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Updated: 3/1/2022

Intramedullary Osteosarcoma

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https://devupload.orthobullets.com/topic/8014/images/Case A - prox tibia - xray ap - Parson_moved.png
https://devupload.orthobullets.com/topic/8014/images/Case A - prox tibia - mri axial - Parson_moved.png
https://devupload.orthobullets.com/topic/8014/images/Case E - femur shaft - xray ap - Parsons_moved.png
https://devupload.orthobullets.com/topic/8014/images/Case E - femur shaft -T2 MRI - Parsons_moved.png
https://devupload.orthobullets.com/topic/8014/images/2a_moved.jpg
https://devupload.orthobullets.com/topic/8014/images/2b_moved.jpg
https://devupload.orthobullets.com/topic/8014/images/Case A - prox tibia - xray lat - Parson_moved.png
https://devupload.orthobullets.com/topic/8014/images/Histology A_moved.png
  • SUMMARY
    • Intramedullary Osteosarcomas are malignant, aggressive, osteogenic bone tumors most commonly found in the distal femur or proximal tibia. Patients are typically children or young adults that present with rapidly progressive pain and swelling.
    • Diagnosis is made with a biopsy showing malignant mesenchymal cells with significant atypia and the presence of lacey osteoid.  
    • Treatment is usually neo-adjuvant chemotherapy, wide surgical resection, followed by adjuvant chemotherapy. 
  • Epidemiology
    • Incidence
      • make up approximately 2.4% of all pediatric cancers
      • ~5 cases per 1 million people per year
    • Demographics
      • usually occurs in children and young adults
        • bimodal distribution of occurrence
          • majority occur in the second decade of life
          • the second peak in occurrence is in elderly patients with Paget's disease and in those who have received prior radiation
        • affects males more than females and black/Hispanic patients more than white patients
    • Location
      • the most common site is the distal femur and proximal tibia
      • other common sites include the proximal humerus, proximal femur, and pelvis
  • ETIOLOGY
    • Grading and staging
      • most commonly diagnosed as MSTS┬áStage IIB (high grade, extra-compartmental, no metastases)
        • approximately 90% of diagnosed osteosarcomas are high-grade
      • 20-25% of patients present with radiographically detectable metastases
        • the lung is the most common site of metastasis (~80%)
        • bone is the second most common site
    • Genetics
      • tumor suppressor genes (AD inheritance)
        • Retinoblastoma tumor suppressor gene (Rb) predisposes to osteosarcoma
        • P53 tumor suppressor gene mutations (ie patients with Li-Fraumeni syndrome) predisposes to a high incidence of breast cancer and osteosarcoma
      • risk increased in Rothmund Thomson syndrome
        • AR inheritance, mutations in RECQL4 gene
        • 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
  • Symptoms
    • Symptoms
      • pain and swelling are the usual initial presenting symptoms
      • the median time of onset of symptoms to diagnosis is 4 months
    • Physical exam
      • inspection
        • swelling
        • palpable mass
        • tenderness to palpation over area of concern
      • motion
        • may have decreased range of motion if large soft-tissue mass
      • neurovascular
        • can cause nerve or vascular compression with mass effect
      • provocative tests
        • pain with axial loading if lower extremity lesion
  • 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 evidenced by soft-tissue shadow
    • MRI with and without contrast
      • must include entire involved bone
      • used to determine:
        • soft tissue and 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
    • PET-CT
      • no clear role for PET-CT imaging in osteosarcoma
      • potential uses include correlating percent necrosis to  standard uptake values (SUVs) in pre- and post-chemotherapy setting
      • can differentiate between benign and malignant pulmonary nodules
  • STUDIES
    • Serum Labs
      • elevated alkaline phosphatase and lactate dehydrogenase can suggest more aggressive disease
    • 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 (GCT) of bone
          • GCT of bone does not display the degree of cellular atypia or osteoid production
      • 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 the definitive surgeon
  • DifferentiaL
      • Osteosarcoma Differential
      • Destructive bone lesion in young patients
      • Treatment is Wide Resection & Chemotherapy
      • Osteosarcoma (intramed. & periosteal)
      • o
      • o
      • Ewing's sarcoma
      • o
      • o
      • Leukemia
      • o
      • Lymphoma
      • o
      • Eosinophilic granuloma
      • o
      • Osteomyelitis
      • o
      • Desmoplastic fibroma
      • o
      • MFH / fibrosarcoma
      • o
      • Dedifferentiated chondrosarcoma
      • o
      • Secondary sarcoma
      • o
      • Rhabdomyosarcoma (soft tissue)
      • o
      • ASSUMPTIONS: (1) Younger patient is < 40 yrs; (2) assuming no impending fracture
  • Treatment
    • Operative
      • neoadjuvant chemotherapy, limb salvage resection, followed by adjuvant chemotherapy
        • indications
          • high-grade osteosarcoma
        • chemotherapy
          • preoperative chemotherapy given for 8-12 weeks followed by maintenance chemotherapy for 6-12 months after surgical resection
            • standard chemotherapy is methotrexate, doxorubicin (adriamycin), and cisplatin (MAP therapy)
          • >90% 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
          • limb salvage is preferred whenever possible
          • overall survival in osteosarcoma is equal after limb salvage vs. amputation to deal with the local extent of disease
          • rotationplasty is a great surgical option that optimizes the patient's function and is most commonly done in a pediatric population
      • wide surgical resection alone
        • indications
          • low grade surface osteosarcoma such as parosteal osteosarcoma
      • amputation
        • indications
          • the only absolute indication for amputation is when a functional AND disease-free extremity cannot be achieved with limb salvage
          • pathologic fracture 
            • can undergo limb salvage if the fracture is stabilized throughout neoadjuvant chemotherapy (ex-fix or cast)
          • encasing neurovascular bundle
          • enlarging during preop chemo AND adjacent to neurovascular bundle
  • COMPLICATIONS
    • Progression of disease, including local recurrence and development of metastases
    • Prosthetic joint infection
      • occurs in 2-10% of patients
    • Aseptic loosening
      • Occurs in 7-13% of patients
    • Amputation secondary to limb salvage failure
  • Prognosis
    • 76% long-term survival with modern treatment
    • Poor prognostic factors include
      • advanced stage of disease (most predictive of survival)
      • response to neoadjuvant chemotherapy (as judged by percent tumor necrosis of resected specimen)
        • >90% necrosis is a good prognostic indicator
      • tumor site and size
      • expression of P-glycoprotein
      • high serum alkaline phosphatase
      • high lactate dehydrogenase
      • vascular involvement
      • positive surgical margins
      • type of chemotherapy regimen
        • multi-agent chemotherapy with methotrexate, doxorubicin, and cisplatin is superior to single-agent chemotherapy

