm, what is true:
- 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
A
ANSWER: D
2013
- 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
21
Q
- Regarding GCT, all of the following are TRUE EXCEPT:
a. Commonly involves the epiphysis
b. Most often seen in patients <20yo
c. Commonly osteogenic
d. Can cause soft tissue extension and formation of a neocortex
A
ANSWER: B
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.
22
Q
- GCT lesions, what is true:
- Treated by en-block resection
- Can erode through cortex, and invade into soft tissues
- Have no potential to metastasis to the chest
A
ANSWER: B
2013
JAAOS 2013 - GCT of Bone
- 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
23
Q
- All are true about treating GCT with curettage and cement vs. bone graft except?
- Can immediately weight bear
- Easier detection of recurrence
- Decreases local recurrence
- Exothermic reaction can be helpful
A
ANSWER: C
2008, 2011
- 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
24
Q
- ABC in a 5 yo distal femur with slight valgus. All of the following are true except:
- 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.
A
ANSWER: C
2013
JAAOS 2012- ABC
- 20% recurrence rate
- MRI showing fluid fluid levels is not pathognomonic but highly suggestive of ABC
- Some cases of regression, but not common
25
Q
- Chordoma involves S3 to S5. What is the appropriate treatment
- Wide resection and chemo
- Wide Resection, chemo, and radiation
- Wide resection and radiation
- Wide resection
A
ANSWER: D
2013
- OKU MSK Tumors
- “Like chondrosarcomas, chordomas are insensitive to chemotherapy and RT because of their slow growth…Surgery is the best option for a cure; however, because of the complex anatomy caused by the location of these neoplasms and the large size on presentation, chordomas are difficult to resect with wide margins….The usefulness of RT has not been established, but may be of benefit if you have positive resection margins.”
26
Q
- What tumor is not typically located in the posterior spinal elements?
- ABC
- Chordoma
- Osteoblastoma
- Osteoid Osteoma
A
ANSWER: B
2016
- JAAOS – Benign Tumors of the Spine
- Osteoid Osteoma “approximately 10% occur in the vertebrae, primarily in the posterior elements”
- Most common cause of painful scoliosis in the adolescent population
- Osteoblastoma:
- Spine accounts for 28-36% of osteoblastomas
- “typically arise in the posterior elements, although extension into the vertebral body is common with larger tumors”
- Most common in lumbar spine
- ABC:
- 15% of primary spine tumors
- Boriani (spine 2001) most lesions in posterior elements, 70% thoracolumbar
- JAAOS – Chordoma of the Sacrum
- Typically found in the midline of the neuroaxis, arise from intraosseous notochordal remnants
- 50% sacrococcygeal, 35% sphenooccipital, 15% mobile spine
27
Q
- MFH of bone treatment (PUS)
- Chemo and radiation
- Surgical excision and chemo
- Surgical excision and radiation
- Surgical excision
A
ANSWER: B
2011, 2013
- AAOS OKU: MSK Tumors 2 (p. 198)
- Most patients can be treated surgically with a limb-sparing surgical resection in lieu of amputation. In many cases, patients are treated with preoperative (induction or neoadjuvant) chemotherapy, and postoperative (adjuvant) chemotherapy…Radiation is rarely used in the treatment of MFH of bone, although it may be used for treatment of an unresectable tumor or for treatment following resection with close or microscopically positive margins.
- Nishida J (Cancer 1997) Malignant fibrous histiocytoma of bone
- Prognosis similar to osteosarcoma
- Some patients had a good response to radiation therapy
- Majority of patients had surgery alone
- Jeon DG (CORR 2011) MFH of bone and osteosarcoma show similar survival and chemosensitivity
- Patients with MFH-B and osteosarcoma have similar survival rates and histologic responses to chemotherapy
28
Q
- Given a AP Pelvis xray with fibrous dysplasia with destroyed hip. What is the best type of bone graft to use.
- Cortical allograft
- Cancellous allograft
- Cortical autograft
- Cancellous autograft
A
ANSWER: A
2013
DiCaprio M (JBJS 2005) Current Concepts Review Fibrous Dysplasia
29
Q
- Given an x-ray showing fibrous dysplasia. What is this not associated with or not at risk for?
