eMedicine Specialties > Orthopedic Surgery > Neoplasms

Osteosarcoma: Workup

Author: Charles T Mehlman, DO, MPH, Director, Musculoskeletal Outcomes Research, Associate Professor, Division of Pediatric Orthopedic Surgery, Cincinnati Children's Hospital Medical Center
Coauthor(s): Timothy P Cripe, MD, PhD, Professor of Pediatric Hematology/Oncology, University of Cincinnati; Director, Translational Research Trials Office, Department of Pediatrics, Cincinnati Children's Hospital Medical Center
Contributor Information and Disclosures

Updated: Mar 28, 2008

Workup

Laboratory Studies

  • Most of the laboratory studies that are obtained relate to the use of chemotherapy. It is important to assess organ function before administering chemotherapy and to monitor function after chemotherapy. The only blood tests with prognostic significance are lactic dehydrogenase (LDH) and alkaline phosphatase (ALP). Patients with an elevated ALP at diagnosis are more likely to have pulmonary metastases. In patients without metastases, those with an elevated LDH are less likely to do well than are those with a normal LDH.
  • Important laboratory studies include the following:
    • LDH
    • ALP (prognostic significance)
    • Complete blood cell (CBC) count, including differential
    • Platelet count
    • Liver function tests: Aspartate aminotransferase (AST), alanine aminotransferase (ALT), bilirubin, and albumin
    • Electrolyte levels: Sodium, potassium, chloride, bicarbonate, calcium, magnesium, phosphorus
    • Renal function tests: blood urea nitrogen (BUN), creatinine
    • Urinalysis

Imaging Studies

  • Plain films – Primary, posteroanterior (PA), and lateral chest views
    • Obtain plain films of the suspected lesions in 2 views. No single feature on a radiograph is diagnostic.
    • Osteosarcoma lesions can be purely osteolytic (approximately 30% of cases), purely osteoblastic (approximately 45% of cases), or a mixture of both.
    • Elevation of the periosteum may appear as the characteristic Codman triangle. Codman described this entity in 1909, stating, "In many cases near the junction of the healthy bone with the tumor, there is a reactive new bone formation beneath the periosteum. At the edge of the tumor, this layer of new bone ends abruptly and gives a characteristic appearance in the skiagraph [radiograph]."3
    • Extension of the tumor through the periosteum may result in a so-called sunburst appearance (approximately 60% of cases). Obtain an image of the entire bone and adjacent joint to assess for skip lesions or joint involvement. Telangiectatic osteosarcomas are often very cystic and can be mistaken for an aneurysmal bone cyst.
  • CT scanning
    • Obtain a CT scan of the primary lesion and a CT scan of the chest (high resolution).
    • CT scanning of the primary lesion helps delineate the location and extent of the tumor and is critical for surgical planning.
    • CT scanning of the chest is more sensitive than is plain film radiography for assessing pulmonary metastases. Ideally, obtain the CT scan of the chest before performing a biopsy to avoid ambiguity that can arise from postanesthesia atelectasis.
  • MRI
    • MRI of the primary lesion is the best method to assess the extent of intramedullary disease as well as associated soft-tissue masses and skip lesions.
    • This imaging modality is perhaps the single most important study for accurate surgical staging of the lesion with use of the Enneking staging system.
  • Radionuclide bone scanning with technetium-99 (99m Tc)-methylene diphosphonate (MDP/MDI)
    • It is important to evaluate for the presence of metastatic or multifocal disease with a bone scan.
    • Subsequently, obtain an image of abnormal areas with CT scanning or MRI.
  • Echocardiography or multiple gated acquisition scanning: Assess cardiac function before, and at various intervals following, treatment with Adriamycin.

Other Tests

  • Audiography: Hearing loss is an adverse effect of cisplatin. Hearing loss typically occurs during treatment. Once treatment is completed, obtaining audiograms is not typically a part of long-term follow-up care.

Diagnostic Procedures

  • Biopsies should be performed by an orthopedic surgeon (see Surgical therapy).
  • Definitive resection
    • Resections of the primary lesion and of any pulmonary metastases are essential for cure.
    • These resections should be performed by orthopedic (primary lesion) and thoracic surgeons (pulmonary metastases) (see Surgical therapy).
    • Presurgical (neoadjuvant) chemotherapy often aids the surgeon in performing the resection by shrinking tumors as well as enables the assessment of histopathologic tumor responsiveness, a major predictor of outcome.

Histologic Findings

Two elements are important to the histologic examination of the tumor. The first can be assessed on the biopsy, the tumor type. The second can be assessed on the definitive resection following chemotherapy, the response to treatment.

In general, the characteristic feature of osteosarcoma is the presence of osteoid in the lesion, even at sites distant from bone (eg, lung). Although osteoid formation is usually obvious, electron microscopy occasionally may be required to reveal this process. Stromal cells may be spindle-shaped and atypical, with irregularly shaped nuclei.

A number of different histologic types of osteosarcoma exist. The conventional type is the most common in childhood and adolescence and has been subdivided based on the predominant features of the cells (osteoblastic, chondroblastic, fibroblastic), although the subtypes are clinically indistinguishable.

The telangiectatic type contains large, blood-filled spaces and is seen commonly in adolescence and early adulthood. The parosteal type usually arises from the bone cortex, has an intermediate prognosis, and can be seen in childhood or adulthood. It most commonly arises on the distal posterior aspect of the femur. Periosteal osteosarcoma is a low- to intermediate-grade tumor that typically arises immediately below the periosteum in children. It most frequently involves the tibia.

