BONE TUMORSBONE TUMORS

Dr.Ankit GujarathiDNB resident,DMH

INITIAL EVALUATION• Carried out in 4 phases –• 1st phase – involvesHigh index of suspicion for tumorsRoutine X-raysRoutine lab facilitiesMeticulous historyThorough physical examination• 2nd phase – is prebiopsy regional evaluation, to determine size,

location and type of tissue .• 3rd phase – is the actual biopsy.• 4th phase – is undertaken if presumptive clinical & path evidence

suggestive of malignancy, search for mets is done, using CT scan of lung & Tc-99 bone scan.

PRESENTING SYMPTOMS PainMassAn abnormal radiographic finding detected during evaluation of

unrelated problemPAIN:- is most frequent symptom -deep constant pain, poorly localised, worse at night.MASS:- rate of enlargement is important.

HISTORY OF THE PATIENT• AGE:- most imp information, because of their presentation in specific

age group.1st decade- usually ABC ,SBC2nd decade-Chondroblastoma,osteochondroma,osteosarcoma,Ewings3rd decade- GCT4th decade- chondrosarcoma5th decade- Multiple myeloma• SEX:- less imp than ageSome tumors like GCT are more in females• RACE:- little imp, Ewings rare in African descent• H/O any exposure to radiation.• carcinogens- methylcholanthrene, zinc beryllium silicate, beryllium

oxide.

PHYSICAL EXAMINATION• Evaluation of patient’s general health• TUMOR MASS should be measured & its location, shape,

consistency,mobility,tenderness,local temp & change with position should be noted.

• SKIN & SUBCUTANEOUS TISSUE :Small dilated superficial veins overlying the mass are produced by

large tumorsCafé-au-lait spots & subcutaneous neurofibromas indicate Von

Recklinghausen’s diseaseA venous malformation on the same of body as the cartilaginous

tumor is an indicator of Maffucci Syndrome REGIONAL LYMPH NODES: sign of metastatic disease.

OTHER TESTS

• Hemoglobin• CBC• ESR• CRP• Serum alkaline phosphatase, sr.ca• Electrophoresis & urinary Bence Jones protein

INVESTIGATIONS• X-RAY• CT SCAN• MRI• TECHNETIUM BONE SCAN-This type of scan uses a

very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone.

• PET- Positron Emission Tomography uses radioactive glucose to locate cancer. This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera.

What to see in X rays

• What is the precise location of the lesion?- cortex or medulla; epiphysis, metaphysis or diaphysis)?

• Is there any evidence of underlying bone abnormality ?• Is the lesion multifocal?• Does the tumour have a well-defined margin? Is there a rim

of sclerotic bone? The presence of a well-defined margin and a sclerotic rim strongly suggests a benign non-growing lesion.

• Is there evidence of significant cortical expansion or destruction? These findings are seen with locally aggressive or malignant tumours.

• Is there an associated periosteal reaction and, if so, of what type?

• Is there a soft tissue mass?

PATTERNS OF GROWTH and BONEDESTRUCTION

• Geographic• Moth-eaten• Permeative

TYPES OF PERIOSTEAL REACTION

Benign• None• SolidMore aggressive or malignant• Lamellated or onion-skinning• Sunburst• Codman’s triangle

BIOPSY • According to the tumor size and type (malignant or benign)

and the biopsy's purpose (to remove the entire tumor or only a small tissue sample), there are two types of biopsies used in bone cancer diagnosis. These are: needle biopsy and incisional biopsy.

• 1. Needle biopsy: During this procedure, a small hole is made in the affected bone and a tissue sample from the tumor is removed.

• There are two types of needle biopsies:• 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.

Principals of biopsy• Should be done after all investigation done.• Surgeon should be familiar with incisions for limb salvage

surgery. • If Tourniquet is used, the limb is elevated before inflation but

should not be exsanguinated by compression.• Avoid transverse incisions.• Enter thru single compartment(single muscle belly).• Minimal retraction.• Drain thru same incision.

• Biopsy from periphery of lesion.

STAGINGEnneking described the most widely used staging system for benign

bone tumors . Many benign bone tumors have the potential to present at, and

progress through, various stages during their disease course. Stage 1-LATENT, it do not have any characteristics of growth or

progressive change, may resolve spontaneously. Stage 2-ACTIVE, lesion deform the host bone but remain contained in

bone, require intralesional curettage. Stage 3-AGGRESSIVE , tumor extend beyond the bone, require

complete work-up and a removal with wide margins to avoid possible local recurrence.

• The staging system for malignant tumors adopted by the Musculoskeletal Tumor Society, and originally developed by Enneking is based on the histological grade, the local extent, and the presence or absence of metastasis.

