Bone TumorsBone Tumors

Long Bones: Description

• Epiphysis• Metaphysis

• Diaphysis

• Metaphysis• Epiphysis

Long Bones: Description

• Bone– Periostium– Cortical bone– Cancelleous bone

• Medullary cavity (CANAL)• Bone marrow

• Blood supply• Nerve supply

Bones: Structure

• Organic– Cells– Collagen– Mucopolysaccharides– Water

• Inorganic– Mineral-based components

Bone cells: Tissues• Bony

– Osteoblasts– Osteoclasts– Osteocytes

• Cartilaginous- chondroblasts- chondrocytes

• Fibrous- Fibroblasts - fibrocytes

Classification:

Benign:Osteoma.Osteoid osteoma.OsteochondromaChondroma.Benign giant-cell tumors.

Malignant :Osteosarcoma.Chondrosarcoma.Malignant giant-cell tumors.Fibrosarcoma.Ewing's Tumor.Reticulum-cell sarcoma.Plasma cell tumor or multiple myeloma.Metastatic tumors.

Tissue of origin: Bone , Cartilage, Fibrous Others.

Benign Malignant Bone - Osteoma - Osteosarcoma - OsteoblastomaCartilage - Chondroma - Chondrosarcoma - Chondroblastoma - Osteochondroma - chondromyxoid fibromsFibrous Fibroma FibrosarcomaOthers GCT? Ewing’s

Myeloma

Tumour-like conditions of bone

• Bone cysts– Simple bone cyst– Aneurysmal bone cyst

• Fibrous-osseous lesions– Fibrous dysplasia

• Eosinophilic granuloma (Langerhans histiocytosis)• Osteochondroma - ?hamartoma

OSTEOMAIVORY

OSTEOMA

It arises from membranous bone of Skull .SmallSessileSmooth surface Slowly growingSimulating ivory (Compact & dense bone)

6.S

OSTEOMA

• Benign, Often craniofacial in location• Hamartomatous / reactive not true tumor.• Histologically are woven and lamellar bone

(closely resemble normal bone).• Never turn malignant .• Gardner Syndrome: multiple, Osteoma,

osteochondroma, GIT polyps, skin tumors. Autosomal Dominant, Colon Cancer.

IVORY OSTEOMASites:

Outer table of Skull disfigurementInner table pressure on brainOrbit interfere with eye movements

Paranasal sinus sinusitisAuditory meatus prevent hearingNeck of mandible interfere with jaw movementsTreatment : Excision biopsy.

Osteoma Lateral radiograph of the skull (a) and axial CT scan (b) demonstrate an ossific nodule (arrow) arising from the outer table of the skull

Benign painful lesion arises from osteoid mesenchyme Reparative bone lesion ? .Age: 10-30 YsSite : Long bones( shaft of femur, tibia ) [every bone expect mandible].

PainFew months duration by night Nagging ( like toothache)Referred to neighboring jointRelieved by salicylate.

Examination:Localized tenderness – palpable thickening of a superficial bone like tibiaX-ray :• Radiolucent area; • Circumscribed.• In one side of cortex.• ˂ 1 cm in diameter.• Surrounded by dense bone

sclerosis .• Nidus (small dense

shadow of calcified Osteoid) inside tumor.

OSTEOID OSTEOMA MACROSCOPICALLY

Radiographic Features

Nidus

D . DSclerosing Ch. O.M of GarreBenign Bone cyst.Brodie’s absces Osteogenic sarcomaEosinophilic granulomaEwing’s sarcomaNon ossifying fibroma

N.E: Nodule: Reddish; firm; small.M.E: Vascular osteoblastic tissue - uncalcified matrix - central calcified area

Treatment : = surgical excision after exact radiographic localization.= Radiofrequency ablation

OSTEOBLASTOMA• Clinically similar to osteoid osteoma

(large)• Also known as giant osteoid osteoma.• Common location -- vertebral column• Histology similar – but rare nidus.• Can be locally aggressive• Therapy - curettage/resection with bone

graft.

