Non-odomtogenics
tumors
Non-odomtogenics tumors
• Tumor :
A solid mass of tissue that forms when abnormal
cells group together.
• Types of tumor:
1- Benign
2- Malignant
Non-odomtogenics tumors
• Tumors develop from other tissues within the jaws
that are not related to the teeth, such as bone or soft
tissue cells
Non-odomtogenics tumors
Benign Malignant
• Osteoma
• Osteosarcoma
• Multiple Osteomas in Gardner’s
Syndrome • Chondrosarcoma
• Central Haemangioma • Ewing’s Sarcoma
(Intraosseous Haemangioma) • Myeloma
• Melanotic Neuroectodermal • Solitary Plasmacytoma
Tumour of Infancy
• Burkitt’s Lymphoma
1- Osteoma
• Definition/Description
A benign tumour composed of mature compact
and/or cancellous bone
occur rarly in the jaw.
Types:
1- peripheral or periosteal
2-central or endosteal osteoma
• Aetiology:
Cause is unknown but injury or muscle traction may be
contributing factors.
• Clinical Features:
- Mandibular body (lingual to premolars) and the condyle are the
sites of predilection.
- Solitary and asymptomatic.
- Slow growing.
- Limitation and deviation of mouth opening in condylar osteoma.
a slow growing hard painless
lesion of over two years duration. Note a
round nodule on the right anterior surface
of the mandible adjacent to teeth cantral
and lateral
• Radiographical Features
- Round sclerotic mass (peripheral and central types).
- Dense sclerotic mass at the periphery and central trabeculation
(periosteal osteoma)
Panoramic radiograph
of osteoma revealing a
radiopaque lesion
(red arrows).
• Differential Diagnosis
- Condensing osteitis (central type)
- Focal chronic sclerosing osteomyelitis (central
type)
• Management
- Small asymptomatic osteoma needs no
treatment
- Large and symptomatic osteoma should be
removed surgically
2-Multiple
Osteomas in Gardner’s Syndrome
• Definition
- A rare genetic disorder characterized by multiple
intestinal polyps, osteomas of the cranium or mandible,
epidermal cysts and fibromas, and abnormalities of the
teeth
- Intestinal polyps tend to progress to colorectal cancer
• Aetiology
- Mutations in adenomatous polyposis coli gene
• Clinical Features
- Colonic polyps
- Multiple bony swellings of the
craniofacial bones
- Multiple epidermoid cysts
- Impacted/supernumerary teeth
- Odontomas
- Fibrous neoplasms (desmoid tumours)
- Any of the above four findings suggest Gardner’s syndrome
Extraoral osteomas: multiple diffuse
bilateral swellings along the mandibular
border.
• Radiographical Features
- Presence of dense bony masses (osteomas), odontomas,
supernumerary teeth and other dental anomalies
Posteroanterior radiograph shows
extra-oral osteomas along the
mandible (white arrows).
• Differential Diagnosis
- Other familial intestinal polyposis syndromes
- Isolated forms of osteomas with other dental
anomalies
• Management
- Surgery for intestinal polyps, skin tumours and
jaw lesions
- Good prognosis
3- Central Haemangioma
(Intraosseous Haemangioma)
• true benign neoplasm of vascular origin, rare in the
maxillofacial bones.
• Aetiology
- Endothelial proliferation which differentiates into
blood vessels.
• Clinical Features
- Bone involvement is usually of cavernous type; it is congenital
- Mandibular ramus is mostly affected (premolar/molar area)
- Female predilection
- Peak incidence: second and fifth decade
- Asymptomatic in early stages
- More aggressive lesions are symptomatic: painless swelling, loose
teeth and bleed from the gingival
margins.
• Radiographical Features
- Cyst-like radiolucency; often with soap-bubble appearance
- Formation of reactive spicula produced by the lesion is a classic
radiographical feature of intrabony
haemangioma
Occlusal radiograph revealing a well-defined
lesion surrounded by a
sclerotic margin, containing reactive bone
spicules which are characteristic for
intraosseous cavernous
hemangioma.