Stage Grade Local Extent Metastasis 1A low intracompartmental - 1B low extracompartmental - 2A high intracompartmental - 2B high extracompartmental - 3 any any + Grade is determined by histological parameters. Low-grade tumors

generally have few mitotic figures, little if any cellular atypia, and have a relatively non-infiltrative growth pattern. High-grade tumors tend to have marked cellular atypia, hyperchromatism, and nuclear pleomorphism. They often demonstrate an infiltrative growth pattern

Musculoskeletal Tumour Society staging

(surgical margins)• Radical- Normal tissue extracompartmental• Wide -Beyond reactive zone through normal

tissue within compartment• Marginal- Within reactive zone-extracapsular• Intralesional -Within lesion

Site of bone involvement

WHO CLASSIFICATION

1.Bone Forming Tumors-• Osteoid Osteoma • Osteoblastoma 2. Cartilage forming

Tumors- • Osteochondroma• enchondroma • Chondroblastoma • Chondromyxoid fibroma 3. Giant Cell Tumor

4.Other-• Solitary bone cyst • Aneurysmal bone cyst • Metaphyseal fibrous

defect • Fibrous dysphasia • Osteofibrous Dysplasia • Myositis ossificans 5. Benign vascular tumors

tumors .

1.OSTEOCHONDROMA • solitary osteochondroma (exostosis) or multiple

osteochondromatosis .• Solitary Osteochondroma is a developmental abnormality

of bone. When a stalk is present, the structure is called pedunculated. When no stalk is present, it is called sessile.

• An osteochondroma growth usually stops at maturity. • Solitary osteochondromas-most common benign bone

tumor. • It is seen in patients aged 10 to 30. It occurs equally in

males and females.It is thought to arise during skeletal growth when bone grows away from the growth plate instead of in line with it.

SYMPTOMS

• The most common symptom of an osteochondroma is a painless mass near the joints.

• Most common skeletal sites include metadiaphyses of the femur and tibia at the knee (35%), proximal femur and humerus, pelvis and scapula.

DIAGNOSIS

• A plain X-ray will show the bony growth.

• MRI scan may be used to look for cartilage on the surface of the bony growth.

• CT scan may also be used.

TREATMENTNonsurgical Treatment

Most of the time, solitary osteochondroma is not removed surgically. Observe it & take regular X-rays to keep track of any changes.

Surgical TreatmentWhen surgery is recommended, it is best to wait until growth complete (a mature skeleton by X-ray evaluation) before removing a solitary osteochondroma. This decreases the chance of the tumor growing back. Surgery may be considered if the osteochondroma: Is causing pain with activity Puts pressure on a nerve or blood vessel Has a large cap of cartilage The osteochondroma is removed at the level of the normal bone.

Discussion• This tumor does not occur in bones with membranous type

of ossification.• Multiple osteochondromas represent an autosomal dominant

hereditary disorder and are associated with bone deformities.• Recurrence may develop when a portion of cartilage cap is

left behind.• Malignant transformation is more often seen in multiple

hereditary osteochondromas. Most common secondary malignancy is chondrosarcoma. (The earliest pathologic sign of malignant transformation to a low-grade chondrosarcoma is increased thickness of the cartilage cap, cap thickness will exceed 2cm)

MULTIPLE OSTEOCHONDROMATOSIS

• Multiple osteochondromatosis is also called multiple osteocartilaginous exostosis, multiple hereditary exostosis (MHE), familial osteochondromatosis, multiple hereditary osteochondromatosis, or Diaphyseal aclasia.

• It affects males more often than females. It usually happens within the first three decades of life.

• Alterations in genes called EXT genes are thought to be the cause of this disease. There is increased risk of malignant transformation.

SYMPTOMS

• Symptoms are the same as solitary osteochondroma. The number and location of osteochondromas varies. A patient may have deformities of the forearms, a short stature, and knock-knees and ankles. This is caused by abnormal growth in the bones affected by the osteochondromas.

• Symptoms and signs of a tumor becoming cancerous are: Growth of an osteochondroma after puberty Pain at the site of an osteochondroma A cartilage cap >2cm

2.ENCHONDROMA

• Benign cartilage tumor.• They are often found in patients between 10 and 20 years

of age. • very common in the small bones of the hand and feet• In rare cases, multiple tumors can appear as part of a

syndrome.• These syndromes are Ollier's disease and Maffucci's

syndrome.• Ollier’s disease-multiple enchromatosis• Maffucci’s disease-multiple haemangiomas

+enchondromas

SYMPTOMS

• Enchondromas are usually painless, found during an X-ray evaluation of another problem in the same area. When these tumors appear in the hands or feet, or in multiple lesions, they can deform the bone, lead to pathologic fracture.

• The deformities in patients with Ollier's disease and Maffucci's syndrome can be quite severe.

