Updated: 5/4/2019

Intramedullary Osteosarcoma

Topic
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0
0
Questions
28
0
0
Evidence
26
0
0
Videos
1
Cases
2
Techniques
3
https://upload.orthobullets.com/topic/8014/images/Case A - prox tibia - xray ap - Parson_moved.png
https://upload.orthobullets.com/topic/8014/images/Case A - prox tibia - mri axial - Parson_moved.png
https://upload.orthobullets.com/topic/8014/images/Histology A_moved.png
https://upload.orthobullets.com/topic/8014/images/poikiloderma.jpg
https://upload.orthobullets.com/topic/8014/images/poikilo.jpg
https://upload.orthobullets.com/topic/8014/images/Case E - femur shaft - xray ap - Parsons_moved.png
Introduction
  • Intramedullary osteosarcoma is the most common primary sarcoma of bone
    • the most common malignancy of bone is metastatic disease
    • the most common primary malignancy of bone is myeloma
  • Age & location
    • usually occurs in children and young adults
      • bimodal distribution of occurrence
        • majority occur in the second decade of life
        • second peak in occurrence is in elderly patients with Paget's disease
    • most common site is the distal femur and proximal tibia
    • other common sites include proximal humerus, proximal femur, and pelvis
  • Malignancy
    • most commonly diagnosed as Stage IIB (high grade, extra-compartmental, no metastases)
    • 10-20% of patients present with pulmonary metastases (obtain CT of chest)
      • lung is most common site of metastasis
      • bone is second most common site
  • Genetics
    • patients who carry the Retinoblastoma tumor suppressor gene (Rb) are predisposed to osteosarcoma
    • risk increased in Rothmund Thomson syndrome 
      • AR inheritance, mutations in RECQL4 gene, chr 8q24.3
      • sun-sensitive facial poikiloderma rash (pigmentation, thinned skin, prominent blood vessels)   
      • absent eyelashes, eyebrows, hair  
      • juvenile cataracts, teeth abnormalities
      • osteosarcoma, fibrosarcoma, gastric adenocarcinoma, cutaneous BCC and SCC
  • Prognosis
    • 76% long-term survival with modern treatment
    • poor prognostic factors include
      • advanced stage of disease (most predictive of survival)
      • response to chemotherapy (as judged by percent tumor necrosis of resected specimen)
      • tumor site and size
      • expression of P-glycoprotein
      • high serum alkaline phosphatase
      • high lactic dehydrogenase
      • vascular involvement
      • surgical margins
      • type of chemotherapy regimen
Symptoms
  • Symptoms
    • rapidly progressive pain, fever, and swelling
  • Physical exam
    • may feel mass on examination
Imaging
  • Radiographs
    • characteristic blastic and destructive lesion  
      • sun-burst or hair on end pattern of matrix mineralization
    • periosteal reaction (Codman's triangle)
    • large soft tissue mass with maintenance of bone cortices
  • MRI
    • must include entire involved bone to determine
      • soft tissue involvement
      • neurovascular involvement
      • presence skip metastases   
        • if skip metastases are found, this is equivalent to metastatic (stage III) disease
  • Bone scan
    • very hot in osteosarcoma
    • useful to evaluate extent of local disease and presence of bone metastases
  • CT
    • chest CT is required at presentation to evaluate for pulmonary metastases   
Labs
  • Elevated alkaline phosphatase
    • may be 2-3 times normal value
Histology
  • Characteristics
    • tumor cells show significant atypia, and produce "lacey" osteoid
    • stroma cells show malignant characteristics with atypia, high nuclear to cytoplasmic ratio, and abnormal mitotic figures
  • May have mixed histology with different combinations of chondroblastic,osteoblastic, or fibroblastic looking cells
    • depends on the subtype of osteosarcoma
  • Giant cells may be present in giant cell rich osteosarcoma
    • often confused with giant cell tumor of bone
  • Diagnosis
    • biopsy is required to obtain tissue diagnosis and institute therapy 
    • improper biopsy techniques are associated with increased rates of complications
      • biopsy should be performed by the surgeon responsible for definitive treatment of the sarcoma, or after discussion with this surgeon 
Treatment
  • Operative
    • multi-agent chemotherapy and limb salvage resection       
      • indications
        • high grade osteosarcoma
      • chemotherapy  
        • preoperative chemotherapy given for 8-12 weeks followed by maintenance chemotherapy for 6-12 months after surgical resection
        • 98% necrosis after neo-adjuvant chemotherapy is good prognostic sign
        • expression of multi-drug resistance (MDR) gene tends to have a poor prognosis
          • tumor cells can pump chemotherapy out of cell with MDR expression
          • present in 25% of primary lesions and 50% of metastatic lesions
      • surgical technique
        • trend towards limb salvage whenever possible 
        • overall survival in osteosarcoma is equal after limb salvage vs. amputation to deal with local extent of disease
        • rotationplasty is a great surgical option which optimizes the patient's function, and most commonly done in a pediatric population  
    • wide surgical resection 
      • indications
        • indicated in low grade osteosarcoma such as parosteal osteosarcoma
    • amputation
      • indications
        • pathologic fracture
        • encasing neurovascular bundle
        • enlarging during preop chemo AND adjacent to neurovascular bundle
Differentials & Groups
 
