Neoplasms of the Bone

Question 1

1. Contrast benign and malignant neoplasms of the bone.

Answer 1

• Benign:
  
  • Asymptomatic, grows slowly and by expansion -
  • displaces teeth and expands cortex, symmetrical, does not metastasize.
• Malignant:
  
  • usually symptomatic, rapid growth, invades and destroys adjacent structures, often asymmetrical,
  • ragged or poorly defined borders and destroys cortex, lays down bone outside cortex, metastasizes.

Question 2

Classify neoplasms by origin, including: Bone

Answer 2

• Benign:
  
  • Exostoses (not neoplastic,
  • Osteoma,
  • Osteoid osteoma,
  • Osetoblastoma.
• Malignant:
  
  • Osteosarcoma,
  • Osteogenic sarcoma

Question 3

Classify neoplasms by origin, including: Cartilaginous

Answer 3

• Benign:
  
  • Chondroma,
  • Chondromyxoid fibroma,
  • Benign chondroblastoma
  • Malignant:
    
    • Chondrosarcoma

Question 4

Classify neoplasms by origin, including: Fibrous

Answer 4

Benign: Desmoplastic fibroma.

Question 5

Classify neoplasms by origin, including: Marrow

Answer 5

Malignant: Ewing’s sarcoma, Multiple myeloma

Question 6

Classify neoplasms by origin, including: Metastatic disease

Answer 6

Covered previously

Question 7

3. Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of benign neoplasms of bone, including: Exostoses

Answer 7

• Localized proliferation of bone that arises from the cortical plate, torus palatinus & mandibularis, single or multiple.
• Buccal area of the maxillary or mandibular alveolar ridge is the most common site, often bilateral, can occur on lingual/palatal areas.
• Adults - bone hard elevated nodular lesion. Asymptomatic, thin overlying mucosa may ulcerate easily.

Question 8

3. Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of benign neoplasms of bone, including: Osteoma

Answer 8

• A localized proliferation of bone - involves medullary bone or cortical bone (may be indistinguishable from tori), produces a bony hard, elevated, smooth surfaced mass.
• Variable type of bone - dense, compact cotrical like bone without significant marrow, and canellous bone with trabecular pattern and marrow.
• Almost always in craniofacial skeleton.
• Solitary lesions, asymptomatic, predilection for sinuses, limited growth potential, may be a manifestation of Gardner’s Syndrome.

Question 9

What are the characteristics of Gardner’s Syndrome?

Answer 9

• Autosomal dominant disorder.
• Multiple osteomas (jaws, angle of mandible area, frontal bone, frontal and ethmoidal sinuses, bones outside head), supernumerary teeth, multiple epidermoid cysts, demoid tumors,
• Multiple adenomatous polyps of colon and rectum = 100% incidence of malignant transformation.

Question 10

3. Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of benign neoplasms of bone, including: Osteoid osteoma

Answer 10

• True neoplasm of bone, rare <1% of all bone tumors.
• Usually seen in <30 years of age (85%), males 2:1,
• Noctural pain, relieved by Aspirin,
• Radiographic appearance of a “target”, small lesion less than 1cm in diameter, Central opacity, peripheral radiolucent “halo”, Corticated rim.

Question 11

3. Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of benign neoplasms of bone, including: Osteoblastoma

Answer 11

• True neoplasm of bone, rare <1% of all bone tumors, Mandible is most common site,
• Wide age ran usually less than 30 yrs, Males 2:1, Larger lesions over 2cm in diameter, pain is a common symptom, not nocturnal or relieved by Aspirin,
• May produce significant expansion and deformity, may be well-defined or ill-defined, peripheral corticated rim +-, central calcified rim +-

Question 12

3. Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of benign neoplasms of bone, including: Chondroma

Answer 12

• Benign neoplasm of cartilage origin.
• Adults usually 20-40 year range in extremities, Very rare in head and neck region,
• Most lesions diagnosed as chrondroma are low grade chondrosarcomas.

