Bone Neoplasms Flashcards
an uncommon neoplasm that probably arises from periodontal ligament fibroblasts.
central ossifying fibroma
could be confused with focal cemento-osseous dysplasia in the past (also osteoporotic bone marrow defect, fibrous dysplasia)
central ossifying fibroma
*note: cemento-osseous dysplasia is more apical
What region of the mouth is most affected by central ossifying fibroma? Age and gender?
- mandibular premolar/molar
- adult female 3rd-4th decade
What are the signs and symptoms of central ossifying fibroma?
- well-circumscribed radiolucency with variable central opacity
- asymptomatic swelling, root divergence, facial deformity with larger lesions
- can range in lucency/opacity
Describe the histology of central ossifying fibroma
- lesional tissue separates readily from host bone, often in one piece
- cellular fibrous CT with calcified trabeculae/spherules (resembles cellular cementum or woven bone)
- similar histology to fibrous dysplasia or cemento-osseous dysplasia so radiographic correlation is helpful
What is the treatment of central ossifying fibroma? Prognosis?
- treatment: enucleation
- prognosis: very good; recurrence is 12%
What is a benign osseous tumor, primarily affecting the craniofacial bones (especially paranasal sinuses)?
osteoma
Tori probably represent the majority of ___.
osteomas
*note: tori are usually static and develop but don’t enlarge afterward whereas osteomas keep enlarging
What are the signs and symptoms of osteoma? Where is it most frequently located?
- painless slow enlarging bone mass
- most often in mandibular body and condylar area
Describe the histology of an osteoma.
normal bone +/- marrow tissue
What other condition is associated with osteoma?
Gardner Syndrome
Describe how Gardner syndrome is acquired. How often? What family of disease?
- autosomal dominant; APC gene, chromosome 5
- 1 in 8,000-16,000
- familial adenomatous polyposis syndromes
What are the characteristics of Gardner Syndrome?
- mulitple osteomas of the facial bones (skull, paranasal sinuses, mandible)
- epidermoid cysts
- desmoid tumors
ALSO: - impacted supernumerary teeth, or less commonly, odontomas
- development of precancerous polyps of the colon
*note: presence of osteomas helps to distinguish Gardner from cleidocranial dysplasia
What can Gardner’s syndrome radiographically resemble?
florid cemento-osseous dysplasia or osteitis deformans
__% of Gardner syndrome patients develop adenocarcinoma of the colon by age 30; __% by the 5th decade
50%
100%
Describe the management of Gardner syndrome.
- prophylactic colectomy
- removal of problematic cysts and osteomas
- removal of impacted teeth/odontomas with prosthodontic work as needed
- genetic counseling
What condition does some think is a neoplasm while others think it is a reactive lesion?
central giant cell granuloma
What age is most susceptible to a central giant cell granuloma? Gender? Area of oral cavity?
- broad age range (60% of patients
True or false: The teeth in a central giant cell granuloma are non-vital.
FALSE. Teeth are vital
*note: these are soetimes treated endodontically mistakenly
How do central giant cell granulomas appear radiographically? Histologically?
- appear unilocular when small –> multilocular and expansile when larger
- cellular granulation tissue with numerous benign multi-nucleated giant cells; RBC extravasation and hemosiderin deposits
What does a brown tumor of hyperparathyroidism resemble histologically?
central giant cell granuloma
What is the treatment of central giant cell granuloma? Prognosis?
- aggressive curettage, or alternate non-surgical therapies
- prognosis good, recur 20%
What is the difference between primary and secondary hyperparathyroidism?
- PRIMARY: due to parathyroid hyperplasia, parathyroid adenoma, parathyroid carcinoma
- SECONDARY: due to renal failure, which is responsible for poor calcium retention and altered vitamin D metabolism
What are the signs and symptoms of hyperparathyroidism?
- radiographic loss of lamina dura and “ground glass” trabeculae pattern
- “brown tumor”: unilocular/multilocular radiolucencies may develop
- renal osteodystrompy (diffuse jaw enlargement with chronic renal failure)
Describe the histology of hyperparathyroidism.
- brown tumors show vascular granulation tissue with numerous multinucleated giant calls and extravasated erythrocytes
- IDENTICAL TO CENTRAL GIANT CELL GRANULOMA
What is renal osteodystrophy and what condition is it related to?
- hyperplastic response of the bone in patients with poorly-controlled secondary hyperparathyroidism related to end-stage renal disease; prominent jaw enlargement may result
- note: distinguish from Paget’s disease with alkaline phosphatase test (high AP in Paget disease)
What is the treatment for hyperparathyroidism?
