Updated: 6/21/2021

Intramedullary Osteosarcoma

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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/Case E - femur shaft - xray ap - Parsons_moved.png
https://upload.orthobullets.com/topic/8014/images/Case E - femur shaft -T2 MRI - Parsons_moved.png
https://upload.orthobullets.com/topic/8014/images/2a_moved.jpg
https://upload.orthobullets.com/topic/8014/images/2b_moved.jpg
https://upload.orthobullets.com/topic/8014/images/Case A - prox tibia - xray lat - Parson_moved.png
https://upload.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 tumor cells with significant atypia and the presence of lacey osteoid.  
    • Treatment is usually neo-adjuvant chemotherapy, limb salvage surgical resection, followed by adjuvant chemotherapy. 
  • Epidemiology
    • Demographics
      • 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
    • Anatomic location
      • most common site is the distal femur and proximal tibia
      • other common sites include proximal humerus, proximal femur, and pelvis
  • EIOLOGY
    • 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
      • tumor suppressor genes
        • patients with mutations in the Retinoblastoma tumor suppressor gene (Rb) are predisposed to osteosarcoma
        • patients with mutations in the P53 tumor suppressor gene are at risk for Li-Fraumeni syndrome which is associated with a high incidence of breast cancer and 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
  • 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
      • used 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
  • STUDIES
    • Serum 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
  • DifferentiaL
    • Osteosarcoma Differential
      Destructive bone lesion in young patients

      Treatment is Wide Resection & Chemotherapy

      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
  • 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
          • 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
  • 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)
        • Greater than 90% is a good prognostic indicator
      • tumor site and size
      • expression of P-glycoprotein
      • high serum alkaline phosphatase
      • high lactic dehydrogenase
      • vascular involvement
      • surgical margins
      • type of chemotherapy regimen

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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%

(3390/3586)

2

Genetic screening for EXT1 and EXT2 gene mutations

1%

(48/3586)

3

NSAIDS and single-dose radiation therapy

1%

(20/3586)

4

Intravenous antibiotics

3%

(93/3586)

5

Observation

0%

(17/3586)

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%

(152/3442)

2

Intramedullary osteosarcoma

50%

(1719/3442)

3

Parosteal osteosarcoma

37%

(1281/3442)

4

Giant cell tumor

4%

(124/3442)

5

Osteoblastoma

4%

(141/3442)

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%

(136/3819)

2

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

8%

(287/3819)

3

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

4%

(144/3819)

4

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

5%

(196/3819)

5

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

79%

(3027/3819)

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%

(338/4251)

2

Eosinophilic granuloma

14%

(581/4251)

3

Lymphoma

1%

(52/4251)

4

Osteosarcoma

61%

(2581/4251)

5

Osteoblastoma

15%

(645/4251)

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%

(465/4256)

2

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

5%

(199/4256)

3

Surgical excision followed by adjuvant chemotherapy and radiation therapy

7%

(314/4256)

4

Neoadjuvant chemotherapy, surgical excision, adjuvant chemotherapy

74%

(3157/4256)

5

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

2%

(92/4256)

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%

(34/2468)

2

Above knee amputation

17%

(414/2468)

3

Rotationplasty

63%

(1545/2468)

4

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

14%

(353/2468)

5

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

4%

(110/2468)

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%

(320/3009)

2

Figure C

15%

(445/3009)

3

Figure D

11%

(335/3009)

4

Figure E

26%

(773/3009)

5

Figure F

37%

(1104/3009)

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%

(19/1912)

2

distal femur > proximal tibia > proximal humerus > axial skeleton

88%

(1676/1912)

3

distal femur > axial skeleton > proximal tibia > proximal humerus

1%

(27/1912)

4

distal femur > proximal tibia > axial skeleton > proximal humerus

8%

(162/1912)

5

proximal tibia > distal femur > axial skeleton > proximal humerus

1%

(14/1912)

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%

(16/2442)

2

Hanging arm sling

3%

(74/2442)

3

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

4%

(94/2442)

4

Consultation with pediatric oncology

1%

(23/2442)

5

Tumor biopsy performed by interventional radiology

91%

(2217/2442)

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

2%

(41/1697)

2

Intramedullary nailing

1%

(16/1697)

3

Intravenous antibiotics

1%

(16/1697)

4

Radiation therapy

2%

(42/1697)

5

Preoperative chemotherapy, wide resection, and postoperative chemotherapy

93%

(1573/1697)

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%

(3/1201)

2

Low serum level of alkaline phosphatase at diagnosis

80%

(965/1201)

3

Large tumor volume

2%

(19/1201)

4

Inadequate surgical margins following resection

0%

(6/1201)

5

70% histologic tumor necrosis after pre-operative chemotherapy

17%

(203/1201)

L 2 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%

(15/2965)

2

IB

4%

(104/2965)

3

IIA

14%

(428/2965)

4

IIB

78%

(2298/2965)

5

IIIA

4%

(105/2965)

L 2 C

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

QID: 254
1

Bone

7%

(132/1861)

2

Liver

2%

(30/1861)

3

Lung

89%

(1657/1861)

4

Kidney

0%

(5/1861)

5

Lymph nodes

2%

(33/1861)

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%

(17/2502)

2

Chemotherapy and radiation therapy

2%

(54/2502)

3

Surgery alone

13%

(333/2502)

4

Surgery and chemotherapy

75%

(1876/2502)

5

Surgery and radiation therapy

8%

(210/2502)

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%

(1071/1873)

2

Inter-lesional

4%

(68/1873)

3

Wide

7%

(128/1873)

4

Marginal

30%

(556/1873)

5

Radical

2%

(43/1873)

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%

(28/1344)

2

Neoadjuvant chemotherapy, surgical excision, adjuvant chemotherapy

85%

(1137/1344)

3

Surgical excision followed by radiation therapy

2%

(32/1344)

4

Neoadjuvant chemotherapy, surgical excision, radiation therapy

8%

(114/1344)

5

Surgical excision followed by adjuvant chemotherapy

2%

(31/1344)

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%

(14/1835)

2

Malignant fibrous histiocytoma

5%

(98/1835)

3

Chondrosarcoma

6%

(110/1835)

4

Ewing's sarcoma

9%

(172/1835)

5

Osteosarcoma

78%

(1434/1835)

L 2 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%

(457/800)

2

chemotherapy only

1%

(5/800)

3

surgery only

31%

(248/800)

4

radiation and surgery

10%

(79/800)

5

radiation only

0%

(4/800)

L 5 D

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