Question 13

3. Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of benign neoplasms of bone, including: Chondromyxoid fibroma

Answer 13

• Rare in jaws, desmoplastic fibroma - benign locally aggressive fibrous boney neoplasm. bone equivalent of soft tissue fibromatosis (desmoid tumors),
• Commin in children and young adults, asymptomatic, 85% in mandible (posterior & ramus),
• Radiolucency, unilocular to multilocular, borders well defined or poorly defined. Tx: curettage with 70% recurrence, resection with 20% recurrence

Question 14

3. Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of benign neoplasms of bone, including: Benign chondroblastoma

Answer 14

• Rare in jaws, desmoplastic fibroma - benign locally aggressive fibrous boney neoplasm. bone equivalent of soft tissue fibromatosis (desmoid tumors),
• Commin in children and young adults, asymptomatic, 85% in mandible (posterior & ramus),
• Radiolucency, unilocular to multilocular, borders well defined or poorly defined.
• Tx: curettage with 70% recurrence, resection with 20% recurrence

Question 15

Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of fibrous benign neoplasms, including: Desmoplastic Fibroma

Answer 15

• Rare in jaws, desmoplastic fibroma - benign locally aggressive fibrous boney neoplasm. bone equivalent of soft tissue fibromatosis (desmoid tumors),
• Common in children and young adults, asymptomatic, 85% in mandible (posterior & ramus),
• Radiolucency, unilocular to multilocular, borders well defined or poorly defined.
• Tx: curettage with 70% recurrence, resection with 20% recurrence

Question 16

Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of fibrous benign neoplasms, including: Osteosarcoma

Answer 16

• A malignant neoplasm of osteoblastic cells. Produces osteoid matrix that may or may not calcify.
• Conventional, parosteal and periosteal, extraskeletal, post radiation, in Paget’s disease,
• Entirely radiolucent lesions - matrix production without matrix calcification, mixed lucent-opaque lesions - variable degrees of matrix calcification.
• Densely opaque lesions - extensive matrix calcification.
• **The most common primary malignancy of bone -
• 7% in jaws, mandible = maxilla, swelling & pain, Loosening of adjacent teeth, paresthesia/anesthesia. Ill defined lesion - blends into surrounding bone. Worrisome features:
• Symmetrical widening of the periodontal membrane space (earliest change), alveolar bone production above lesel of normal crest, irregular root resorption or a “spiked” root form associated with irregular lucency. “Sunburst” or “Sun-ray” appearance - seen in 25% of osteosarcomas - may be best appreciated on occlusal radiographs.

Question 17

Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of fibrous benign neoplasms, including: Osteogenic sarcoma

Answer 17

• “Juxtacortical Osteosarcoma” -
• osteosarcoma arising on the cortical surface rather than in intramedullary location. Initial growth is outward, generraly have a better prognosis overall.
• Two subtypes - Parosteal & Periosteal.
• Tx: Radical ablation surgery the mainstay of treatment, local recurrence a major problem, preoperative chemotherapy has improved prognosis in some studies, jaw lesions metastasize less frequently than long bone diesease, overall survival between 30-80%.

Question 18

Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of fibrous benign neoplasms, including: Chondrosarcoma

Answer 18

• A malignant bone neoplasm producing cartilage but not bone.
• Cartilage matrix may show dystrophic calcification or maturation to bone - radiographic lesions may be lucent, mixed or opaque. Relatively unusual lesion in the jaws.
• Normal cartilage is largely absent in the jaws, a vestigial remnant in the anterior maxilla condyles.
• Most over 50 yrs, increases in 6-7th decades, males more affected, swelling, non-painful, loosening of adjacent teeth, paresthesia/anesthesia. Maxilla more than mandible.
• Radiographic - poorly defined asymmetric lesion, completely lucen to mixed to predominately opaque.
• Tx: Radical surgical ablation, only about 12% metastasize to challege is local recurrence - which can take years, so 5 yr survival is not good indicator of survival.
• New studies show better prognosis than for osteosarcoma. 5 year - 65-90%, 10 year - 50-70%, 15 year - slightly worse.

Question 19

Describe characteristics, clinical presentation, typical population and location, treatment and associated syndrome (if applicable) of fibrous benign neoplasms, including: Ewing’s sarcoma

Answer 19

• Uncertain cell of origin. Bone marrow?
• Neuroectodermal origin!
• Consistent genetic defect - translocation 11;22,
• 6-10% of all primary bone tumors, pelvic bones and femur account for >50% of all cases.