Intramedullary Osteosarcoma

Author: Patrick O'Donnell

Topic updated on 04/26/13 8:09pm

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 portends 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

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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Qbank (14 Questions)

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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? Topic

Review Topic

1. Through knee amputation
2. Above knee amputation
3. Rotationplasty
4. Extra-articular resection, endoprosthetic reconstruction, and free flap coverage
5. Extra-articular resection, allograft prosthetic composite, and free flap coverage

PREFERRED RESPONSE ▶

DISCUSSION: While intra-articular extension of osteosarcoma is a rare presentation, understanding of the various treatment options available is crucial for the treating surgeon. Often joint contamination occurs secondary to a previous surgical procedure when a malignant neoplasm isn't considered in the differential diagnosis. While limb salvage may be possible with joint contamination, optimization of function and limitation of the number of future surgical procedures needs to be considered. Rotationplasty (Illustrations A and B) offers the treating surgeon the ability to perform a safe and negative margin resection while at the same time maximizing patient function and limiting the number of future re-operations.

Damron et al review the various joint-related tumors which mimic sports-related injuries in their instructional course lecture. They suggest that appropriate preoperative imaging and consideration of malignant conditions in the differential diagnosis of any suspicious condition will increase the chances that the treating surgeon correctly identifies the neoplasm and avoids a procedure which compromises limb-salvage.

Quan et al retrospectively reviewed 27 cases of peri-articular osteosarcoma, specifically looking for intra-articular involvement. They suggest that intra-articular involvement of osteosarcoma is very rare as articular cartilage serves as a good boundary to tumor expansion. Joint contamination from an incorrect surgical procedure is a much more likely cause of intra-articular extension.

Incorrect answers
1) Through knee amputation - with joint contamination, a through knee amputation would not provide an appropriate negative margin resection and therefore contaminate the distal resection margin with tumor
2) Above knee amputation - while this would provide a safe resection level, the level of function would not be as great as with a rotationplasty
4 & 5) Both of these options would be available for limb salvage, however they would not result in as high a level of function as a rotationplasty. In addition these procedures would likely require many subsequent operations given the higher rates of complication and the need for future prosthetic lengthening/exchange.

Illustrations: A

REFERENCES:

1. Damron TA, Morris C, Rougraff B, Tamurian R. Diagnosis and treatment of joint-related tumors that mimic sports-related injuries. Instr Course Lect. 2009;58:833-47. PMID:19385590 (Link to Abstract)

2. Quan GM, Slavin JL, Schlicht SM, Smith PJ, Powell GJ, Choong PF. Osteosarcoma near joints: assessment and implications. J Surg Oncol. 2005 Sep 1;91(3):159-66. PMID:16118770 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Jan Szatkowski MD, Ben Taylor MD,

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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? Topic

Review Topic

FIGURES: A

1. Figure B
2. Figure C
3. Figure D
4. Figure E
5. Figure F

PREFERRED RESPONSE ▶

DISCUSSION: Figure F shows diffuse cellular atypia with nuclear pleomorphism and "lacey" osteoid matrix production which are consistent with the diagnosis of osteosarcoma. Patients who carry mutations in the retinoblastoma gene are predisposed to this malignancy. Figure B displays numerous multinucleated giant cells and represents a giant cell tumor. Figure C shows Ewing's sarcoma, which consists of densely packed uniform small blue-stained cells in sheets. The cells have scant cytoplasm without distinct borders. Figure D is consistent with the microscopic appearance of an osteochondroma. It displays the benign cartilagenous cap at the left of the image, and the bony cortex at the right. Figure E shows a chondrosarcoma. The tissue is recognizable as cartilage, and there are chondrocytes in clear spaces, but there is no orderly pattern. At the bottom of the figure, the neoplasm can be seen invading and destroying bone.

REFERENCES:

1. Damron TA. Orthopaedic Surgery Essentials, Oncology and Basic Science. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:177-185.