T. derived from cartilage forming C.T. ( chonroid + myxoid ).Age: < 20 Ys.Site: metaphysis of long bones.S&S: mild pain & mass.X-ray : Eccentric well demarcated; radiotranslucent lesion.Surrounded with slight sclerosis.

N.E: Rubbery tissue (not mucinous); Whitish; Firm.

M.E: Cells: Stellate, spindle shaped.Matrix: Fibrous & chondroid.

TreatmentCurettage & bone grafting.

Osteochondroma

OSTEOCHONDROMA(osteo-cartilagenous exostosis)

The commonest B.TPedunculated [ cartilage-capped exostosis]Sessile

Pedunculated [ cartilage-capped exostosis]

Sessile

• Age: 10-25 Ys• Site:

Metaphysis of long bones.

• S & S: Painless swelling

Origin ?:Displaced part of the epiphyseal cartilage Perverted activity of periosteum.

•Osteochondromas are mushroom shaped and range in size from 1 to 20 cm. •The outer layerouter layer of the head of the osteochondroma is composed of benign hyaline cartilage varying in thickness•Newly formed bone forms the inner portioninner portion of the head and stalk, with the stalk cortex merging with the cortex of the host bone.

Pain may be due to:Pressure on Nerve; Tendon, muscleAdventious bursitisFr. Of the stalk Malignant changes

OSTEOCHONDROMA

Bony swelling; Well defined Over the surface of the bone Continuous with the outer cortex of the mother boneA cauliflower mass

X-ray:

OSTEOCHONDROMAN.E: Stalk; of cortical & cancellous boneCap; of growing cartilageBase; directed away from epiphysis

BaseCap

Stalk

OSTEOCHONDROMA

Osteochondroma

Osteochondroma:

OsteochondromaOsteochondromaA cauliflower massA cauliflower mass

Complications: 1. Sarcomatous changes ( chondrosarcoma):

Pain Rapid growth Invasion of the mother bone Recurrence after excision.

2. Fracture of the Pedunculated Osteochondroma3. Bursitis4. Compression of Nerve5. Hitching of a tendon6. Bony block to the joint

Treatment: Excision & Biopsy

non symptomatizing tumors may be left alone and observed by repeated clinical and radiological examination.

Osteochondroma microscopy:Osteochondroma microscopy:

2-Hereditary or multiple Osteochondroma (Metaphyseal Aclasis):

It is a developmental disease of bone commonly affecting the bones of the forearm and leg. The bones may be deformed with irregular broad ends and the stature may be stunted. Only those tumors causing; pain or dysfunction are removed.

Malignant change is liable to occur (5-15%).

The three characteristic features of the disease are:Multiple osteochondromata Stunted stature. Lack of remodeling of the metaphysis.

PedunculatedSessile

OSTEOCHONDROMA

Multiple Hereditary Exostosis

ChondromChondromaa

B.T of cartilage tissue.Age: 10- 50 Ys.Site: Short bones of hands & feet. Metaphysis of long bonesRibs, pelvic bone & scapula.S &S : Swelling , mild pain Path. Fr.

CHONDROMA

X-ray: Well defined radiotransclucent area

Enchondroma (centrally situated) little or no expansion

Ecchondroma (eccentric growth) cortical expansion

Enchondroma Ecchondroma

Enchondroma

Ecchondroma

• N.E:Bluish whiteCircumscribedGritty feeling.

• M.E: lobules of hyaline cartilage+ fibrous partitions ± areas of calcifications; ossification & mucoid degeneration

Pathological fracture

Sarcomatous change (Long bones & Flat bones)Rapid growthPainRecurrence after

excision

•Curettage •+ Bone Graft

Multiple chondromatosisFailure of remodelingUnilateral dwarfismDeformities

Multiple chondromatosisMultiple soft tissue haemangioma

Uncommon T.Chondroblastoma is a benign neoplasm arising from immature, cartilaginous cells (chondroblasts).Epiphysial

Origin: chondroblastAge : 10 – 20 Ys.MaleFemale; 3:1Site: epiphysis ( Around knee, upper humerus, upper femur) Clinical Picture.

1. Joint pain , stiffness.2. Swelling & effusion.3. Limping.

• An epiphyseal oval or rounded area of radiotranslucency.