Destructive bone lesion in young patients (1)
     
Treatment is Wide Resection & Chemotherapy (2)
   
Osteosarcoma (intramed. & periosteal)
     
   
Ewing's sarcoma
     
   
Leukemia
           
Lymphoma
           
Eosinophilic granuloma
           
Osteomyelitis
           
Desmoplastic fibroma
           
MFH / fibrosarcoma        
   
Dedifferentiated chondrosarcoma        
   
Secondary sarcoma        
   
Rhabdomyosarcoma (soft tissue)        
   
ASSUMPTIONS: (1) Younger patient is < 40 yrs; (2) assuming no impending fracture
 
IBank
  Location
Age
Xray
Xray
CT
Bone scan
MRI
MRI
Histo
Case A proximal tibia
13
 
Case A prox. tibia
 
Case B distal femur
 
 
Case C distal femur
 
Case D pelvis
 
 
Case E femoral shaft
 
Case F prox. humerus
 
Case F ankle
 
Case G              

 

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Technique Guides (3)
Questions (28)

(OBQ13.109) A 14-year-old immigrant from a developing country had complained of pain playing soccer 4 months prior. Radiographs at that time were read as normal and he was given a prescription for NSAIDs. More recently, his parents noticed he is limping and complaining of pain after playing games. He has had some intermittent fevers and swelling around his thigh. A complete blood workup is performed. Current radiographs and MRI are seen in Figures A and B respectively. What is the next step in treatment
Review Topic

QID: 4744
FIGURES:
1

Biopsy

94%

(2537/2689)

2

Genetic screening for EXT1 and EXT2 gene mutations

1%

(38/2689)

3

NSAIDS and single-dose radiation therapy

1%

(14/2689)

4

Intravenous antibiotics

3%

(74/2689)

5

Observation

0%

(13/2689)

ML 1

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PREFERRED RESPONSE 1

(OBQ13.86) An 11-year-old male presents with left forearm swelling and pain after getting kicked while playing soccer. Figures A through E are the radiographs, bone scan and MRI scan of the forearm and biopsy results. What is the most likely diagnosis? Review Topic

QID: 4721
FIGURES:
1

Chondroblastoma

4%

(124/2769)

2

Intramedullary osteosarcoma

49%

(1351/2769)

3

Parosteal osteosarcoma

38%

(1045/2769)

4

Giant cell tumor

4%

(107/2769)

5

Osteoblastoma

5%

(126/2769)

ML 5

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PREFERRED RESPONSE 2

(OBQ12.228) A 13-year-old presents with persistent leg pain. Representative radiographs are shown in Figures A and B. A biopsy is subsequently obtained and is shown in Figure C. What is the most likely diagnosis?
Review Topic

QID: 4588
FIGURES:
1

Ewing's Sarcoma

7%

(244/3417)

2

Eosinophilic granuloma

14%

(462/3417)

3

Lymphoma

1%

(45/3417)

4

Osteosarcoma

61%

(2076/3417)

5

Osteoblastoma

16%

(544/3417)

