Malignant Non –Odontogenic Tumors of the Jaw Bones Flashcards
Key Concepts of
Malignant Lesions on
Imaging
Rapidly growing and infiltrative
–finger-like extensions into surrounding anatomy
Remember: in some instances, a benign lesion can mimic a malignant
one.
Therefore we should be wary of all the information that is available.
Key Concept of
Malignant Lesions on
Imaging
ill-defined invasive borders followed by bone destruction
Key Concept of
Malignant Lesions on
Imaging
Destruction of the cortical boundary (floor of maxillary antrum) with an adjacent soft tissue mass (arrows)
Key Concept of
Malignant Lesions on
Imaging
Tumor invasion along the periodontal membrane space causing irregular thickening of this space
Key Concept of
Malignant Lesions on Imaging
Multifocal lesions located at root apices and in the papilla of a developing tooth destroying the crypt cortex and displacing the developing tooth in an occlusal direction (arrow)
Key Concept of
Malignant Lesions on Imaging
Four types of effects on cortical bone and periosteal reaction, from top to the bottom:
•cortical bone destruction without periosteal reaction
•laminated periosteal reaction with destruction of the cortical bone and the new periosteal bone
•destruction of cortical bone with periosteal reaction at the periphery forming Codman’s triangles
•a spiculatedor sunray type of periosteal reaction
Key Concept of Malignant Lesions on Imaging
Bone destruction around existing teeth, producing an appearance of teeth floating in space.
Chondroma & Chondrosarcoma
- *Chondroma –** benign
- *Chondrosarcoma**‐ malignant
- Both are listed here because
- a benign cartilaginous tumor central In the jaw is extremely rare (or may not exist)
- Lesions tend to recur many times and eventually metastasize ( Basically chondromas are not really benign)
Chondrosarcoma
Charcterstics
- Malignant tumor that forms cartilage
- 10% of all primary bone tumors, but rare in the jaws
Chondrosarcoma
Clinical presentations
- Patient’s chief complaint is painless swelling, may be associated with tooth mobility
-
Symmetric widening of the PDL space
- Can be initial presentation with chondrosarcoma and osteosarcoma
-
Along radicular surface of the tooth there is the same rate of widening all the way down the tooth
- In contrast to periodontal disease, where there is a triangular shaped loss of space
Chondrosarcoma
Radiographically
-
Poorly defined radiolucency, often with scattered radiopaque foci
- Radiopaque foci can be seen since the cartilage in the tumor can ossify
What is this radiographic finding?
Chondrosarcoma
- its consistent widening as opposed to seen in periodontitis and inflammatory disease
Case
CC of loose teeth wanted extractions and a
denture
Chondrosarcoma
- Ill defined lesion of anterior maxilla
- Areas of radiolucency
- Classic area of moth‐eaten look
- Circular area of radiolucency with trabecular
- pattern
- Patient left without surgery, not heeding medical advice
Then patient came back
▪ Someone was willing to do the dentures for her
▪ CC‐ denture was not fitting
▪ Expansion of cortical plate
▪ Hyperkeratotic because of denture rubbing
▪ Still has malignancy
▪ Advise for surgery
▪ Refused again
Then the lesion kept on Lesion still growing
▪ Metastasize to lungs
▪ Admitted to breathing issues
▪ About 5 ½ years from initial dx to
pt passing away
What is this clinical finding?
Chondrosarcoma
- Alveolar process and floor of mouth affected
- Limitations of movement of the tongue
Chondrosarcoma
Treatment
-
Radical surgical excision on initial treatment
-
Maxillectomy/Mandibulectomy
- If anterior region they remove the entire anterior portion of the jaw
-
Maxillectomy/Mandibulectomy
- These lesions don’t respond to radiation or chemotherapy
- Although used as adjuncts for lesions that can’t be treated surgically
-
For example a posterior sinus lesion since that is the base of the skull
- These patients have poorer prognosis than those with more accessible sites such as the mandible
- Prognosis is poorer than for osteosarcoma (which contrasts with the prognosis in extragnathicsites)
- Treatment failure (and mortality) is usually due to uncontrolled local disease not metastasis
Why any diagnosis of chondroma in the jaws should be viewed with suspicion?
