Bone tumors

Overview
• Primary bone cancers make up less than 0.2% of all cancers.
• In 2014, an estimated 3020 people were diagnosed in the US and
1460 will die from the disease.
• Osteosarcoma (35%), chondrosarcoma (30%) and Ewing’s sarcoma
(16%) are the three most common forms of bone cancer.
• Rare: High grade undifferentiated pleomorphic sarcoma (UPS) of
bone, fibrosarcoma, chordoma and giant cell tumor of bone (GCTB).

Chondrosarcoma
• These characteristically produce cartilage matrices from neoplastic tissue
devoid of osteoid
• More common in older adults
• Pelvis and proximal femur are most common sites
• Conventional chondrosarcoma of the bone makes up 85% of all
chondrosarcomas
• It is divided as follows:
1. Primary or central lesions arising from normal appearing bone
preformed from cartilage
2. Secondary or peripheral tumors arising from pre-existing benign cartilage
lesions such as enchondromas or cartilaginous portion of
osteochondroma

Overview of clinical characteristics and therapeutic options in all subtypes of chondrosarcoma
of bone.
Gelderblom H et al. The Oncologist 2008;13:320-329
©2008 by AlphaMed Press

Histology of grade I, II, and III chondrosarcoma.

Histology of rare chondrosarcoma subtypes.

Radiological presentation of a conventional low-grade chondrosarcoma.

Flowchart of the surgical management of central and peripheral chondrosarcoma.

Flowchart of the surgical management of local recurrence in central chondrosarcoma.

NCCN Recommendations
• Histologic grade and tumor locations are the most important variable
determining choice of primary treatment.
• Wide excision or intralesional excision with or without adjuvant should be
considered for resectable low grade and intracompartmental lesions.
• Wide excision is preferred treatment for pelvic low grade
chondrosarcomas.
• High grade (grade II, III), clear cell or extracompartmental lesions, if
resectable, should be treated with wide excision with negative margins.
• Postop treatment with proton and/or photon beam RT may be used in
chondrosarcomas of skull base and axial skeleton.
• RT may be considered in unresectable cases or for palliation.

Chordoma
• They arise from embryonic remnants of notochord
• More common in older adults
• Predominantly arise in the axial skeletal with the sacrum (50-60%), skull
base (25-35%) and spine (15%)
• Classified into 3 variants: conventional, chondroid and dedifferentiated
• Conventional: most common, lack cartilaginous or mesenchymal
components
• Chondroid present with cartilage remnants and features of chordoma
• Dedifferentiated chordomas have features of high grade pleomorphic
spindle cell soft tissue sarcoma, follow aggressive course
• Chordomas of spine and sacrum present with localized deep pain or
radiculopathy, cervical ones cause airway obstruction or dysphagia or
present with oropharyngeal mass

Ewing’s sarcoma
• Ewing’s sarcoma is the second most frequent primary malignant bone
cancer, after osteosarcoma.
• It is a small round-cell tumor typically arising in the bones.
• It is slightly more common in boys (55:45 male:female ratio).
• Most common age of diagnosis is the second decade of life, although
20%–30% of cases are diagnosed in the first decade.

Localization
• Ewing’s sarcoma demonstrates a predilection for
the trunk and long bones.
• In the truncal skeleton, the pelvis predominates,
followed by the scapula, vertebral column, ribs and
clavicle.
• Of the long bones, the most common site is the
femur, followed by the humerus, tibia and bones of
forearm in that order.

Localization
• As opposed to osteosarcoma, Ewing’s sarcoma of the
long bones tends to arise from the diaphysis rather than
the metaphysis.
• Ewing’s sarcoma has a strong potential to metastasize.
• Metastases most commonly occur in the lungs and bone.
• More than 10% of patients present with multiple bone
metastases at initial diagnosis. While metastases in the
lungs, bone, bone marrow, or a combination thereof are
detectable in approximately 25% of patients, metastases
to lymph nodes are rare.
• Extra-skeletal Ewing’s sarcomas present with rapid
growth and frequent distant metastases, similarly to
Ewing’s sarcoma of bone.