ML 3

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PREFERRED RESPONSE 4

(OBQ06.197) A 13-year-old girl presents with right thigh pain and constitutional symptoms for 8 weeks. The patient's serum LDH and alkaline phosphatase are elevated. A radiograph of the right femur is shown in Figure A. Computed tomography (CT) of the chest reveals no pulmonary metastases. Magnetic resonance imaging (MRI) of the right femur shows erosion of the tumor through the cortex into the adjacent soft tissue, but no skip lesions are seen. A biopsy specimen is shown in Figures B and C. What stage does this patient's case most appropriately represent using the Enneking Staging System for Musculoskeletal Tumors? Review Topic

QID: 383
FIGURES:
1

IA

1%

(11/2169)

2

IB

4%

(76/2169)

3

IIA

15%

(319/2169)

4

IIB

77%

(1671/2169)

5

IIIA

4%

(83/2169)

ML 2

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PREFERRED RESPONSE 4
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(OBQ08.159) You are asked to consult on a 8-year-old child in the emergency department with the pathologic fracture seen in Figure A. In preparation for transfer to a sarcoma center for treatment, all of the following are correct steps in the child's management EXCEPT? Review Topic

QID: 545
FIGURES:
1

Pain management

1%

(10/1670)

2

Hanging arm sling

2%

(41/1670)

3

Tumor staging, including bone scan, CT of chest, MRI of the entire bone

4%

(68/1670)

4

Consultation with pediatric oncology

1%

(13/1670)

5

Tumor biopsy performed by interventional radiology

91%

(1528/1670)

ML 1

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PREFERRED RESPONSE 5

(OBQ09.193) Which of the following most accurately lists the most common location of osteosarcoma in decreasing frequency? Review Topic

QID: 3006
1

axial skeleton > distal femur > proximal tibia > proximal humerus

1%

(19/1368)

2

distal femur > proximal tibia > proximal humerus > axial skeleton

87%

(1190/1368)

3

distal femur > axial skeleton > proximal tibia > proximal humerus

1%

(15/1368)

4

distal femur > proximal tibia > axial skeleton > proximal humerus

9%

(126/1368)

5

proximal tibia > distal femur > axial skeleton > proximal humerus

1%

(9/1368)

ML 1

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PREFERRED RESPONSE 2

(OBQ08.46) A 19-year-old woman presents with dull right thigh pain for the past 2 months. Radiographs and a biopsy specimen are shown in Figures A-C. What is the most appropriate definitive treatment? Review Topic

QID: 432
FIGURES:
1

Nonsteroidal anti-inflammatory drugs with follow-up x-ray in 6 months

3%

(33/1230)

2

Intramedullary nailing

1%

(8/1230)

3

Intravenous antibiotics

1%

(13/1230)

4

Radiation therapy

2%

(28/1230)

5

Preoperative chemotherapy, wide resection, and postoperative chemotherapy

93%

(1139/1230)

ML 1

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PREFERRED RESPONSE 5

(OBQ04.39) A 14-year-old child presents to your office with 6 months of worsening knee pain. Figures A through D show the respective radiographs, bone scan, MRI, and histology. What treatment protocol has been shown to maximize survival in patients with this diagnosis? Review Topic

QID: 100
FIGURES:
1

Neoadjuvant chemotherapy followed by surgical excision

2%

(24/1017)

2

Neoadjuvant chemotherapy, surgical excision, adjuvant chemotherapy

85%

(868/1017)

3

Surgical excision followed by radiation therapy

3%

(27/1017)

4

Neoadjuvant chemotherapy, surgical excision, radiation therapy

7%

(73/1017)

5

Surgical excision followed by adjuvant chemotherapy

2%

(23/1017)

ML 1

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PREFERRED RESPONSE 2

(OBQ06.243) What is the most common site of metastasis for osteosarcoma? Review Topic

QID: 254
1

Bone

7%

(110/1533)

2

Liver

2%

(24/1533)

3

Lung

89%

(1364/1533)

4

Kidney

0%

(5/1533)

5

Lymph nodes

2%

(26/1533)

ML 1

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PREFERRED RESPONSE 3

(OBQ13.70) An 11-year-old boy presents with knee pain, warmth and swelling after a fall. Radiographs, MRI, bone scan and biopsy of the knee are seen in Figures A through E. What is the most appropriate treatment plan? Review Topic

QID: 4705
FIGURES:
1

Curettage and targeted intravenous antibiotics for 6 weeks.

