Diseases & Tumors of Bone
DR. ZAMEER PASHA
Bone Tumors
 Bone tumors are comparatively rare but are very important due to their
high malignant potential.
 Bone tumors can be primary or metastatic.
 Bone forming tumors are – osteoma, osteoid osteoma, osteoblastoma &
osteosarcoma.
Osteoma
 Osteoma is a rare benign, slow growing lesion. Considered more as a
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hamartoma rather than a true tumor.
It is almost exclusively seen I the flat bones of the skull and face.
It may be seen as part of gardner’s syndrome.
It may grow into the paranasal sinus or protude out of the orbit.
Appears as a dense radiopaque mass
Histologic features – consists of a well differentiated mature lamellar
bony trabaculae seperated by fibrovascular tissue.
Osteoid osteoma & Osteoblastoma
 Osteoid osteoma and osteoblastoma are closely related benign tumors
occuring in children and young adults.
 Both the tumors are histologically very similar.
 Osteoid osteoma is small [less than 1 cm] and painful tumor, located in
cortex of long bones with reactive bone formation. Appears as a
radiolucent lesion with sclerotic border. The pain is relieved by asprin
 Osteoblastoma is larger in size usually painless, located in the medulla
of vertebra, ribs and long bones. No reactive bone formation, when
painful the pain is not relieved by asprin.
Osteosarcoma
 Osteosarcoma is the third most common cancer in adolescence.
 Incidence is slightly higher in males, occurs chiefly in young persons.
 A number of variants of osteosarcoma are seen (1)conventional types
(ie, osteoblastic, chondroblastic, fibroblastic) (2) multifocal, (3)
telangiectatic, ( 4) Small cell, (5) Intraosseous well-differentiated, (6)
Intracortical, 7) Periosteal, 8) Paraosteal, (9) High-grade and
(10)extraosseous.
 Swelling and pain particularly with activity of the involved bone are the
early features of the neoplasm.
 Oral manifestations - the most common presenting symptoms of the
patients were swelling of the involved area, often producing facial
deformity, and pain, followed by loose teeth, paresthesia, toothache,
bleeding, nasal obstruction and a variety of other manifestations
 Radiographic features: small streaks of bone radiate outward from
approximately 25% of these tumors. This produces a sunray (sunburst)
pattern. , the periosteum is elevated over the expanding tumor mass in
a tent-like fashion. At the point on the bone where the periosteum
begins to merge (edge of the tent), an acute angle between the bone
surface and the periosteum is created. This is called Codman’s
triangle. resorption of the alveolar bone resulting in uniform widening
of the periodontal ligament space .
 Histologic features: the characteristic feature of osteosarcoma is the
presence of osteoid formed by malignant osteoblasts in the lesion. It is
further subdivided on the basis of the predominant features of the cells
(ie, osteoblastic, chondroblastic, fibroblastic).
Ewing’s Sarcoma
 Ewing's sarcoma is a sarcoma of the bone which is highly malignant
seen in age group of 5-20 years.
 Arises from primary neuroectodermal cells.
 Three variants seen –
Classical [skeletal] ewings sarcoma
Soft tissue sarcoma
Primitive neuroectodermal tumor
Clinical features include pain tenderness and swelling of the affected area
accompanied by fever, leucocytosis and elevated ESR
Radiographic feature of ‘onion skin’ appearance due to periosteal bone
formation is characteristic.
Osteoarthritis
 This is the most common form of chronic disorder of synovial joints. It
is characterized by progressive degenerative changes in the articular
cartilages, particularly in weight-bearing joints.
 Types –
Primary : occurs in elderly due to aging, steady increase in clinical
symptoms.
Secondary: may appear at any age, related to previous injury,
fracture or inflammation with the concerned joint.
The patient usually complains of - joint stiffness, discomfort, pain and
diminished mobility.
Hard bony enlargements of the terminal phalanges are known as
‘Heberden’s nodes’
Marfan Syndrome
 Marfan syndrome is a spectrum of disorders caused by a heritable
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genetic defect of FBN1 gene on chromosome 15, bands q15-q23.
It codes for the connective tissue protein, fibrillin.
The skeleton typically displays multiple deformities including
arachnodactyly ,dolichostenomelia (ie, long limbs relative to trunk
length). The shape of the skull and face is characteristically long and
narrow. hyperextensibility of joints with habitual dislocations, kyphosis
and flat foot. In the cardiovascular system, aortic dilation, aortic
regurgitation, and aneurysms are the most worrisome clinical findings.
a high, arched palatal vault is very prevalent and may be a constant
finding. Bifid uvula and malocclusion is also reported.
multiple odontogenic cysts of the maxilla and mandible have
occasionally been reported.
Osteopetrosis (Marble Bone Disease; Albers-Schonberg
Disease)
 Osteopetrosis is a rare hereditary bone disease in which failure of
osteoclastic bone resorption leads to increased bone mass. However,
the bone has poor mechanical properties.
 Three distinct forms of the disease are based on age and clinical
features. These are adult onset, infantile, and intermediate.
 Infantile osteopetrosis (also called malignant osteopetrosis)
is diagnosed early in life. Failure to survive and growth retardation are
symptoms. Manifestations include deafness, proptosis, and
hydrocephalus. Dentition might be delayed. Osteomyelitis of the
mandible is common due to a deficient blood supply. Defective osseous
tissue tends to replace bone marrow, which can cause bone marrow
failure with resultant pancytopenia.
 Adult osteopetrosis (also called benign osteopetrosis)-
Approximately one half of the patients are asymptomatic, and the
diagnosis is made incidentally Bony defects are common and include
cranial nerve entrapment neuropathies, Bones are fragile and might
fracture easily.
 Oral Manifestations: The medullary spaces of the jaws are
remarkably reduced, there is a marked predilection for the
development of osteomyelitis, this is a complication of dental
extraction. Fracture of the jaw during tooth extraction, even when the
extrac tion is performed without undue force, may also occur.
 It has been reported that the teeth are of defective quality, enamel
hypoplasia, microscopic dentinal defects and arrested root development all having been described.
Down Syndrome [trisomy 21]
 Down syndrome is a frequent form of mental retardation associated
with characteristic morphologic features due to chromosomal
aberrations.
 The major features of Down syndrome are mental retardation,
characteristic head appearance, flat facies with increased interocular
distance (hypertelorism), depressed nasal bridge, flat occiput, and
broad short neck. Narrow, upward and outward slating of the palpebral
fissures. Skeletal anomalies include short stature; broad and short
hands, feet, and digits.
 Oral manifestations: Small mouth with protrusion of the tongue
(macroglossia) with difficulty in eating and speaking, scrotal tongue,
hypoplasia of the maxilla, delayed tooth eruption, partial anodontia,
enamel hypoplasia, juvenile periodontitis, and cleft lip. Juvenile
periodontitis is a feature of Down syndrome.
Massive Osteolysis [Vanishing Bone, Gorham Disease]
 Massive osteolysis is an unusual and uncommon disease characterized
by spontaneous, progressive resorption of bone with ultimate total
disappearance of the bone.
 The disease, which may or may not be painful, begins suddenly and
advanced rapidly until the involved bone is replaced by a thin layer of
fibrous tissue surrounding a cavity.
 The patient may present with pain or facial asymmetry or both. One of
the consistent findings in the disease has been pathologic fracture
following minor trauma.
 There is no specific treatment. Radiation therapy has been of benefit in
some cases, while surgical resection has stopped the progress of the
disease in others. Left untreated, the disease commonly progresses to
total destruction of the involved bone.