Symptoms
Ewing’s sarcoma typically progresses quite rapidly.
Skeletal lesions typically progress to large tumors
that form in soft tissues within a few weeks.
The earliest symptom is pain. At ﬁrst, the pain can be
intermittent and mild, but rapidly progresses to the
point at which it becomes so intense as to require the
use of analgesic drugs.
When the tumor is vertebral or pelvic in origin, the
pain may be accompanied by paresthesia and treated
by irradiation.

Symptoms
• Pain often does not completely disappear during the night
• Pain without defined trauma adequate to explain the symptoms,
lasting longer than a month, continuing at night, or with any other
unusual features should therefore prompt early imaging studies.

Histologic and immunohistochemical features of Ewing’s sarcoma/pPNET.
Bernstein M et al. The Oncologist 2006;11:503-519

The reciprocal translocation between chromosomes 11 and 22 results in the formation of an
ews-fli1 fusion gene on the abnormal chromosome 22 that codes for a chimeric transcription
factor with the N-terminal transcriptional regulatory domain deriving from ews and the ets-
specific DNA-binding domain derived from fli1.

Imaging Evaluation of Ewing Sarcoma
Imaging in Ewing sarcoma can help to :
(a) detect and accurately assess the extent of
disease prior to treatment.
(b) evaluate for the presence of metastatic or
recurrent disease.
• (c) monitor therapy response.

Imaging Evaluation of Ewing Sarcoma
• Conventional radiography and magnetic resonance
(MR) imaging of the primary tumor.
• Tomography (CT) to evaluate for pulmonary
metastases.
• Bone scintigraphy to identify osseous metastases.

DIAGNOSTIC IMAGING
• PLAIN RADIOGRAPH :
• The initial imaging investigation of a
suspected bone tumor is a radiograph in
two planes. Tumor-related osteolysis and
periosteal reactions suggest a diagnosis of
primary malignant tumor.
Periosteal reactions, the reactive
osteogenesis of the periosteum, are
caused by extra-osseous extension of the
tumor.

DIAGNOSTIC IMAGING
Several types of periosteal reactions have been
observed:
 an ‘onion skin’ or ‘onion-peel appearance’ is a
prominent multi-layered reaction,
 a ‘sunburst’ or ‘spiculae’ pattern is a perpendicular
reaction, while ‘Codman’s triangle’ is a triangular lifting
of the periosteum from the bone at the site of
detachment.
 Typically, Ewing’s sarcoma appears as an ill-deﬁned,
permeative, or focally moth-eaten, destructive
intramedullary lesion accompanied by a periosteal
reaction (‘onion skin’) that affects the diaphyses of
long bones.
 The sunburst type of periosteal reactions can present,
but is less common in comparison with its occurrence in
osteosarcoma.

MRI
• The most precise deﬁnition of the local
extent of bone tumors, including the degree
of expansion into the intra medullary
portion and the relationship of the lesion to
adjacent blood vessels and nerves, is
provided by MRI.
• When malignant bone tumors are
suspected, MRI is routinely performed for
staging and surgical planning.
• MRI is particularly important in the imaging
of Ewing’s sarcoma.

MRI
MRI typically demonstrates lesions that involve large segments of the
intramedullary cavity, which extend beyond the area indicated by plain
radiographs.
MRI can also evaluate the extent of soft tissue masses, which can be quite
large.
MRI is widely used to assess responses to neoadjuvant
chemotherapy or irradiation, because regression of the extra skeletal
tumor mass can be precisely deﬁned.
Currently, MRI is the standard imaging method for such evaluation. Recent
studies have demonstrated, however, that PET, thallium-201 scintiography
and dynamic MRI provide more valuable information than MRI for
assessment of therapeutic responses.

Staging investigations at diagnosis.