- Pleomorphic undifferentiated sarcoma= MFH
- Chondrosarcoma
- Osteosarcoma
- Fibrosarcoma
A
ANSWER: 2020-2021 consensus asnwer is A
[B (was previously changed to A)]
2016
- JAAOS Fibrous Dysplasia 2004
- Malignant transformation in about 0.5% of patients with monostotic disease and 4% with McCune-Albright
- Malignant degeneration to osteosarcoma most common, but fibrosarcoma, chondrosarcoma or MFH sometimes develops
- Ruggieri (Cancer 1994)
- Review of 28 malignant transformations:
- 19 osteosarcoma, 5 fibrosarcoma, 3 chondrosarcoma, 1 MFH
- Yabut (CORR 1988)
- Review of literature – 83 cases
- Osteosarcoma (40 cases)
- Fibrosarcoma (22 cases)
- Chondrosarcoma (11 cases)
30
Q
- What is true about fibrous dysplasia:
- It is a developmental malformation of bone
- Monostotic is often symptomatic
- Polyostotic is more common
- Xray and MRI are necessary for diagnosis
A
ANSWER: A
2015
- JAAOS 2004/JBJS 2005 Current Concepts
- 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
31
Q
- What is a characteristic of an NOF
- Epiphyseal
- Eccentric
- Multi-loculated
- Less than 1cm
A
ANSWER: B
2012
- JAAOS 2004 - Metaphyseal Fibrous Defects
- Non-ossifying fibromas are larger than fibrous cortical defects, occupy an eccentric location in the medullary cavity of long-bone metaphyses and usually involve but do not breach the cortex
32
Q
- 8 year old boy with proximal phalanx fracture. Shown well circumscribed lesion with stippled calcification. What to do?
- Amputate (O’Neill style)
- Open biopsy
- Reduce and immobilize
- CRPP
A
ANSWER: C
2011, 2012
JAAOS 2016 - Enchondroma of the Hand
- Traditionally, enchondroma-related pathologic fractures have been treated with 1-2 months of immobilizations to allow healing before curettage (adults)
- I asked Dhaliwal about this and he agrees that the majority of people still allow the fractures to heal first then treat the enchondroma down the road once ROM regained
33
Q
- What is the most common primary malignant tumor in the hand?
- MFH
- Osteosarcoma
- Chondrosarcoma
- Fibrosarcoma
A
ANSWER - C
2014
- JAAOS 2006 Malignant Tumors of the Hand and Wrist
- “Chondrosarcoma is the most common malignant bone tumor in the hand”
- Although osteosarcoma is the most common primary bone tumor in children and adolescents, fewer than 40 cases in the hand have been reported
- Baumhoer D (JHS Eur 2010) Tumours of the hand: a review on histology of bone malignancies
- Primary malignant tumours of the hands are extremely rare
- benign bone tumours were much more common, representing 12% of all non-malignant postcranial lesions, of which nearly 50% were enchondromas.
- Malignant bone tumours of the hand comprise basically all types of bone sarcomas. However, according to the literature and to our experience, chondrosarcomas outnumber by far all variants of osteosarcomas, Ewing sarcomas/ PNETs (primitive neuroectodermal tumours) as well as other tumours, e.g. fibrosarcomas, leiomyosarcomas and angiosarcomas, the latter generally being reported as single cases.
