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Chapter 34: Sarcomas

Brigitte Widemann; Tito Fojo; Jean-Yves Blay

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INTRODUCTION

Primary Malignant Bone Tumors: Epidemiology

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Incidence: 3,020 (male: 1,680; female: 1,340. Estimated new cases for 2014 in the United States) 1.1 per 100,000 males, 0.8 per 100,000 females
Deaths: Estimated 1,460 in 2014 (male: 830; female: 630)
Median age: 42 years
Male to female ratio: 1.5:1
 

Siegel R et al. CA Cancer J Clin 2014;64:9–29

Surveillance, Epidemiology and End Results (SEER) Program, available from http://seer.cancer.gov (accessed in 2013)

Classification: WHO Classification of Malignant Bone Tumors

Osteogenic tumors

Osteosarcoma

  1. Conventional

    • Chondroblastic

    • Fibroblastic

    • Osteoblastic

  2. Telangiectatic

  3. Small cell

  4. Low-grade central

  5. Secondary

  6. Parosteal

  7. Periosteal

  8. High-grade surface

Ewing sarcoma/primitive neuroectodermal tumor

Ewing sarcoma

 

Cartilage tumors

Chondrosarcoma

  1. Central, primary, and secondary

  2. Peripheral

  3. Dedifferentiated

  4. Mesenchymal

  5. Clear cell

Fibrogenic tumors

Fibrosarcoma

 

Fibrohistiocytic tumors

Malignant fibrous histiocytoma

 

Giant cell tumor

Malignancy in giant cell tumor

 

Notochordal tumors

Chordoma

 

Vascular tumors

Angiosarcoma

 

Smooth muscle tumors

Leiomyosarcoma

 

Lipogenic tumors

Liposarcoma

 

Miscellaneous tumors

Adamantinoma

 

Hematopoietic tumors (see respective sections)

  1. Plasma cell myeloma

  2. Malignant lymphoma, NOS

Work-up

  1. History and physical examination

  2. Laboratory tests: CBC with differential; electrolytes; liver function tests; mineral panel, including alkaline phosphatase; lactate dehydrogenase

  3. Plain films of affected bone

  4. Chest x-ray (PA and lateral)

  5. CT scan of chest, abdomen, and pelvis (particularly chest because 80% of metastatic lesions occur here)

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

  7. Bone scan to identify skip lesions within affected bones or distant metastatic disease

  8. Bone marrow aspirate 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

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]
G1 Low grade, uniform cell type without atypia, few mitoses
G2 High grade, atypical nuclei, mitoses pronounced
Site [T]
T1 Intracompartmental = Confined within limits of periosteum
T2 Extracompartmental = Breach in an adjacent joint cartilage, bone cortex (or periosteum) fascia lata, quadriceps, and joint capsule
Lymph Node Involvement and Metastases [M]
M0 No identifiable skip lesions or distant metastases
M1 Any skip lesions, regional lymph nodes, or distant metastases
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Surgical Staging
Enneking Staging System of Malignant Bone Tumors
Stage   G T M
IA Low grade, intracompartmental G1 T1 M0
IB Low grade, extracompartmental G1 T2 M0
IIA High grade, intracompartmental G2 T1 M0
IIB High grade, extracompartmental G2 T2 M0
IIIA Low or high grade, intracompartmental with metastases G1/2 T1 M1
IIIB Low or high grade, ...

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