2. Hornicek FJ. Osteosarcoma of bone. In: Schwartz HS, ed. Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:163-174.

Question Authors:

Joshua Blomberg MD, Patrick McCulloch MD, John Badylak MD,

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

Review Topic

1. axial skeleton > distal femur > proximal tibia > proximal humerus
2. distal femur > proximal tibia > proximal humerus > axial skeleton
3. distal femur > axial skeleton > proximal tibia > proximal humerus
4. distal femur > proximal tibia > axial skeleton > proximal humerus
5. proximal tibia > distal femur > axial skeleton > proximal humerus

PREFERRED RESPONSE ▶

DISCUSSION: The distal femur is the most common location of osteosarcoma, followed by the proximal tibia, the proximal humerus, and then the spine and sacrum in decreasing frequency. The reference by Mankin et al reviewed 648 patients with centrally located osteosarcoma reviewed over a 30 year period at their institution. They found 222 cases involved the distal femur while 109 involved the proximal tibia. The proximal humerus ranked 3rd with 63 of the cases, and the spine/sacrum was farther down the list with 19 cases. The most profound independent variable that affected patient survival rate was use of chemotherapy. This was followed (in order) by the patient's age, MSTS stage of the tumor, percentage of cells killed after neoadjuvant therapy, tumor size, and anatomic site.

REFERENCES:

1. Mankin HJ, Hornicek FJ, Rosenberg AE, Harmon DC, Gebhardt MC. Survival data for 648 patients with osteosarcoma treated at one institution. Clin Orthop Relat Res. 2004 Dec;(429):286-91. PMID:15577500 (Link to Abstract)

Question Authors:

Daniel Stahl MD,

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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? Topic

Review Topic

FIGURES: A

1. Nonsteroidal anti-inflammatory drugs with follow-up x-ray in 6 months
2. Intramedullary nailing
3. Intravenous antibiotics
4. Radiation therapy
5. Preoperative chemotherapy, wide resection, and postoperative chemotherapy

PREFERRED RESPONSE ▶

DISCUSSION: The clinical presentation, radiographs, and pathology are consistent with osteosarcoma. The radiographs show a mixed lytic/blastic destructive lesion. The pathology specimen shows pleomorphic osteoblastic cells and lacey osteoid. Therefore, the answer to this question is neo-adjuvant chemo and wide resection. In the referenced study by Bacci et al they reported that the “The five-year disease-free survival and overall survival rates were 60.7% (limb salvage) and 68.5% (amputation), respectively, with no significant difference between patients undergoing amputation and those undergoing resection.”

REFERENCES:

1. Bacci G, Ferrari S, Lari S, Mercuri M, Donati D, Longhi A, Forni C, Bertoni F, Versari M, Pignotti E. Osteosarcoma of the limb. Amputation or limb salvage in patients treated by neoadjuvant chemotherapy. J Bone Joint Surg Br. 2002 Jan;84(1):88-92 PMID:11837839 (Link to Abstract)

2. Simon MA. Limb salvage for osteosarcoma. J Bone Joint Surg Am. 1988 Feb;70(2):307-10. Review. PMID:3277972 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Derek Moore,

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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? Topic

Review Topic

FIGURES: A

1. Pain management
2. Splinting of the extremity
3. Tumor staging, including bone scan, CT of chest, MRI of the entire bone
4. Consultation with pediatric oncology
5. Tumor biopsy performed by interventional radiology

PREFERRED RESPONSE ▶

DISCUSSION: Tumor biopsy, even if performed by interventional radiology, can contaminate the future resection of this malignant appearing proximal humeral lesion, making limb salvage not possible. Pain control, splinting, staging/consultation with pediatric oncology will not negatively affect this child's oncologic outcome in the way that an inappropriate biopsy can. The referenced article by Mankin et al. summarizes the efforts of the 1982 Musculoskeletal Tumor Society's (MSTS) review of biopsies performed either by surgeons or at facilities not accustomed to treating bone and soft tissue tumors. Errors, complications, and changes in the course and outcome were two to twelve times greater (p < 0.001) when the biopsy was done in a referring institution instead of in a treatment center. As such, it is the stance of the MSTS that the biopsy should be performed under the direction of the treating surgeon.

