Bone Tumor, Primary Malignant

• Bharti Rathore, M.D.

 Basic Information

Definition

Primary malignant bone tumors are invasive and anaplastic and have the ability to metastasize. Most arise from the marrow (myeloma), but tumors may develop from bone, cartilage, fat, and fibrous tissues. Leukemia and lymphoma are excluded from this discussion.

Fibrosarcoma and Liposarcoma

They are extremely rare and similar to tumors arising in soft tissue.

Osteosarcoma

A rare primary malignant tumor of bone characterized by malignant tumor cells that produce osteoid or bone. Several variants have been described: parosteal sarcoma, periosteal sarcoma, multicentric, and telangiectatic forms.

Chondrosarcoma

A malignant cartilage tumor that may develop primarily or secondarily from transformation of a benign osteocartilaginous exostosis or enchondroma.

Ewing’s Sarcoma

A malignant tumor of the bone or soft tissues characterized by the presence of small, round, blue cells on immunohistochemical evaluation.

Multiple Myeloma

A neoplastic proliferation of plasma cells in the bone marrow.

Synonyms

Multiple myeloma:

1. 1.

   Plasma cell myeloma

2. 2.

   Plasmacytoma

ICD-10CM CODES
C41.9	Malignant neoplasm of bone and articular cartilage, unspecified
C40.00	Malignant neoplasm of scapula and long bones of unspecified upper limb
C40.01	Malignant neoplasm of scapula and long bones of right upper limb
C40.02	Malignant neoplasm of scapula and long bones of left upper limb
C40.10	Malignant neoplasm of short bones of unspecified upper limb
C40.11	Malignant neoplasm of short bones of right upper limb
C40.12	Malignant neoplasm of short bones of left upper limb
C40.20	Malignant neoplasm of long bones of unspecified lower limb
C40.21	Malignant neoplasm of long bones of right lower limb
C40.22	Malignant neoplasm of long bones of left lower limb
C40.30	Malignant neoplasm of short bones of unspecified lower limb
C40.31	Malignant neoplasm of short bones of right lower limb
C40.32	Malignant neoplasm of short bones of left lower limb
C40.80	Malignant neoplasm of overlapping sites of bone and articular cartilage of unspecified limb
C40.81	Malignant neoplasm of overlapping sites of bone and articular cartilage of right limb
C40.82	Malignant neoplasm of overlapping sites of bone and articular cartilage of left limb
C40.90	Malignant neoplasm of unspecified bones and articular cartilage of unspecified limb
C40.91	Malignant neoplasm of unspecified bones and articular cartilage of right limb
C40.92	Malignant neoplasm of unspecified bones and articular cartilage of left limb
C41.0	Malignant neoplasm of bones of skull and face
C41.4	Malignant neoplasm of pelvic bones, sacrum and coccyx
C41.9	Malignant neoplasm of bone and articular cartilage, unspecified

Epidemiology & Demographics

Table 1 summarizes incidence, in decreasing order, of lesions that may present as a primary bone tumor.

Multiple Myeloma

1. •

   The most common tumor in bone

2. •

   Age at onset: usually >40 yr

3. •

   Male/female ratio of 2:1

Osteogenic Sarcoma

1. •

   Average age at onset: 10 to 20 yr

2. •

   Males afflicted more often than females

3. •

   Parosteal sarcoma in older patients

Chondrosarcoma

1. •

   Age at onset: 40 to 60 yr

2. •

   Male/female ratio of 2:1

Ewing’s Sarcoma

Age at onset: 10 to 15 yr

Physical Findings & Clinical Presentation

Multiple Myeloma

1. •

   May present as a systemic process or, less commonly, as a “solitary” lesion

2. •

   Early manifestations: anorexia, weight loss, and bone pain; majority of cases present initially with back pain that often leads to the detection of a destructive skeletal lesion

3. •

   Other organ systems eventually become involved, resulting in more bone pain, anemia, renal insufficiency, and/or bacterial infections, usually as a result of the dysproteinemia typical of this disorder

4. •

   Possible secondary amyloidosis, leading to cardiac failure or nephrotic syndrome

Osteosarcoma

1. •

   Most originating in the metaphysis

2. •

   50% to 60% around the knee

3. •

   Possible pain and swelling, but otherwise healthy patient

4. •

   Osteosarcoma in conjunction with Paget’s disease, manifested primarily as a sudden increase in bone pain