3%

(112/3229)

2

Curettage, cancellous bone grafting, non-weightbearing until visible callus is seen on follow-up radiographs.

8%

(247/3229)

3

Curettage, placement of antibiotic beads, intravenous antibiotics for 6 weeks, removal of beads and placement of bone graft substitute at 6 weeks.

4%

(118/3229)

4

Neoadjuvant chemotherapy, physeal-sparing resection, intercalary bone allograft, locked plate fixation, postoperative adjuvant chemotherapy.

5%

(167/3229)

5

Neoadjuvant chemotherapy, wide resection of the distal one-third of the femur, reconstruction with a custom implant, postoperative adjuvant chemotherapy.

79%

(2562/3229)

ML 2

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PREFERRED RESPONSE 5

(OBQ12.175) A 13-year-old boy presents with left leg pain for 10 weeks. He reports a remote history of trauma while falling off of a trampoline 12 weeks ago. He reports constitutional symptoms for the past 4 weeks. The patient's serum LDH and alkaline phosphatase are elevated. A radiograph of the left knee is shown in Figures A and B. A bone scan is displayed in Figure C. Magnetic resonance imaging (MRI) is shown in Figure D and E. A biopsy specimen is shown in Figure F. What is the most appropriate next step in management? Review Topic

QID: 4535
FIGURES:
1

Neoadjuvant chemotherapy, surgical excision, adjuvant radiation therapy

11%

(383/3579)

2

Surgical irrigation and debridridement followed by 6 weeks of culture directed intravenous antibiotics

5%

(168/3579)

3

Surgical excision followed by adjuvant chemotherapy and radiation therapy

7%

(241/3579)

4

Neoadjuvant chemotherapy, surgical excision, adjuvant chemotherapy

75%

(2686/3579)

5

Surgical irrigation and debridridement with antibiotic cement spacer placement followed by 6 weeks of culture directed intravenous antibiotics

2%

(76/3579)

ML 3

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PREFERRED RESPONSE 4

(OBQ07.121) A 13-year-old girl presents with knee pain for 2 months especially at night. She denies fevers and weight loss. Her physical exam reveals a painful thigh mass. A radiograph is shown in Figure A. What is the next most appropriate step in managment? Review Topic

QID: 782
FIGURES:
1

Repeat radiographs in 3 months with observation

1%

(13/1987)

2

External beam radiation and chemotherapy

0%

(7/1987)

3

Surgical biopsy and culture directed intravenous antibiotics

2%

(30/1987)

4

Neoadjuvant chemotherapy followed by wide excision and adjuvant chemotherapy

5%

(104/1987)

5

MRI of the entire bone, whole body bone scan and CT chest

92%

(1825/1987)

ML 1

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PREFERRED RESPONSE 5

(OBQ06.78) A 17-year-old male presents with increasing right shoulder pain and swelling. There is no history of trauma and he is otherwise healthy. Representative clinical photograph, radiograph, bone scan, MRI, and histology are shown in Figures A through E. What is the most appropriate treatment for this patient? Review Topic

QID: 189
FIGURES:
1

Observation

1%

(15/2141)

2

Chemotherapy and radiation therapy

2%

(47/2141)

3

Surgery alone

14%

(298/2141)

4

Surgery and chemotherapy

74%

(1592/2141)

5

Surgery and radiation therapy

8%

(178/2141)

ML 2

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PREFERRED RESPONSE 4

(OBQ04.164) A 10-year-old boy has 2 months of right knee pain that started at summer camp. The patient denies constitutional symptoms. There is no lymphadenopathy present. CT of the chest shows no signs of metastatic disease. Imaging studies and biopsy results are shown in Figures A-E. What is the most likely diagnosis? Review Topic

QID: 1269
FIGURES:
1

Synovial sarcoma

1%

(12/1462)

2

Malignant fibrous histiocytoma

5%

(69/1462)

3

Chondrosarcoma

6%

(82/1462)

4

Ewing's sarcoma

8%

(124/1462)