REFERENCES:

1. Mankin HJ, Mankin CJ, Simon MA. The hazards of the biopsy, revisited. Members of the Musculoskeletal Tumor Society. J Bone Joint Surg Am. 1996 May;78(5):656-63. PMID:8642021 (Link to Abstract)

2. Menendez LR (ED): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, AAOS, 2002, pp29-34

Question Authors:

Patrick O'Donnell MD/PhD,

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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? Topic

Review Topic

1. High histologic grade
2. Low serum level of alkaline phosphatase at diagnosis
3. Large tumor volume
4. Inadequate surgical margins following resection
5. 70% histologic tumor necrosis after pre-operative chemotherapy

PREFERRED RESPONSE ▶

DISCUSSION: Alkaline Phosphatase is a marker for bone turnover and an increased level indicates tumor activity. Therefore, elevated (not low) serum alkaline phosphatase is a risk factor for progression in patients with an extremity-based osteosarcoma without metastasis.

Bacci et al conducted a multivariate analysis of risk factors and found that age < or = 14 years, high serum levels of alkaline phosphatase, tumor volume > 200 mL, a two-drug regimen chemotherapy, inadequate surgical margins, and poor histologic response to treatment all were independent risk factors for events like tumor recurrence, metastasis or death.

Meyers et al performed a similar study in 1992 and found that disease free survival correlated with serum lactate dehydrogenase (LDH), alkaline phosphatase, primary tumor site, race, and histologic response to preoperative chemotherapy.

REFERENCES:

1. Meyers PA, Heller G, Healey J, Huvos A, Lane J, Marcove R, Applewhite A, Vlamis V, Rosen G. Chemotherapy for nonmetastatic osteogenic sarcoma: the Memorial Sloan-Kettering experience. J Clin Oncol. 1992 Jan;10(1):5-15. PMID:1370176 (Link to Abstract)

2. Bacci G, Longhi A, Versari M, Mercuri M, Briccoli A, Picci P. Prognostic factors for osteosarcoma of the extremity treated with neoadjuvant chemotherapy: 15-year experience in 789 patients treated at a single institution. Cancer. 2006 Mar 1;106(5):1154-61. PMID:16421923 (Link to Abstract)

Question Authors:

Charles Jobin MD, Michael Hughes MD, Ben Taylor MD,

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(OBQ07.121) A 17-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? Topic

Review Topic

FIGURES: A

1. repeat radiographs in 3 months with observation
2. external beam radiation
3. intravenous antibiotics
4. wide excision
5. MRI of the entire bone, whole body bone scan, CT chest

PREFERRED RESPONSE ▶

DISCUSSION: The radiograph shows a destructive lesion of the distal femur. As this is likely a malignancy (osteosarcoma) it should be staged prior to biopsy, as described by Simon and Finn. CT of the chest, bone scan, and MRI of the femur should be performed before biopsy to look for skip lesions. CT looks for metastasis to lung tissue, which is the most common site of disseminated disease caused by malignant primary bone and soft-tissue tumors. Observation with follow-up radiographs is not appropriate because this lesion is painful and radiographically aggressive. While infection should be suspected in children with pain and radiographic changes concerning for osteomyelitis, IV antibiotics are inappropriate because the patient’s history does not suggest an infectious etiology and further noninvasive workup is necessary prior to invasive methods. Radiation and wide excision should not be performed prior to proper work-up.

REFERENCES:

1. Simon and Finn: Diagnostic strategy for bone and soft tissue tumors. Instr Course Lect. 1994; 43: 527-536 PMID:9097183 (Link to Abstract)

Question Authors:

Greg Galano MD,

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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? Topic

Review Topic

FIGURES: A

1. Observation
2. Chemotherapy and radiation therapy
3. Surgery alone
4. Surgery and chemotherapy
5. Surgery and radiation therapy

PREFERRED RESPONSE ▶

DISCUSSION: The clinical presentation is consistent with osteosarcoma. Treatment for this patient would consist of neoadjuvant chemotherapy, surgical excision with reconstruction, and adjuvant chemotherapy.