Prognostic Factors
• Favorable:
• Distal site of primary disease
• Tumor volume <100 ml
• Normal LDH at presentation
• Absence of metastatic disease at presentation
ESFT in spine and sacrum has worse prognosis.

Enneking classification of surgical intervention.

Giant cell tumor of the bone
• 10 bone neoplasm
• Generally benign
• Potential for :
• Recurrence
• Pulmonary metastasis
• Frank malignancy

Epidemiology
• 5-10% 10 bone tumors
• 20% benign bone tumors
• F : M 1.5 : 1
• 70-80% age 20-40
• Epiphyseal

Incidence
• Ends of long bones
• >50% about knee
• High recurrence rate
• 1-2% benign pulm. Mets
• 10 malignant GCT <1%
• Rare polyostotic form <1%

Presentation
• Pain x wks. – mos.
• Swelling
• Mass
• Pathologic #
• Neuro deficit (spine / sacrum)
• incidental

Radiology
• Lytic lesion
• Epipyseal
• Eccentric or central
• Narrow zone transition
• Cortical thinning
• expansile
• No sclerotic margin

Imaging
• Occ. Cortical breakthrough
• +/- soft tissue mass
• Extend to subarticular cortex
• Typically no host response
• Often large @ presentation

Other modalities
• CT
• Integrity cortical rim
• MRI
• Assess subchondral breakthrough
• Bone Scan
• Suspect multicentri loci
• ie. HAND

Histology
• Fibrohistiocytic origin
• Multinucleated giant cells
• Mononuclear stroma
• Round / ovoid / spindle
• Indistinct cell membrane
• Giant cells 20 fusion stromal cells

Enneking Staging
Stage 1 Stage 2 Stage 3
Pt % 10-15% ~70% 10-15%
Symptoms asymp pain pain
Radiograph sclerotic
rim
expanded
cortex
cortical
perforation
Histology benign benign benign

Biopsy
• Necessary for Dx
• Tumor principles
• Histologic grade not helpful
• R/O 10 malignant GCT
• Occ assoc.
• ABC
• Pagets

Tx
• Traditionally:
• Intralesional curettage / resection & bone graft
• Recurrence 35-42%
• En Bloc resection
• Recurrence ~10%
• Multiple complications
• Adjuvant

Curettage
• Wide decortication (windowing)
• Curettage / high speed burr
• Aggressive
• Choice of adjuvant

Adjuvant Tx
• Radiation - ~10% sarcomatous degeneration
• PMMA, Liquid N2, Phenol, CO2 laser, Electrocautery
• Local extension of margin
• Kill residual foci

PMMA
• Fill tumor cavity
• Heat kill of tumor cells?
• Effect size dependent
• 8-26% recurrence
• Easy recurrence detection
• Degenerative changes

Cryotherapy
• 3 freeze thaw cycles
• Irrigate cartilage with cool saline
• Circumferential necrosis
• “difficult”
• Complications
• Soft tissue injury
• Late fractures

Phenol
• Wash cavity
• Alcohol rinse
• 10-20% recurrence

Enbloc Resection
• Expendable bones
• Prox fibula / Distal ulna
• High recurrence with other Tx
• Hand / Distal radius
• Recurrence
• Pathologic #
• Joint involvement
• Osteochondral allograft reconstruction

Spine
• < 3% vertebrae above sacrum
• All levels affected equally
• Affects vertebral body c ext. pedicle
• Resection with stabilization
• Often incomplete
• ?radiation as adjuvant (low dose 3000 Gyc)
• Incomplete excision
• Local recurrence

Sacrum / Pelvis
• Intalesional excision
• Adjuvant
• +/- radiation

Pelvis
• GCT often vascular
• Pre-op angiography
• ? embolization

• Classic X-ray findings:
1. Codman's triangle (periosteal elevation)
2. Sunburst pattern
3. Bone destruction
Osteosarcoma

Codman's triangle
Osteosarcoma

Bone tumors

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