BONE TUMOUR
BY SHREYA VEKARIYA
GUIDED BY DR.DHRUVI PATEL (PT)
CONTENTS
• EWING’S SARCOMA
• CHONDROBLASTOMA
• CHONDROSARCOMA
• REFERENCES
EWING’S SARCOMA
• Ewing’s sarcoma is a rare, highly malignant primary bone tumour
primarily affecting children and young adults, accounting for 10–14%
of all malignant bone tumours. It was first described by James Ewing in
1928.
Epidemiology
• Age: Most commonly affects individuals between 10–20 years, though
it can present up to 30 years. Around 80% of cases occur between 4–25
years.
• Sex: More common in males.
Rare overall, but second most common bone tumor in children and
adolescents after osteosarcoma.
Common sites affected
• Bones:
• Long bones (about 2/3 of cases): femur, tibia, fibula, humerus
(in that order).
• Flat bones (about 1/3): pelvis, scapula, ribs, and calcaneum.
• Location in bone:
• Diaphysis (shaft) of long bones is the most common site.
• Less commonly found in metaphysis in atypical cases.
Pathology
Gross pathology
• Grey-white, soft, and friable tumour tissue, often resembling
pus.
• Involves a large portion or the entire medullary cavity.
• Bone expansion with elevated periosteum and sub-periosteal
new bone formation (in layers).
• The tumour ruptures through the cortex early and infiltrates
adjacent soft tissues.
• May show hemorrhagic foci and cystic changes.
• Occasionally exhibits multicentric origin.
Histopathology
• Small, round, uniform cells resembling lymphocytes.
• Cells are arranged in sheets or cords with minimal
intercellular substance.
• Pseudorosette formation is common (tumour cells
surrounding a central clear area without fibril); true rosettes
are less frequent.
• Necrosis is common.
• No giant cells or new bone formation seen.
• Perivascular arrangements ("perithelioma" pattern).
• Tumour cells stain positive for glycogen (PAS-positive).
• Negative on silver stain (helps differentiate from reticulum cell
sarcoma).
Clinical Features
• Pain and swelling in the affected area (most commonly
intermittent, worsens at night).
• Often presents with constitutional symptoms: fever, chills,
sweating, anemia, leukocytosis, mimicking acute
osteomyelitis.
• Skin over tumour may be red with dilated veins.
• Pain may follow minor trauma, but this is usually incidental.
• Exacerbation and remission phases are characteristic.
Radiological features
• Classic appearance is a lytic lesion in the diaphysis with
cortical destruction.
• Periosteal reaction is deposited in layers—this produces the
characteristic "onion peel" appearance.
• Other patterns:
• Moth-eaten or "cracked ice" appearance.
• Permeative margins.
• In flat bones: lytic lesion with minimal new bone formation.
• May have a predominant soft tissue mass with little cortical
destruction.
• Can mimic osteomyelitis or soft tissue sarcoma in atypical
cases.
Ewing’s Sarcoma Chronic Osteomyelitis
Sequestrum Rare Common
Cloacae Absent Well-defined
Periosteal Reaction Onion-peel (aggressive) Smooth, solid
Location Diaphysis Metaphysis
Constitutional symptoms Present, mimics infection Often present
Other differentials:
• Osteosarcoma
• Malignant lymphoma
• Reticulum cell sarcoma (differentiated by silver stain)
Investigations
• X-ray: moth-eaten, lytic, or sclerotic lesion in diaphysis; onion-
peel appearance.
• Biopsy: essential for diagnosis (histopathological
confirmation).
• Special tests:
• PAS stain for glycogen (positive).
• Urine vma: to rule out neuroblastoma in children.
• Immunohistochemistry and electron microscopy for definitive
diagnosis.
Metastasis and spread
• Highly aggressive, rapid hematogenous spread to:
• Lungs
• Other bones (skull, vertebrae, ribs)
• Bone-to-bone secondaries are common
Treatment Approach
General
• Multimodal therapy is the standard: chemotherapy +
radiotherapy ± surgery
Radiotherapy
• Tumour is highly radiosensitive, melts rapidly with radiation
(“melts like snow”), but with high recurrence.
• Dose: 6000 rads (or 4000 + 1000 boost to tumour).
• Used especially in axial skeleton tumours or when surgery is
not feasible.
Cemotherapy
• Controls systemic disease and reduces recurrence.
• Standard regimens include:
• Vincristine
• Cyclophosphamide
• Adriamycin (doxorubicin)
• Newer drugs: ifosfamide, cisplatin, etoposide
(epipodophyllotoxin)
• Given in cycles every 3–4 weeks for 12–18 cycles.
Surgery
• Limb-salvage or conservative resections preferred.