Osteosarcoma is the most common primary sarcoma of bone. It usually occurs in children and young adults with the majority occuring in the second decade of life.

Quan et al retrospectively reviewed 27 cases of peri-articular osteosarcoma, specifically looking for intra-articular involvement. They suggest that intra-articular involvement of osteosarcoma is very rare as articular cartilage serves as a good boundary to tumor expansion. Joint contamination from an incorrect surgical procedure is a much more likely cause of intra-articular extension.

Figure B is a radiograph that shows a destructive, non-geographic, bone-forming lesion in the proximal humerus.Figure C shows the lesion is hot on bone scan. Figure D is an MRI concerning for intra-articular tumor contamination which would alter the surgical resection. Figure E is a histology slide that shows atypical malignant cells on a background of malignant osteoid.

REFERENCES:

1. Menendez LR (ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2002, pp 175-186.

2. Quan GM, Slavin JL, Schlicht SM, Smith PJ, Powell GJ, Choong PF. Osteosarcoma near joints: assessment and implications. J Surg Oncol. 2005 Sep 1;91(3):159-66. PMID:16118770 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Ben Taylor MD,

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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 no skip lesions, and erosion of the tumor through the cortex into the adjacent soft tissue. 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? Topic

Review Topic

FIGURES: A

1. IA
2. IB
3. IIA
4. IIB
5. IIIA

PREFERRED RESPONSE ▶

DISCUSSION: This patient's history, imaging, and biopsy are consistent for osteosarcoma. The patient's biopsy specimen displays a high grade osteosarcoma with atypical nuclei and mitoses present, and therefore is a II as there is no evidence of metastasis (Low grade= I and High grade=II, presence of metastases=III). The question stem states the lesion is extracompartmental on MRI as the tumor has eroded through the cortical bone and breeched the "compartment" of the femur and is therefore a B (Intracompartmental=A, Extracompartmental=B). Therefore this patient has a IIB osteosarcoma of the distal femur. It is not a 2B, as the lesion is malignant. The Enneking staging system for benign lesions is detailed with arabic numerals (1=inactive, 2=active, and 3=aggressive).

The article by Meyers et al revealed that adjuvant chemotherapy improves disease-free survival (DFS) for patients with osteosarcoma. They found that the 5-year DFS was 76% for patients aged less than or equal to 21 years. A table showing the Enneking classification system is shown in illustration A. A gross pathology picture taken of this patients extirpated osteosarcoma is shown in Illustration B.

Illustrations: A

REFERENCES:

2. Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res. 1980 Nov-Dec;(153):106-20. PMID:7449206 (Link to Abstract)

Question Authors:

Michael Hughes MD,

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

Review Topic

1. bone
2. liver
3. lung
4. kidney
5. lymph nodes

PREFERRED RESPONSE ▶

DISCUSSION: The most common site of metastasis for osteosarcoma is the lung via hematogenous spread. When a patient is diagnosed with osteosarcoma, a chest CT is recommended as part of the initial staging process. Bone is the second most common site.

REFERENCES:

1. Lawrence R. Menendez (Ed.), Orthopaedic Knowledge Update: Musculoskeletal Tumors, American Academy of Orthopaedic Surgeons.

2. Malignant bone tumors. Gibbs CP Jr, Weber K, Scarborough MT. Instr Course Lect. 2002;51:413-28. PMID:12064130 (Link to Abstract)

Question Authors:

Greg Galano MD, Derek Moore,

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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? Topic

Review Topic

1. Intra-lesional
2. Inter-lesional
3. Wide
4. Marginal
5. Radical

PREFERRED RESPONSE ▶

DISCUSSION: Based on the Enneking classification of tumor resections, this would be classified as a intra-lesional resection as there are residual tumor cells in the resection margin of the femur. Intra-lesional resections occur in the lesion, as in this case, and are not appropriate for malignant sarcomas. For osteosarcomas, it is common to "sample" the marrow margin at the level of the bone cut during resection.