• Flecks of calcification.

• Thin overlying cortex.

X-ray

Pathology

N.E: Brownish, sand-like and gritty lesion.

N.E

M.E: Rounded or polyhedral chondroblasts. + scattered giant cells +{ Area of focal necrosis and calcification }.

M.E

D.D : Giant cell tumor Chondroma Chondrosarcoma .

Treatment: Curettage + bone

graft.

CT Scan

`MRI

Femoral head

Radiograph of epiphyseal lesion (hip).

Giant cell tumorGiant cell tumor (Osteoclastoma(Osteoclastoma))

Pathology

Incidence:age --- between 20 and 30 Years.

Site ---originates in the fused epiphysis and involves the metaphysis by extension.

Commonest sites are the lower end of the femur, upper end of the tibia and lower end of the

radius. It may also occur in other bones, as the humerus, the ulna, the fibula and occasionally in the small bones of the hands and

feet.

This bone tumor is composed of a fibroblastic stroma and multi-nucleated giant cells.

Gross appearance:

The tumor causes expansion of the bone and the cortex is greatly thinned out. Bone trabeculae may be left behind and these give the tumor the soap bubble appearance in the X-ray film. The cut section shows a soft dark red, hemorrhagic mass causing expansion of the end of the bone.

Microscopic appearance:Two types of cells are encountered:

Spindle shaped or oval cells which are the basic tumor cells.

Multinucleated giant cells of the osteoclastic type. In determining the degree of malignancy more attention must be paid to the stromal cells and not the giant cells.

Pathological types: 3 grades of the tumor can be identified,

grade I tumors have a benign course (98%), grade III tumors are frankly malignant whereas

grade II tumors show an intermediate.

Malignant tumors are characterized by frequent mitosis and predominance of anaplastic cells,

whereas in benign tumors mitosis is infrequent and numerous giant cells are observed.

Clinical Features:•The common feature is a dull aching pain

over the affected site, associated with the appearance of slowly growing tumor of the extreme end of the bone,

•IN big Tumor egg-shell crackling may be detected over it. The nearby joint may be painful, limited mobility and with effusion

•The patient may present with a pathological fracture and this may be the result of trivial trauma..

Expansion of the extreme end of the bone. (At the fused epiphysis).1. Soap bubble appearance.2. The joint line is not invaded except very

late3. Sharp limitation of the tumor from the

surrounding bone and soft tissues. There is always some sclerosis between the tumor and the shaft (operculum or medullary plug).4. Pathological fracture is a frequent

radiological finding.

X-ray features:

Giant cell T. ChondroblastomaAge <20 Ys 10 – 20 YsX-ray -Large translucent area

– in fused epiphysis. -No calcification or

Ossification -Extends to metaphysis

-Small translucent area.-Areas of ossification

M.E -Spindle cell Stroma

–many Giant cells -Polyhedral cells + focal

necrosis & calcification + -few giant cells

Behavior Aggressive Benign

•For definitive diagnosis and •For grading the tumor.

•The neoplastic part of the tumor is the mesenchymal cells

rather than the giant cell element.

Biopsy:

Treatment:

•Benign Tumors:i.Resection if not disturbing the function of the part, e.g. tumor of the fibula.

ii. If the tumor is found in a main bone it is treated by curettage and bone grafting.

iii.This form of treatment carries the risk of recurrence in 40%. If recurrence occurs, biopsy is performed again, if malignancy is found the bone is resected and replaced by an allograft or synthetic prosthesis.

•Malignant Tumors: Whether primary or secondary they should be treated by amputation.•Inaccessible Tumors: Such as tumors of the spine are treated by irradiation.

OsteosarcomaOsteosarcoma

•This is the most common and most malignant bone tumor. •Age -- between 10-20 years -- after the age of 50 years, it

may occur on top of Paget's disease ) •Site in bone : in the metaphysis, •bones affected in order of

frequency are the femur, tibia, humerus, pelvis and fibula.

Pathology:

Predisposing factors:

Trauma; although a history of trauma is common there is no proof that trauma predisposes to bone sarcoma. The tumor was found common in children exposed to radio-active materials.