• Differential Diagnosis
- Ameloblastoma
- Odontogenic myxoma
- Fibrous dysplasia
- Aneurysmal bone cyst
- Central giant cell granuloma
• Management
-Non-invasive radiotherapy
- Intralesional injection of sclerosing agents and selective arterial embolization
- Curettage and radiation
- Resection followed by osseous reconstruction
- Note: lesion should not be opened, or associated tooth extracted; these
procedures may be fatal
due to excessive haemorrhage
• Prognosis
-Favourable
4-Melanotic Neuroectodermal
Tumour of Infancy
• Melanotic neuroectodermal tumour of infancy is a rare, destructive,
benign, pigmented neoplasm of neural crest origin seen in early
infancy.
• Frequency
- Rare; 90% are in infants
- Median age five month
- Slightly more common in males
• Aetiology
- Unknown, tumour originates from neural crest.
• Clinical Features
- Occurs in the first few months of life
- Anterior maxilla commonly involved (60–80%), mandible (5.8%)
- Painless, non-ulcerative, locally aggressive, bluish black
(pigmented) tumour mass.
- Destroys underlying bone.
- Prevents tooth development.
A three-month-old female infant depicting, a rapidly
growing bluish mass on the anterior aspect of the maxilla.
Growth involves the right upper vestibule, alveolar ridge,
and anterior hard palate. There is an embedded tooth in
the lesion
• Radiographical Features
- Destruction of the underlying bone.
- Displacement of developing teeth.
• Differential Diagnosis
- Eruption cyst.
- Congenital epulis.
• Management
- Surgery, Variable prognosis.
- High recurrence rate: (10–60%); 15% in the first year of surgery.
- Malignant transformation: 6.6%
5- Osteosarcoma
• Definition/Description
A malignant neoplasm of the bone that forms abnormal bone or osteoid,
Rare; 5% of osteosarcomas arise in the jaw.
• Aetiology
- 10% of jaw lesions are radiation induced
Predisposing Factors
- Hereditary retinoblastoma
- Paget’s disease of the bone
- A history of fibrous dysplasia or trauma
• Clinical Features
- Median age is 30–40 years
- Maxilla and mandible are equally affected
- Local pain
- Numbness and facial dysesthesia
- Loose teeth
- Trismus and difficulty in mouth opening
- Nasal obstruction Note the bone forming mass in the right mandible with
considerable displacement of teeth in the region.
- Bleeding
- Firm mass fixed to the underlying bone
- Systemic features: fever, headache, weight loss
• Radiographical Features
- Irregular bone destruction: a moth eaten appearance.
- Periosteal elevation and new bone formation.
- ‘sun ray’ appearance is often seen.
• Differential Diagnosis
- Chondrosarcoma
- Fibrous dysplasia
- Osteomyelitis
- Osteoma
- Myositis ossificans
- Cemento-osseous
- Dysplasia
• Management
- Radical surgery (hemi-mandibulectomy).
- Recurrence in 50% of cases
- Five-year survival is 70% for tumors of less than 5 cm in
diameter and 0% for those greater than 15 cm.
6- Ewing’s Sarcoma
• Definition/Description
An uncommon round cell malignant bone tumour with an
aggressive course. Affecting mainly children and young adults.
• Frequency
- Approximately 4% in the bones of the head and neck, with
1% occurring in the jaws (predominantly mandible)
- Marked predilection for white populations
• Aetiology/Risk Factors
- Unknown
- Genetically related to primitive peripheral neuroectodermal
tumour via translocations.
- Chromosomal translation, gene rearrangement and expression
of the MIC2 gene.
• Clinical Features
- Slight male preponderance.
- Presenting signs and symptoms: swelling, pain, loose teeth,
paraesthesia, ulceration, trismus and toothache.
- Extraoral features: exophthalmos, ptosis, epistaxis, otitis media
and sinusitis, fever, anaemia.
- Rise in erythrocyte sedimentation rate, moderate leucocytosis
and an increase in serum LDH are common.
• Radiographical Features
- Osteolysis (moth eaten appearance), cortical erosion, and
periostitis.
• Differential Diagnosis
- Osteosarcoma
- Eosinophilic granuloma
- Histiocytosis X
- Giant cell tumour Cropped panoramic radiograph shows
radiolucent ill-defined osteolytic
- Lymphoma lesion with bony spiculations,
teeth displacement and missing.
- Primitive rhabdomyosarcoma
- Neuroectodermal tumour of infancy
- Chondrosarcoma
• Management
- Radiotherapy
- Chemotherapy
• Prognosis
- Five-year
- survival: 54–74%
7- Myeloma(Multiple Myeloma)
• Definition/Description
Haematological malignancy characterized by the multicentric
proliferation of a single clone of abnormal immunoglobulin-
secreting plasma cells.
