4. OVERVIEW
• Bone tumors are very diverse in morphology and
biological potential (can be no big deal or rapidly
fatal)
• MOST bone tumors are benign lesions
• Most benign lesions are seen <30 years of age
• A new bone tumor in the elderly is more likely to be
malignant
• No bone is safe (though most primaries are in long
bones)
• Location in the bone gives important Dx info
• More common benign lesions typically present as
incidental findings (non-painful, stable size)
• Be cautious with painful lesions and those that grow
relatively fast (over weeks or months)
• Pathological fracture can be the first sign of tumor
5. • Bone neoplasms are very difficult to diagnose
specifically on radiologic testing alone
• So why is radiology important?
– Exact location of lesion
– Extent of growth/metastasis
– Aggressiveness
• Best test for Dx= X-ray
• Best test for staging= CT or MRI
• Quick shout out to the pathologists– histologic grade
is the most important prognostic feature of bone
sarcomas and essential for staging most of the bone
tumor types.
8. BIOPSY
• Most conclusive test because it confirms if the tumor is
malignant or benign, the bone cancer type (primary or
secondary bone cancer), and stage.
• TYPES-
• 1. Needle biopsy: During this procedure, a small hole is made
in the affected bone and a tissue sample from the tumor is
removed.
two types -
• Fine needle aspiration: During this procedure, the tissue
sample is removed with a thin needle attached to a syringe.
• Core needle aspiration: During this procedure, the surgeon
removes a small cylinder of tissue sample from the tumor with
a rotating knife like device.
• 2. Incisional biopsy: During this procedure, the surgeon cuts
into the tumor and removes a tissue sample.
9. ENNEKING STAGING
Enneking described the most widely used staging system for benign
bone tumors .
The stages are denoted by the Arabic numerals 1, 2, and 3, whereas
malignant bone tumors are classified by Roman numerals (I, II, III).
Stage 1-LATENT- low biological activity, well marginated , often
incidental ,may resolve spontaneously. -NOF
Stage 2-ACTIVE-Symptomatic,limited bone destruction, may present
with pathological fracture- ABC
Stage 3-AGGRESSIVE- Bone destruction/Soft tissue extension, require
complete work-up and a removal with wide margins to avoid possible
local recurrence. -GCT
10. SURGERY PRINCIPLES
• Surgical margin is described by one of four terms—
intralesional, marginal, wide, or radical.
• Intralesional Resection--Plane of surgical
dissection is within the tumor.
• Often described as “debulking” because it leaves
behind gross residual tumor.
• Marginal resection--achieved when the closest plane
of dissection passes through the pseudocapsule.(
surrounding reactive tissue around tumor )
• For most benign lesions and some low-grade
malignancies
16. ◾ Benign lesions of hyaline cartilage
◾ Common in adults; M=F
◾ Common sites – Phalanges of the hand, small bones of hand and feet
◾ Asymptomatic usually, discovered incidentally/after a
pathologic fracture
◾ Intramedullary canal – “Enchondroma”
◾ Olliers’ Disease – Multiple enchondromatosis - hereditary
– cartilaginous tumors appear in the large and small tubular bones and in
flat bones – due to failure of normal enchondral ossification. malignant
degeneration to chondrosarcoma
◾ Olliers’ + Haemangiomas = Maffucci syndrome
17. ◾ Imaging - Lobulated areas of stippled calcification
(Popcorn/punctate calcification), Minimal cortical erosion
(except in hand).
◾ Histology - Benign-appearing hyaline cartilage.
Treatment
◾ Solitary Enchondromas – Observation with serial
radiographs. If stable radiographically, no further
intervention is indicated.
◾ Symptomatic – Curretage
◾ Multiple enchondromatoses – Deformities treated by
osteotomy
18.
19. ◾ More developmental malformations than true neoplasms
◾ Originate within the periosteum as small cartilaginous
models
◾ Slight male predominance. 2nd-3rd decade. Metaphysis of
long bones
◾ Multiple hereditary exostoses (MHE) is autosomal
dominant —mutation of EXT1 or EXT2
◾ Presentation - Mass; may be painful secondary to irritation of
soft tissue structures, fracture, or overlying bursa.
◾ Two types – Pedunculated or Sessile. Pedunculated more
common.
20. ◾ Imaging studies – Plain radiograph/CT/MRI (Confirmatory) –
Pedunculated or sessile bone lesion that communicates with
the intramedullary canal of the host bone + Cartilage cap
◾ Histological features – Similar to epiphysis of the bone which
undergoes enchondral ossification
◾ Treatment – Observation
◾ if asymptomatic : En- bloc resection
◾ if symptomatic, with removal of the cartilage cap.
◾ Malignant degeneration – rare – 1% solitary, 5% in multiple
hereditary exostoses.
21.
22.
23. ◾ Replacement of normal bone and marrow by fibrous tissue
and small, woven spicules of bone. Monostotic/Polyostotic.