- PRIMARY: remove source of excess hormone
- SECONDARY: better control of serum calcium levels, parathyroidectomy, or renal transplant
What is the prognosis of hyperparathyroidism?
fair to good (no risk of osteosarcoma like Pagets does)
What is the most common primary bone malignancy?
ostosarcoma
How common is osteosarcoma? What age?
- 1,000 new cases in the USA annually; only 6-8% affect the jaws
- bi-modal age distribution = major peak at 2nd decade and lesser peak after 6th decade
How does osteosarcoma of the jaw differ from that of long bones?
- LONG BONES: knee area or pelvis; mean age 18 years
- JAWS: maxilla = mandible; mean age 33-39 years
What are the signs and symptoms of osteosarcoma?
- initial complaint of pain, followed by swelling, loose teeth, or paresthesia
- mixed radiopaque/radiolucent will ill-defined borders
- symmetrically widened PDL
- “sunburst” pattern is uncommon
- note: wide PDL usually only seen in malignancies; PDL is entry for sarcoma
- note: mottled expansion = bone forming malignancy
- note: differential diagnoses = peripheral ossifying granuloma and focal cemento-osseous dysplasia
Describe the histology of an osteosarcoma.
- infiltrating sheets of malignant spindle-shaped or angular lesional cells
- direct production of osteoid/bone
- mitotic activity can vary field to field
- osteoblastic, chondroblastic, and fibroblastic differentiation
*note: 3 IHC markers help diagnose
What is the treament and prognosis of osteosarcoma?
- TREATMENT: radical surgery with chemo (in some cases)
- PROGNOSIS: 5-year survival 60-70%, death is more often due to uncontrolled local disease than metastasis (which is lower overall in gnathic vs extragnathic lesions)
What is the condition that is a rare malignancy of cartilaginous differentiation?
chondrosarcoma
Which is more common: osteosarcoma or chondrosarcoma?
osteosarcoma (2x more common)
What age is most susceptible to chondrosarcoma? Gender? Area of the body? Area of the oral cavity?
- adults 4th to 6th decade
- males
- femur, pelvis, ribs
- posterior mandible, anterior maxilla
*note: only 0.1% of all head and neck malignancies
What are the signs and symptoms of chondrosarcoma?
- pain, swelling, or loose teeth
- poorly defined radiolucency with variable amounts of radiopacity
- larger lesions may appear multilocular
- widened PDL of teeth in area of tumor (like osteosarcoma)
Describe the histology of chondrosarcoma.
characterized by invasive lobules of atypical cells showing cartilaginous differentiation
What grades of chondrosarcoma are recognized? What are most jaw lesions?
- grades I-III recognized
- lower grade (I or II)
True or false: Chondrosarcomas have a very rapid proliferation and leave areas of necrosis due to blood vessels not being able to keep up.
true
True or false: Chondrosarcomas do not spread via metastasis.
FALSE; metastasizes via blood and can spread from the jaws to the vertebral column or elsewhere
What is the treatment for chondrosarcoma? Prognosis?
- TREATMENT: radical surgery
- PROGNOSIS: depends on the location and histopathologic grade of the tumor
- 5 year survival = 87%; 10-year survival = 71%
- death is usually due to direct extension of the tumor, involving vital structures
What is the most common form of cancer to involve the bone?
metastatic disease
In metastasis, most carcinomas travel via ___ while most sarcomas travel via ___.
- lymphatics
- hematogenously
*note: not a hard and fast rule
How do metastatic deposits from malignancies below the neck affect the jaws?
Batson’s paravertebral plexus of veins which lack valves
What age is most susceptible to metastatic disease? Gender? Area of the oral cavity?
- mean age 43-52 years
- no gender predilection
- posterior mandible (because that is where there is hematopoietic marrow so it can catch the cells)
What are the signs and symptoms of metastatic disease?
- paresthesia
- tooth mobility
- swelling
- hemorrhage
- pathologic fracture
- trismus
- failure of tooth socket to heal
- poorly defined radiolucency (less commonly, mixed radiolucency/radiopacity)
Describe the histology of metastatic disease.
lesions mimic the primary malignancy from which they arose
scattered clusters of lesional cells gives “seeded” effect or small deposits of cells in many different places
What are the most common primary malignancies that cause metastatic disease to the jaws?
- breast, lung, colon, thyroid, prostate, kidney, melanoma
__% of jaw metastases represent the initial manifestation of the malignant process
22%
What is the treatment for metastatic disease? Prognosis?
- palliation, usually with radiation therapy, anti-resorptives
- very poor prognosis, most patients die within 1 year