Diagnosis

• They are appear as small (less than 5 cm) lobe-shaped white tumors in the middle of the bone, white areas of the tumor usually show a pattern of rings and arcs that indicates the tumor contains cartilage.

Characteristics of a more aggressive tumor or malignant

chondrosarcoma include: Scalloping of the inner surface of the bone Thickening of the bone cortex Destruction of the bone by the tumor Soft-tissue mass Bone erosion that is growing Erosion surrounded by reactive bone

• CT or MRI scan give a more complete picture of the bone around the tumor. .

• Under the microscope, enchondromas have islands of cartilage that surrounds them. Usually, cartilage is not found in the center of bones. However, these tumors in the hand and foot or in Ollier's disease or Maffucci's syndrome may contain more odd-looking cartilage.

• Low-grade chondrosarcomas (cancers) look more cellular under a microscope than enchondromas and there is less normal bone in the tumor.

TREATMENT Nonsurgical Treatment• Most enchondromas require no treatment at all. When

needed, treatment for enchondroma can vary due to the difficulty in determining the aggressiveness of the lesion.

• Stage 1 latent lesions - that show no symptoms can be watched with regular X-rays to ensure the tumor is not growing.

Surgical Treatment• Scraping out and filling of the cavity with bone graft or

other filling substances back. • Tumors that cause pathologic fractures are usually treated

by allowing the fracture to heal. Then, the tumor is scraped out to prevent another fracture.

• Malignant tumors are either scraped out or the entire bone around the lesion must be removed. This decision is made depending on the grade of the tumor. The grade of the tumor is determined by imaging studies and biopsy.

3.CHONDROBLASTOMA

• Sometimes called Codman's tumor, a chondroblastoma is a rare type of benign bone tumor that originates from cartilage. Chondroblastoma most often affects the ends of the long bones in the arms and legs at the hip, shoulder, and knee.

• Chondroblastoma is a rare bone tumor that can affect people of all ages, most common between the ages of 10 and 20 years. This type of tumor is also more common in males than females.

Originates from immature cartilage producing cells called chondroblasts.

SYMPTOMS

• Symptoms of chondroblastoma may vary depending on the location of the tumor. Symptoms may include:

pain in the knee, hip, and shoulder joint. withered or shrunken appearance of the muscle near the

affected bone impaired mobility of the adjacent joint fluid accumulation in the joint adjacent to the affected

bone

DIAGNOSIS• X-RAY : The diagnosis of chondroblastoma can usually be

made by radiograph.The most common site for chondroblastoma is the epiphysis. The lesion is lytic with well defined margins and can be from 1-6cm in size. Scalloping or expansion of cortical bone may be present. Fine calcifications, either punctate or in rings, may be visible.

• MRI & CT : Cysts are present about 20% of the time and both MRI and CT can define the fluid levels. CT is also useful for defining the relationship of the tumor to the joint, integrity of the cortex, and intralesional calcifications.

Diagnosis

• Epiphyseal location of the tumor above the open

• growth plate, and "benign" radiological features (good demarcation and a

• sclerotic rim).

Histology• uniform, polygonal cells that

are closely packed. These primitive cells are derived from the epiphyseal cartilage plate and have abundant cytoplasm. A scant chondroid matrix may be superimposed by a pericellular deposit of calcification that appears like "chicken-wire".

TREATMENT• The goal for treatment of chondroblastoma is to remove

the tumor and prevent damage to the end of the affected bone.

• Treatment may include: Surgical removal of the tumor -

Biopsy and curettage with possible use of adjuvant liquid nitrogen or phenol, or a mechanical burr.

Bone grafting.

4.OSTEOID OSTEOMA• Osteoid Osteoma is a benign bone lesion with a nidus of <2 cm

surrounded by a zone of reactive bone. • Second decade,M>F• The proximal femur is the most common location followed by the

tibia, posterior elements of the spine, and the humerus.It is found in the diaphysis or the metaphysis of the proximal end of the bone.

SYMPTOMS :-• It has a distinct clinical picture of dull pain that is worse at night and

disappears within 20 to 30 minutes of treatment with non-steroidal anti-inflammatory medication.

• Joint pain may be present with a periarticular lesion and synovitis can occur secondary to an intraarticular lesion.

DIAGNOSIS• radiolucent nidus surrounded

by a dramatic reactive sclerosis in the cortex of the bone

• diagnostic features include (1) a sharp round or oval lesion

that is (2) less than 2 cm in diameter, (3) has a homogeneous dense

center.• CT is the preferred method of

evaluation.• Radiologic D/D includes

osteoblastoma, osteomyelitis, arthritis, stress fracture and enostosis

TREATMENT

• Osteoid osteoma will resolve without treatment.• If the patient does not wish to endure the pain and

prolonged use of non-steroidal anti-inflammatory medications, surgical removal or percutaneous ablation of the nucleus is indicated.

