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A. Types of Neoplastic Cells (all are Sarcomas)navigator

  1. Usually occur in children or young adults
  2. Osteogenic, Chondrogenic, Fibrinogenic
  3. Benign
    1. Osteoid Osteoma
    2. Osteochondroma (Exostosis)
    3. Enchondroma
  4. Malignant
    1. Osteosarcoma
    2. Chondrosarcoma
    3. Giant Cell Tumor
    4. Ewing's Sarcoma
    5. Synovial Sarcoma

B. Osteoid Osteoma [1]navigator

  1. Benign tumor (no malignant potential) found in diaphyseal portion of long bone
  2. Children usually affected
  3. Sclerotic (dense) focus of benign osteoblastic tissue
  4. Painful lesions in most cases; muscle atrophy is rarer
  5. Well defined edge on standard radiographic studies
  6. Tumors grow slowly or not at all
  7. Malignant transformation has not been reported
  8. Treatment
    1. May be ablated with catheter directed radiofrequency waves
    2. Surgical therapy may be required - en bloc surgical excision of lesion
    3. Primary goal is control of pain
  9. Spontaneous regression has occurred but is difficult to predict

C. Osteochondroma (Exostosis)navigator

  1. Developmental Defect
  2. Cartilage covered benign bone outgrowth
  3. If it occurs next to a joint then usu asymptomatic
  4. Often looks like cauliflower lesion
  5. Congenital, multifocal forms

D. Enchondromanavigator

  1. Benign cartilage inclusion within bone
  2. Looks like infarcts because it gets calcified
  3. Probably developmental
  4. Congenital, multifocal form

E. Osteosarcoma [2] navigator

  1. Characteristics
    1. Most common primary malignant bone tumor
    2. Metaphysis involved, usually distal femur / proximal tibia
    3. Sarcoma with bizarre anaplastic spindle cells yields osteoid
    4. Frequency 5.6 per million children under the age of 15
    5. About 25% present with metastatic disease
    6. Rarely occurs in the maxillofacial region [3]
  2. Risk Factors for development of Osteosarcoma
    1. Hereditary Retinoblastinoma (germline Rb mutations)
    2. Paget's Disease
    3. Osteomyelitis
    4. Radiation Therapy
    5. Enchondromatosis
    6. Hereditary Multiple Exostoses
    7. Fibrous Dysplasia
    8. Increased risk with simian virus 40 (SV40) DNA [4]
  3. Genetic Defects
    1. Associated with Retinoblastoma (Rb) gene mutation/deletion
    2. P-glycoprotein (Pgp1, mdr1) can be expressed and is associated with poor outcome
    3. Increased risk in patients with Li-Fraumeni Syndrome (p53 germline mutations)
  4. Treatment
    1. Wide resections (5cm margins generally recommended)
    2. Surgical resection of primary tumor should be done in both local and metastatic cases
    3. If localized, post-operative adjunctive radiotherapy strongly considered
    4. Adjuvant or neoadjuvant chemotherapy improves survival to 75-80% in nonmetastatic cases
    5. Strongly consider adjuvant chemotherapy for young age, moderate or high grade tumors
    6. 50% survival with amputation
    7. Limb sparing surgery being developed in combination with initial chemotherapy
  5. Combination Chemotherapy [2]
    1. No differences between two drug and multidrug regimens in non-metastatic disease [5]
    2. Two drug regimen consisted of doxorubicin and cisplatin for 18 weeks (6 cycles)
    3. About 30% of treated cases show good histopathological responses
    4. Doxorubicin, methotrexate, ifosfamide and cisplatin have been recommended
    5. Salvage therapy with ifosfamide and etoposide may be considered
    6. Ifosfamide and etoposide added to standard therapy for Ewing's Sarcoma improves outcome for non-metastatic (but not metastatic) disease [6]
  6. Prognosis
    1. Larger tumors, higher grade, associated with poor outcomes
    2. Presence of large degree of necrosis in tumor associated with poorer outcome also
    3. P glycoprotein (drug resistance) expression strongly associated with poor outcome
    4. Five year survival with operable, non-metastatic osteosarcoma is 45-75% [2,5]
    5. Three to 5 year survival for metastatic disease is 14-50% [2]

F. Chondrosarcomanavigator

  1. Affects mostly older adults
  2. Malignant cartilage tumor
  3. Can calcify, with rough edge, not demarginated
  4. Low or high grade

G. Ewing's Tumor navigator

  1. High grade, small, differentiated, round cell bone sarcoma
    1. Unclear etiology; associated with (11;22) translocation
    2. Abnormal mRNA transcripts in a subset of tumor cells
  2. Peak incidence in second decade of life
  3. Usually occurs on diaphysis of long bones
  4. Patients present with pain and swelling
  5. Variant high-grade synovial cell sarcomas can occur [7]
  6. Intensive chemotherapy with surgery and/or radiation affords up to 70% cure rates
  7. Ifosfamide and etoposide added to standard therapy for Ewing's Sarcoma improves outcome for non-metastatic (but not metastatic) disease [6]
  8. Prognosis [6]
    1. Metastatic disease: 22% 5 year survival
    2. Non-metastatic disease: 55-70% (higher with ifosfamide + etoposide)

H. Giant Cell Tumornavigator

  1. Osteoclast like giant cells
  2. Lucent lesion in bone

References navigator

  1. Gebhardt MC, Rosenthal DI, Arnell PM. 2005. NEJM. 352(11):1122 (Case Record) abstract
  2. Arndt CAS and Crist WM. 1999. NEJM. 341(5):343
  3. Dodson TB, Caruso PA, Nielsen GP. 2004. NEJM. 350(3):267 (Case Record) abstract
  4. Vilchez RA, Kozinetz CA, Arrington AS, et al. 2003. Am J Med. 114(8):675 abstract
  5. Souhami RL, Craft AW, Van der Eijken JW, et al. 1997. Lancet. 350(9082):911 abstract
  6. Grier HE, Krailo MD, Tarbell NJ, et al. 2003. NEJM. 348(8):694 abstract
  7. Cohen IJ, Issakov J, Avidgad S, et al. 1997. Lancet. 350(9091):1679