5

Osteosarcoma

80%

(1171/1462)

ML 2

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PREFERRED RESPONSE 5

(OBQ07.63) In patients with an extremity-based osteosarcoma without metastasis, all of the following are risk factors for disease progression and poor outcomes EXCEPT? Review Topic

QID: 724
1

High histologic grade

0%

(3/942)

2

Low serum level of alkaline phosphatase at diagnosis

81%

(759/942)

3

Large tumor volume

1%

(13/942)

4

Inadequate surgical margins following resection

0%

(4/942)

5

70% histologic tumor necrosis after pre-operative chemotherapy

17%

(158/942)

ML 2

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PREFERRED RESPONSE 2

(OBQ04.7) A 30-year-old man has had leg pain for 6 months. A lesion is identified in the proximal femur and biopsy it taken. Histology is shown in Figure A and is consistent with a low-grade intramedullary osteogenic sarcoma. Additional imaging studies confirm that this is an isolated lesion with no metastasis. What is the standard treatment for this type of lesion? Review Topic

QID: 118
FIGURES:
1

chemotherapy and surgery

57%

(282/496)

2

chemotherapy only

0%

(2/496)

3

surgery only

31%

(155/496)

4

radiation and surgery

10%

(52/496)

5

radiation only

1%

(3/496)

ML 5

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PREFERRED RESPONSE 3

(OBQ10.32) A 16-year-old boy presents with a 6-month history of increasing knee pain and swelling. He is otherwise healthy, but the child has a known retinoblastoma gene mutation. A mass is found on the posterior aspect of his knee, and a biopsy is taken. A radiograph is shown in Figure A. Which of the following biopsy specimens in Figures B through F most likely fits with this patient's history, exam, and radiograph? Review Topic

QID: 3120
FIGURES:
1

Figure B

10%

(247/2496)

2

Figure C

16%

(389/2496)

3

Figure D

11%

(282/2496)

4

Figure E

26%

(649/2496)

5

Figure F

36%

(905/2496)

ML 4

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PREFERRED RESPONSE 5

(OBQ11.72) A 8-year-old boy presents with knee pain and an effusion. Biopsy and staging studies show a distal femoral osteosarcoma with contamination of the knee joint. Which of the following treatment options will provide this child with the best chance of local control and the highest level of function? Review Topic

QID: 3495
1

Through knee amputation

2%

(31/1975)

2

Above knee amputation

16%

(322/1975)

3

Rotationplasty

63%

(1240/1975)

4

Extra-articular resection, endoprosthetic reconstruction, and free flap coverage

14%

(285/1975)

5

Extra-articular resection, allograft prosthetic composite, and free flap coverage

5%

(91/1975)

ML 3

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PREFERRED RESPONSE 3

(OBQ05.120) A 13-year-old boy is diagnosed with osteosarcoma of his distal femur. The treating surgeon samples the bone marrow from the resection margin of the femur during definitive limb salvage surgery and finds microscopic evidence of tumor. Assuming no change in the resection level, what type of resection would this be considered? Review Topic

QID: 1006
1

Intra-lesional

57%

(845/1480)

2

Inter-lesional

4%

(54/1480)

3

Wide

6%

(89/1480)

4

Marginal

31%

(457/1480)

5

Radical

2%

(31/1480)

ML 3

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PREFERRED RESPONSE 1
ARTICLES (38)
POSTS (5)
Evidence
intra articular versus extra articular resection in osteosarcoma around joint
  • Pathology
  • - Intramedullary Osteosarcoma
Evidence
The use of extracorporeally irradiated autografts in pelvic reconstruction following tumour resection
  • Pathology
  • - Intramedullary Osteosarcoma
Evidence
skip lesions are poor prognostic indicator for osteosarcoma
  • Pathology
  • - Intramedullary Osteosarcoma
Evidence
Effects of resection margins on local recurrence of osteosarcoma in
  • Pathology
  • - Intramedullary Osteosarcoma
Evidence
Rotationplasty: Beauty is in the Eye of the Beholder
  • Pathology
  • - Intramedullary Osteosarcoma
VIDEOS (1)
CASES (2)
Topic COMMENTS (31)
Private Note