Staging

The purpose of staging tumors is to stratify risk groups. The conventional staging used for other solid tumors is not appropriate for skeletal tumors because these tumors rarely involve lymph nodes or regional spread. Rather, the staging devised and introduced by Enneking in 1980 is based on grade, extracompartmental spread, and whether or not metastases are present. This system applies to all musculoskeletal tumors (both bone and soft tissue). The Enneking staging system (also referred to as the staging system of the Musculoskeletal Tumor Society) has been credited with bringing order to the surgical treatment of a group of tumors for which treatment was previously approached rather haphazardly.

The key components to the staging system are the histologic grade of the tumor (low grade vs high grade), the anatomic location of the tumor (intracompartmental vs extracompartmental), and the absence or presence of metastatic disease. The staging system is typically depicted as follows:

  • Low-grade tumor, intracompartmental – I-A
  • Low-grade tumor, extracompartmental – I-B
  • High-grade tumor, intracompartmental – II-A
  • High-grade tumor, extracompartmental – II-B
  • Any tumor with evidence of metastasis – III

The definition of "a compartment" is a central and crucial concept related to the Enneking staging system. In general, a compartment may be defined as any individual bone (ie, each bone is a compartment unto itself), intra-articular space (ie, a purely intra-articular lesion is intracompartmental), and clearly identified fascially enclosed space (eg, the anterior compartment of the lower leg). Many of these compartments are the same ones that a surgeon would release in the setting of compartment syndrome; these relate much more to soft-tissue tumors than to bone tumors such as an osteosarcoma.

Some areas of the body are considered to be extracompartmental by definition according the Enneking staging scheme. These areas include the antecubital fossa, the inguinal region, the popliteal space, and intrapelvic and paraspinal lesions. Because of the unique challenges of spinal tumors, an entirely separate staging system has been proposed for these areas by Weinstein, Boriani, and Biagin. It is referred to as the WBB staging system and was introduced in 1996. This system focuses on the general anatomic location about the spine (conceptualizing a spinal segment as if it were the face of a clock), as well as the specific anatomic location about the spine (eg, extraosseous soft-tissue extension into muscular areas vs intradural extraosseous extension). Just as spinal anatomy is complex, the WBB staging system is complex, but its use is slowly increasing.

For osteosarcoma, the foremost initial question regarding staging is whether the tumor has metastasized. Other features of the tumor, although not technically used in the staging, may impact the prognosis. These include the LDH and ALP measurements (see Lab studies), site of primary tumor (mostly related to ease of complete resection), histologic response to chemotherapy, and cause of the disease (patients with osteosarcomas arising from Paget disease have a particularly poor prognosis). Patients with isolated jaw lesions tend to do better and have a lower incidence of metastases.

  • Stage I – Low-grade lesions
  • Stage II – High-grade lesions
  • Stage III – Metastases
    • Substage A – Intracompartmental lesion (intramedullary lesion for bone tumors)
    • Substage B – Extracompartmental lesion (extramedullary spread for bone tumors)
  • Site of primary tumor
    • Distal extremity – Best
    • Distal femur – Intermediate
    • Axial skeleton – Worst
  • Size of the initial tumor: In a retrospective study by Kim et al, the records of 331 patients with stage II osteosarcoma who underwent surgery and chemotherapy were reviewed.26 The authors found that the initial tumor size appears to be associated with histologic response and is an important prognostic factor in osteosarcoma.
  • Histologic response: Patients with tumors that have a good histologic response (the definition of which is still under debate) to preoperative chemotherapy appear to have a better prognosis, although this still is under investigation.

More on Osteosarcoma

Overview: Osteosarcoma
Workup: Osteosarcoma
Treatment: Osteosarcoma
Follow-up: Osteosarcoma
Multimedia: Osteosarcoma
References
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Further Reading

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Keywords

osteogenic sarcoma, osteoblastic osteosarcoma, chondroblastic osteosarcoma, fibroblastic osteosarcoma, multifocal osteosarcoma, high-grade intramedullary osteosarcoma, typical osteosarcoma, classic osteosarcoma, conventional osteosarcoma, variant osteosarcoma, primary osteosarcoma, synchronous osteosarcoma, metachronous osteosarcoma, unicameral bone cyst, osteofibrous dysplasia, Campanacci tumor, periosteal osteosarcoma, malignant bone cancer, bone cancer

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Contributor Information and Disclosures

Author

Charles T Mehlman, DO, MPH, Director, Musculoskeletal Outcomes Research, Associate Professor, Division of Pediatric Orthopedic Surgery, Cincinnati Children's Hospital Medical Center
Charles T Mehlman, DO, MPH is a member of the following medical societies: American Academy of Pediatrics, American Fracture Association, American Medical Association, American Orthopaedic Foot and Ankle Society, American Osteopathic Association, Arthroscopy Association of North America, North American Spine Society, Ohio State Medical Association, Pediatric Orthopaedic Society of North America, and Scoliosis Research Society
Disclosure: Nothing to disclose.

Coauthor(s)

Timothy P Cripe, MD, PhD, Professor of Pediatric Hematology/Oncology, University of Cincinnati; Director, Translational Research Trials Office, Department of Pediatrics, Cincinnati Children's Hospital Medical Center
Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Miguel A Schmitz, MD, Consulting Surgeon, Department of Orthopedics, Klamath Orthopedic and Sports Medicine Clinic
Miguel A Schmitz, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Society for Sports Medicine, and Arthroscopy Association of North America
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Sean P Scully, MD, PhD, Professor, Department of Orthopedics, University of Miami
Sean P Scully, MD, PhD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, International Society on Thrombosis and Haemostasis, and Society of Surgical Oncology
Disclosure: Nothing to disclose.

CME Editor

Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital
Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association of Physicians of Indian Origin, American College of International Physicians, and American College of Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD, Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami School of Medicine
Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society
Disclosure: Nothing to disclose.

 
 
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