- Since 20% of chondrosarcomas of the jaw are initially misdiagnosed as chondromas ► any diagnosis of chondromain the jaws should be viewed with suspicion
-
All cartilaginous tumors arising in the jaws should be excised widely
- (>60% of cartilaginous tumors of the jaw recur and ~7% metastasize to the lung and/or bone )
Case
- 83 year old female with nodular areas under denture on anterior mandibular ridge
- ▪ c/c of her denture rocking
Can see in the anterior region there’s an
elevation
histology shows it’s not chondrosarcoma
because it contained Cutright lesion
papule or nodule on alveolar ridge
- Osseous and/or chondromatous metaplasia within the soft tissue of the gingiva
o Lesion is NOT central in bone or connected to bone
▪ NOT a malignant lesion
o Thought to be reactive metaplasia due to a poorly fitting denture
Osteosarcoma
_Demographics & Location_MD > MX, Male > Female
- Most common malignant bone tumor in the jaws is metastatic disease
-
Most commonly primary (meaning started at this location) malignant bone tumor in patients under 40 years old
- 2nd most common overall after multiple myeloma
-
Mean age at presentation for jaw lesions is 33 years old, 10‐15 years older than that for long bones
*
Osteosarcoma
Clinically & Radiographically
- Swelling and pain are the common presenting symptoms (25% have “toothache”)
- Can also have loosening of teeth,** **paresthesia of lip** and **nasal obstruction
- Symmetric widening of the PDL is often an early radiographic change
- Lesions vary from dense sclerotic, mixed sclerotic and radiolucent to all radiolucent
Osteosarcoma
Treatment
- Important to distinguish from chondrosarcoma as treatments are different
- Osteosarcoma is currently treated with pre-op multi-agent chemotherapy followed by surgery
-
Radiation therapy alone is insufficient for cure
- Favorable jaw site – MD symphysis
- Worst site – MX sinus
- 5 year survival is ~ 20% (up to 80% if caught early and treated with radical resection)
What is this clinical finding?
Osteosarcoma
▪ Swelling on left side of face
▪ Difficult opening
What is this clinical finding?
Osteosarcoma
- See something in the operculum
- Infection in third molar?
What is this radiographic finding?
Osteosarcoma
- AP Plain Film
- Most of jaw was missing
- Radiolucency affecting entire ramus and condyle
What is this radiographic finding?
Osteosarcoma
- Classic sunburst pattern
- Fuzzy appearance on outer edges of cortex
What is this radiographic finding?
Osteosarcoma
- cloudy bone formation on surface of cortex on facial and lingual aspect
What is this clinical finding?
Osteosarcoma
a patient with swelling with side of face
What is this radiographic finding?
Osteosarcoma
▪ Lytic lesion
▪ Slightly ill defined
▪ Loss of bone in the inferior aspect of mandible
Peripheral (juxtacortical) Osteosarcoma
Location
- Arise on the surface of the bone (vs. medullary site for usual forms of osteosarcoma)
- Usually long bones
What are the two types of Peripheral (juxtacortical) Osteosarcoma ?
parosteal – well differentiated, but will recur with less than an en bloc or radical surgery
periosteal – higher grade with prominent cartilaginous component
Parosteal Peripheral Osteosarcoma
Charcterstics
o Mushroom like growth on bone surface
o No elevation of periosteum
o No new bone formation
o Low grade
Periosteal Peripheral Osteosarcoma
Charcterstics
o Usually sessile growth on bone surface
o Elevation of periosteum
o New bone fills in space under periosteum
o Prognosis is better than medullary osteosarcoma but worse
thanparosteal type
Langerhans Cell
Disease
also known as
?
- Histiocytosis X (old name)
- Langerhans cell granuloma
- Eosinophilic granuloma
- Langerhans cell histiocytosis
Langerhans cell histiocytosis
Etiology
Etiology unclear
o Demonstration that LCH cells are clonal, along with the recent discovery of activating BRAF mutations in LCH cells, ►strongly suggests that LCH is a neoplastic disease
Langerhans cell histiocytosis
Demographics & Location
- Males>>> Females
- More than half of cases seen under the age of 10
- Jaw affected in 10 ‐20% of cases
Langerhans Cell
Histiocytosis
Clinically & Radiographically
Clinically:
Common sites: skull, rib, vertebrae, and mandible
▪ Often have associated pain or tenderness
Radiographically:
▪ In jaws we see loss of alveolar bone in molar area
o Mimics severe periodontitis
Types of Langerhans Cell
Disease
List 3
▪ Acute disseminated form (Letterer‐Siwe disease)
▪ Chronic disseminated form (Hand‐Schuller‐Christian Disease,
▪ Eosinophilic Granuloma (chronic localized)
Acute disseminated form (Letterer‐Siwe disease)
charcterstics
- Multisystem (bone, skin, liver spleen and lymph nodes)
- Infants
- high mortality
- rapidly progressive
Chronic disseminated form (Hand‐Schuller‐Christian Disease, Multifocal Eosinophilic Granuloma)
Charcterstics
- Unisystem (frequently bone, but also skin and viscera)
- Children
- fairly high mortality
- more chronic progression
Eosinophilic Granuloma (chronic localized)
Charcterstics
- Solitary or multiple bone lesions without visceral involvement
- Adults
- very low mortality
- Some reports of association with smoking marijuana
- In these cases the lungs are involved
The site of invovelement of Langerhans Cell
Disease depends on ?