5.Osteoblastoma• Osteoblastoma is a rare bone-producing neoplasm that

closely resembles osteoid osteoma on microscopic examination.

• By definition, all osteoblastomas are larger than 1.5cm.• Osteoblastomas tend to arise in the axial skeleton,

involving the spine and the sacrum.• Osteoblastomas do not produce

prostaglandin/prostocyclin .• Peak incidence is in the second and third decades of life.

6.UNICAMERAL BONE CYST• A unicameral (simple) bone cyst is a cavity found

within a bone that is filled with straw-colored fluid. It is a benign condition.

• UBC are usually found in patients younger than 20 years.

• Location-proximal humerus or proximal femur. Less common locations include the pelvis, ankle (talus), or heel (calcaneus).

• There is no known cause.

SYMPTOMS

• Most unicameral bone cysts have no symptoms. Some are discovered incidentally. Other unicameral bone cysts are not noticed at all.

• If a unicameral bone cyst is thinning the bone, there may be pain with weightbearing activities.

• If there is a pathologic fracture through the cyst, the affected arm or leg may have pain, swelling, and deformity.

DIAGNOSIS

• plain X-rays to show a hollow cavity in the metaphyseal portion of the bone.

• There is no associated bone expansion. A well-defined cortical rim can be seen.

• Fallen leaf sign-path gnomic of #.

TREATMENT Nonsurgical Treatment• A unicameral bone cyst without pain or other symptoms

can be watched with repeated X-rays Surgical Treatment• Only unicameral bone cysts at risk of a pathologic fracture

need treatment.• If the unicameral bone cyst is painful, growing larger, or in

a location that may fracture, may be treated by aspiration and injecting it with a steroid or with demineralized bone from the bone bank.

• A unicameral bone cyst in a structurally compromising location may need surgery and fixation.

7.Aneurysmal bone cyst

• locally destructive, blood-filled reactive lesions of bone and are not considered to be true neoplasm's.

• Any bone may be involved, but the most common locations include the proximal humerus, distal femur, proximal tibia and spine.

• F>M

DIAGNOSIS

• circumscribed,loculated• Radiolucent lesion

producing Blowout expansion of the bone.

• Double-density fluid level and intralesional septations usually indicates an aneurysmal bone cyst.

Treatment

• Extended curettage and grafting with a bone graft substitute. Because the lesion may produce heavy bleeding, tourniquet control is advised. Marginal resection sometimes is indicated for lesions in expendable bones.

• Arterial embolization.

8.GIANT CELL TUMOR• Benign/aggressive tumor.• Ages of 20 and 40 years.• F>M• Giant cell tumors are named for the way they look under

the microscope. Many "giant cells" are seen.• Giant cell tumor of bone occurs almost exclusively in the

end portion of long bones next to the joints (epiphysis)- distal femur,proximal tibia, distal radius .

• In rare cases, this tumor may spread to the lungs.

Giant Cell Tumor Osteoclastoma of Bone in Rib"Soap Bubble" appearance on X-ray

SYMPTOMS

• The bone involved is generally tender. There may be swelling in the area around it.

• The first symptom is pain in the area of the tumor. There may be pain with movement of a nearby joint. Pain generally increases with activity and decreases with rest. Pain is usually mild initially, but it progressively increases. Occasionally the bone weakened by the tumor may break and cause sudden onset of severe pain.

DIAGNOSIS• Giant cell tumors appear on X-rays as destructive (lytic)

lesions next to a joint. Occasionally, the involved area of bone can be surrounded by a thin rim of white bone that may be complete or incomplete. The bone in the area of the tumor is sometimes expanded.

• MRI and CT scans better evaluate the area of involvement.• bone scan generally shows a "hot spot" in the bone where

the tumor is. An X-ray or CT scan of the chest will often be done to look for possible spread to the lungs.

Treatment• If not treated, these tumors will continue to grow and

destroy bone. Surgery has proven to be the most effective treatment for giant cell tumors.

Nonsurgical Treatment• Radiation therapy. • Embolization- a process where the blood vessels supplying

the tumor are occluded by means of a catheter threaded through the main arteries. Blocking the blood supply to the tumor can cause it to shrink and even disappear because the tumor loses its source of nutrition.

• Interferon

Surgical Treatment• If treatment is limited to curettage and bone grafting alone,

the tumor may come back (recur) up to 45 percent of the time.

• Scooping out the tumor (curettage) is generally performed. This techniques cause a window in the bone that can be filled with a bone graft.

• Enhancing the curettage with a high-speed burr or with the use of agents such as liquid nitrogen, hydrogen peroxide, or phenol, followed by placement of bone cement decreases the recurrence rate to 10 percent to 29 percent.

• In the rare cases where the tumor has spread to the lungs, surgical removal of the tumor in the lungs results in cure 75 percent of the time.

Thank you