The referenced article by Enneking is the original description of resection margins used in soft tissue sarcomas. Because this referenced article was published when the surgical treatment for soft tissue sarcomas didn't include adjuvant therapy, the results aren't as applicable today. Today with adjuvant therapy, many sarcomas which are close to neurovascular structures can be safely resected without sacrifice of these critical structures.

Wrong Answers:
Answer 2: The Enneking classification does not use the term inter-lesional.
Answer 3: Wide resections are those where the tumor compartment is maintained but a "cuff" of normal tissue surrounds the resected specimen. If you think of the tumor as the fruit of an orange, a wide resection takes a normal cuff of tissue (the rind) around the tumor in 360 degrees (Illustration A).
Answer 4: Marginal resections are those which occur through the periphery of the tumor in the zone of the pseudocapsule where tumor cells can be located.
Answer 5: Radical resections are those in which the entire "compartment " of the tumor is removed. In the case of Illustration A, a radical resection would require removal of the entire femur as that is the "compartment".

Illustrations: A

REFERENCES:

1. Menendez JR, ed. OKU: Musculoskeletal Tumors. 2002. American Academy of Orthopaedic Surgeons. Rosemont, IL.

2. Enneking WF, Spanier SS, Malawer MM. The effect of the Anatomic setting on the results of surgical procedures for soft parts sarcoma of the thigh. Cancer. 1981 Mar 1;47(5):1005-22. PMID:7226034 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Patrick McCulloch MD, Dave Marcu MD,

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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? Topic

Review Topic

FIGURES: A

1. chemotherapy and surgery
2. chemotherapy only
3. surgery only
4. radiation and surgery
5. radiation only

PREFERRED RESPONSE ▶

DISCUSSION: The histology shows "lacey" osteoid with minimal cellular atypia. This is consistent with low-grade intramedullary osteogenic sarcoma. Low grade central osteogenic sarcoma is an uncommon form of osteosarcoma that is characterized by a long premorbid history and is compatible with prolonged survival after treatment. Treatment is surgery with wide margins alone and chemotherapy and radiation are not indicated. Good local control is associated with an excellent long term survival.

Choong et al. reviewed the long term follow-up of 20 patients diagnosed with low grade osteogenic sarcoma and found the 5 year survival rate was 90% and at 10 years was 85%. Local recurrence is a key feature in most cases and is typically the result of inadequate surgical margins frequently arising from initial misdiagnosis. Although amputation generally is successful for primary and recurrent tumors, limb salvage surgery is a definite option.

Kurt et al reviewed 80 well-differentiated osteosarcomas. They found local excision was almost always associated with recurrence. Wide excision was almost never followed by recurrence. The recurrent tumor was a high-grade, conventional osteosarcoma in 15% of the patients, and this was associated with a poor prognosis. They recommend wide excision as the treatment of choice for this very rare variant of osteosarcoma.

REFERENCES:

1. Choong PF, et al. Low grade osteogenic sarcoma: a long term follow-up of 20 patients. Clin Orthop, 322:198-206. 1990 PMID:8542697 (Link to Abstract)

2. Kurt AM, et al. Low grade intraosseous osteosarcoma. Cancer, 65:14-18-28. 1990 PMID:2306687 (Link to Abstract)

Question Authors:

Derek Moore, John Badylak MD, Ben Taylor MD,

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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? Topic

Review Topic

FIGURES: A

1. Neoadjuvant chemotherapy followed by surgical excision
2. Neoadjuvant chemotherapy, surgical excision, adjuvant chemotherapy
3. Surgical excision followed by radiation therapy
4. Neoadjuvant chemotherapy, surgical excision, radiation therapy
5. Surgical excision followed by adjuvant chemotherapy