• Frequency
- About 1% of all malignancy and 10% of haematologic
malignancy.
- Common between 50 and 80 years of age.
- Occurs twice as often in men as in women.
• Aetiology :Unknown
• Clinical Features
- Early signs and symptoms: fatigue, bone pain, fever, anaemia,
nephropathy and weight loss.
- Posterior region of the mandible is the preferred sites.
- Odontalgia, paraesthesia, tooth mobility, gingival haemorrhage
and ulcerations in later stages of multiple myeloma.
• Radiographical Features
- Multiple radiolucent defects (punched out appearance) in the skull and
the jaw with no sclerotic outline of the punched out radiolucent
lesions.
Radiograph shows multiple punched-out
radiolucencies involving the skull bones, facial
bones, and mandible.
• Differential Diagnosis
- Plasmacytoma (solitary)
- Brown tumour
- Metastatic lesions
- Chronic osteomyelitis
- Arteriovenous malformations
- Langerhans’ cell disease.
• Management
- Incurable but life can be extended for many years
- Combination of chemotherapy, corticosteroids, proteasome inhibitors (bortezomib) and thalidomide
analogues.
- Localized lesions may be treated with radiotherapy.
- Bisphosphonates for those with a tendency for myeloma related bone fractures
- Bone marrow or stem cell transplants for some patients .
- Prognosis
- Fair.
- Survival rate of six to seven years following diagnosis and treatment.
8-Solitary Plasmacytoma
• Definition/Description
- Plasmacytoma is a monoclonal, neoplastic proliferation of plasma
cells that usually arises within bone marrow or soft tissue sites.
• Frequency
- Rare in the jaws
-
• Aetiology/Risk Factors
- uncertain
• Clinical Features
- May be the first manifestation of a subsequent multiple myeloma
- Mean age 55 years
- Predilection for males
- Often detected incidentally on radiography
- Bone paraesthesia or pain and swelling
- Pathological fracture
- Haemorrhage
• Radiographical Features
- Well-defined solitary radiolucent lesion with no sclerotic outline.
- Microscopically, sheets of neoplastic plasma cells with varying degree of
differentiation.
ill-defined radiolucency involving the
entire right side of the mandible
• Differential Diagnosis
- Reactive inflammatory lesions such as plasma cell gingivitis
- Non-Hodgkin lymphoma
- Malignant melanoma
- Ameloblastoma
- Odontogenic myxoma
- Giant cell lesions
- Multiple myeloma
• Management
- Radiotherapy (offers better result)
- Chemotherapy (alone has little effect)
- Surgery
- Combination of surgery and radiotherapy offer best results
• Prognosis
- Course is relatively benign
- Survival rate is 50–80% at 10 years
- If recurrence is present, survival rate drops to 16%
9- Burkitt’s Lymphoma
• Definition/Description
The most aggressive malignant solid tumour of B
lymphocytes grouped under the umbrella of non-
Hodgkin B-cell lymphoma.
• Immunodeficiency-associated Burkitt lymphoma is
associated with HIV infection (or those who received
allografts) in older patients.
• Aetiology/Risk Factors
- Epstein–Barr virus is associated with 95% of the ‘endemic’ cases.
- Infection of B cell lymphocytes with the virus causes the disease.
- Malaria is a risk factor in endemic form of Burkitt’s lymphoma.
-
• Clinical Features
- 50–70% of endemic Burkitt’s lymphoma affects the jawbone.
- Peak prevalence is around seven years.
- Greater predilection for males.
- Predominantly affects posterior maxilla.
- Facial swelling and severely mobile and displaced teeth are common presenting symptoms.
- Premature exfoliation of teeth is common.
- Lymphadenopathy is present.
Tumour in a seven-year-old boy with a several-month
History of jaw swelling, which had been treated with antibiotics.
The tumour was ulcerated, infected and draining
• Differential Diagnosis
- Abscesses
- Apical lesion
- Ameloblastoma
- Undifferentiated carcinomas
- Sarcoma
• Management/Prognosis
- Fatal if not treated
- Treated preferentially with intensive chemotherapy
• Five-year survival rates 75–95%, depending on the stage of the
lesion at the time of diagnosis.