Gross appearance:At first the disease is confined to the bone giving rise to a fusiform mass which gradually fades into the shaft.

The consistency of the tumor varies as there is bone formation as well as bone destruction. Therefore, the mass may be soft in areas, while it is firm or hard in other areas.

The usual colour is grey but owing to a great liability to haemorrhage, necrosis and cystic formation, the colour varies much. The periosteum is raised . Deposition of fine specules of bone around the stretched blood vessels between the raised periosteum and the cortex gives the characteristic Sun Ray Appearance in the x-ray. In late cases the periosteum is invaded and the soft tissues are infiltrated.

Microscopic appearance:

The characteristic appearance is pleomorphism, the cells vary widely, some are spindle shaped or spheroidal and giant cells are frequent.

The stroma also varies widely; it may be hyaline and fibrous, cartilagenous, myxomatous, osteoid or osseous.

The nature of the stroma may give different names to the tumor, thus it may be called osteolytic when bone formation is scanty or absent, sclerosing when bone formation is great and telangiectatic when it is greatly vascular.

The blood vessels of an osteogenic sarcoma are thin walled, and ill developed and in. some areas tumor cells form the walls of blood sinuses and this explains the early spread by the blood stream.

Clinical Features:

Pain:Pain is the initial complaint and usually precedes the

appearance of the swelling by weeks or months it is due to early involvement of the periosteum as usually there is a history of trauma which precipitates the discomfort, although in some cases the pain comes gradually and there is no relation to trauma.

Swelling:There is a tender fusiform mass arising at the region of the

metaphysis and gradually fading into the shaft. The swelling increases rapidly in size with marked deterioration in general health. The mass usually feels warm, there may be dilated veins on the surface and some sympathetic effusion in nearby joint.

Diagnosis: x-ray:

Bone destruction which starts in the medulla of the metaphyseal part of the bone and then affects the cortex.

a. Irregular bone formation which may be in the form of Sun Rays. Subperiosteal deposition at the angle between the raised periosteum and the shaft (Codmann's triangle), or scattered areas of new bone. b. Soft tissue mass due to early extra osseous extension of the tumor.c. The epiphyseal plate resists the invasion by the tumor.d. Pathological fracture may be seen in the osteolytic variety; bone destruction is the main feature while bone formation is scanty or absent. However, the shadow of the shaft (Ghost of the bone) can always be seen inside the tumor.

Open biopsy: Confirms the diagnosis

Management:The classic treatment: which was applied for many years ago, was the complete amputation. As the tumor always infilterates along the medulla of the bone, the whole bone affected should be amputated. For example, above knee amputation should be done for tumor affecting the upper end of tibia.The recent approach for management : Done in 3 steps

Neo-adjuvant chemotherapy : pre-operative chemotherapy.Operation : done as a salvage procedure , as the complete excision of the lesion only or radical excision of the compartment containing the tumor . Followed by reconstructive procedures like replacement of the removed part by bone graft. Adjuvant chemotherapy: Post-operative chemotherapy

Prognosis:

-- extremely grave, -- survival period is not more than 12 months

This is due to early invasion of the blood vessels and the development of pulmonary metastases. At the time the tumor is diagnosed, tumor cells are growing and the lung is usually affected, even if a chest radiogram appears normal.

-- The 5 y. survival rate is about 20%, -- it may increase to 60% after amputation. --- recently survival rate improved to 70 %

CHONDROSARCOMACHONDROSARCOMA

It is a slowly growing tumor arising from chondroblasts.

It is usually of 2 types:

Primary chondrosarcoma: usually occurs between the ages of 30-60 y.Secondary chondrosarcoma: which arises 2ry to a pre-existing chondroma.

Chondrosarcoma is common in pelvis, and in bones developed from cartilage. But it may also affect long bones (in metaphysis).

Clinically: Clinically: It presents with dull aching pain associated with the development of a slowly growing swelling usually firm and not tender.

In the X-ray film the tumor appears as a large area of translucency with scattered spots of calcification and a varying degree of destruction of the cortex.