◾ Common – 1st to 3rd decades. M=F
Can occur in the epiphysis, metaphysis or diaphysis
◾ Presentation – Pain, deformity, cutaneous pigmentation and
endocrine abnormalities (McCune Albright Syndrome),
sexual precocity, intramuscular myxoma (Mazabraud
syndrome) and thyroid disease.
◾ Histology – Irregularly woven bone spicules with a fibrous
stroma
24. ◾ Imaging – Ground glass appearance, granular with a well-
defined sclerotic rim. Small areas of cartilaginous metaplasia
and cystic changes may be present
◾ Treatment – Surgical treatment – significant deformity or
pathological fracture or pain – intramedullary
fixation/osteotomy. Treatment with bisphosphonates for
extensive disease
25. ◾ A.K.ACampanacci disease
◾ Ossifying fibroma of long bones
◾ Common 2nd-3rd decade of life – usually affecting the tibia and
fibula
◾ Presentation –Asymptomatic unless there’s a pathological fracture –
anterior bowing.
◾ Radiologically – Multicentric radiolucent lesions of the cortex of the
tibia
◾ Histologically – Irregular trabeculae with prominent osteoblastic
rimming in a loose fibrous stroma
◾ Treatment – Observation, Fractures usually non-operatively, deformity
correction.
26.
27. ◾ Developmental/reactive lesion
◾ 1st – 2nd decades. M:F = 2:1
◾ Any extremity; most common – proximal humerus and femur. Ilium
and calcaneum.
◾ Active during skeletal growth and heal spontaneously at
maturity
◾ Asymptomatic unless pathological fracture
◾ Radiologically – Centrally located, purely radiolucent lesion which
concentrically expands the cortex. No cortical destruction
◾ Cyst filled with straw colored fluid.Thin fibrovascular lining.
28. ◾ Observation
◾ Aspiration
◾ Injection of steroids/bone marrow/bone graft
substitutes
◾ Curretage
Treatment
Aneurysmal bone cyst ?
29.
30. ◾ Relatively rare
◾ Adults, M=F
◾ Asymptomatic, discovered as incidental findings.
◾ Radiology – Well defined lucencies with a thin rim of reactive
bone.
◾ Histologically – Fatty tissue with focal areas of necrosis.
◾ Surgery – only if symptomatic – simple curretage
31. ◾ Common benign bone lesion. 10% population has
asymptomatic lesions in vertebral bodies.
◾ Skull, long bones uncommon.
◾ Usually incidental findings.
◾ Rarely symptomatic unless there is vertebral collapse or
nerve root or cord compression.
◾ Radiographic appearance – Characteristic thickened vertically
oriented trabeculae – “Jailhouse” appearance
Cross section – “Polka-dot” pattern
32.
33. ◾ MRI – bright onT1 andT2 weighted images.
◾ Asymptomatic – no treatment required
◾ Vertebral collapse with neurological deficit –
decompression with spinal stabilization.
Long bones – extended curretage
Pre-op embolizationto prevent blood loss.
Low dose radiation – risk of
malignant transformation
34. Giant-cell tumor of bone
(osteoclastoma)
Description: benign but locally aggressive tumor composed of giant cells that
arise from the bone marrow [1]
Epidemiology: peak incidence between 20 and 40 years
Clinical features
Found in the epiphysis or metaphysis of long
bones (especially knee region)
Pathological fractures
Local pain and swelling
Limited range of motion
Diagnostics
X-ray: multicystic osteolytic lesions (soap-bubble appearance)
Histopathology
Mononuclear, RANKL-expressing cells (neoplastic)
Multinucleated giant cells (are reactive and resemble osteoclasts)
Treatment: curettage and bone grafting or en-bloc resection to minimize
recurrence rate
Prognosis: risk of malignant degeneration increases with age; may spread to
the lungs
35. When to do what?
Curettage , bone cement
(adjuvant therapy)
and
k wire fixation
radiolucent core (osteoid), surrounded by perifocal sclerosis (nidus)
Gardner syndrome: extracolonic manifestation of familial adenomatous polyposis (FAP) that includes osteomas of the skull or mandible, hypertrophy of the retinal pigment epithelium, adrenal adenomas, desmoid tumors, dental abnormalities, and cutaneous lesions
hereditary multiple exostoses Definition: autosomal dominant disorder with multiple exostoses
GNAS1 codes for the α subunit of the Gs protein (Gsα).
Mutation → constitutive activation of certain Gs-cAMP coupled pathways → inhibition of mesenchymal differentiation into osteoblasts → lack of osteocytes → skeletal lesions composed largely of mesenchymal cells → weak, imperfect bone with fibrous tissue
blood-filled cysts
Idiopathic
secondary to malignant or other benign bone diseases
spine and the metaphysis of the femur and tibia.
surgical curettage and bone grafting
Prognosis: high risk of recurrence