Site of involvement depends on clinical form
- Neoplastic proliferation of Langerhans cells
- Langerhans cells
- Dendritic mononuclear cells normally found in the epidermis and mucosa
- Antigen presenting cells to T lymphocytes
- Langerhans cells
What is this clinical finding?
Langerhans Cell
Disease
Infant with Acute disseminated type
▪ See lesions on head/ear
What is this clinical finding?
Langerhans Cell
Disease
we see lesions on maxilla
What is this clinical finding?
Langerhans Cell Disease
▪ Older child
▪ Chronic disseminated form
▪ Alveolar ridge involvement
▪ Lot of bone loss and mobility
▪ Painful to brush
What are these clinical findings?
Langerhans Cell Disease
Torus and molar involvement
Chronic disseminated form (Hand-Schuller-Christian Disease, Multifocal Eosinophilic Granuloma)
What is its classic Triad?
- Exophthalmus
- Diabetes insipidus
- Lytic defects of bone
Chronic disseminated form of Langharan Disease
Clinically and radiographically
Clinically:
- we see mobility of teeth
Radiographically:
- picture of child with “teeth floating in air”
- Sharply punched out radiolucent lesions, ill- defined radiolucency in some cases
Eosinophilic Granuloma
Charcterstics & Demographics
- Elderly men often present with lung lesions (increased incidence with smoking)
- Localized lesions, usually affecting one bone
- If found to be an accessible solitary lesion, treatment is usually curettage and possibly low dose RT
- in some instances it can be initial presentation of systemic disease – but that would typically be in children or young adults
What is this clinical finding?
Eosinophilic
Granulations
Erythematous area
What is this radiographic finding?
▪ Child with disseminated form
▪ Punched out radiolucency in the skull
What is this clinical finding?
▪ Child with bone loss surround the teeth
▪ Floating teeth
disseminated form
What is this radiographic finding?
▪ Floating teeth
▪ Only attached by soft tissue due to extensive bone loss
disseminated form
What is this radiographic finding?
Eosinophilic granuloma
Langerhans Cell
Histiocytosis
Histology
It contain rod shape called
Birbeck granules
What is the differential diagnosis for Alveolar Bone Loss in Children?
o Juvenile periodontitis
o Langerhans cell histiocytosis
o Papillon‐Lefevre syndrome
o Cyclic neutropenia/agranulocytosis
o Burkitt’s Lymphoma
Multiple Myeloma
charcterstics
Monoclonal Expansion of malignant plasma cells
- Plasma cells make a lot of immunoglobulin ► So we will see a lot of immunoglobulin
Multiple Myeloma
Demographics and Location
- Most common in 40‐70 year old
- Means 63 years old
- Bones most commonly involved include ribs, vertebrae and skull
- 70‐90% will have jaw involvement at some point
Multiple Myeloma
Clinically
- Present with bone pain (>70%) and pathologic fractures
- Anemia, thrombocytopenia and neutropenia due to crowding out of normal cells within bone marrow by proliferating malignant cells
- 50‐60% have Bence‐Jones proteins in urine (light chains, usually kappa)
▪ Solitary plasmacytoma Vs Extramedullary plasmacytoma Vs Multiple myeloma – multifocal disseminated disease
▪ Solitary plasmacytoma
-
Individual lesion in bone
- When affecting oral cavity, ~95% of the cases are ramus and angle of the mandible
- In many cases p_rogress to more systemic disease_
Extramedullary plasmacytoma
- individual soft tissue lesion (not central In bone)
Multiple myeloma
- multifocal disseminated disease
Multiple Myeloma
Radiographically
- “punched out” radiolucencies (no sclerotic margin) often with an irregular outline
Multiple Myeloma
Lab findings
- Elevated M spike in serum
- Elevation of immunoglobulin in serum (hyperglobulinemia) most commonly IgG
- Deposition of amyloid in tissues (macroglossia)
Multiple Myeloma
Treatmet
Treatment can include :
- chemotherapy with or without Radtiaion Therapy
- bone marrow transplant
- interferon
- antibodies made against tumor cells
- thalidomide
- Even with treatment, many patients do not survive more than 18‐24 months, however treatment modalities are improving
- Older patients are treated less aggressively
What is this radiographical finding?