• Debulking or wide excision based on location and spread.
• Amputation is rare, used in advanced local cases.
Prognosis
• Historically poor (5-year survival <10%), but with
modern treatment:
• 2-year survival: Up to 50–75%
• 5-year survival: Improved to 30–40%
• Unfavorable prognostic factors:
• Male sex
• Pelvic or humeral involvement
• Presence of distant metastasis
CHORNDROBLASTOMA
• Definition:
• Chondroblastoma is a benign, but locally aggressive tumor that arises
from cartilaginous tissue.
• It commonly affects the epiphysis (end part) of long bones, especially
around the knee joint (distal femur, proximal tibia).
• Age group:
• Typically seen in adolescents and young adults (10–25 years old), more
common in males.
• Bone involvement:
• It arises in the cancellous (spongy) bone and gradually destroys the
internal structure.
• Multiple calcium deposits are seen inside the tumor, due to abnormal
cartilage mineralization.
• Radiological features:
• Well-defined lytic lesion (bone destruction visible on x-ray).
• Surrounded by a sclerotic rim (dense bone border).
• Mottled or speckled appearance due to calcium deposits within the
tumor.
• Symptoms:
• Pain near the affected joint (often chronic).
• Swelling or tenderness.
• Restricted movement if near a joint.
• Treatment:
• Curettage (scraping out the tumor).
• Followed by bone grafting to fill the cavity.
• Recurrence is possible, so follow-up is essential.
.
CHONDROSARCOMA
• It is the second most frequent malignant bone tumor after
osteosarcoma.
• Arises from cartilage-producing cells (chondrocytes).
• Malignant tumor, but typically slow-growing compared to
osteosarcoma.
• Classification
• Primary/secondary: secondary tumors develop when benign cartilaginous
tumors are irradiated.
• Peripheral/central/juxtacortical: depending on the situation of the tumor
within the bone.
• Low, medium and highgrade malignancy depending on the cellularity.
• Location
Common sites:
• Proximal femur
• Proximal humerus
• Ribs
• Scapula
• Pelvis (innominate bones)
• Upper femora
Rare sites:
• Hands and feet (except calcaneus)
• Sex:
More common in males.
• Age:
• Typically affects 20–60 years.
• Rare below 20 years.
• Peak incidence: Sixth decade (~60 years).
• Clinical features
Symptom duration:
• < 2 years in 75% of cases.
• < 5 years in the rest.
Pain:
• Not prominent (unlike osteosarcoma).
• Becomes symptomatic after cortical breach or fracture.
Palpable mass:
• Firm, attached to bone.
• Tumor may grow to large size.
• Radiological features
Central tumors
• Central lytic lesion with calcification.
• Classic appearance:
• Fluffy, cotton wool, popcorn, or breadcrumb pattern.
• Affects:
• Metaphysis or diaphysis of long bones.
• Rarely the epiphysis.
• More calcification in slow-growing tumors.
• Soft tissue invasion, but little/no periosteal reaction.
Peripheral tumors
• Very large.
• Central part heavily calcified.
Juxtacortical tumors
• Arise adjacent to the cortex.
Diagnosis
• Biopsy is essential for confirmation.
• Risk of soft tissue seeding,
• Small biopsy scar within planned resection area is
crucial.
Treatment
• Surgical resection is the mainstay.
• Low/medium grade lesions:
• Require wide excision.
• Examples:
• Forequarter amputation (shoulder girdle).
• Hindquarter amputation (pelvic girdle).
• High grade lesions:
• Require radical marginal excision.
• Others:
• Chemotherapy: role is controversial.
• Radiotherapy:
• Used palliatively for:
• Inoperable or massive tumors.
• Inaccessible anatomical sites.
Prognostic factors
Factors indicating poor prognosis:
• Location:
• Tumors in axial skeleton and proximal long bones.
• Age:
• More aggressive in younger patients.
• Cytological indicators of high-grade malignancy:
• ↑ Water & calcium (85%)
• Dna > 5.5 μg/mg
• Protein > 350 μg/mg
• Ch-4-so₄ ↑ / ch-6-so₄ ↓ (ratio >1)
• ↓ Keratin sulphate
• Galactosamine/xylose ratio >10
• Hexosamine <75 μg/mg
• Tumor size:
• Larger tumors = higher malignancy risk
• Secondary chondrosarcoma:
• More malignant than primary tumors.
References
Ebnezar J. Essentials of Orthopaedics. 4th ed. New Delhi:
Jaypee Brothers Medical Publishers; 2010.
Maheshwari J. Essential Orthopaedics. 5th ed. New Delhi:
Jaypee Brothers Medical Publishers; 2015.
