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PRIMARY MALIGNANT BONE TUMORS

Epidemiology

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Epidemiology
Incidence: 3600 (male: 2120; female: 1480. Estimated new cases for 2020 in the United States) 1.1 per 100,000 males, 0.8 per 100,000 females
Deaths: Estimated 1720 in 2020 (male: 1000; female: 720)
Median age: 42 years
Male to female ratio: 1.5:1

American Cancer Society. Cancer Facts & Figures 2020. Atlanta: American Cancer Society; 2020

2020 WHO Classification of Malignant Bone Tumors

  1. Chondrogenic tumors

    Chondrosarcoma, grades 1

    Chondrosarcoma, grades 2

    Chondrosarcoma, grades 3

    Periosteal chondrosarcoma

    Clear cell chondrosarcoma

    Mesenchymal chondrosarcoma

    Dedifferentiated chondrosarcoma

  2. Osteogenic tumors

    Low-grade central osteosarcoma

    Osteosarcoma NOS

      Conventional osteosarcoma

      Telangiectatic osteosarcoma

      Small cell osteosarcoma

    Parosteal osteosarcoma

    Periosteal osteosarcoma

    High-grade surface osteosarcoma

    Secondary osteosarcoma

  3. Fibrogenic tumors

    Fibrosarcoma NOS

  4. Vascular tumors of bone

    Epithelioid hemangioendothelioma NOS

    Angiosarcoma

  5. Osteoclastic giant cell-rich tumors

    Giant cell tumor of bone, malignant

  6. Notochordal tumors

    Chordoma NOS

      Chondroid chordoma

    Poorly differentiated chordoma

    Dedifferentiated chordoma

  7. Other mesenchymal tumors of bone

    Adamantinoma of long bones

      Dedifferentiated adamantinoma

    Leiomyosarcoma NOS

    Pleomorphic sarcoma, undifferentiated

    Bone metastases

  8. Hematopoietic neoplasms of bone

    Plasmacytoma of bone

    Malignant lymphoma, non-Hodgkin, NOS

    Hodgkin disease, NOS

    Diffuse large B-cell lymphoma NOS

    Follicular lymphoma NOS

    Marginal zone B-cell lymphoma NOS

    T-cell lymphoma NOS

    Anaplastic large cell lymphoma NOS

    Malignant lymphoma, lymphoblastic, NOS

    Burkitt lymphoma NOS

    Langerhans cell histiocytosis NOS

    Langerhans cell histiocytosis, disseminated

    Erdheim-Chester disease

    Rosai-Dorfman disease

Adapted from: Choi JH, Ro JY. Adv Anat Pathol 2021;28:119–138. (note: non-malignant tumors have been omitted from this list)

Work-up

  1. History and physical examination

  2. Laboratory tests: CBC with differential; electrolytes including calcium, phosphorus, and magnesium; renal function tests including blood urea nitrogen (BUN) and creatinine; liver function tests including liver enzymes and total bilirubin; alkaline phosphatase; lactate dehydrogenase

  3. Plain films of affected bone

  4. CT scan of chest, abdomen, and pelvis (particularly chest because 80% of metastatic lesions occur here). CT scan may be performed in conjunction with FDG-PET. High resolution chest CT scan should be performed

  5. MRI with gadolinium contrast to ascertain extent of the tumor, involvement of surrounding neurovascular structures, invasion of the adjacent joint, and the presence of skip metastases

  6. Technetium-99 bone scan to identify skip lesions within affected bones or distant metastatic disease

  7. Whole body 18-fluoro-deoxyglucose positron emission tomography (FDG-PET)/CT is more routinely being performed particularly for Ewing sarcoma

  8. Bilateral bone marrow aspirate and biopsy for light microscopy examination in the case of Ewing sarcoma

  9. No radiologic studies are pathognomonic, so bone biopsy remains essential to diagnosis

  10. Echocardiogram or MUGA scan to determine cardiac ejection fraction as clinically indicated

  11. Audiogram before cisplatin chemotherapy

  12. Fertility preservation to be offered when feasible

Surgical Staging

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Surgical Staging

The surgical system as described by Enneking et al. is based on the GTM classification. Stage is determined by 3 different subcategories: grade (G), location or site (T), and lymph node involvement and metastases (M)

Grade (G)...

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