- Punched out radiolucency
- Lytic radiolucency without cortication
MM
Multiple Myeloma
What is this radiographical finding?
- more punched out radiolucency in iliac crest in the image
- Bone marrow biopsy usually done in this area since it’s frequently involved
multiple Myeloma
MM
What is this radiographical finding?
Multiple Myeloma
▪ Radiolucency without sclerotic border
▪ Multiple and separated
What is this clinical finding
Swelling of gingiva
▪ Plasmacytoma
Ewing Sarcoma, Ewing’s family of sarcomas
Charcterstics
▪ Highly malignant, undifferentiated, small round cell tumor/small blue cell tumor
o The cell of origin is in question, may be of neural crest origin
Ewing Sarcoma
Demographics and Location
- Primarily a disease of children and adolescents (90% of patients are between 5 and 30 years old) ▪ >60% males ▪
- Twice as common in mandible
- Caucasians>>>>Blacks and Asians
- Make up ~6% of primary malignant bone tumors
- In the jaws, the ratio of primary tumor to metastasis is 14 to 1
- 50% of cases in the femur and pelvic bones
- ~1% occur in the jaw bones
Ewing Sarcoma
Clinically
- Patients often present with pain, swelling, fever and elevated ESR (similar to signs of inflammation)
- May be misdiagnosed as an infection or osteomyelitis
- Paresthesia and loosening of teeth are common findings with jaw lesions
Ewing Sarcoma
Radographically
- an irregular lytic lesion with ill‐defined margins
- Root resorption may be seen
- May see thickening of the periosteum with a characteristic “onion skinning” pattern (like with peripheral periostitis)
- seen more in long bones
What is this gross finding?
Ewing Sarcoma
▪ Long bone
▪ Large expansion
What is this radiographical finding?
Ewing Sarcoma
- an _expansion of tissu_e
- Dissolution of bone in that area
Ewing Sarcoma
Treatment
- Combined therapy with multi‐agent chemotherapy, radiation therapy and surgery has led to 40% ‐ 80% survival rates
- Gnathic Ewing sarcoma has a lower mortality rate than all other primary sites
What are the most common carcinomas that metastasize to the jaw
The most common carcinomas to jaw: Breast Lung Thyroid and Colon Kidney Prostate ▪
“B. L. T. and Cold Kosher Pickle”
What is this radiographical finding?
Metastatic Carcinoma to Jaw Bones
▪ A. Metastatic breast carcinoma surrounding the apical half of the second and third molar roots and extending inferiorly. It has destroyed the inferior border of the mandible.
What is this radiographical finding?
Metastatic Carcinoma to Jaw Bones
B. Bilateral metastatic lesions from the lung destroying the mandibular rami.
What is this radiographical finding?
Metastatic Carcinoma to Jaw Bones
D. Destruction of the left mandibular condyle (arrows) from a thyroid metastatic lesion
What is this radiographical finding?
Metastatic Carcinoma to Jaw Bones
A. Partial panoramic image of prostate metastatic lesions involving the body and ramus; note the sclerotic bone reaction (arrows).
What is this radiographical finding?
Metastatic Carcinoma to Jaw Bones
B. Occlusal image of prostate lesions causing sclerosis and spiculated periosteal reaction (arrows)
What is this radiographical finding?
Metastatic Carcinoma to Jaw Bones
C. Periapical image of a metastatic lesion of breast carcinoma; note the irregular widening of the periodontal membrane spaces and patchy sclerotic bone reaction, especially around the roots of the molars
Malignant tumors of jaw
Review
Metastatic tumors to the jaw are more common than primary lesions
● Think osteosarcoma, chondrosarcoma, or osteoblastic metastasis (breast or prostate) if lesion radiographically looks malignant and has radiopaque internal pattern
● Ewing’s sarcoma is clinically accompanied by signs of inflammation
●Multiple myeloma characteristically presents as multiple, well‐ defined, punched‐out radiolucencies
● Bone affected by Non‐Hodgkin’s lymphoma radiographically appears to be dissolving
● Langerhans cell histiocytosis characteristically presents as scooped‐ out radiolucencies at the mid‐root level
● Remember to include metastatic carcinoma to jaw as a radiographic differential diagnosis for malignant lesions