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(OBQ18.85) An 8-year-old patient presents with 3 months of increasing pain and swelling of the left knee. Radiographs and MRI are in figures A-C, with histology in figure D. Which of the following surgical techniques has been shown to have higher self-reported outcomes and lower revision surgeries in pre-adolescent patients?

QID: 212981
FIGURES:
1

Intercalary allograft reconstuction

7%

(127/1941)

2

Endoprosthetic reconstruction

12%

(230/1941)

3

Amputation

13%

(249/1941)

4

Van Nes rotationplasty

63%

(1226/1941)

5

Transepiphyseal resection with bone transport

5%

(90/1941)

L 3 A

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(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

QID: 4744
FIGURES:
1

Biopsy

95%

(3452/3651)

2

Genetic screening for EXT1 and EXT2 gene mutations

1%

(48/3651)

3

NSAIDS and single-dose radiation therapy

1%

(21/3651)

4

Intravenous antibiotics

3%

(95/3651)

5

Observation

0%

(17/3651)

L 1 B

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(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?

QID: 4721
FIGURES:
1

Chondroblastoma

4%

(154/3484)

2

Intramedullary osteosarcoma

50%

(1742/3484)

3

Parosteal osteosarcoma

37%

(1295/3484)

4

Giant cell tumor

4%

(125/3484)

5

Osteoblastoma

4%

(143/3484)

L 5 B

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(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?

QID: 4705
FIGURES:
1

Curettage and targeted intravenous antibiotics for 6 weeks.

4%

(136/3876)

2

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

7%

(290/3876)

3

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

4%

(146/3876)

4

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

5%

(199/3876)

5

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

79%

(3074/3876)

L 2 A

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(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?

QID: 4588
FIGURES:
1

Ewing's Sarcoma

8%

(343/4306)

2

Eosinophilic granuloma

14%

(586/4306)

3

Lymphoma

1%

(53/4306)

4

Osteosarcoma

61%

(2615/4306)

5

Osteoblastoma

15%

(654/4306)

L 3 A

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(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?

QID: 4535
FIGURES:
1

Neoadjuvant chemotherapy, surgical excision, adjuvant radiation therapy

11%

(467/4289)

2

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

5%

(200/4289)

3

Surgical excision followed by adjuvant chemotherapy and radiation therapy

7%

(319/4289)

4

Neoadjuvant chemotherapy, surgical excision, adjuvant chemotherapy

74%

(3179/4289)

5

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

2%

(93/4289)

L 3 A

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(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?