Chondrosarcoma

1. •

   Tumor most commonly involving the pelvis, upper femur, and shoulder girdle

2. •

   Painful swelling

Ewing’s Sarcoma

1. •

   Painful soft tissue mass often present

2. •

   Possibly increased local heat

3. •

   Midshaft of a long bone usually affected (in contrast to other tumors)

4. •

   Weight loss, fever, and lethargy

Diagnosis

Differential Diagnosis

1. •

   Osteomyelitis

2. •

   Metastatic bone disease

Laboratory Tests

1. •

   Slightly elevated alkaline phosphatase in osteosarcoma

2. •

   In Ewing’s sarcoma: reflective of systemic reaction; includes anemia, an increase in white blood cell count, and an elevated sedimentation rate

3. •

   In multiple myeloma:

   1. 1.

      Bence Jones protein in the urine

   2. 2.

      Anemia and elevated sedimentation rate

   3. 3.

      Characteristic dysproteinemia on serum protein electrophoresis showing a monoclonal spike

   4. 4.

      Elevated quantitative immunoglobulins, beta-2 microglobulin, and elevated kappa:lambda ratio upon serum-free light chain assay

   5. 5.

      Rouleaux formation in the peripheral blood smear

   6. 6.

      Often presence of hypercalcemia

Imaging Studies

1. •

   Table 2 summarizes a systemic approach in diagnosing osseous tumors or tumor-like lesions.

   TABLE2 Systemic Approach in Diagnosing Osseous Tumors or Tumor-Like LesionsFrom Pope TL, Bloem HL, et al.: Musculoskeletal imaging, ed 2, Philadelphia, 2015, Elsevier.

   1. Categorize the radiograph (normal, variant, tumor-like lesion, tumor).

   2. Determine the prevalence of lesions in relation to the age of the patient.

   3. Determine the prevalence of lesions in the affected bone.

   4. Is the lesion solitary, or are there multiple lesions?

   5. Determine the prevalence of osseous lesions in the affected part of the bone.

   6. Analyze the radiograph in detail.

   7. Analyze additional information (MR, CT, clinical and laboratory data, and so on).

   8. Perform a biopsy, if needed, based on the comprehensive imaging findings.

2. •

   Classic osteogenic sarcoma penetrates the cortex early in many cases.

   1. 1.

      A blastic (dense), lytic (lucent), or mixed response may be seen in the affected bone (Fig. 1).

      

   2. 2.

      An aggressive perpendicular sunburst pattern may be present as a result of periosteal reaction, and peripheral Codman’s triangles are often noted.

   3. 3.

      Margins of the tumor are poorly defined.

3. •

   Speckled calcifications in a destructive radiolucent lesion are usually suggestive of chondrosarcoma.

4. •

   Ewing’s sarcoma is characterized radiographically by mottled, irregular destructive changes with periosteal new bone formation. The latter may be multilayered, producing the typical “onion skin” appearance.

5. •

   Typical roentgenographic finding in multiple myeloma is the “punched out” lesion with sharply demarcated edges.

   1. 1.

      Multiple lesions are usual.

   2. 2.

      Diffuse osteoporosis may be the only finding in many cases.

   3. 3.

      Pathologic fractures are common.

Treatment

The evaluation and treatment of malignant bone tumors are complicated. Diagnostic studies and treatment should be supervised by an orthopedic cancer specialist and oncologist.

Disposition

1. •

   In the past 20 yr, dramatic improvements have been made in the treatment protocols for osteosarcoma with the use of adjuvant multidrug regimens and limb-sparing surgery.

2. •

   Prognosis of multiple myeloma has markedly improved with introduction of several newer therapies, including proteasome inhibitors, immunomodulatory drugs, histone deacetylase inhibitors, and monoclonal antibodies.

3. •

   Prognosis for Ewing’s sarcoma has improved with a combination of chemotherapy, local resection, and radiation therapy.

4. •

   Chondrosarcomas are not sensitive to chemotherapy or radiation, and prognosis depends on the grade of the tumor and the ability to obtain an adequate resection.

Pearls & Considerations

Early diagnosis is important because most tumors have not metastasized at the time of initial presentation.

Related Content

Multiple Myeloma (Related Key Topic)

Sarcoma (Related Key Topic)