Metastases to distant sites particularly to the lungs occur later than in the case with most other bone sarcomas.

Treatment: Treatment: Complete local excision. Amputation may be needed for tumors of the limbs especially if big or if they recur after local excision.

Ewing's TumourEwing's Tumour

This forms about 10% of all malignant bone tumors.

between the ages of 5 and 15 years. History of trauma is common. Pain, fever and tender swelling follow and the condition may be

easily mistaken for osteomyelitis for the following reasons:

1. General constitutional disturbances.2. The severe pain and local tenderness. 3. The rapid appearance of the swelling which may be

warm as in osteomyelitis. 4. The occasional appearance of leucocytes. A soft

necrotic cellular material is obtained by biopsy and the pathologist may even mistake it for pus.

The following points may help the diagnosis:

A very high leucocytic count is in favour of osteomyelitis.

Osteomyelitis usually responds rapidly to antibiotic therapy.

Ewing tumor responds quickly to irradiation but recurs rapidly.

Gross Appearance:

Bones affected are tibia, humerus, femur, iliac bonebut no bone is exempt. It affects the metaphysis and also the diaphysis.

The tumor is formed of necrotic brain like material which invades the bone and leads to bone destruction and formation of successive layers of new bone giving rise to the characteristic Onion Peal Appearance in the X-ray.

New bone formation may also obliterate the medullary cavity and for this reason pathological fracture is rare.

Microscopic Appearance:

It is a round cell sarcoma arranged in columns or sheets but may be grouped around

blood vessels and give rise to an angiotheliomatous appearance.

Spread of the tumor:

Local in the bone both longitudinally and transversely, the bony canals are full of tumor cells.

Blood spread to lungs and other bones. It is the only bone tumor which gives secondaries in bones. (Multiple myeloma is generalized from the start).

Lymphatic spread to the local lymph nodes.

Treatment:•The accepted treatment is irradiation & • chemotherapy followed by

amputation. •Although it is highly sensitive

recurrence is the title and the prognosis is bad. •Combination of radiation and multiple

drug chemotherapy has improved the 5 years survival rate to 60%.

Multiple MyelomaMultiple Myeloma

This is a diffuse tumor of the whole skeleton arising in the bone marrow and its cells may resemble plasma cells.

Occasionally a solitary myeloma is described.

The tumor is characterized by over-production of monoclonal immunoglobulins, which are excreted in urine as Bence Jones protein. And forms myloma band in plasma elctrophoresis

Clinical Features:

Common between 40 and 60 years of age and more frequent in males.

General weakness, weight loss and backache.

Tenderness on palpation or percussion of the affected bone.

Superficial bones may be felt tumefied.

A pathological fracture may be the first presentation,

Radiological findings:X-ray shows multiple punched out areas of radio-translucency. These are common in the skull.

The diagnosis should be suspected when lytic lesions are found in a patient with anaemia and elevated sedimentation rate and elevated serum Calcium.

DiagnosisSerum and urine electrophoresis and immunophoresis shows elevated M protein (Immunoglobulins or Bence Jones protein).

Biopsy of bone marrow.

Treatment

Measures to relieve pain as rest and sedatives.

General supportive measures as diet, Blood transfusion and anabolic drugs.

Measures to control the growth of the tumor as chemotherapy and radiotherapy.

Surgical fixation of pathological fractures to improve quality of life.

Metastatic Carcinoma

Bone secondaries are more commonly seen than the primary bone tumors .

•The skeleton is a common site for metastatic carcinoma.

•The common sites for the primary are the thyroid, the breast, the lung, the kidney, the adrenal and the prostate.

•Secondaries commonly affect the skull, the sternum, the spine the pelvic bones and the limbs above the knee and elbow. They are very rare in the bones of the forearm, hand and foot.

•Most of the secondaries are osteolytic in nature, but secondaries from a prostatic carcinoma are usually Osteosclerotic (Rarely the breast may give osteosclerotic secondaries).

Clinical pictures:

Sometimes secondaries in bone may be the first presentation of the primary which remains silent for a variable time.