PREFERRED RESPONSE ▶

DISCUSSION: The history, images, and histology all suggest a high grade osteosarcoma. The appropriate treatment includes neoadjuvant chemotherapy, surgical excision, and adjuvant chemotherapy. The difference between neoadjuvant and adjuvant therapy is their relationship to the surgical excision. You could, for example in high grade soft tissue sarcomas, have neoadjuvant chemotherapy and neoadjuvant radiation therapy followed by surgical excision. It is just a matter of nomenclature and the effective protocols of management of the specific disease. In the referenced article by Bruce Rougraff, he and Mike Simon compared the aforementioned management combined with either limb salvage or amputation for the surgical resection. Turns out, while local recurrence and need for additional surgery is higher in patients undergoing limb salvage surgery, the overall mortality is about the same between the two groups. Limb salvage patients also function better - which is why the tumor society now encourages limb salvage when appropriate.

REFERENCES:

1. Koval, KJ. Orthopedic Knowledge Update 7. Rosemount, IL, AAOS, 2002, pp 155-181

2. Rougraff BT, Simon MA, Kneisl JS, Greenberg DB, Mankin HJ. Limb salvage compared with amputation for osteosarcoma of the distal end of the femur. A long-term oncological, functional, and quality-of-life study. J Bone Joint Surg Am. 1994 May;76(5):649-56. PMID:8175811 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD,

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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? Topic

Review Topic

FIGURES: A

1. Synovial sarcoma
2. Malignant fibrous histiocytoma
3. Chondrosarcoma
4. Ewing's sarcoma
5. Osteosarcoma

PREFERRED RESPONSE ▶

DISCUSSION: The age, imaging and histology are consistent with an osteosarcoma. The radiograph shows an aggressive (lytic, mottled, sclerotic) appearing lesion around the distal femur metadiaphyseal region. The T2 weighted MRI image shows a significant soft tissue mass which appears to arise from the distal femur with destruction of the adjacent cortex. The biopsy shows an infiltrative pattern with elements of osteoid and bone.
Treatment for osteosarcoma includes neoadjuvant chemotherapy, wide resection, and adjuvant chemotherapy.

REFERENCES:

1. Menedez LR (ed): Orthopaedic Knowledge Update: Musuloskeletal Tumors. Rosemont, IL, American Academy of Orthopedic Surgeons, 2002, pp 305-312

2. Simon MA, Springfield D (eds): Surgery for bone and soft-tissue tumors. Philadelphia, PA Lippincott-Raven, 1998, 756pp.

Question Authors:

Paul D. Kim MD, Patrick McCulloch MD, Ben Taylor MD,

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Cases

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Tibial osteosarcoma in a 12-year-old boy (C1166)

Pathology - - Intramedullary Osteosarcoma

HPI - 12-year-old boy presents with 2 months of knee pain and a limp. Biopsy and stag...

What surgical treatment would you propose for a 12-year-old with a proximal...

3/6/2012

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Evidence & References Show References

Level of Evidence 3 (Retrospective Cohort Study or Case-Control Study)

Enneking WF, Spanier SS, Malawer MM. The effect of the Anatomic setting on the results of surgical procedures for soft parts sarcoma of the thigh. Cancer. 1981 Mar 1;47(5):1005-22. PMID:7226034 (Link to Abstract)
Mankin HJ, Hornicek FJ, Rosenberg AE, Harmon DC, Gebhardt MC. Survival data for 648 patients with osteosarcoma treated at one institution. Clin Orthop Relat Res. 2004 Dec;(429):286-91. PMID:15577500 (Link to Abstract)

Level of Evidence 4 (Case Series)

Meyers PA, Heller G, Healey J, Huvos A, Lane J, Marcove R, Applewhite A, Vlamis V, Rosen G. Chemotherapy for nonmetastatic osteogenic sarcoma: the Memorial Sloan-Kettering experience. J Clin Oncol. 1992 Jan;10(1):5-15. PMID:1370176 (Link to Abstract)
Quan GM, Slavin JL, Schlicht SM, Smith PJ, Powell GJ, Choong PF. Osteosarcoma near joints: assessment and implications. J Surg Oncol. 2005 Sep 1;91(3):159-66. PMID:16118770 (Link to Abstract)

Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions, Personal Observations, and Biomechanic Studies)

Damron TA, Morris C, Rougraff B, Tamurian R. Diagnosis and treatment of joint-related tumors that mimic sports-related injuries. Instr Course Lect. 2009;58:833-47. PMID:19385590 (Link to Abstract)
Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res. 1980 Nov-Dec;(153):106-20. PMID:7449206 (Link to Abstract)

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Menendez JR, ed. OKU: Musculoskeletal Tumors. 2002. American Academy of Orthopaedic Surgeons. Rosemont, IL.
Menendez LR (ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2002, pp 175-186.

Undefined

Bacci G, Ferrari S, Lari S, Mercuri M, Donati D, Longhi A, Forni C, Bertoni F, Versari M, Pignotti E. Osteosarcoma of the limb. Amputation or limb salvage in patients treated by neoadjuvant chemotherapy. J Bone Joint Surg Br. 2002 Jan;84(1):88-92 PMID:11837839 (Link to Abstract)
Bacci G, Longhi A, Versari M, Mercuri M, Briccoli A, Picci P. Prognostic factors for osteosarcoma of the extremity treated with neoadjuvant chemotherapy: 15-year experience in 789 patients treated at a single institution. Cancer. 2006 Mar 1;106(5):1154-61. PMID:16421923 (Link to Abstract)
Choong PF, et al. Low grade osteogenic sarcoma: a long term follow-up of 20 patients. Clin Orthop, 322:198-206. 1990 PMID:8542697 (Link to Abstract)
Damron TA. Orthopaedic Surgery Essentials, Oncology and Basic Science. Philadelphia, PA: Lippincott Williams & Wilkins; 2008:177-185.
Hornicek FJ. Osteosarcoma of bone. In: Schwartz HS, ed. Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2007:163-174.
Koval, KJ. Orthopedic Knowledge Update 7. Rosemount, IL, AAOS, 2002, pp 155-181
Kurt AM, et al. Low grade intraosseous osteosarcoma. Cancer, 65:14-18-28. 1990 PMID:2306687 (Link to Abstract)
Lawrence R. Menendez (Ed.), Orthopaedic Knowledge Update: Musculoskeletal Tumors, American Academy of Orthopaedic Surgeons.
Malignant bone tumors. Gibbs CP Jr, Weber K, Scarborough MT. Instr Course Lect. 2002;51:413-28. PMID:12064130 (Link to Abstract)
Mankin HJ, Mankin CJ, Simon MA. The hazards of the biopsy, revisited. Members of the Musculoskeletal Tumor Society. J Bone Joint Surg Am. 1996 May;78(5):656-63. PMID:8642021 (Link to Abstract)
Menedez LR (ed): Orthopaedic Knowledge Update: Musuloskeletal Tumors. Rosemont, IL, American Academy of Orthopedic Surgeons, 2002, pp 305-312
Menendez LR (ED): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, AAOS, 2002, pp29-34
Rougraff BT, Simon MA, Kneisl JS, Greenberg DB, Mankin HJ. Limb salvage compared with amputation for osteosarcoma of the distal end of the femur. A long-term oncological, functional, and quality-of-life study. J Bone Joint Surg Am. 1994 May;76(5):649-56. PMID:8175811 (Link to Abstract)
Simon and Finn: Diagnostic strategy for bone and soft tissue tumors. Instr Course Lect. 1994; 43: 527-536 PMID:9097183 (Link to Abstract)
Simon MA, Springfield D (eds): Surgery for bone and soft-tissue tumors. Philadelphia, PA Lippincott-Raven, 1998, 756pp.
Simon MA. Limb salvage for osteosarcoma. J Bone Joint Surg Am. 1988 Feb;70(2):307-10. Review. PMID:3277972 (Link to Abstract)

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Intramedullary Osteosarcoma

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