34
Q
- Tumors; all except:
- Retinoblastoma (it said RF-1) is associated with increased risk of OS
- Neurofibrillin (NF-1) gene is associated with malignant nerve tumors
- MET with Chondrosarcoma
- NF and Astrocytoma
- Sudbury = cool (none of the above)
A
ANSWER: E
- 2012
- Recall issues, all schools had different stems
- Other schools had NF and CNS tumors –> which would be correct
- Retinoblastoma definitely associated with osteosarcoma
- NF associated with MPNST
- Evans ()
- Risk of developing and dying from MPNST 8-13% (lifetime)
- Bikowska-Opalach B (Histo Histopath 2014) Pilocytic astrocytoma: a review of genetic and molecular factors…
- The most important causative factors seem to be NF1 gene inactivation and BRAF gene overexpression
- MET = multiple enchondromatosis (?) then yes IF Maffucci
35
Q
- Conditions associated with secondary development of Chondrosarcomas (all except)
- Olliers
- Mafuccis
- MHE
- McCune Albright syndrome
A
ANSWER: D
2008, 2012
Ollier’s Disease –> multiple enchondromatosis –> chondrosarcoma
Mafucci’s Disease –> multiple enchondromatosis –> chondrosarcoma
MHE –> osteochondroma –> chondrosarcoma
McCune Albright –> fibrous dysplasia –> fibrosarcoma, osteosarcoma, MFH
36
Q
- All are true about multiple enchondromas (Oilliers or Maffuci’s) except?
- 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.
A
ANSWER: D
2011
JAAOS 2010 - Secondary Chondrosarcoma
- No genetic predisposition for inheritance of Ollier or Maffucci
- Maffucci has near 100% transformation into chondrosarcoma classically, studies show more accurate is probably 15-20%
- Patients are at higher risk for chondrosarcoma, but it has the same prognosis as isolated chondrosarcoma
- According to Peter Ferguson –> secondary chondrosarcomas have a BETTER prognosis than de novo ones
- From Orthobullets on Ollier’s
- Involved bones are dysplastic, with shortening and bowing
37
Q
- All of the following are associated with osteosarcoma EXCEPT?
- Radiation
- Paget’s
- Li Fraumeni
- Hand-Schuller-Christian
A
ANSWER - D
2014
- JAAOS - Osteosarcoma
- Genetic pre-disposition commonly involves mutations in tumor suppressor genes…Li Fraumeni syndrome and retinoblastoma
- Post-radiation and Paget’s Sarcoma
- Hand Schuller Christian —> eosinophilic granuloma (exopthalmos, diabetes insipidus)
38
Q
- All of the following genetics and tumor syndromes have been found to be associated, except:
- Retinoblastoma (Rp-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
A
ANSWER: B
2016
- Rb/Osteosarc
- JAAOS 2009 – Osteosarcoma
- “Tumor suppressor genes normally regulate the cell cycle; mutations will result in uncontrolled cell proliferation as seen in Li-Fraumeni syndrome, involving the p53 gene, or retinoblastoma, involving the RB1 gene”
- NF-1 and malignant CNS
- JAAOS – Orthopedic Manifestations of NF
- Increased risk of MPNST, melanoma, leukemia, rhabdomyosarcoma, pheo, carcinoma, pancreatic endocrine tumors and astrocytic tumors
- Astrocytomata is benign CNS tumor, MPNST is PNS
- Definitely evidence out there than CNS tumors associated with NF
- EXT/Osteochondroma
- JAAOS 2005 Manifestations of HME
- Chromosome/Ewings:
- JAAOS 2010 – Ewing’s Sarcoma Family of Tumors
64
Q
- Which tumor is not associated with a translocation?
- Synovial Sarcoma
- Osteosarcoma
- Ewing Sarcoma
- Liposarcoma
A
ANSWER: B
2008
87
Q
- 30 year old with groin pain. Shown an image of a hip with what I thought looked like extensive synovial chondromatosis (?). No arthritic changes. How should you manage this?
- Surgical hip dislocation and synovectomy
- Chemotherapy and resection
- Radiation and resection
- Total hip arthroplasty
A
ANSWER: A
2018
-indication for either hip scope or surgical hip dislocation
88
Q
- A patient is confirmed to have a high grade osteosarc. I think it’s a female and she might be 18. You are shown a clinical image (its in the calcaneus, the calc is fractured and it extended to where the Achilles would insert). What is the best treatment?
- Neodjuvant chemo, negative margin resection surgery, reconstruction
- Neoadjuvant chemo, BKA
- Radiation
- Immediate BKA
A
ANSWER: B
2018