Metastatic carcinoma in bones usually gives rise to anaemia, bone pains, pathological fracture and sometimes bony mass.

A mass is common with hypernephroma and thyroid carcinoma .

Compression paraplegia may occur due to secondaries in the spine.

Renal cell carcinoma

Investigations:X-ray a secondary appears as a round well defined area of radiotranslucency

Bone scan with 99m TcHDP, which is of a great value for detection of the silent secondaries.

The Treatment: is palliative and may be in the form of radiotherapy, hormonal therapy, antimitotic drugs or radioactive isotopes according to the nature of the primary lesion.

Surgical fixation of a long bone is indicated when there is a pathological fracture, and prophylactic fixation of long bones with metastatic lesions is indicated when the lesion involves more than half of the circumference or when the length of the tumor is greater than the bone diameter .

FibrosarcomaFibrosarcoma

This is a rare malignant tumor of bone. The tumor is solid, firm and whitish in colour and it may affect any bone. It may occur in any part of the bone. The commonest sites are the distal femur and proximal tibia and have two types:

Periosteal type: Develops from the periosteum and grows on the surface. It causes erosion of the cortex producing a concave defect in the bone and some reactive bone may develop at the ends of the tumor.

Medullary or central type: Arises from the interior of the bone causing bone destruction and without biopsy it is difficult to diagnose.

Histological structure:

Similar to fibrosarcoma of the soft tissues with different grades of differentiation.

Treatment:

Wide local excision may be sufficient for highly differentiated tumors, but anaplastic tumors need amputation as the recurrence rate after local excision is high. Irradiation and chemotherapy are ineffective. The 5 years survival rate is 30-35%.

BONE CYSTS

Simple (Unicameral ) bone cyst.Aneurysmal bone cyst.Parathyroid osteodystrophy

(osteitis fibrosa cystica)Hydatid cyst.

Simple (Unicameral ) Bone cyst)

Unilocular fluid filled cyst.Age: <15 Ys. M > FSite: Ends of long bones abutting on E.P.(active); away of E.P. (latent).S&S: Pain swellingPathological Fr.

Radiolucent area Oval;CentralMetaphyseal near E.P.Thin cortex ± Path. Fr.

Calcaneus

Treatment: Curettage & Bone grafting

Tumor-like ; non pulsating bone lesion.The bone is expanded (Ballooned).Age: <20 Ys.Site: Metaphysis of long bone.; Vertebra ( arch & transverse process).S&S: pain & swelling.Spine : signs of compression

An expansile cystic lesion2nd decade of life.Any bone in the body.Although benign, the ABC can be locally aggressive and cause extensive weakening of the bony structure and impinge on surrounding tissues.

ABC

• ABC is consisting of blood-filled spaces separated by connective tissue septa containing trabeculae or osteoid tissue and osteoclast giant cells.

• Although benign, the ABC can be a rapidly growing and destructive bone lesion.

The expansile nature of the lesions can cause:Pain . Swelling .Deformity.Disruption of growth plates.Neurologic symptoms (depending on its location).Pathologic fracture.

X-ray : Eccentric;Expansile; translucent area. Clearly defined margin; sclerosed edge. Thin cortex

Aneurysmal Bone Cyst

• N.E: Cavity filled with few thin shreds & blood.

• M.E • Fibroblastic

spindle cells• + few giant cells• Blood filled

spacesM.E

Treatment Curettage & Bone grafting.Prognosis: Recurrence.

• Treatment: Intralesional curettage; however, recurrence is not uncommon.En bloc resectionSelective arterial embolization.Curettage with locally applied adjuvants such as liquid nitrogen or phenol.

FIBROUS DYSPLASIA

• Etiology is unknown.• Dysplastic proliferation of fibrous

tissue & bone tissue in localized area(s) of skeletal bone

• Three Types: – monostotic, – polyostotic, – polyostotic with endocrinopathies.

Polyostotic with endocrinopathies

• 3 to 5% of cases• café au lait spots and

precocious sexual development (McCune-Albright syndrome)

• Associated with other endocrine disorders.

Fibrous Dysplasia:

Fibro-osseous tissue – dysplastic

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