QID: 3495
1

Through knee amputation

1%

(34/2500)

2

Above knee amputation

17%

(417/2500)

3

Rotationplasty

63%

(1570/2500)

4

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

14%

(356/2500)

5

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

4%

(111/2500)

L 3 B

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(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?

QID: 3120
FIGURES:
1

Figure B

11%

(328/3066)

2

Figure C

15%

(448/3066)

3

Figure D

11%

(349/3066)

4

Figure E

26%

(784/3066)

5

Figure F

37%

(1125/3066)

L 4 C

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

QID: 3006
1

axial skeleton > distal femur > proximal tibia > proximal humerus

1%

(19/1937)

2

distal femur > proximal tibia > proximal humerus > axial skeleton

88%

(1698/1937)

3

distal femur > axial skeleton > proximal tibia > proximal humerus

1%

(28/1937)

4

distal femur > proximal tibia > axial skeleton > proximal humerus

8%

(164/1937)

5

proximal tibia > distal femur > axial skeleton > proximal humerus

1%

(14/1937)

L 2 C

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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?

QID: 545
FIGURES:
1

Pain management

1%

(18/2517)

2

Hanging arm sling

3%

(79/2517)

3

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

4%

(98/2517)

4

Consultation with pediatric oncology

1%

(24/2517)

5

Tumor biopsy performed by interventional radiology

91%

(2281/2517)

L 1 A

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(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?

QID: 432
FIGURES:
1

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

3%

(43/1715)

2

Intramedullary nailing

1%

(16/1715)

3

Intravenous antibiotics

1%

(16/1715)

4

Radiation therapy

3%

(43/1715)

5

Preoperative chemotherapy, wide resection, and postoperative chemotherapy

93%

(1588/1715)

L 1 A

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(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?

QID: 724
1

High histologic grade

0%

(4/1253)

2

Low serum level of alkaline phosphatase at diagnosis

81%

(1010/1253)

3

Large tumor volume

2%

(20/1253)

4

Inadequate surgical margins following resection

1%

(7/1253)

5

70% histologic tumor necrosis after pre-operative chemotherapy

17%

(207/1253)

L 1 C

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(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?

QID: 383
FIGURES:
1

IA

1%

(16/3003)

2

IB

3%

(104/3003)

3

IIA

14%

(433/3003)

4

IIB

78%

(2329/3003)

5

IIIA

4%

(106/3003)

L 2 C

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

QID: 254
1

Bone

7%

(135/1904)

2

Liver

2%

(30/1904)

3

Lung

89%

(1696/1904)

4

Kidney

0%

(5/1904)

5

Lymph nodes

2%

(34/1904)

L 1 B

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(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?

QID: 189
FIGURES:
1

Observation

1%

(18/2532)

2

Chemotherapy and radiation therapy

2%

(54/2532)

3

Surgery alone

13%

(336/2532)

4

Surgery and chemotherapy

75%

(1901/2532)

5

Surgery and radiation therapy

8%

(211/2532)

L 2 A

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(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?

QID: 1006
1

Intra-lesional

57%

(1076/1881)

2

Inter-lesional

4%

(68/1881)

3

Wide

7%

(128/1881)

4

Marginal

30%

(559/1881)

5

Radical

2%

(43/1881)

L 3 D

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(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?

QID: 100
FIGURES:
1

Neoadjuvant chemotherapy followed by surgical excision

2%

(29/1368)

2

Neoadjuvant chemotherapy, surgical excision, adjuvant chemotherapy

84%

(1155/1368)

3

Surgical excision followed by radiation therapy

2%

(33/1368)

4

Neoadjuvant chemotherapy, surgical excision, radiation therapy

8%

(115/1368)

5

Surgical excision followed by adjuvant chemotherapy

2%

(33/1368)

L 1 B

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(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?

QID: 1269
FIGURES:
1

Synovial sarcoma

1%

(15/1880)

2

Malignant fibrous histiocytoma

5%

(101/1880)

3

Chondrosarcoma

6%

(111/1880)

4

Ewing's sarcoma

9%

(177/1880)

5

Osteosarcoma

78%

(1465/1880)

L 3 D

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(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?

QID: 118
FIGURES:
1

chemotherapy and surgery

57%

(468/816)

2

chemotherapy only

1%

(5/816)

3

surgery only

31%

(253/816)

4

radiation and surgery

10%

(79/816)

5

radiation only

0%

(4/816)

L 5 D

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